Mental Retardation - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

MENTAL RETARDATION A M EDICAL D ICTIONARY , B IBLIOGRAPHY , AND A NNOTATED R ESEARCH G UIDE TO I NTERNET R E FERENCES ...

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MENTAL

RETARDATION A M EDICAL D ICTIONARY , B IBLIOGRAPHY , AND A NNOTATED R ESEARCH G UIDE TO I NTERNET R E FERENCES

J AMES N. P ARKER , M.D. AND P HILIP M. P ARKER , P H .D., E DITORS

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ICON Health Publications ICON Group International, Inc. 4370 La Jolla Village Drive, 4th Floor San Diego, CA 92122 USA Copyright 2004 by ICON Group International, Inc. Copyright 2004 by ICON Group International, Inc. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without written permission from the publisher. Printed in the United States of America. Last digit indicates print number: 10 9 8 7 6 4 5 3 2 1

Publisher, Health Care: Philip Parker, Ph.D. Editor(s): James Parker, M.D., Philip Parker, Ph.D. Publisher's note: The ideas, procedures, and suggestions contained in this book are not intended for the diagnosis or treatment of a health problem. As new medical or scientific information becomes available from academic and clinical research, recommended treatments and drug therapies may undergo changes. The authors, editors, and publisher have attempted to make the information in this book up to date and accurate in accord with accepted standards at the time of publication. The authors, editors, and publisher are not responsible for errors or omissions or for consequences from application of the book, and make no warranty, expressed or implied, in regard to the contents of this book. Any practice described in this book should be applied by the reader in accordance with professional standards of care used in regard to the unique circumstances that may apply in each situation. The reader is advised to always check product information (package inserts) for changes and new information regarding dosage and contraindications before prescribing any drug or pharmacological product. Caution is especially urged when using new or infrequently ordered drugs, herbal remedies, vitamins and supplements, alternative therapies, complementary therapies and medicines, and integrative medical treatments. Cataloging-in-Publication Data Parker, James N., 1961Parker, Philip M., 1960Mental Retardation: A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References / James N. Parker and Philip M. Parker, editors p. cm. Includes bibliographical references, glossary, and index. ISBN: 0-597-84035-0 1. Mental Retardation-Popular works. I. Title.

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Disclaimer This publication is not intended to be used for the diagnosis or treatment of a health problem. It is sold with the understanding that the publisher, editors, and authors are not engaging in the rendering of medical, psychological, financial, legal, or other professional services. References to any entity, product, service, or source of information that may be contained in this publication should not be considered an endorsement, either direct or implied, by the publisher, editors, or authors. ICON Group International, Inc., the editors, and the authors are not responsible for the content of any Web pages or publications referenced in this publication.

Copyright Notice If a physician wishes to copy limited passages from this book for patient use, this right is automatically granted without written permission from ICON Group International, Inc. (ICON Group). However, all of ICON Group publications have copyrights. With exception to the above, copying our publications in whole or in part, for whatever reason, is a violation of copyright laws and can lead to penalties and fines. Should you want to copy tables, graphs, or other materials, please contact us to request permission (E-mail: [email protected]). ICON Group often grants permission for very limited reproduction of our publications for internal use, press releases, and academic research. Such reproduction requires confirmed permission from ICON Group International Inc. The disclaimer above must accompany all reproductions, in whole or in part, of this book.

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Acknowledgements The collective knowledge generated from academic and applied research summarized in various references has been critical in the creation of this book which is best viewed as a comprehensive compilation and collection of information prepared by various official agencies which produce publications on mental retardation. Books in this series draw from various agencies and institutions associated with the United States Department of Health and Human Services, and in particular, the Office of the Secretary of Health and Human Services (OS), the Administration for Children and Families (ACF), the Administration on Aging (AOA), the Agency for Healthcare Research and Quality (AHRQ), the Agency for Toxic Substances and Disease Registry (ATSDR), the Centers for Disease Control and Prevention (CDC), the Food and Drug Administration (FDA), the Healthcare Financing Administration (HCFA), the Health Resources and Services Administration (HRSA), the Indian Health Service (IHS), the institutions of the National Institutes of Health (NIH), the Program Support Center (PSC), and the Substance Abuse and Mental Health Services Administration (SAMHSA). In addition to these sources, information gathered from the National Library of Medicine, the United States Patent Office, the European Union, and their related organizations has been invaluable in the creation of this book. Some of the work represented was financially supported by the Research and Development Committee at INSEAD. This support is gratefully acknowledged. Finally, special thanks are owed to Tiffany Freeman for her excellent editorial support.

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About the Editors James N. Parker, M.D. Dr. James N. Parker received his Bachelor of Science degree in Psychobiology from the University of California, Riverside and his M.D. from the University of California, San Diego. In addition to authoring numerous research publications, he has lectured at various academic institutions. Dr. Parker is the medical editor for health books by ICON Health Publications. Philip M. Parker, Ph.D. Philip M. Parker is the Eli Lilly Chair Professor of Innovation, Business and Society at INSEAD (Fontainebleau, France and Singapore). Dr. Parker has also been Professor at the University of California, San Diego and has taught courses at Harvard University, the Hong Kong University of Science and Technology, the Massachusetts Institute of Technology, Stanford University, and UCLA. Dr. Parker is the associate editor for ICON Health Publications.

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About ICON Health Publications To discover more about ICON Health Publications, simply check with your preferred online booksellers, including Barnes&Noble.com and Amazon.com which currently carry all of our titles. Or, feel free to contact us directly for bulk purchases or institutional discounts: ICON Group International, Inc. 4370 La Jolla Village Drive, Fourth Floor San Diego, CA 92122 USA Fax: 858-546-4341 Web site: www.icongrouponline.com/health

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Table of Contents FORWARD .......................................................................................................................................... 1 CHAPTER 1. STUDIES ON MENTAL RETARDATION ........................................................................... 3 Overview........................................................................................................................................ 3 The Combined Health Information Database................................................................................. 3 Federally Funded Research on Mental Retardation..................................................................... 13 E-Journals: PubMed Central ....................................................................................................... 64 The National Library of Medicine: PubMed ................................................................................ 65 CHAPTER 2. NUTRITION AND MENTAL RETARDATION ............................................................... 111 Overview.................................................................................................................................... 111 Finding Nutrition Studies on Mental Retardation.................................................................... 111 Federal Resources on Nutrition ................................................................................................. 113 Additional Web Resources ......................................................................................................... 113 CHAPTER 3. ALTERNATIVE MEDICINE AND MENTAL RETARDATION ......................................... 115 Overview.................................................................................................................................... 115 National Center for Complementary and Alternative Medicine................................................ 115 Additional Web Resources ......................................................................................................... 121 General References ..................................................................................................................... 122 CHAPTER 4. DISSERTATIONS ON MENTAL RETARDATION ........................................................... 123 Overview.................................................................................................................................... 123 Dissertations on Mental Retardation......................................................................................... 123 Keeping Current ........................................................................................................................ 161 CHAPTER 5. CLINICAL TRIALS AND MENTAL RETARDATION ..................................................... 163 Overview.................................................................................................................................... 163 Recent Trials on Mental Retardation......................................................................................... 163 Keeping Current on Clinical Trials ........................................................................................... 167 CHAPTER 6. PATENTS ON MENTAL RETARDATION ..................................................................... 169 Overview.................................................................................................................................... 169 Patents on Mental Retardation.................................................................................................. 169 Patent Applications on Mental Retardation .............................................................................. 178 Keeping Current ........................................................................................................................ 188 CHAPTER 7. BOOKS ON MENTAL RETARDATION ......................................................................... 189 Overview.................................................................................................................................... 189 Book Summaries: Federal Agencies............................................................................................ 189 Book Summaries: Online Booksellers......................................................................................... 190 The National Library of Medicine Book Index ........................................................................... 198 Chapters on Mental Retardation................................................................................................ 199 Directories.................................................................................................................................. 202 CHAPTER 8. MULTIMEDIA ON MENTAL RETARDATION .............................................................. 205 Overview.................................................................................................................................... 205 Video Recordings ....................................................................................................................... 205 Bibliography: Multimedia on Mental Retardation .................................................................... 206 CHAPTER 9. PERIODICALS AND NEWS ON MENTAL RETARDATION ........................................... 207 Overview.................................................................................................................................... 207 News Services and Press Releases.............................................................................................. 207 Newsletter Articles .................................................................................................................... 210 Academic Periodicals covering Mental Retardation .................................................................. 210 APPENDIX A. PHYSICIAN RESOURCES .......................................................................................... 215 Overview.................................................................................................................................... 215 NIH Guidelines.......................................................................................................................... 215 NIH Databases........................................................................................................................... 217 Other Commercial Databases..................................................................................................... 221

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APPENDIX B. PATIENT RESOURCES ............................................................................................... 223 Overview.................................................................................................................................... 223 Patient Guideline Sources.......................................................................................................... 223 Associations and Mental Retardation........................................................................................ 232 Finding Associations.................................................................................................................. 234 APPENDIX C. FINDING MEDICAL LIBRARIES ................................................................................ 237 Overview.................................................................................................................................... 237 Preparation................................................................................................................................. 237 Finding a Local Medical Library................................................................................................ 237 Medical Libraries in the U.S. and Canada ................................................................................. 237 ONLINE GLOSSARIES................................................................................................................ 243 Online Dictionary Directories ................................................................................................... 244 MENTAL RETARDATION DICTIONARY ............................................................................. 245 INDEX .............................................................................................................................................. 317

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FORWARD In March 2001, the National Institutes of Health issued the following warning: "The number of Web sites offering health-related resources grows every day. Many sites provide valuable information, while others may have information that is unreliable or misleading."1 Furthermore, because of the rapid increase in Internet-based information, many hours can be wasted searching, selecting, and printing. Since only the smallest fraction of information dealing with mental retardation is indexed in search engines, such as www.google.com or others, a non-systematic approach to Internet research can be not only time consuming, but also incomplete. This book was created for medical professionals, students, and members of the general public who want to know as much as possible about mental retardation, using the most advanced research tools available and spending the least amount of time doing so. In addition to offering a structured and comprehensive bibliography, the pages that follow will tell you where and how to find reliable information covering virtually all topics related to mental retardation, from the essentials to the most advanced areas of research. Public, academic, government, and peer-reviewed research studies are emphasized. Various abstracts are reproduced to give you some of the latest official information available to date on mental retardation. Abundant guidance is given on how to obtain free-of-charge primary research results via the Internet. While this book focuses on the field of medicine, when some sources provide access to non-medical information relating to mental retardation, these are noted in the text. E-book and electronic versions of this book are fully interactive with each of the Internet sites mentioned (clicking on a hyperlink automatically opens your browser to the site indicated). If you are using the hard copy version of this book, you can access a cited Web site by typing the provided Web address directly into your Internet browser. You may find it useful to refer to synonyms or related terms when accessing these Internet databases. NOTE: At the time of publication, the Web addresses were functional. However, some links may fail due to URL address changes, which is a common occurrence on the Internet. For readers unfamiliar with the Internet, detailed instructions are offered on how to access electronic resources. For readers unfamiliar with medical terminology, a comprehensive glossary is provided. For readers without access to Internet resources, a directory of medical libraries, that have or can locate references cited here, is given. We hope these resources will prove useful to the widest possible audience seeking information on mental retardation. The Editors

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From the NIH, National Cancer Institute (NCI): http://www.cancer.gov/cancerinfo/ten-things-to-know.

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CHAPTER 1. STUDIES ON MENTAL RETARDATION Overview In this chapter, we will show you how to locate peer-reviewed references and studies on mental retardation.

The Combined Health Information Database The Combined Health Information Database summarizes studies across numerous federal agencies. To limit your investigation to research studies and mental retardation, you will need to use the advanced search options. First, go to http://chid.nih.gov/index.html. From there, select the “Detailed Search” option (or go directly to that page with the following hyperlink: http://chid.nih.gov/detail/detail.html). The trick in extracting studies is found in the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Journal Article.” At the top of the search form, select the number of records you would like to see (we recommend 100) and check the box to display “whole records.” We recommend that you type “mental retardation” (or synonyms) into the “For these words:” box. Consider using the option “anywhere in record” to make your search as broad as possible. If you want to limit the search to only a particular field, such as the title of the journal, then select this option in the “Search in these fields” drop box. The following is what you can expect from this type of search: •

Comparison of the Interplak and Manual Toothbrushes in a Population with Mental Retardation-Developmental Disabilities (MR-DD) Source: SCD. Special Care in Dentistry. 17(5): 133-136. September-October 1997. Summary: A major dental problem confronting persons with mental retardation or developmental disabilities (MR-DD) is poor dental hygiene, which can result in an increased incidence of gingivitis and periodontal disease. Studies indicate that the most obvious reason for the poor oral hygiene of such persons is their inability to clean the oral cavity adequately. This article reports on a study undertaken to determine whether there was a difference in oral health between a group of residents with MR-DD using the Interplak and a group using manual toothbrushes. Fifty-six residents from four Franklin County group homes completed the study. Each group home was divided into

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two study groups, one using the Interplak and the second using manual toothbrushes. Each subject was seen for initial recording based on the Gingival Index (Loe) and Simplified Oral Hygiene Index (Greene and Vermillion), followed by a thorough prophylaxis. These measurements were recorded again at three, six, and nine months, with a final recording at 12 months. Analysis of the data indicated significant improvement in the Gingival Index over a 12-month period for the residents using the Interplak. In addition, the data showed a trend in which residents who brushed by themselves, with either a manual toothbrush or Interplak, had the same or lower gingival, debris, and calculus scores than those who were assisted with their toothbrushing. 5 tables. 16 references. (AA-M). •

Administration of Chlorhexidine to Persons with Mental Retardation Residing in an Institution: Patient Acceptance and Staff Compliance Source: SCD. Special Care in Dentistry. 16(2): 53-57. 46-52. March-April 1996. Summary: Chlorhexidine is beneficial in maintaining the oral health of persons diagnosed with mental retardation. This article reports on a study that evaluated two methods of chlorhexidine administration for patient acceptance and direct-care staff compliance among 44 institutionalized individuals diagnosed with mental retardation. The authors stress that proper daily oral administration depends upon the compliance of caregivers as well as patient acceptance. If caregivers are non-compliant or if patients are overly resistant to daily applications of chlorhexidine, its benefits will not be realized. 1 table. 14 references. (AA-M).

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Appliance Therapy for Chronic Drooling in a Patient With Mental Retardation Source: Special Care in Dentistry. 14(1): 30-32. January-February 1994. Summary: Chronic drooling is a frequent finding in conditions affecting function of the muscles of the orofacial complex. In this article, the authors describe appliance therapy for chronic drooling. They present a case report of an individual with cerebral palsy and mental retardation that illustrates the use of a simple appliance to align the patient's lip to provide opportunity for a lip seal when swallowing or at rest in a patient with a Class II malocclusion. The appliance resulted in a significant decrease in drooling in this patient. The authors note that, ultimately, the success of any appliance therapy is dependent on patient compliance and motivation of care providers to see that treatment recommendations are followed. 3 figures. 12 references. (AA-M).

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You May Be Treating Children with Mental Retardation and Attention Deficit Hyperactive Disorder in Your Dental Practice Source: Journal of Dentistry for Children. 67(4): 241-245. July-August 2000. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: Diagnosis of attention deficit hyperactivity disorder (ADHD) focuses on three main components of the disorder: impulsiveness, hyperactivity, and inattention. In a dental setting, the behavior management of children with ADHD can be challenging. In this article, the authors consider the further complications of providing care to children with both ADHD and mental retardation. Deinstitutionalization and mainstreaming these youngsters with complicating developmental and psychological disorders will continue to increase the demand for dental and medical services by community practitioners. The authors discuss the prevalence of mental retardation,

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statistics regarding the dual diagnosis, complicating factors (including psychosocial and academic factors), and the practitioner's perspective on providing care to this population. The authors remind readers that more than 85 percent of individuals with mental retardation fall into the 'mild' range. Three appendices offer the diagnostic criteria for ADHD and attention deficit disorder (ADD), and a list of specific dental management considerations for the child with both ADHD and mental retardation. 27 references. •

Management of the Patient with Mental Retardation: A Case Report Source: PROBE. 32(1): 18-22. January-February 1998. Contact: Available from Canadian Dental Hygienists Association (CDHA). 96 Centrepointe Drive, Nepean, Ontario, Canada K2G 6B1. (800) 267-5235. Fax (613) 2247283. Summary: During the course of their careers, dental professionals frequently interact with individuals with mental retardation. This article identifies the basic information that dental professionals need to know when treating people with mental retardation. The authors emphasize that, in order to provide the best possible quality of care, dentists should be aware of the etiology of mental retardation, the medical and dental conditions associated with this disability, and the services which can be incorporated into a treatment plan focused on improving the oral health of these individuals. The authors describe a case report of the dental hygiene services that were provided to a patient with mental retardation. Limitations when managing this case centered around the patient's poor hand coordination, mouthbreathing habit, mental status, and short attention span, all of which impaired her ability to improve her oral hygiene condition. These limiting factors are frequently exhibited in persons with mental retardation and force dental professionals to provide care which may not be ideal but is the best that can be provided for that patient due to the circumstances. Recommendations for patients similar to the one presented in this case include frequent recalls, educational services for both the patient and their parent or caregiver, and a team approach to providing the best possible dental care. 7 figures. 20 references. (AA-M).

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Dental Care for Children with Mental Retardation: Thoughts About the Americans with Disabilities Act Source: Journal of Dentistry for Children. 65(6): 487-491. November-December 1998. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 North Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: Except for concerns relative to HIV discrimination, limited attention within the context of the Americans With Disabilities Act (ADA) has been directed to the difficulties in securing needed dental care for individuals with disabilities. In particular, there has been little to no discussion regarding ADA issues and the difficulties in obtaining needed dental care for persons with mental retardation, including more than a half million children. This article provides an introduction to the demographics of children with mental retardation and their need for dental services, thereby providing a framework for discussion of the dimensions of the problem. Increasing numbers of practitioners are, and will be, called upon to provide the needed care for people with mental retardation who reside in the general community. As a result of deinstitutionalization, many children may require behavior management techniques, including physical support or restraints, sedation or hospital dentistry, and general anesthesia to provide needed services. 3 tables. 29 references.

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Deinstitutionalization of Children with Mental Retardation: What of Dental Services? Source: Journal of Dentistry for Children. 67(6): 413-417. November-December 2000. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 North Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: For more than three decades, changing social policies, favorable legislation for people with disabilities, and class action legal decisions, which delineated the rights of people with mental retardation, have led to deinstitutionalization. This article considers the impact of deinstitutionalization of children with mental retardation on the dental services and dental care they receive. Many of the community residential facilities that have taken the place of the institutions are too small in size to provide adequate monitoring of health care, including dental care. The authors provide an overview of the deinstitutionalization of people with mental retardation or developmental disabilities at the national and state levels; and the delivery or lack of dental services for this population of residents in community residential facilities. The authors provide statistical information to support their discussion of these issues. The authors also discuss professional dental education, the revision of the presently cumbersome Medicaid system of payment for dental services, and the need for the profession to prepare itself to care for this special population. 2 tables. 27 references.

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Communication Disorders and Children with Mental Retardation Source: Child and Adolescent Psychiatric Clinics of North America. 8(1): 61-75. January 1999. Contact: Available from W.B. Saunders Company. 6277 Sea Harbor Drive, Orlando, FL 32887-4800. (800) 654-2452. Summary: Mental retardation (MR) is a condition measured by intellectual functions (IQ) and adaptive behavior (communication, self care, home living, social skills, community use, self direction, health and safety, functional academics, leisure, and work). With a pragmatic perspective, this article addresses the communication characteristics of children with MR from the outlook of rehabilitation professionals who recognize that communication is a shared and valued social tool between children and their communication partners. The author highlights the communication styles presented by children with MR, specifically during interactions between the child and the child's parents, siblings, teachers, and professionals. The author also includes a description of the communication characteristics (including developmental status, expressive receptive language and phonology, and social communication), assessment formats relevant to the MR population, and intervention protocols that reflect daily routines and educational opportunities. The author concludes that within both the pragmatics orientation and the revised definition of MR, the discussion of communication disorders for children with MR has begun to deemphasize a developmental content form protocol for assessment and intervention. Instead, the current model suggests the importance of communication stimulation and support in daily routines. 57 references.

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Children with Mental Retardation and Epilepsy: Demographics and General Concerns Source: Journal of Dentistry for Children. 67(4): 268-274. July-August 2000.

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Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: Providing dental care for children with mental retardation can be a challenge and can be complicated by multiple disabilities, family attitudes, and the unavailability of practitioners to provide care. This article reports on demographics and general concerns regarding the provision of dental care to children with mental retardation and epilepsy (the two more frequent types of major neurological impairments in childhood). The authors describe epilepsy, the drugs used to treat epilepsy, the prevalence of epilepsy, attitudes toward children with epileptic seizures, the prevalence of mental retardation, the existence of both conditions in the same child, and the dental practitioner's perspective. The authors offer an appendix in which they outline specific strategies for dental care of youngsters with epilepsy; a second appendix lists the prevalence rate of mental retardation by state (1993 figures). 3 tables. 40 references. •

Improving Health Care Communication for Persons with Mental Retardation Source: Public Health Reports. 107(3): 297-302. May-June 1992. Summary: There has been little effort directed to training health care professionals in behaviors and attitudes that are effective in communicating with persons with mental retardation. This article reports on an education program that provided a self-study instructional text and a 20-minute companion video, both focused on providing methods of communicating with a patient with mental retardation in medical and dental care settings. The authors' results show that resident physicians, medical students, nurses, and nursing assistants improved their communication skills, knew more about mental retardation, and were more proactive in health care interviews following training. 2 tables. 16 references. (AA-M).

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Understanding the Siblings of Children with Mental Retardation Source: Journal of Dentistry for Children. 67(5): 345-349. September-October 2000. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: This article builds on a discussion presented in an earlier article on the general family issues attendant to the dental treatment of children with mental retardation and other disabilities. This review article extends the discussion with emphasis on the personal and family difficulties faced by siblings of children with disabilities, and how these issues may affect care that is provided for them in dental practices. The impact for siblings may arise from a variety of factors, including the effects of parental stress, disproportionate parental attention and resources devoted to the child with a disability, family isolation and stigmatization, and decreased social and recreational activities. These factors can result in negative and positive effects. The authors review each of these risk factors and the likely result for the siblings, discussing each age group separately. The authors conclude that the complicating realities of family life that include children with mental retardation and other disabilities must be considered in the practitioner's attempt to work with the sibling to improve patient management and provide the necessary care in the dental office and needed home care follow up. The authors summarize suggested strategies for dental care practitioners who are working with this patient population. 21 references.

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Communication Intervention for Adults with Severe Mental Retardation Source: Topics in Language Disorders. 13(3): 47-60. May 1993. Summary: This article describes the general communication characteristics and needs of adults with severe mental retardation. Topics include the heterogeneity of this population, developmental stages, nonverbal communication, and associated behavior characteristics. The authors discuss a transactional approach to intervention for adults who do not communicate symbolically in any mode. This approach reflects four basic assumptions regarding the nature of human communication and the goals of communication intervention; they are that communication is a basic right, adults with severe mental retardation are appropriate candidates for communication intervention, communication intervention targets for adults should be functional, and communication development and use reflect a transactional process. 1 figure. 59 references. (AA-M).

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Children With Mental Retardation Grow Older Source: Journal of Dentistry for Children. 66(4): 266-272. July-August 1999. Summary: This article discusses pediatric dental care for children with mental retardation, focusing on the delivery of care for these children as they grow older. The authors note that the particular difficulties in caring for these special patients may require the training and expertise of pediatric dentists well beyond the period when the dental care of teenagers and young adults ordinarily is provided by general dentists. The authors discuss epidemiologic concerns, including the life expectancy changes in this population, attitudes toward and care of persons with severe disabilities, the transition to adulthood for individuals with mental retardation, the role of race, ethnicity and religion, the impact on families that include young children with mental retardation, siblings of retard and developmentally disabled individuals, aging parents, financial concerns, and issues of sexuality. The authors conclude by encouraging dentists to educate themselves about the ongoing needs and difficulties faced by children with mental retardation and their families as they (both) age, and to provide the necessary services for these populations. An appendix to the article lists the prioritized ten services needed by children and adults with mental retardation in each of 5 age groups, as reported by parents. 76 references.

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Ageing, Alzheimer Disease and Mental Retardation Source: Journal of Intellectual Disability Research. 38(Part 3): 233-239. June 1994. Summary: This article discusses problems that face the mentally retarded as their life expectancies expand, namely old-age- associated dementias, particularly Alzheimer's disease (AD), which may have severe consequences for affected adults with mental retardation. The authors explore the physiology of dementia and AD. Adults with Down syndrome are known to be at particularly high risk for developing AD-type neuropathy, but less is known about AD neuropathology in mentally retarded adults without Down syndrome. Studies suggest that a substantial proportion of the older mentally retarded population will develop AD pathology, whether or not they have Down syndrome. Current data from the authors' research support the hypothesis that beta-amyloidosis initiates the cascade of pathological changes leading to dementia associated with AD, although the underlying mechanisms responsible for initiation of this beta-fibrillosis remain unknown. Previous studies have not been able to relate neuropathology to ante-mortem deterioration in performance measures among adults with mental retardation in any clear way. The authors currently plan to conduct research that may provide a basis for distinguishing between normal aging and changes

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in functioning associated with AD, and provide added insights into the processes underlying AD pathogenesis. 52 references. •

Alzheimer Project: Formulating a Model of Care for Persons With Alzheimer's Disease and Mental Retardation Source: American Journal of Alzheimer's Disease. 10(4): 13-16. July-August 1995. Summary: This article examines the Alzheimer Project, an Alzheimer's disease (AD)/mental retardation care management process designed for the community based residential care setting. The author discusses what can be done to overcome the caregiving obstacles that are present in cognitively-impaired patients, such as those dually diagnosed with Down Syndrome (DS) and AD. This caregiving project is the result of the unique care required by a patient with DS who was diagnosed with AD. Medical technology is extending the life of people with DS beyond 50 years and many are developing AD. The project's ultimate goal is to provide care that supports the patient's medical, behavioral, and emotional needs throughout the course of disease progression. Using the project as an example, the following areas are addressed: the adoption of a new methodology of care for the DS/AD patient, meeting staff needs for continuing educational and DS/AD case management support, choosing day programs to meet the patient's present and future needs, and providing care for patients in the final stages of DS/AD. The author also discusses home adaptations that include lighting and color. 4 references.

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Desensitization: Strengths and Limitations of Its Use in Dentistry for the Patient with Severe and Profound Mental Retardation Source: Journal of Dentistry for Children. 67(4): 250-255. July-August 2000. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: This article explores the strengths and limitations of desensitization as a technique to help patients with severe and profound mental retardation cope with the fear and anxiety of going to the dentist. Poor experiences in the dental chair can influence the behavior of a person with mental retardation and developmental disabilities (MRDD), just as it can with people in the general population. The person can then become sensitized to the next dental experience. The authors explore ways the dentist can use ongoing desensitization, in conjunction with restraint, to deal effectively with the person who has severe and profound MRDD, fear, anxiety, and difficult behavior. The goal is to reduce fear and to encourage the person to become calm and secure while effective dental treatment is provided. The authors describe the desensitization process in some detail, including a history of its use in medical and dental settings. The authors remind readers of the imperative of having all the necessary documentation completed and the informed consent of each patient before the incorporation of desensitization and appropriate restraint programs. 27 references.

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Communication in Adults with Mental Retardation Source: Topics in Language Disorders. 13(3): 1-8. May 1993. Summary: This article provides an overview of social changes emerging over the last 20 years that affect the lives of adults with mental retardation (AMR). The author focuses on the social analysis of communication activities and the functioning of individuals with mild or moderate cognitive deficits. The author also discusses aging and mental

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retardation. The author notes that, in some conversational contexts, adults with mental retardation (AMR) may need a guide, a supportive interrupter, or a conversational colleague; the role of adaptation is stressed. The author hopes that the next era of communication progress will include a model of social orientation in which family, friends, and professional specialists will combine in creating social conditions that are favorable for each individual with mental retardation. 20 references. (AA-M). •

Oral Health of Institutionalized Individuals with Mental Retardation Source: American Journal on Mental Retardation. 98(5): 656-660. March 1994. Summary: This article reports on a study in which a random 10 percent (n=702) of the residents of the Texas Department of Mental Health and Mental Retardation institutions were given dental examinations. Findings were compared with results from a similar survey of 1,077 residents by the same investigator 9 years earlier. Data were analyzed for oral pathology, unmet dental needs, contribution of dental care to habilitation, and effectiveness of the dental service system. Some improvements were found in dental condition, although the results were sometimes difficult to interpret. Some differences may be related to changes in client age and average length of stay and to a shift toward institutionalization of individuals with more severe disabilities. Other differences appear to be the result of better dental care in state institutions. 1 table. 7 references. (AA-M).

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Access to Dental Health Care for Persons with Mental Retardation in Community Residential Settings Source: New England Journal of Human Services. 11(1): 14-19. 1992. Summary: This article reports on a study of access to dental health care for persons with mental retardation in community residential settings. The study was designed to gather information from dental health care providers directly, thereby providing a perspective which is essential to the development of concrete plans to enhance the willingness and capacity of dental health care professionals to provide services to this population. The authors include a discussion of the patterns and trends revealed in the survey, using the data to highlight the major problems and issues dentists face when managing the care of patients with mental retardation. Topics covered include the need for back-up services, professional training issues, reinforcing the need for routine oral home care, working with uncooperative patients, patient compliance, attention span concerns, emergencies, financial considerations, and patient education.

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Effect of Polyol-combinant Saliva Stimulants on S. mutans Levels in Plaque and Saliva of Patients with Mental Retardation Source: SCD. Special Care in Dentistry. 22(5): 187-193. September-October 2002. Contact: Available from Federation of Special Care Organizations in Dentistry. 211 East Chicago Avenue, Chicago, IL 60611. (312) 440-2660. Summary: This article reports on a study that investigated the effect of chewable salivastimulants on Streptococcus mutans levels in dental plaque and paraffin-stimulated whole saliva among 98 mentally disabled participants. Over 64 days, the participants chewed one of four saliva-stimulating tables five times per day. The tables contained one of the following: xylitol (X), sorbitol (S), xylitol and erythritol (XE) or sorbitol and erythritol (SE). Interproximal (between the teeth) dental plaque and stimulated whole saliva were sampled at baseline, Day 36, and Day 64. There was a statistically significant

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reduction of S. mutans in plaque and saliva counts in Groups X and XE. The percentage of S. mutans in total streptococci increased significantly in dental plaque in Group S but decreased in the other groups. The results suggest that xylitol-containing saliva stimulants may be more effective than sorbitol-containing products in controlling some caries-associated parameters in people who are mentally disabled. 1 figure. 4 tables. 12 references. •

Inter-Relationship Among Degree of Mental Retardation, Living Arrangements, and Dental Health in Adults with Mental Retardation Source: SCD. Special Care in Dentistry. 17(1): 7-12. January-February 1997. Summary: This article reports on a study undertaken to investigate the interrelationship among the degree of mental retardation, living arrangement, and dental health in adults with mental retardation. One hundred and thirty-two adults between the ages of 21 and 40 years who had mental retardation were examined on two occasions, one year apart. All subjects had regular dental care for at least 10 years. The clinical examinations included bite-wing radiographs and were made by the same dentist. The degree of mental retardation was assessed by a professional psychologist. The results show that the degree of mental retardation as well as living arrangements influence the dental health of persons with mental retardation. Subjects with mild retardation had higher caries incidence and caries prevalence compared with subjects with moderate or severe mental retardation. From a preventive dental health perspective, special attention should be focused on subjects with mild mental retardation who are not living in institutions. 2 figures. 4 tables. 20 references. (AA-M).

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Children with Mental Retardation: Stigma and Stereotype Images Are Hard to Change Source: Journal of Dentistry for Children. 66(5): 343-347. September-October 1999. Contact: Available from American Society of Dentistry for Children. John Hancock Center, 875 North Michigan Avenue, Suite 4040, Chicago, IL 60611-1901. (312) 943-1244. Summary: This article, from the Journal of Dentistry for Children, addresses the difficulties of stereotypes and stigma associated with children with mental retardation. The authors note that the inappropriate stigma and stereotype images of children (and adults) with mental retardation have an impact on the daily lives of these youngsters and may well affect the implementation of needed health services. The authors review and question some long held and more recent attitudes and beliefs regarding individuals with mental retardation, in an effort to improve the availability of dental services for these children. The authors describe how children perceive and relate to other children with mental retardation, how adults perceive and relate to children with mental retardation, and how health care providers can recognize and help others to recognize the abilities of children with mental retardation. 37 references.

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Making Minds Meet: Assessment of Conversational Topic in Adults with Mild to Moderate Mental Retardation Source: Topics in Language Disorders. 13(3): 36-46. May 1993. Summary: This describes a strategy for assessing conversational topics in adults with mild to moderate mental retardation. The author presents a framework for the nature of 'topic' as it relates to adults with mental retardation. The framework consists of three levels of analysis: topic initiations, topic maintenance, and topic changes or disruptions.

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Mental Retardation

The author presents information on how topic identification difficulties exhibited by these adults is then used to design appropriate and effective intervention programs. Numerous examples of conversations between adults with mental retardation and clinicians or between peers with mental retardation are presented. 15 references. (AAM). •

Depression and the Onset of Dementia in Adults With Mental Retardation Source: American Journal on Mental Retardation. 96(5): 502-511. March 1992. Summary: This journal article describes a study that assessed the relationship between depressive symptoms and the onset of Alzheimer's disease in adults with Down syndrome or other mental retardation. Depression and declines in functioning were assessed in 61 adults with Down syndrome and a comparison group of 43 adults with mental retardation due to other symptoms. The subjects, who ranged from 20 to 60 years of age, were administered a neuropsychological battery to assess declines in function. Caregiver reports were used to assess subjects' adaptive behavior and depression. The results suggest that depression and dementia are associated in persons with Down syndrome, but not in persons with mental retardation due to other causes. All the subjects with Down syndrome who were identified as declining in function were also depressed, and 43 percent of those who were depressed were identified as declining. Only in the Down syndrome group was a greater severity of depression related to poorer performance on measures of mental age, memory, and adaptive skills. The implications of these results for treatment are discussed. 45 references.

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HIV/AIDS Risk Factors and Antibody Testing for People With Mental Retardation/Developmental Disabilities Source: HIV / AIDS & Mental Hygiene; April 1993. Contact: YAI National Institute for People with Disabilities Incorporated, AIDS Professional Education Program, 460 W 34th St 11th Fl, New York, NY, 10001-2382, (212) 273-6517, http://www.yai.org. New York University, School of Education Health Nursing and Arts Professions, Department of Health Studies, AIDS/SIDA Mental Hygiene Project, 35 W 4th St Ste 1200, New York, NY, 10012, (212) 998-5614. Summary: This journal article discusses HIV/AIDS risk factors and antibody testing as they relate to people with mental retardation and developmental disabilities. It describes why these populations are at risk, identifies significant risk behaviors which could potentially transmit HIV infection, and applies this information to issues of testing.

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Aging and Dementia Among Adults With Mental Retardation and Down Syndrome Source: Topics in Geriatric Rehabilitation. 13(3): 49-64. March 1998. Summary: This journal article reviews the literature on aging and dementia among people with mental retardation (MR) and Down's syndrome (DS). It reviews the epidemiology of Alzheimer's disease (AD) in the general population, AD neuropathology, and estimates of the risk of AD among adults with MR and DS. It explores factors complicating the diagnosis of dementia in people with MR, including the lack of standard assessment protocols for people with MR, limited communication skills in many people with MR, presence of other medical conditions that may mimic dementia, and difficulties in distinguishing AD from pseudodementia and in recognizing subtle signs of early AD. This article focuses on the association between AD and DS, methodological issues in research with the DS population, possible

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explanations for the discrepancy between the neuropathologic and clinical profiles of AD in the older DS population, and challenges in differentiating between dementia and developmental aging in DS. The article concludes with a discussion of the implications for future research and clinical practice. 109 references.

Federally Funded Research on Mental Retardation The U.S. Government supports a variety of research studies relating to mental retardation. These studies are tracked by the Office of Extramural Research at the National Institutes of Health.2 CRISP (Computerized Retrieval of Information on Scientific Projects) is a searchable database of federally funded biomedical research projects conducted at universities, hospitals, and other institutions. Search the CRISP Web site at http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen. You will have the option to perform targeted searches by various criteria, including geography, date, and topics related to mental retardation. For most of the studies, the agencies reporting into CRISP provide summaries or abstracts. As opposed to clinical trial research using patients, many federally funded studies use animals or simulated models to explore mental retardation. The following is typical of the type of information found when searching the CRISP database for mental retardation: •

Project Title: A MURINE MODEL OF SMITH-MAGENIS SYNDROME Principal Investigator & Institution: Boerkoel, Cornelius F.; Molecular and Human Genetics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-SEP-1999; Project End 31-AUG-2004 Summary: Smith-Magenis syndrome (SMS) is a multiple congenital anomaly mental retardation syndrome associated with a heterozygous deletion of human chromosome l7p11.2. This microdeletion syndrome has an estimated birth incidence of 1 in 20-25,000, making it one of the most frequently observed chromosomal deletions in humans. Clinical features include mental retardation, peripheral neuropathy, short stature, minor craniofacial anomalies, short fingers, microcornea, developmental defects of the heart and kidneys, and neurobehavioral abnormalities. The complex phenotype suggests deletion of several contiguous genes and is hypothesized to result from haploinsufficiency. Although several genes have been identified in the SMS common deletion interval, their contribution to this complex phenotype remains speculative. Chromosome 17p11.2 is syntenic to the 32-34 cM region of murine chromosome 11. Several genes have been mapped to both the mouse and human regions of synteny. Other genes in l7p11.2 also likely have murine homologues. This proposal seeks to characterize which gene or group of genes is responsible for SMS by using chromosome engineering to construct deletions of those regions of mouse chromosome 11 that are syntenic for the human SMS deletion interval. Extensive characterization of the engineered mice will then be performed to determine the consequences of gene haploinsufficiency. These analyses will contribute to the understanding of the molecular basis of the SMS chromosomal microdeletion syndrome and will have potent

2

Healthcare projects are funded by the National Institutes of Health (NIH), Substance Abuse and Mental Health Services (SAMHSA), Health Resources and Services Administration (HRSA), Food and Drug Administration (FDA), Centers for Disease Control and Prevention (CDCP), Agency for Healthcare Research and Quality (AHRQ), and Office of Assistant Secretary of Health (OASH).

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implications for human development and biology. The career development aims of this proposal have been designed to provide the candidate with the tools necessary for human disease gene identification and analysis as well as with the capability of developing murine models of human disease. Baylor College of Medicine provides an environment that is unparalleled for developing murine models and that is ideally suited to prepare highly motivated individuals for careers in academic medicine. The Mentored Scientist Development Award would further facilitate this process. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: ACTION TREMOR & DEMENTIA IN MALE CARRIERS OF FRAGILE X Principal Investigator & Institution: Grigsby, James P.; Medicine; University of Colorado Hlth Sciences Ctr P.O. Box 6508, Grants and Contracts Aurora, Co 800450508 Timing: Fiscal Year 2003; Project Start 15-SEP-2003; Project End 30-JUN-2008 Summary: (provided by applicant): The purpose of this application is to define the clinical features of a newly identified, progressive neurologic disorder consisting of intention tremor, ataxia, and dementia, with generalized brain atrophy and inclusion bodies, among older men with the fragile X premutation. Fragile X syndrome (FXS) is a developmental disorder involving a trinucleotide repeat expansion (CGG) in the fragile X mental retardation 1 gene (FMR1). The full mutation is associated with mental retardation among males and milder impairment of cognition among females. Carriers of the FXS gene are said to possess the premutation, a smaller trinucleotide expansion (55 to 200 CGG repeats). The premutation generally has been associated with a normal or nearly normal cognitive and anatomic phenotype, but recent data suggest the premutation phenotype may include subtle developmental anomalies. Our research over the past two years suggests that a subgroup of adult males with the premutation develop a neurologic disorder resembling some of the spinocerebellar ataxias. It is first clinically apparent when the men are in their fifties or sixties, and is characterized by action tremor and other motor findings, dementia, and generalized brain atrophy. The prevalence of this disorder has not been definitively established, but our data suggest it may be between 20 and 78 per I00,000 males, which is common enough that it would represent an important public health problem. Since our previous submission of this application, we have obtained neurologic, radiologic, neuropsychological, and neuropathologic data on a number of additional patients, and the condition has become the focus of study for other investigators in the United States. and abroad. The proposed study will compare a sample of FXS carrier males with this tremor-ataxia disorder to an age- and education-matched sample of carrier males without tremor or other signs of neurologic disorder, and a matched group of healthy men without FXS involvement. The measures used include a comprehensive standardized neurological evaluation, neuropsychological examination, functional assessment, MRI of the brain, and basic molecular studies. Data will be collected at baseline, and at 18-month and 36-month follow-up points. Data analysis will include between-groups and repeated measures methods. The results of this study will provide important information on the clinical features of this previously unidentified disorder, including its rate of progression and its relationship to the FXS premutation. Further knowledge of the nature of this phenotype, and of its association with the FMR1 gene, is of substantial clinical importance for the differential diagnosis, management, and appropriate treatment of movement disorders among older males. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: ADAPTATION IN FAMILIES OF CHILDREN WITH MR Principal Investigator & Institution: Floyd, Frank J.; Professor of Psychology; Psychology; Georgia State University University Plaza Atlanta, Ga 30303 Timing: Fiscal Year 2001; Project Start 08-SEP-1987; Project End 31-AUG-2003 Summary: Family adaptation to children with mental retardation involves mutual and continuous influences among children and family members throughout the life course. Because data on continuity and longer-term outcomes are limited, the present investigation evaluates how patterns of mutual influence unfold throughout childhood, adolescence, and into adulthood. The investigation proposes to collect a fourth wave of data for a prospective, longitudinal study of 200 children with mild and moderate mental retardation and their families who began the study up to 12 years earlier when the children were 6 to 18 years old. The sample consists of three age cohorts, and the four waves of data will provide overlapping assessments to evaluate changes across two major developmental transitions, adolescence and young adulthood. Telephone interviews, mailed surveys, and face-to-face interviews will be conducted with the participants who have MR, their mothers, fathers, selected siblings, and, as needed, other key informants to assess current adaptive functioning, maladaptive behaviors, and occupational and social functioning for the person with MR, along with personal wellbeing for family members, and the quality of family relationships. Their extensive data on earlier child functioning and family relationships will allow the investigators to construct hierarchical linear models to focus on understanding mechanisms of influence surrounding three developmental issues: 1) how families promote social development and adaptive functioning for the child 2) how caregiving affects the personal well-being of parents and siblings, and 3) how family relationships adapt over the life course. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: BAYLOR MENTAL RETARDATION RESEARCH CENTER Principal Investigator & Institution: Zoghbi, Huda Y.; Professor and Investigator; Molecular and Human Genetics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-AUG-1988; Project End 31-JUL-2004 Summary: They Baylor College of Medicine (BCM) Mental Retardation Research Center (MRRC) was established August 1, 1988 and has been continuously funded with a renewal of funding August 1, 1993. The overall goal of the Baylor MRRC is to use a multidisciplinary approach to move mental retardation research into a new phase aimed at understanding pathogenesis and implementing therapy. The specific objectives are: (1) To enhance mental retardation research activities at BCM by focusing the research efforts on etiology, diagnosis, prevention, pathogenesis, treatment and amelioration of MRDD; (2) To continue to promote a multi-disciplinary approach to mental retardation research by improving interactions between Center investigators and by continuing to develop and to apply cutting edge technology; (3) To facilitate the research efforts and to enhance the productive of project investigators through cost effective and efficient research core units; (4) To recruit new investigators into the field of mental retardation research through scientific interactions with Center investigations and through the New Program Development Awards; and (5) To promote scientific and collaborative interactions with investigators outside Baylor ho have demonstrated a major commitment to study mental retardation. The research projects will be supported by the Administrative Core (A) and by seven Research Cores: Biomedical Assessment (B); Genomics (C); Tissue Culture (D); Transgenic Mice (E); Embryonic Stem Cell

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Technology (F); Neurobehavior and Synaptic Plasticity (G): and Developmental Neuropathology (H). There are 45 faculty participants, including 37 research projects investigators and 49 research projects. The research score of the BCM MRRC will include the following nine topic areas: Developmental neurobiology, inborn errors of metabolism, genetic/cytogenetic disorders, infectious diseases, diagnosis, early intervention, psychobiological processes, and epidemiology. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BEHAVIORAL AND BIOCHEMICAL MECHANISMS OF SELF INJURY Principal Investigator & Institution: Symons, Frank J.; Assistant Professor; Educational Psychology; University of Minnesota Twin Cities 200 Oak Street Se Minneapolis, Mn 554552070 Timing: Fiscal Year 2001; Project Start 01-JUL-1997; Project End 31-AUG-2004 Summary: Why some people with mental retardation and/or autism repeatedly and persistently injure themselves, some so severely to the point of tissue damage and often times permanent scarring, has remained a mystery eluding a single solution. Unraveling this mystery poses paradoxial biomedical and behavioral science questions and creates deeply troubling problems for practitioners and family members of affected individuals. Over the past decade, many cases of self-injurious behavior (SIB) have been treated successfully using behavioral interventions that teach communication and other functional skills. Practical problems of implementation, costs associated with long-term treatment, and cases with no clear social profile appearing about 1/3 of the time suggest, however, that there is still much to be learned about why people self-injure. Our overall goals are to improve treatment, refine diagnosis, and to clarify mechanisms underlying different forms of self-injury. Given the severity of self-injury, it is surprising that few of the models have examined in more detail the relation between variables common to SIB and the neurophysiology of pain regulation. The main objective of this project is to evaluate the validity of several of these variables as possible predictors of response to self-injury. Treatments will be based on the hypothesis that some forms of self-injury involve intense stimulation of body sites sufficient to elicit the release and receptor binding of endogenous opioid peptides. Accordingly, treatments will include transcutaneous electric nerve stimulation (TENS)(an opioid agonist treatment) or naltrexone (an opioid antagonist treatment). Predictors will include observationallybased measures of the environmental functions of self-injury, body site location and intensity of self-injury, and salivary baseline levels of three bioactive substances (substance P, metenkephalin, & cortisol). Following initial identification of subjects (age range 4-25) with mold to profound mental retardation and/or autism, our first aim is to observe and describe in detail how frequently self- injury occurs, what its duration and intensity is, and where on the body it is directed. Following this characterization, substance P, met-enkephalin, and cortisol will be noninvasively examined through saliva as markers for altered pain transmission and predictors of response to treatment. After screening and SIB subtyping (i.e., social, nonsocial, or mixed) 37 subjects whose self-injury is primarily nonsocial or mixed will be evaluated over a 16-week period with TENS and the opiate antagonist naltrexone for self-injury. Subjects whose self-injury is primarily socially motivated will be evaluated with TENS and the opiate antagonist naltrexone for self-injury. Subjects whose self-injury is primarily socially motivated will be evaluated with TENS and receive behavioral interventions through a technical assistance service delivery model. Three- and six-month follow-ups will be conducted for each subject.

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Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BEHAVIORAL TECHNOLOGY FOR TEACHING MATCHING SKILLS Principal Investigator & Institution: Mahon, Karen L.; Praxis, Inc. 13 West St Belmont, Ma 02478 Timing: Fiscal Year 2001; Project Start 01-JUN-2001; Project End 31-MAY-2003 Summary: This application seeks support for an SBIR Phase II project to complete development and evaluation of a computer-based product, StartMatching!, which is intended primarily for teaching children with mental retardation, autism, and other intellectual disabilities. The product addresses a pivotal skill --generalized identity matching to sample -- which is a target of many current programs for teaching this population (and also for teaching young typically developing children). In identity matching, students are presented with an array of two- or three-dimensional stimuli and required to select the item that physically matches (i.e., is identical to) a sample; hence the name "matching to sample." The project has two major objectives. First, based on a successful prototype developed in Phase I, we will develop a full-feature version of the StartMatching! product, in anticipation of commercialization. Second, we will verify that the product (1) can be implemented effectively by its intended users and (2) teaches matching skills reliably in settings in which it will be routinely used. PROPOSED COMMERCIAL APPLICATION: The product being developed and evaluated may have a significant impact on special education practice for a variety of student populations. As such, the product is potentially marketable to educators, psychologists, other professionals, and parents. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: BIOCHEMICAL RETARDATION

AND

GENETIC

ASPECTS

OF

MENTAL

Principal Investigator & Institution: Jungalwala, Firoze B.; Professor; Eunice Kennedy Shriver Center; Univ of Massachusetts Med Sch Worcester Office of Research Funding Worcester, Ma 01655 Timing: Fiscal Year 2001; Project Start 01-SEP-1977; Project End 30-NOV-2005 Summary: There is no text on file for this abstract. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BIOLOGICAL BASIS OF MENTAL RETARDATION Principal Investigator & Institution: Schwartz, Nancy B.; Professor; Pediatrics; University of Chicago 5801 S Ellis Ave Chicago, Il 60637 Timing: Fiscal Year 2001; Project Start 01-JUN-1975; Project End 31-DEC-2003 Summary: The unifying goal of this program project proposal is to define the principles governing normal nervous system developmental processes that may lead to brain dysfunction and mental retardation. The studies for the most part are basic in nature, focusing on various aspects of the cell and its environment that are involved in overall developmental processes; including examination of the extracellular matrix (ECM), cell membrane gap junctions, cytoskeletal proliferation. The derivative information from these proposed studies should define specific loci where normal developmental processes. The first project focuses on the structure and function of brain proteoglycans. The emphasis is on control of one of the major chondroitin sulfate proteoglycans

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(aggrecan) and elucidation of the role this important ECM component plays in neuronal development. The second project will examine intracellular communication which may play a significant role in embryonic neural development by specifically studying Connexin45, a subunit gap junction protein with special permeability properties. The third project focuses on function of NF1 protein in central nervous system neurons and astrocytes using a combination of biochemical, molecular biology and confocal microscopy techniques in tissue culture models from the chick embryos and human CNS cell lines. The fourth project is aimed toward understanding the pathogenesis of Batten disease. The aim is to develop diagnostic biochemical assays for the enzymes deficient in CLN2 (PPT) and CLN2 (endoprotease) and to determine the cause of neuronal death. A comprehensive multi-disciplinary approach using biochemical, molecular, morphologic and cell culture techniques will be used in all four projects. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: BOLD FMRI IN MR RESEARCH TO MAP LEARNING HISTORIES Principal Investigator & Institution: Schlund, Michael W.; Assistant Professor; Kennedy Krieger Research Institute, Inc. Baltimore, Md 21205 Timing: Fiscal Year 2003; Project Start 26-SEP-2003; Project End 31-AUG-2005 Summary: (provided by applicant): Successful development and adaptation requires, in part, proper performances in terms of how to respond, when to respond, and what consequences may follow responding. Defining characteristics of developmental disabilities and mental retardation are deficits in responding appropriately to environmental stimuli associated with various contingencies. Primary among these are the learning requirements to reach proper levels of responding. To understand the neural substrates underlying learning processes in healthy adults, previous PET and fMRI investigations image brain regions during delivery of reinforcers or punishers. The extent to which similar brain regions are involved in discriminating among stimuli associated with different contingencies is unclear, but equally important to understanding the neural processes involved in learning. This application presents data from a preliminary investigation using BOLD fMRI to examine relations between a history of discrimination learning and neural patterns of activation in a small number of adult human subjects. Preliminary results show stimuli associated with a reinforcement contingency produced a wide range of activation patterns when contrasted with a stimulus not associated with a contingency. Before conducting studies comparing subjects with and without differing levels of mental retardation, the investigators need to replicate their preliminary work with a sufficient number of non-mental retardation subjects to determine the usefulness of the stimulus-based imaging approach in either differing between conditioning histories or identifying neural substrates that map these histories. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CELL DEVELOPMENT

ADHESION

MOLECULES

IN

NERVOUS

SYSTEM

Principal Investigator & Institution: Lemmon, Vance P.; Professor; Neurosciences; Case Western Reserve University 10900 Euclid Ave Cleveland, Oh 44106 Timing: Fiscal Year 2001; Project Start 02-APR-2001; Project End 31-MAR-2006 Summary: (From the Applicant's Abstract): The neural cell adhesion molecule L1 is found on some classes of migrating neuronal precursors in the developing nervous system and on almost all projection axons in both the central nervous system and

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peripheral nervous system. Not surprisingly, it has been implicated in the fasciculation of axon bundles and in migration of some neural precursors in various in vitro systems. In the early 1990's it was shown that mutations in the L1 gene in humans cause severe mental retardation (corpus callosum hypoplasia, adducted thumbs, spastic paraplegia, and hydrocephalus). We have analyzed individuals with different mutations in the L1 gene and discovered that mutations that lead to a loss of L1 expression are much more severe than mutations that only alter the cytoplasmic domain of L1. However, mutations of the cytoplasmic domain are sufficient to cause axon guidance failures and mental retardation. Recently, we and others have analyzed the L1 knock-out mouse and discovered that it has a phenotype remarkably similar to humans with X-linked hydrocephalus. This includes hydrocephalus, abnormal development of the corticospinal tract, and hypoplasia of the cerebellar vermis and corpus callosum. In this project we propose to test the hypothesis that L1 mediated adhesion is essential for normal development of the cerebellar vermis and that the function of the L1 cytoplasmic domain is essential for development of the corticospinal tract. To do this we will generate new mouse lines with specific alterations in the L1 cytoplasmic domain. We will also analyze mice in which the 6th Ig domain of L1 has been removed, deleting the RGD sequence in L1, allowing us to evaluate the difference between L1 homophilic binding and L1-integrin interactions during brain development. Finally, we will undertake the first careful analysis of cerebellar development in mice with altered or absent L1. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CELLULAR/MOLECULAR PATHOPHYSIOLOGY OF MENTAL RETARDATION Principal Investigator & Institution: Lipton, Stuart A.; Director, Degenerative Disease; Burnham Institute 10901 N Torrey Pines Rd San Diego, Ca 92037 Timing: Fiscal Year 2001; Project Start 01-SEP-1992; Project End 31-AUG-2004 Summary: The unifying theme of the revised, competing renewal of this Program Project is a cellular and molecular approach to developmental neurology in an attempt to uncover processes leading to neonatal brain injury and mental retardation. Four inter-related projects are planned. They all concern the influence of overstimulation of the N-methyl-D-aspartate (NMDA) subtype of glutamate receptor and related signaling pathways to neuronal damage during hypoxic-ischemic brain injury and AIDS. Hypoxic -ischemic insults are known to result in mental retardation and developmental delay. Only recently, however, has it become apparent that AIDS is associated with an approximately 50% incidence in delayed milestones and frank cognitive decline in children infected with HIV-1. This Program Project Group of Investigators and others have shown that at least part of this damage to the nervous system appears to be mediated by excessive NMDA receptor activation that is not adequately treated by currently available therapeutic regimens. This grant plans to study the underlying molecular mechanisms that can be used to down regulated NMDA receptor activity in aq clinically tolerant manner using a combination of techniques ranging from molecular biology and gene-targeting to patch-clamp recording. Two types of clinically tolerated NMDA antagonists are being developed: (1) open- channel blockers of the memantine class of compounds, and (2) nitric oxide (NO) related species (using nitroglycerin). Additional novel agents include a combinatorial drug, nitro-memantine, which targets NO species to NMDARs via the open-channel blocker. In projects, these novel, clinically-tolerated NMDA antagonists will be tested in animal models of hypoxicischemic and HIV-related brain injury. These projects will also explore signaling

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pathways downstream to NMDAR activity which mediate neuronal necrosis or apoptosis and involve nitric oxide and caspases. Rodent models consisting of transgenic and knockout mice will be used. Project has cloned, characterized and generated mice deficient in a new NMDAR subunit NR3A. The CORE supplies support for statistics, tissue culture, and magnetic resonance imaging/spectroscopy (MRI/MRS). An important feature of this Program Project is its multi-disciplinary approach using molecular biology, electrophysiology and imaging techniques both in vitro and in vivo. The team has a proven track record of hypothesis testing to help elucidate the pathogenesis of hypoxic-ischemic and AIDS-related injury in the brain, and then of using this knowledge for drug development, leading to human clinical studies. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CENTER GRANT FOR RESEARCH IN MENTAL RETARDATION Principal Investigator & Institution: Crnic, Linda S.; Professor of Pediatrics and Psychiatry; Pediatrics; University of Colorado Hlth Sciences Ctr P.O. Box 6508, Grants and Contracts Aurora, Co 800450508 Timing: Fiscal Year 2001; Project Start 01-AUG-1988; Project End 31-JUL-2003 Summary: This application is for continued support of the administrative and research cores of the BF Stolinsky Laboratories Mental Retardation Research Center at the University of Colorado Health Sciences Center. This MRRC began its operations in 1968, and most of its investigators and core are now housed in a new and larger facility on the UCHSC campus. The research of all investigators that utilize the cores impacts substantially on mental retardation and developmental disabilities. Most of their work is on heritable cause of central nervous system dysfunction, with a primary focus on inborn errors of metabolism and specifically on disorders of mitochondria. Substantial effort is also directed to environmental causes of MRDD, including trace metal deficiencies and viral infections, and how these conditions affect neuropsychological processes and cognitive development. Studies on genetic (metabolic) causes of central nervous system dysfunction focus on glutaric acidemia type I and II (Goodman & Frerman), cystathionine beta-synthase deficiency (Kraus), propionic acidemia (Kraus), and Down syndrome (Patterson & Gardiner)); the primary aim of these studies is to better understand pathogenesis, so that efforts to treat can have a more science-based rationale. Studies of environmental causes of MRRD examine the impact of zinc deficiency in infancy and gestation (Krebs), and of latent viral infections in the newborn period (Crnic) on subsequent cognition. Others focus on developmental (learning) disorders such as schizophrenia (Ross, Leonard), dyslexia, attention deficit hyperactivity disorder, and autism (Rogers & Pennington; early events in neuronal development of the human CNS (Pfenninger); and neuromuscular defects related to sodium channels (Caldwell). Core facilities for which support is sought include: a) administration, b) Cell Biology, c) Chemistry/Mass Spectrometry, e) Large Insert Library Screening, f) Developmental Neuropsychology, g) Animal Housing and Assessment, and h) Instrument maintenance and Glassware Washing services. Core e) is new to this application. We also propose an association with the MRRC at Baylor College of Medicine, in which investigators at each institute can learn techniques from one another and, in some cases, utilize each others cores. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CHARACTERIZATION OF A NOVEL FRAGILE X INTERACTING GENE Principal Investigator & Institution: Zarnescu, Daniela C.; Cell Biology; Emory University 1784 North Decatur Road Atlanta, Ga 30322 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2006 Summary: (provided by applicant): Fragile X syndrome is the most frequent form of inherited mental retardation, affects about 1 in 3,500 males and to date has no cure. Patients have a pleiotropic phenotype that includes mental retardation, facial dismorphia as well as attention deficit and hyperactivity disorder. The disease is caused by mutations in the Fmr1 gene which has a single homolog in Drosophila: dFmr1. To unravel novel players with key roles in the disease mechanism of fragile X syndrome, we recently developed and conducted a genetic screen for dominate modifiers of dFmr1 over-expression in Drosophila. We identified a single major autosomal modifier which we mapped to the lethal (2)giant larvae (I(2)gl)locus). I(2)gl is a component of the cytoskeleton and loss of function mutations lead to neoplastic tumors. On one hand, Lgl bindsmyosin II and interacts genetically with both myosin II and V and on the other hand, FMR protein is involved in transport and translational regulation of target mRNAs. In addition, the latter associates with myosin V to form a common RiboNuclear Particle (RNP). Taken together this data suggest that Lgl and FMR associate physically in a protein complex, perhaps an RNP equiped with molecular motors which enable its travels on the major cellular highways comprised of microtubule and microfilament networks. Specific predictions of this model will be tested in the proposed project: i) I(2)gl phenotypes should overlap with those of dFmrl mutants; ii) 1(2)gl interacts genetically with dFmr1; iii) Lgl and Fmr1 associate in a common protein complex, be it directly, or through an intermediate partner. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CHILD DEVELOPMENT AND MENTAL RETARDATION Principal Investigator & Institution: Piven, Joseph; Professor; Psychiatry; University of North Carolina Chapel Hill Office of Sponsored Research Chapel Hill, Nc 27599 Timing: Fiscal Year 2001; Project Start 01-SEP-1977; Project End 31-JUL-2003 Summary: The North Carolina Mental Retardation and Developmental Disability Research Center (MRRC) is a diverse and coordinated program to advance knowledge concerning the etiology and treatment of mental retardation. The causes of mental retardation and developmental disabilities are multiple. The few hundred suspected or identified represent only a fraction of the total number of causes. Many moderate to severe conditions of mental retardation have biological causes identifiable with currently available technology; however, biological causes of the majority of less severe mental retardation are presently unknown. The boundary between biological and psychosocial factors in mental retardation is becoming less distinct, not only because progress in biological sciences has led to the identification of more causes, but more importantly because there is increasing awareness of the importance of the interactive influence of psychological events of biology and of biological processes on psychological consequences. This broad-based approach remains the guiding principle of the MRRC. Thus, our research programs encompass the broadest range of concepts, disciplines and methodologies, from social and behavioral sciences to fundamental molecular genetics and developmental neurobiology. While preserving this diversity, we focus much of our work on eight major areas on research emphasis: (1) early intervention effectiveness and processes, (2) family processes and interventions, (3) language, cognitive, and social

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Mental Retardation

processes, (4) neurohormones and maternal behavior, (5) brain development: molecular genetics, biochemistry, toxicology and pathology, (6) neurogenetic disorders including fragile X syndrome, (7) neurotransmitter pharmacology and brain function, and (8) interaction of the immune system and the brain. MRRC researchers represent diverse disciplines and university departments. The majority of MRRC research projects are conducted in two university Centers: The Neuroscience Center (UNCNC, Kunihiko Suzuki, Interim Director) and the Frank Porter Graham Child Development Center (FPG, Don Bailey, Director). The MRRC operates as an integrated program to establish an optimal interdisciplinary environment by providing an Administrative ore and four scientific core units: Information Technology (Richard Mailman, Director), Design and Statistical Computing (Market Burchinal, Director), Observational Methods (Maria Boccia, Director) and Morphology- Pathology (Kinuko Suzuki, Director). These research efforts are augmented by existing training programs in mental retardation and related fields, a State-supported Genetic Counseling Program, and a visiting lecturer seminar series. The UNC-Chapel Hill and the State of North Carolina provide extensive support to the MRRC. By efficiently coordinating specialized core support services and by providing a common administrative framework to these diverse research programs, the MRRC achieves a cost- effective means for conducting research that seeks eventual understanding, treatment, and prevention of mental retardation and developmental disabilities. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: RETARDATION

CHROMOSOME

REARRANGEMENTS

AND

MENTAL

Principal Investigator & Institution: Lupski, James; Professor; Molecular and Human Genetics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 11-JUN-2001; Project End 31-MAY-2006 Summary: Chromosomal abnormalities are responsible for a significant fraction of children with mental retardation. Advances in molecular cytogenetic techniques as well as the tremendous progress of the Human Genome Project, have now made it possible to determine the molecular basis of chromosomal abnormalities. From patients with specific chromosome rearrangements including (i) balanced translocations, (ii) interstitial deletion/duplication and (iii) terminal deletion. The q11 region of chromosome 22 is susceptible to a multitude of rearrangements including translocations, leading to congenital anomalies and malignant disorders. Deletions and duplications of 17p11.2 are associated with Smith-Magenis syndrome and a mental retardation syndrome, respectively. Both the 22qll and 17p11.2 rearrangements may be mediated by low-copy repeat sequences that occur in the vicinity of recurrent chromosome breakpoints. Novel terminal deletions of the 1p36 region delineate a new mental retardation disorder with a different mechanism of rearrangement since the deletion end-points very significantly among patients. The three chromosomal regions will serve as models for our Program. We will examine the intervals that harbor the chromosome breakpoints by fluorescence in situ hybridization in situ hybridization (FISH) and pulsefield gel electrophoresis (PFGE) mapping approaches and breakpoint junctions will be cloned and sequenced to determine the mechanism of rearrangement. This projects will be integrated with a fourth project to determine the evolutionary basis of these regions that are prone to breakage in humans. Our functional genomics approach will capitalize on vast resources of DNA samples, cell lines, and Genome Project, as well as cell lines and reagents from non-human primates, that are available to the individual Project P.I.'s. Results from these studies will greatly impact our understanding of chromosome

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rearrangement causing mental retardation, will likely lead to development of novel diagnostic tools and may change the way medical genetics is practiced with regard to families having an individual with a chromosome rearrangement. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MUTATIONS

CLINICAL

PATHOPHYSIOLOGY

OF

RETT

AND

MECP2

Principal Investigator & Institution: Glaze, Daniel G.; Associate Professor Pediatrics & Neurolo; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 23-JUL-2001; Project End 31-MAY-2006 Summary: Project 2 will study the clinical pathophysiology of Rett Syndrome (RTT) and MECP2 mutations. Three specific aims will be addressed. Mutations in the MECP2 gene have been shown to cause a wide phenotypic spectrum ranging from mild learning disabilities to classic RTT in females and several neonatal encephalopathy in males. Females with some features of RTT or mental retardation of unknown etiology may have mutations in the MECP2 gene. MECP2 mutations may also be responsible for variant cases and have a specific phenotype/genotype formulation distinct from classic RTT. (1) To define the phenotypic spectrum of children with MECP2 mutations, we will study 150 girls who are known to display autistic features, learning disabilities, or nonsyndromic mental retardation of unknown etiology and 25 siblings or near relatives of girls with RTT who have associated learning or behavior problems. (2) To carry out extensive phenotype/genotype correlation studies in RTT, we will study 200 girls with classic RTT and 50 girls with atypical RTT. To accomplish these two specific aims, clinical data will be collected and blood will be obtained for DNA mutational analysis to be conducted by r. Zoghbi (Project 1). (3) The combined results of this program project will improve our understanding of the pathogenesis and enhance our ability to make the diagnosis of RTT and extend diagnosis to other neurodevelopmental disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CLINICAL SYNDROMES ASSOCIATED WITH MTDNA POINT MUTATIONS Principal Investigator & Institution: De Vivo, Darryl C.; Sidney Carter Professor of Neurology; Columbia University Health Sciences New York, Ny 10032 Timing: Fiscal Year 2001; Project Start 01-DEC-2000; Project End 30-NOV-2001 Summary: Mitochondrial (mt)DNA point mutations may produce severe clinical manifestations. Multiple organs may be vulnerable but none more than the nervous system. We are engaged in a long-term multi-faceted mutation. We hypothesize that maternally-inherited mtDNA point mutations cause chronic progressive encephalopathies and mental retardation. To date, we have studied 17 families with the 32143 mutation, 2 families with the 8344 mutation, and 1 family each with the 3271, 8993 and the 5537i mutation (Santorelli et al 1997). In the aggregate, 90 research subjects have been admitted to the Irving Center for Clinical Research-60 from families with the 3243 mutation, 15 from families with the 8344 mutation, 3 from families with the 8993 mutation, 8 with the 5377i mutation, and 4 with the 3271 mutation. These 90 subprojects represent probands (22), paternal controls (16) and asymptomatic/oligosymptomatic maternal relatives (52). We plan to follow this patient cohort for the next five years, and to admit additional patients with the 3243 and the 8344 mutation. Restricting entry to these two relative common mutations will allow us to address specific questions regarding pathophysiology, treatment, and prognosis in a more genotypically

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Mental Retardation

homogeneous population. Each family undergoes a comprehensive clinical and geneticmetabolic staging of their medical condition (Specific Aim #1), and responds to a questionnaire that is designed to evaluate the natural history of mtDNA-associated encephalomyopathies (Specific Aim #2). Functional MRI (BOLD effect) and MRSI studies (brain lactate concentrations) will be registered to assess the ability of a brain region to sustain mental work (Specific Aim #3). These patients will be selected according to their ability to participate in these standardized procedures. Selected patients with the "MELAS" 3243 point mutation and elevated brain lactate will receive dichloroacetate or placebo in a randomized, blinded, 2-treatment controlled clinical trial (Specific Aim #4). This trial will incorporate a 3- period crossover study design and will focus on patients with the 3243 mutation-known to be associated with high brain lactate levels and a more devastating, truncated clinical course (Sano et al 1998; Kaufmann et al 1999). Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CLONING GENES THAT CAUSE MENTAL RETARDATION Principal Investigator & Institution: Srivastava, Anand K.; Greenwood Genetic Center 1 Gregor Mendel Circle Greenwood, Sc 29646 Timing: Fiscal Year 2001; Project Start 10-AUG-2000; Project End 31-MAY-2003 Summary: Mental retardation (MR) is a common condition affecting about 2-3% of the human population. It reflects a diversity of potential causes that include both genetic and environmental factors. The causation in at least half of all MR cases is still unknown. The genetic component of MR, which consists of chromosome aberrations (approximately 12%), single gene defects (approximately 10%) and multi-factorial effects (approximately 10%), is likely due to defects in a large number of autosomal and Xlinked genes. The goal of this proposal is to identify, clone, and characterize a selected set of genes involved in brain development and function, which, when defective, cause mental retardation. The long-term objective of our research is to aid in the understanding of brain development and function that play a role in cognitive learning abilities. The characterization of disease-associated translocation breakpoints has proven to be a productive strategy to identify the genes responsible for specific disorders. We have identified several patients with balanced chromosome translocations (X; autosome and autosome; autosome) and MR. The Specific Aims of this proposed research are designed to 1) fine map the translocation breakpoints delineating potential MR loci, 2) identify and isolate the genes, associated with the translocation breakpoints, and 3) characterize and confirm the identify of the MR genes and 4) as an extension of the proposed work, to elucidate the role of encoded proteins in brain development and function. Identification and characterization of the genes involved should be a major advance in the understanding of brain functions critical for development of intellectual and learning abilities as well as facilitating objective diagnosis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: CNS MALDEVELOPMENT AND PERINANTAL INFECTION Principal Investigator & Institution: Britt, William J.; Professor; Pediatrics; University of Alabama at Birmingham Uab Station Birmingham, Al 35294 Timing: Fiscal Year 2003; Project Start 08-SEP-2003; Project End 30-JUN-2008 Summary: (provided by applicant): Perinatal infections, including intrauterine viral and parasitic infections are major causes of central nervous system (CNS) disease in infants and children. In some instances, such as congenital human cytomegalovirus (HCMV),

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these infections occur relatively frequently. Current estimates suggest that congenital HCMV is the most common viral cause of CNS disease and mental retardation in the United States. Although HCMV is a well-described causes of perinatal infections, the pathogenesis of this infection is poorly understood. Studies in humans have been limited to observational studies and current animal models have provided few clues to the mechanism of CNS disease. We have developed a small animal model of perinatal infection that recapitulates several of the more distinct findings of CNS HCMV infection. In particular, we have shown that non-lytic viral infection of the brain is associated with maldevelopment of the newborn brain, including cerebellar hypoplasia and loss of normal granular neuron migration. Disease induction in these animals appears to require both virus replication in the brain and an inflammatory infiltrate in the meninges and parenchyma. In this proposal we will define the mechanisms of disease, including loss of normal cerebellar development that are associated with CNS virus infection. These studies will provide a greater understanding of the pathogenesis of perinatal infections and could lead to improved therapy of these potentially treatable causes of mental retardation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: COGNITIVE STRENGTHS AND WEAKNESSES IN DOWNS SYNDROME Principal Investigator & Institution: Fidler, Deborah J.; Human Develmt & Family Studies; Colorado State University Fort Collins, Co 80523 Timing: Fiscal Year 2003; Project Start 01-JUN-2003; Project End 31-MAY-2005 Summary: (Provided by Applicant): For individuals with Down syndrome, the most common genetic mental retardation syndrome, research is converging on a cognitive profile consisting of poor verbal working memory and relatively stronger visuo-spatial processing. The implications of this research point to changes in the way children with Down syndrome are taught. Yet this information has remained relatively under-utilized by practitioners, possibly because the visuo-spatial processing relative strength remains poorly understood. In this application, the investigator proposes to: in Specific Aim 1, describe the strengths and weaknesses within the visuo-spatial relative strength in Down syndrome; and in Specific Aim 2, explore the relationship between performance on domains within visuo-spatial processing and other domains of processing (i.e. verbal working memory, auditory processing, expressive vocabulary, reading/decoding, logic). In subsequent research endeavors, the investigator also plans to identify strategies for improving auditory-dependent outcomes in Down syndrome (such as language acquisition) through the relative strength in visuo-spatial processing. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: COMMUNICATION OF PEOPLE WITH MENTAL RETARDATION Principal Investigator & Institution: Sherman, James A.; Bureau of Child Research; University of Kansas Lawrence Lawrence, Ks 66045 Timing: Fiscal Year 2001; Project Start 01-DEC-1984; Project End 31-JAN-2005 Summary: Understanding of language and communication is crucial to understanding mental retardation because they control, express, or mediate much of our three major developmental spheres: the intellectual, social, and physical. For this reason, language and communication must be a central focus of research in mental retardation. The proposed research was guided by a conceptualization of human development that stressed the centrality of the developmental cusp an intersection in the interaction of

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Mental Retardation

behavior, skill, perception, or motivation, that is crucial to what should come next in successful negotiation, however, permits a significant set of subsequent developments to occur. The cusps chosen to be studied in the proposed research appear critical to the process of communicative development and are representative of a diversity of points (from early and rudimentary to late and sophisticated). They are representative of the problems of a diversity of people including those with mental retardation. They span the intellectual, social, and physical spheres of development, and seem amenable to realistic intervention. Thus, the results of our research can contribute directly to preventing and/or ameliorating significant aspects of mental retardation. Our plan is to enable persons with mental retardation to: learn how to demonstrate their preferences and choose among them, primarily by learning to discriminate reinforcement contingencies (Project I, Saunders & Saunders); put themselves more frequently in contexts that support and enhance their attempts to communicate, primarily by developing positive social relationships (Project II, Sherman & Sheldon); learn to maintain communication attempts until communication is achieved primarily by learning to make communication repairs (Project III, Brady); and, use language-life skills to affect their own problem-solving abilities, primarily by learning to self-instruct (Project IV, Baer & Grote). Hart (Project V), working from an extensive database of language samples collected in prior years, proposes to continue to increase our understanding of the similarities and differences between the language development of children with Down syndrome and typically developing children, primarily by examining the development of declarative sentences as they may be related to the enhancement of expressive language. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CONFERENCE ON INTELLECTUAL DISABILITY, AGING AND HEALTH Principal Investigator & Institution: Davidson, Philip W.; Pediatrics; University of Rochester Orpa - Rc Box 270140 Rochester, Ny 14627 Timing: Fiscal Year 2002; Project Start 01-SEP-2002; Project End 31-AUG-2003 Summary: (provided by applicant): Over the past few years, NIA has recognized the need for more research on aging in the I/DD population. Few competitive grants addressing these issues have been funded. A new look at research needs is in order. The urgent need for information about the health status of this population was one of the main findings of a report prepared by a task force led by one of this project's CoPrincipal Investigators (Janicki) issued by the World Health Organization (WHO, 2000), funded in part by an R-13 grant from NIA. That report focused on identifying health issues in the target population that would be of concern to policy makers. This proposal is to extend the WHO report process but narrow its focus to developing a research agenda. Our intent is to include biomedical, translational, clinical, and policy research issues. There are three Specific Aims: Specific Aim 1. Using a scientific meeting format, summarize the scientific data on health status of and medical care applications for older adults with I/DD. The meeting will bring together some 40 scientists and related workers from the world community who will present summaries of basic and translational research in several key areas pertaining to health and aging in persons with I/DD. The result will be a report leading to either an edited book or a special issue of a scientific journal. Specific Aim 2. Develop recommendations for future research. As a follow-up to the scientific meeting, the project steering committee will prepare a summary of research issues and recommendations for future research. Specific Aim 3. In collaboration with the American Association on Mental Retardation and the American

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Medical Association, define salient areas for medical concern and surveillance based on the extant literature and practice findings of physical conditions and diseases that appear to have particular incidence in middle age and aging adults with I/DD. This area will lead to a report of recommendations for the establishment of practice guidelines for medical care jointly issued by the AAMR and the AMA. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: /OPPORTUNITY

CONFERENCE--GENETIC

DISEASE

RESEARCH

NEED

Principal Investigator & Institution: Watson, Michael; American College of Medical Genetics 9650 Rockville Pike Bethesda, Md 20814 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2008 Summary: (provided by applicant): The annual meeting of the American College of Medical Genetics (ACMG) brings together a large proportion of basic and clinical investigators of rare genetic diseases. The organizers propose a series of workshops to be held in conjunction with ACMG meetings to consider issues related to identifying needs and opportunities for collaborative research involving rare genetic diseases (RGDs) associated with birth defects, mental retardation and developmental disabilities, and would set the stage for clinical and translational research. RGDs present many challenges to the development of effective research plans. These include infrastructure to identify and register patients with RGDs, their associated databases, identification and/or collection of biological specimens, coordination of rapidly evolving therapeutic interventions, and the coordination of studies of a usually limited number of experts involved with any individual rare condition with expertise from correlative sciences and other specialties. A coordinated and collaborative group would facilitate research into the epidemiology, intervention, and prevention of these rare conditions. It would allow for the integration of the many complementary programs overseen by non-NIH governmental organizations and agencies and, in association with currently funded NIH initiatives such as the Mental Retardation Research Centers and the National Children's Study, be highly synergistic with regard to RGD research. The annual workshops over the five years of this application would evaluate the existing models for facilitating collaborative research such as the consortium and cooperative group models. Specific diseases or classes of diseases would be used to identify the research gaps in the current system, the types of research that would be enabled, and the issues that rare genetic diseases raise that drive the need for collaborative research. The workshops would bring together representatives of government agencies, basic and clinical researchers, consumers and families, women, minorities, and underserved populations and geneticists. The first workshop in March 2004 would focus on the current models for collaborative research, the needs and potential benefits of collaborative research for these conditions, and assess collaborative research systems for a specific disease such as the lysosomal storage diseases or cystic fibrosis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CONSENT CAPACITY OF ADULTS WITH MENTAL RETARDATION Principal Investigator & Institution: Fisher, Celia B.; Director; Psychology; Fordham University Bldg. 540 Bronx, Ny 10458 Timing: Fiscal Year 2001; Project Start 01-SEP-2000; Project End 31-AUG-2003 Summary: The stated objectives of this research are to assess and develop procedures to enhance the capacity of adults with mild and moderate mental retardation (MR) to

28

Mental Retardation

provide informed consent for therapeutic research, and to exert their rights to assent or dissent when surrogate consent is obtained. The project will have two phases, with the first designed to examine the understanding of basic elements of psychotropic treatment including the purpose and nature of the research, risks and benefits, voluntary nature of the participation, and right to withdraw without penalty. The project will also examine comprehension of the consent elements (e.g., goals, role of a scientist, nature of placebo conditions, etc). Consent capacity will also be evaluated within the four psycho-legal standards outlined by Appelbaum and Grisso (1988). In phase 1, persons with mild, moderate and "typical;" intelligence will be respond to a hypothetical vignette describing research utilizing a randomized control trial of a new medication to reduce aggressive behavior. In phase 2, the authors will use data generated from phase 1 to develop and evaluate three formats designed to improve the consent capacity of individuals with mild and moderate MR: 1) a picture book format, 2) a video format, and 3) a supported decision-making format. A significant other is also chosen by the subjects to aid in them during the three format presentations. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: CORE--TRANSGENIC MICE Principal Investigator & Institution: Overbeek, Paul; Professor; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2002 Summary: The primary function of the Transgenic Mouse Core is to generate transgenic mice by microinjection of recombinant DNA into mouse embryo. Transgenic mice are increasingly being used for studies of neuronal development and neuron-specific gene expression. Experiments using transgenic mice include: a) studies of cis-acting regulatory sequences that direct gene expression to specific neuronal cell types; b) studies of inappropriate synthesis of cell surface markers that are essential for normal neuronal morphogenesis; c) studies of neuronal maturation, cell cycle control and apoptosis; and d) studies of axonal path-finding. Such studies contribute to disorders that can lead to mental retardation. The recombinant DNA constructs to be used for making transgenic mice will be generated by the user laboratories, but the core until will purify the DNA fragments for microinjection. After injection, the embryos will be reimplanted into pseudopregnant foster mothers for development to term. After the mice are born and of weaning age, the Core will ear tagged the mice for identification, after which the animals will be transferred to individual investigators for further characterization. Another function to be served by the core is the cryopreservation of transgenic mouse lines. For these experiments, the individual investigators will provide superovulated pregnant females to the Core, and mouse embryos at the one cell stage will be harvested and cryopreserved in artificial insemination straws using standard methods. The success of cryopreservation will be monitored by recovering embryos from one straw and examining the efficiency with which the embryos develop to the blastocyst stage in vitro. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: DIETARY CHOLESTEROL AND DEFECTS IN CHOLESTEROL SYNTHESIS Principal Investigator & Institution: Steiner, Robert; Pediatrics; Oregon Health & Science University Portland, or 972393098 Timing: Fiscal Year 2003; Project Start 20-AUG-2003; Project End 31-JUL-2008

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Summary: (provided by applicant): We wish to study the effects of altering dietary cholesterol (CH) on 2 disorders of CH synthesis: mevalonate kinase deficiency (MKD), and Smith-Lemli-Opitz syndrome (SLOS). SLOS is a multiple congenital anomalies/mental retardation syndrome. MKD causes 2 distinct syndromes, mevalonic aciduria and hyperimmunoglobulinemia D with periodic fever syndrome. Impaired mental and physical development are hallmarks of SLOS and MKD. We hypothesize that long-term supplementation of SLOS subjects with dietary CH with and without statins will raise CH levels, decrease CH precursor synthesis and permit adequate bile acid synthesis. In MKD, the effects of altering dietary CH are unclear. We hypothesize that a low CH diet will be beneficial in MKD, allowing maximal upregulation of HMG CoAR since we postulate that MKD is a disorder of isoprenoid and fatty acid rather than CH synthesis. Mevalonate from HMG CoAR is essential for CH synthesis but also provides isoprenoids essential for cellular function. Furthermore, a normally minor catabolic pathway, the mevalonate shunt, diverts mevalonate from isoprenoid and CH synthesis to the leucine oxidation pathway. Shunting may be protective in SLOS and harmful in MKD. Isoprenoid synthesis and mevalonate shunting may be increased in SLOS due to the enzymatic block distal to the takeoff of these 2 pathways, but decreased in MKD due to the proximal block. In vitro studies are planned to evaluate the effects of perturbations in CH exposure and statins on CH synthesis, mevalonate, isoprenoids, and mevalonate shunt products in SLOS, MKD, and control cells. Isoprenoids and shunt products will also be analyzed in a new SLOS mouse model. Parallel in vivo human studies will look at synthesis of sterols and bile acids, CH absorption, mevalonate excretion as an indicator of HMG CoAR activity, essential fatty acids and leukotrienes, isoprenoids, and mevalonate shunt products in SLOS and MKD, altering dietary CH and statins (SLOS only). The effects of altering dietary CH (and statins in SLOS) on plasma 24-S OH-CH, a measure of brain CH turnover, will be evaluated. We will determine whether mutations in SLOS and MKD genes determine biochemical and clinical phenotype and whether certain genotypes respond differently to altering dietary CH. Studies of metabolism/growth, development, behavior, sleep, feeding, hearing, and vision are performed over the long term to determine if the interventions might be helpful. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DYRK TRANSGENIC MICE AS A MODEL FOR DOWN SYNDROME Principal Investigator & Institution: D'arcangelo, Gabriella; Pediatrics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-APR-2000; Project End 31-MAR-2003 Summary: (adapted from applicant's abstract) Down Syndrome is the most common genetic disease associated with mental retardation, affecting about 1 in every 800 live births. The syndrome results from triplication of chromosome 21 or a segment of it, and it includes facial abnormalities, heart disease, increased frequency of leukemia, earlyonset Alzheimer's disease and mental retardation. The latter is associated with decreased neuronal number and reduced complexity of neuronal processes in many regions of the brain. The entire spectrum of Down Syndrome abnormalities likely results from overexpression of several genes located in a critical region on chromosome 21q22.2, and it is thus a complex trait. However, individual aspects of the syndrome may be attributable to one or few genes. Recently, the human homologue of Drosophila minibrain, also known as Dyrk, has been mapped to the Down Syndrome critical region. Mutant minibrain flies have a reduced number of brain cells, suggesting that the mutated gene encodes a protein involved in neurogenesis. Mammalian Dyrks are dual

30

Mental Retardation

specificity protein kinases with extensinve similarities to Drosophila Minibrain and to the yeast Yak1 protein kinase involved in cell division. Injection of a YAC containing genomic sequences including the Dyrk gene in transgenic mice causes altered neurogenesis and cognitive impairment, suggesting that Dyrk is involved in the brain abnormalities associated with Down Syndrome. To understand whether Dyrk plays a role in the developmental neuropathology underlying the mental retardation aspect of Down Syndrome, the investigators plan to first analyze in detail the normal pattern of expressionand activity of Dyrk in the developing mouse brain. Second, they will create transgenic mice that transiently and specifically express elevated levels of Dyrk in the proliferative zone of the developing brain. Finally, they will analyze in detail the brains of these mice to ascertain whether reduction in the number of neuronal cells, or other defects reminiscent of Down Syndrome, have occurred as a result of Dyrk overexpression in the brain during the period of neurogenesis. Their transgenic mice may serve as a novel animal model to investigate the molecular basis of mental retardation in Down Syndrome. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: EDUCATING PHYSICIANS ABOUT GENETICS AND BRAIN DISORDERS Principal Investigator & Institution: Tanner, T Bradley.; President; Clinical Tools, Inc. 431 W Franklin St, #30 Chapel Hill, Nc 27516 Timing: Fiscal Year 2001; Project Start 20-SEP-2001; Project End 31-MAR-2002 Summary: The expanding field of molecular genetics has quickly outpaced the knowledge of medical providers, due in part to the Human Genome Project. The genetics of brain illnesses are only beginning to be understood, nevertheless, providers treating patients with mental health, substance abuse and neurological disorders need to understand clearly what is and is not known about genetics and brain illnesses. It is likely that genetic aspects of schizophrenia, autism, mental retardation, bipolar disorder, among others, will be further identified in the near future, creating confusion and difficult choices for patients and families. Current and future providers must be prepared to explain and understand genetic susceptibility as it applies to a full range of illnesses. In Phase I, we will develop and evaluate an Internet-based CE program focusing on genetic issues related to alcoholism. We will also use the course the discuss upcoming technologies as well as issues related to discussing genetic risk with a patient. Phase II will produce 7 additional course on a variety of topic related to illnesses of the brain including Mixed Substance Abuse, Alzheimer's Disease, Schizophrenia, Affective Disorders, Attention Deficit, Huntington's Disease, and Mental Retardation. For each course we will highlight: 1) how genetic knowledge may affect patients and be used in clinical practice; 2) communication of genetic knowledge with patients and families: preparing for patients' questions; 3) where relevant, ethical, legal and psychosocial issues related to genetic testing, and 4) understanding emerging technologies and findings in genetics. A multidisciplinary team including representatives from psychiatry, genetics, genetic counseling, and primary care will create the content. We will evaluate the courses' effect on knowledge, clinical skills and self-efficacy using simple randomized studies comparing subjects using the genetics course to subjects using an online CE course on another topic. If successful, the training will expand the capabilities of the existing pool of providers and improve the training of future providers regarding the role of genetics in illnesses of the brain. PROPOSED COMMERCIAL APPLICATIONS: Physicians are required to obtain CME credit to maintain licensure and privileges. Managed care organizations (MCOs), such as large

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HMOs, may be interested in purchasing access to CE that can be offered to participant professionals. Pharmaceutical companies may also want to support development of courses based on the model described in this application. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: EPHB SIGNALING IN DENDRITIC SPINE DEVELOPMENT Principal Investigator & Institution: Ethell, Iryna M.; None; University of California Riverside 900 University Ave Riverside, Ca 92521 Timing: Fiscal Year 2003; Project Start 01-JAN-2003; Project End 31-DEC-2007 Summary: (provided by applicant): This research project will investigate the role of EphB receptor signaling in dendritic spine development. Understanding the molecular basis of dendritic spine morphogenesis is fundamentally important to a variety of inherited developmental disorders associated with mental retardation and autism, including Rett Syndrome and FragileX Syndrome. Patients with these disorders exhibit malformation of dendritic spines. These abnormalities result in synaptic dysfunctions, mental retardation and autism. The molecular mechanisms of dendritic spine abnormalities are not well described and require further investigation. Recently I made an important discovery that spine morphogenesis is controlled by the EphB-type receptor tyrosine kinases (Ethell et al., Neuron, 2001). I showed that expression of kinase-inactive EphB2, which prevents activation of EphB-type receptors in a dominantnegative fashion, blocked spine formation in cultured hippocampal neurons, the dendritic protrusions remained long, thin filopodia, as seen in patients with mental retardation and autism. I hypothesize that EphrinB (ligand)-induced activation of EphB receptors control dendritic spine formation. Preliminary results support this hypothesis and shows that clustered EphrinB2-Fc promotes dendritic spine morphogenesis. In Specific Aim 1, I will conduct experiments with knock out mice in which expression of one or multiple EphB receptors is disrupted to find which of the EphB receptors is responsible for the EphrinB2-induced spine formation. I propose to investigate two possible mechanisms through which EphB receptors may trigger dendritic spine mophogenesis: 1) recruitment of signaling molecules to synaptic membranes; 2) tyrosine phosphorylation of key molecules at postsynaptic sites. In Specific Aim 2, I will identify signaling molecules that may link the signaling of the EphB receptors to its effect on spine formation, by conducting mass-spectrometry analysis of the proteins recruited by EphB2 to dendritic spines upon its activation with EphrinB2-Fc. Preliminary results suggest that RhoGTPases may be responsible for the EphrinB/EphB receptor-mediated dendritic spine formation. In Specific Aim 3, I propose to investigate the molecular mechanism of EphB-mediated regulation of RhoGTPases in dendritic spines and its correlation with EphrinB-induced spine formation. In Specific Aim 4, I will also investigate role of cell adhesion molecules in EphrinB-mediated formation and stabilization of dendritic spines. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: EXPRESSION OF THE FRAGILE X GENE Principal Investigator & Institution: Hagerman, Paul J.; Professor; Biological Chemistry; University of California Davis Sponsored Programs, 118 Everson Hall Davis, Ca 95616 Timing: Fiscal Year 2002; Project Start 01-APR-2002; Project End 31-MAR-2007 Summary: (provided by applicant): Fragile X syndrome (FXS), a trinucleotide (CGG) repeat expansion disorder, is the leading heritable form of mental retardation, and is associated with a variety of learning disorders and behavioral problems in childhood.

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Mental Retardation

FXS is generally believed to arise when the CGG element of the fragile X mental retardation 1 (FMR1) gene expands beyond -200 repeats (full mutation), and the upstream promoter region and CGG repeat become methylated, the latter event leading to transcriptional silencing and the consequent failure to produce FMRI protein (FMRP). However, for males with premutation alleles (55-200 repeats) FMRI mRNA levels in peripheral blood leucocytes are significantly elevated, by as much as ten-fold for males with premutation alleles that exceed 100 repeats, and for males with full mutation alleles that remain unmethylated. These observations suggest that the FMR1 gene may be upregulated. Identification of the reasons for the elevated mRNA levels is the first major objective of the proposed research. It has also been demonstrated that leucocytes from the majority of males with FMRI alleles that are fully expanded and (apparently) fully methylated continue to produce significant levels of FMR1 mRNA. This surprising result, which is not due to transcription from premutation alleles or a small fraction of fullyunmethylated alleles, suggests that the interplay between methylation and silencing is less direct than had been supposed. Identification of the means by which hypermethylated FMR1 genes sometimes escape silencing is the second major objective of the proposed research. These two basic observations have important ramifications for understanding and eventually treating FXS. Elucidation of the signals that regulate transcriptional activity (both for up-regulation and for silencing) will hopefully lead to molecular therapies that can modulate the expression of the endogenous FMR1 gene. Methods of approach will continue to be quantitative (fluorescence) RT-PCR, and will incorporate chromatin immunoprecipitation as an assay for acetylation status of FMR1associated histones. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FETAL ALCOHOL EFFECTS IN MONKEYS: DOPAMINE AND BEHAVIOR Principal Investigator & Institution: Schneider, Mary L.; Professor; Kinesiology; University of Wisconsin Madison 750 University Ave Madison, Wi 53706 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-MAY-2006 Summary: Fetal alcohol syndrome, (FAS) is the leading known cause of mental retardation today and currently represents an enormous problem for our society. The central question addressed by this proposal is whether moderate alcohol exposure constitutes a danger to the developing offspring. To address this issue, we propose to assess the behavior and physiology in 50 monkeys from four conditions: 1) mothers consumed moderate level alcohol daily throughout pregnancy 2) mothers experienced psychological stress; 3) mothers consumed moderate level alcohol and experienced psychological stress; and 4) mothers consumed sucrose (controls) (Schneider et al., 1997). The specific aims are as follows: 1) to characterize dopamine D2 receptor densities in striata of offspring using in vivo PET imaging techniques 2) to characterize dopamine synthesis in these same cohorts, also using PET imaging, and to uncouple presynaptic synthesis of dopamine from postsynaptic receptor binding availability; 3) to evaluate these monkeys with a standard battery of widely accepted tests and measurements, which index cognitive functioning and behavior; and 4) to determine the effects of a dopamine agonist, methylphenidate, on behavior and cognitive performance in this cohort of monkeys. Our primate model has allowed control of the exact timing and level of alcohol exposure to the fetus and the separation of the effects of alcohol from other life-style factors, such as psychological stress The proposed studies provide a unique and unprecedented opportunity not only to better understand the underlying neurobiology of fetal alcohol effects, but also to discover potential in vivo diagnostic

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markers for detecting fetal alcohol- induced brain damage. Increasing our understanding of the association between behavior, cognition, and molecular mechanisms of neuronal function in fetal alcohol-exposed primates could aid in early identification and appropriate treatment of children with prenatal alcohol exposure. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FRAGILE X WORKING MEMORY: A NEURAL NETWORK MODEL Principal Investigator & Institution: Johnson-Glenberg, Mina C.; Waisman Ctr/Mr & Human Devlmt; University of Wisconsin Madison 750 University Ave Madison, Wi 53706 Timing: Fiscal Year 2001; Project Start 01-JUN-2001; Project End 31-MAY-2003 Summary: Fragile X syndrome (fra(X)) is the most common inherited form of mental retardation. It is characterized by constellation of impairments in linguistic, cognitive, social, and sensory/motor skills. Theories differ regarding the underlying psychological cause of the cognitive phenotype; however, many researchers agree that those with Fra(X) experience problems with working memory. A working memory deficient would express itself most profoundly in sequencing tasks. Those with fra(X) perform poor than those behavioral model and simulates task performance with an with an artificial neural network. The network model is based on me urological substrates discovered to be significantly different in those with fra(X); in particular, the hippocampus (HC) appears to be larger. The model will use data from a modified "n-back" task, the sequential card playing tasks. Simultaneous processing matched typically developing participants and those with fra(X) will play this experimenter-designed card game. In this task they will be required to recall either one, two, three, or four cards in a row. The neural network will simulate how these two different populations perform. Those with fra(X) may have an abundance of neurons in the HD; paradoxically, having too many neurons leads to poor memory and slower learning. These neurons are represented by hidden units in the network model, and an overabundance of hidden units means that the model does not abstract important elements from the environment. The network too much idiosyncratic, moist, and extraneous information and this is infelicitous for sequencing. Proper sequencing relies on the activation and storage of meaningful, well-abstracted neuronal patterns. The specific aims of this grant are 1) to complete the development of a game-like, sequential card playing task that can be used to assess visual working memory in a motivating manner, 2) to compare fra(X) and typical populations' performance on the card task, and 3) to implement a neural network that will provide a neurologically-motivated, more process- oriented account of the working memory differences between the two populations. In addition, by adjusting the parameters of the neural network model, individual differences within the fra(X) population can be stimulated. This project represents a step towards research in which network models can help to make recommendations for most optimal remediation strategy based on an individual's performance profile. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: FUNCTION OF FMRP IN THE MOUSE OLFACTORY SYSTEM Principal Investigator & Institution: Larson, John R.; Psychiatry; University of Illinois at Chicago 1737 West Polk Street Chicago, Il 60612 Timing: Fiscal Year 2003; Project Start 01-JUL-2003; Project End 30-JUN-2008 Summary: (provided by applicant): Fragile X syndrome is the most common inherited cause of mental retardation. The disorder is caused by mutation in a gene, FMR1, that

34

Mental Retardation

encodes the fragile X mental retardation protein (FMRP). How loss of FMRP produces mental retardation is not known. FMR1 knockout mice have been produced, providing a mouse model for fragile X syndrome. The experiments proposed in this application will use the mouse olfactory system to investigate the normal function of FMRP and the consequences of the protein's absence in knockout mice. FMRP is normally expressed in olfactory brain structures. The project has three specific aims. First, behavioral analyses will be conducted to determine how lack of FMRP results in impairment of memory formation for olfactory information. Second, electrophysiological methods will be used to determine how absence of FMRP alters synaptic function and synaptic plasticity in the primary olfactory cortex. Third, olfactory stimulation and learning paradigms will be used to determine how expression of FMRP is regulated by neuronal activity in the olfactory system. The results of these studies should provide new information regarding the function of FMRP in the normal brain and may identify behavioral or physiological functions that are reliably disrupted in mice lacking this important protein. Such new information would be vital for evaluating novel treatment strategies for a class of developmental disabilities. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: FUNCTION OF NEUROLIGIN IN SYNAPSE FORMATION IN THE CNS Principal Investigator & Institution: Scheiffele, Peter; Physiology/Cellualr Biophysics; Columbia University Health Sciences New York, Ny 10032 Timing: Fiscal Year 2003; Project Start 01-JAN-2003; Project End 31-DEC-2007 Summary: (provided by applicant): The long-term goal of these studies is to understand the molecular mechanism of synapse formation and target recognition in the central nervous system. The aims of the research proposed in this application are: (1) to dissect the structural basis for the synaptogenic activity of neuroligin, a post-synaptic adhesion molecule that can trigger the assembly of pre-synaptic elements in contacting axons, (2) to identify receptors for neuroligin in the pre-synaptic cell, and (3) to analyze the role of neuroligin in vivo. Aim 1 and Aim 2 will be studied with functional cell-based assays in vitro that permit direct manipulation of the pre- and post-synaptic machinery. The neurexin family of proteins will be studied as candidate neuroligin receptors, but at the same time we will perform an unbiased biochemical purification of all molecules that might associate with neuroligin during synapse formation. To analyze neuroligin function in vivo, hammer-head ribozymes will be generated that specifically degrade neuroligin mRNAs. These ribozymes will be expressed in the cerebellum of mice, either by gene transfer into selected cells with a viral vector or in a transgenic animal. Synapse formation is a crucial process in the generation of neuronal circuits in the brain. Aberrant synapse formation and synaptic dysfunction lead to severe nervous system disorders such as epilepsy, schizophrenia, and mental retardation. Neuroligins themselves have been implicated in mental retardation in humans. Besides nervous system disorders, understanding the mechanism of synapse formation will also be most important for controlling or stimulating the regeneration of synaptic circuits after injury, as for example the connectivity in the spinal cord after injuries that lead to paralysis. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: GENE THERAPY FOR METABOLIC DISORDERS Principal Investigator & Institution: Whitley, Chester B.; Professor; Pediatrics; University of Minnesota Twin Cities 200 Oak Street Se Minneapolis, Mn 554552070

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Timing: Fiscal Year 2001; Project Start 10-JAN-1995; Project End 31-DEC-2002 Summary: The central theme of this program is to exploit recent advances in basic science for t he development of innovative gene therapy strategies, especially for metabolic disorders causing mental retardation. The program is highly focused on identifying and resolving the barriers to clinical gene therapy. In this period, five projects aim to exploit recent innovations t hat would enhance gene delivery and expression or actually correct genomic mutations in vivo. These projects are: Therapy for Hyperammonemia with Genetically-Engineered Bacteria (Tuchman). AdenoAssociated Virus Vector Treatment of Spinocerebellar Ataxia (McIvor) Chimeraplasty for Mutations Associated with Mental Retardation (Kren) Sleeping Beauty Transposon for Gene Therapy (Hackett) Lentiviral Ex Vivo Hematopoietic Stem Cell Gene Therapy for Mucopolysaccharidosis Type 1 (Whitley) The projects utilize a number of models of human metabolic disorders causing brain disease, notably, murine models of mucopolysaccharidosis, hyperammonemia, phenylketonuria and spinocerebellar ataxia. The program share core facilities for administration, microchemicals, quantitative PCR, hematopoietic cell processing, animal resources and viral vector production. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GENETIC CAUSES OF MENTAL RETARDATION Principal Investigator & Institution: Moser, Hugo W.; Director; Kennedy Krieger Research Institute, Inc. Baltimore, Md 21205 Timing: Fiscal Year 2001; Project Start 01-JAN-1978; Project End 31-DEC-2001 Summary: This abstract is not available. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: GENETIC CAUSES OF MENTAL RETARDATION Principal Investigator & Institution: Smith, Kirby D.; Associate Professor; Kennedy Krieger Research Institute, Inc. Baltimore, Md 21205 Timing: Fiscal Year 2002; Project Start 01-JAN-1978; Project End 31-DEC-2006 Summary: For the past 15 years this Program has focused has focused on genetic disorders of peroxisome biogenesis and function. In recent years understanding of the biogenesis and metabolic functions of peroxisomes has expanded significantly. Thirteen of the known 16 peroxisomal genetic disorders manifest mental retardation but the connection between peroxisomal abnormality and mental retardation is still unknown. The peroxisome has been shown to have a much wider range of functions than has been recognized in the past. This Program Project has been characterized by a strong synergism between clinical and basic science and this interaction will continue. The projects and investigators of the proposed Program Project will interface with related basic research projects and with ongoing clinical trials (Moser). Our clinics have identified more than 5000 patients with peroxisomal disorders. While they continue to provide a framework for this proposal, new information will be derived from several mouse and yeast models of peroxisomal biogenesis, function and disease. The present proposal includes 4 projects in addition to administrative and clinical, biochemical, cell, and molecular cores. The first Project (Smith) will determine the function of the X-linked of the X-linked adrenoleukodystrophy (XALD) protein, explore causes of the marked clinical heterogeneity that typifies this disease and test therapeutic modalities in an ALD mouse model. The second Project (Watkins) will investigate the role of fatty acid activating enzymes in XALD and their role in the regulation of very long chain fatty acid homeostasis, particularly in the brain. The third Project (Gould) will resolve outstanding

36

Mental Retardation

questions related to peroxisome biogenesis genetics and disorders and elucidate the biochemistry of a-oxidation and its disorders. The fourth Project (Valle) will use yeast and mouse models to investigate the role of peroxisomal membrane proteins in peroxisome biogenesis, function, and disease. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GENETIC EPIDEMIOLOGY OF THE FMR1 GENE Principal Investigator & Institution: Sherman, Stephanie L.; Professor of Human Genetics; Genetics; Emory University 1784 North Decatur Road Atlanta, Ga 30322 Timing: Fiscal Year 2001; Project Start 05-MAY-1994; Project End 31-MAR-2004 Summary: (Adapted from the Investigator's Abstract): The fragile X syndrome (FXS), a type of inherited mental retardation (MR), is due to the silencing of the FMR1 X-linked gene. In over 95 percent of the cases, the mutation is due to an expansion of an unstable CGG repeat sequence located in the 5' untranslated region of the gene. Once expanded to over 200 repeats, the FMR1 gene is hypermethylated and consequently no message is transcribed. In the previous grant, the investigators suggested that, in addition to this specific mutation and resulting MR, there may also be a phenotype associated with an increased number of CGG repeats, i.e., alleles with 41 to 100 repeats. Although not methylated, the mRNA of such FMR1 alleles may be altered or alter the binding properties of other proteins. Preliminary results suggest that an increased number of CGG repeats may influence an individual's cognitive and behavioral performance and, for females, affect the age at menopause. If confirmed, this gene may be one of the first clearly identified genes to affect cognitive and behavioral skills and will become one of the possible candidate genes that play a role in psychiatric and behavioral disorders. As the frequency of such FMR1 alleles is high in the population, about 3 percent, the impact on the population may be greater than that related to the mutation leading to the FXS. Moreover, a proportion of these high repeat alleles may be unstably inherited, although that proportion is unknown and factors that influence that instability are not fully understood. In this revised application, the researchers propose to survey a cross-section of the general population and FXS families to obtain a large sample of probands with high repeat alleles. First they will assess all probands with neuropsychological tests to confirm or refute the phenotype consequence of these alleles. Second, they will ascertain parents of probands and conduct a case control study to further examine the relationship between repeat number and the age at menopause in carriers with the high repeat alleles. Lastly, they will ascertain first degree relatives of probands to obtain an estimate of the proportion of unstable alleles in the general population. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: GENETICS, MORTALITY AND DEMENTIA IN DOWN SYNDROME Principal Investigator & Institution: Zigman, Warren B.; Reasearch Scientist; Institute for Basic Res in Dev Disabil Developmental Disabilities New York, Ny 10314 Timing: Fiscal Year 2001; Project Start 16-FEB-1999; Project End 31-JAN-2004 Summary: Down syndrome, one of the most common genetic causes of mental retardation associated with genetic factors, occurs in approximately 1.2 per 1000 live births, is typically caused by a nondisjunction of the 21st chromosome during meiosis resulting in a complete trisomy genotype, although atypical forms occur occasionally. Adults with Down syndrome have benefited from the advances in public health practices that have resulted in a dramatic extension in life expectancy. However, Down syndrome is still characterized by increased mortality rates during later stages of life.

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Causes of higher mortality rates later in life may be due to a number of factors, two of which are an increased risk for Alzheimer~s disease and an apparent tendency toward premature aging. Aging processes among adults with Down syndrome have been of interest for over 100 years because of the occurrence of the signs and symptoms of Alzheimer~s disease in the population. Indeed, brain tissue of virtually all adults with Down syndrome over 35 to 40 years of age displays significant accumulations of amyloid plaques, historically considered to be a hallmark of Alzheimer's disease neuropathology presumably due to the triplication and over expression of the gene for beta-amyloid precursor protein located on chromosome 21. The genotypic and phenotypic characteristics of the "oldest old" (i.e., 65 and older) adult population with mental retardation due to Down syndrome will be compared to those of their younger peers (also with Down syndrome) in order to identify genetic risk factors associated with survival and the cognitive declines associated with dementia of the Alzheimer~stype. We have been able to identify a group of 100 people with Down syndrome 65 years of age or older. Investigation of this unique group of individualist will allow us to characterize the phenotype of the ~oldest old~ with Down syndrome and identify genetic and health status factors that are associated with extended survival and successful aging on one hand and the development of DAT on the other. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SYNDROME

GENOTYPE/PHENOTYPE

CORRELATIONS

IN

WILLIAMS

Principal Investigator & Institution: Mervis, Carolyn B.; Distinguished University Scholar and Pro; Psychological and Brain Sciences; University of Louisville University of Louisville Louisville, Ky 40292 Timing: Fiscal Year 2001; Project Start 17-MAY-1996; Project End 31-MAR-2005 Summary: Williams Syndrome (WS) is a complex neurodevelopmental disorder involving mild to moderate mental retardation, an unusual personality profile, infantile hypercalcemia, dysmorphic facial features, and supravalvar aortic stenosis (SVAS). WS is a contiguous gene disorder resulting from submicroscopic deletions of chromosome 7q11.23. The goal of the proposed study is to create a medical and behavioral profile of WS ("a quantifiable assay of the WS behavioral profile") and to use this profile to identify specific genes underlying behavioral features of WS and carryout genotypephenotype correlation studies. The specific aims include ascertainment and characterization of individuals who have features that overlap with WS; 2) identification and characterization of the cardinal features of the phenotype of WS and phenotypes of individuals with smaller WS deletions; 3) identification of genes responsible for specific phenotypic features of WS through deliniation of a refined physical map of the WS region, cloning and characterization of genomic DNA within this region, and identification of new genes in the region. An initial specific goal is to identify genes responsible for the personality characteristics of WS as well as other specific phenotype features. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: GLYCOSPHINGOLIPID RETARDATION

METABOLISM

AND

MENTAL

Principal Investigator & Institution: Dawson, Glyn; Professor; Pediatrics; University of Chicago 5801 S Ellis Ave Chicago, Il 60637 Timing: Fiscal Year 2002; Project Start 01-JUN-1978; Project End 30-NOV-2005

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Mental Retardation

Summary: (provided by applicant): We propose to continue studies on the role of the sphingolipid ceramide, as an inducer of apoptosis in neonatal rat oligodendrocytes: a model for neurodegenerative disease involving mental retardation. We will initially test a hypothesis that cytokine-mediated activation of caspase-8-driven apoptosis involves increased ceramide either from increased de novo synthesis or increased formation from sphingomyelin following acid/neutral sphingomyelinase activation. We will then determine if the cellular site of this ceramide increase during apoptosis is the detergentresistant surface membrane fraction (caveolae) also known as Rafts and verify that these sphingolipid/cholesterol-rich domains are scaffolds for signaling complexes, involving tyrosine phospho-kinases such as Fyn and PP2A phosphatases, which lead to apoptosis. We will then address the question of mechanisms by focusing on the ability of ceramide to inactivate the anti-apoptotic phospho-Akt through phosphatase degradation of Akt-P and BAD-P. Since ceramide does not directly dephosphorylate Phospho-Akt we will test the hypothesis that PTEN phosphoinositide phosphatase is involved, based on the activation of Akt by polyphosphoinositides, their loss of during apoptosis, and evidence for PTEN localization in Rafts. Finally we will attempt to link all these events together based on our recent novel observation that overexpression of Palmitoyl:protein thioesterase leads to protection against killing by ceramide by activating Akt. We will test the hypothesis that this is because decreased palmitoylation of caveolin and signaling molecules such as Ras and Fyn leads to disruption of Raft-based pro-apoptotic signaling. By precisely characterizing the role of ceramide we hope to identify new sphingolipid-based strategies for the therapy of a range of neurodegenerative diseases including mental retardation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: HUMAN ARGINASE AND ARGINASE DEFICIENCY Principal Investigator & Institution: Cederbaum, Stephen D.; Professor; University of California Los Angeles 10920 Wilshire Blvd., Suite 1200 Los Angeles, Ca 90024 Timing: Fiscal Year 2002; Project Start 15-MAR-2002; Project End 30-NOV-2006 Summary: This proposal focuses on the study and exploitation of the mouse knockout model for human arginase I (AI) deficiency and hyperargininemia, a condition which causes neurological and intellectual deterioration and which results in mental retardation. The model will be used to elucidate the function, regulation and evolution of the two arginase genes in man (AI and AII) and to elucidate the pathology and pathophysiology of AI deficiency. The long term goal is to devise strategies to replace arginase in these patients or otherwise mitigate the impact of the hyperargininemia. These will include gene therapy and induction of AII or "autoenzyme replacement therapy". The biochemistry, molecular biology, pathology of the AI-deficient hyperarginemic mouse after protecting it from hyperammonemia will be studied. Approaches used include: plasma and tissue amino acid measurement; enzyme assay; immunoprecipitation; immunohistochemistry; in situ hybridization; animal behavioral testing; and tissue culture. Studies will test two distinct hypotheses for the neurological damage in hyperargininemia, excess nitric oxide (NO) production and increased synthesis of the neurotransmitters, glutamate and/or gamma aminobutyric acid (GABA). The AI and AII knockout animals will e used to explore the role of the two arginases in inflammatory cells, prostate function and kidney biochemistry. Expression of one or the other arginase transgenes in different tissues will play a prominent role in these studies. This project exploits unique models, at a propitious time, in an ideal environment and with ideal collaborators, to study the effects of a metabolic disease that

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causes mental retardation, and its treatment by gene manipulation. Results from these studies should provide relevant to a larger number of disorders of this type. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: ID RETARDATION

OF

GENES

RESPONSIBLE

FOR

X-LINKED

MENTAL

Principal Investigator & Institution: Wang, Tao; Institute 0F Genetic Medicine; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2003; Project Start 01-SEP-2003; Project End 31-AUG-2008 Summary: (provided by applicant): Mental retardation is the most common cause of handicap in children and young adults and accounts for 2-3% in the general population. X-linked mental retardation (XLMR) occurs in 1 in 600 males and is genetically heterogeneous. Among the estimated more than 150-200 responsible loci on the X chromosome, less than 40 genes have been cloned. Delineation of the molecular basis of XLMR will contribute to our understanding of human cognitive development, and will lead to development of strategies for clinical management of XLMR patients. The candidate is interested in the study of the molecular mechanism of XLMR, with a longterm career goal to become a successful clinician scientist. To accomplish this goal, he developed a comprehensive career development plan to be carried at the Johns Hopkins University. There are three key components to the research plan: (1) training in bioinformatics and genomic research; (2) training in the clinical evaluation and care for patients with mental retardation; and (3) training in the patient-oriented clinical investigation. The candidate will attend graduate courses and seminars on genome research and on principles of clinical investigation. He will receive mentored training in clinical evaluation and care for patients with mental retardation. He will be responsible for the development of a clinical research protocol for this project. In addition, the candidate will participate in the weekly Genetic Clinic at the Johns Hopkins Hospital and the Mental Retardation Clinic at the Kennedy Krieger Institute. He has developed a strategy of using human X chromosome-specific cDNA microarray to identify responsible genes. This approach is designed to detect mutations that result in a change in the abundance of mRNA due to mechanism such as promoter mutations, gene deletions, and nonsense or frameshift mutations associated with nonsense-mediated mRNA decay. Once a candidate gene is identified, Northern blot and real-time PCR will be used to verify mRNA reduction followed by mutation analysis in the proband. Additional in vitro and in vivo studies will then be carried out to delineate the molecular mechanism of XLMR for each identified gene. Through the combined laboratory research and clinical training, the candidate wishes to develop a solid knowledge base and gain precious experiences in research and clinical care for patients with mental retardation. This will be invaluable to the advancement of the candidate's career to become an independent physician scientist. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: IDENTIFICATION OF FMRP TARGET RNAS Principal Investigator & Institution: Darnell, Jennifer C.; Lab/Molecular Neurooncology; Rockefeller University New York, Ny 100216399 Timing: Fiscal Year 2001; Project Start 15-APR-2001; Project End 31-MAR-2005 Summary: (Adapted from the applicant's description) This application proposes to test the hypothesis that fragile-X mental retardation results from a failure of FMRP to bind sequence-specific RNA targets, and to identify those target RNAs. Three aspects of this

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Mental Retardation

issue will be definitively addressed. First, FMRP sequence-specific RNA targets will be identified using in vitro RNA selection methods, specifically delineating RNAs that bind to full-length FMRP and to the FMRP KH2 RNA binding domain, in which an I to N mutation is implicated in fragile-X mental retardation. In addition, mutagenesis studies will yield insight into the nucleic acid and amino acid residues that are critical for RNAprotein recognition. Second, the investigators will utilize this information to identify candidate in vivo FMRP RNA targets, using several redundant strategies. Third, they will test the metabolism of these candidate RNAs in vivo in genetically defined FMR null mice. The investigators will utilize FMR null mice as well BAC transgenic technology to rescue the FMR-1 null mice with FMR-1 genes harboring specific mutations in KH2 necessary for sequence-specific RNA binding. Examination of phenotype of mice harboring KH2 point-mutants will allow the investigators to assign aspects of neuronal dysfunction that result from failures in sequence-specific RNA binding. Moreover, comparison of the metabolism of candidate RNAs in mutant with wild-type mice will allow the investigators to conclude that FMRP acts on specific RNA targets in neurons. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: INFRASTRUCTURE FOR RESEARCH ON MENTAL DISABILITIES Principal Investigator & Institution: Stevenson, Roger E.; Director; Greenwood Genetic Center 1 Gregor Mendel Circle Greenwood, Sc 29646 Timing: Fiscal Year 2001; Project Start 15-FEB-1999; Project End 31-JAN-2004 Summary: Autism and mental retardation are common and enigmatic disturbances of brain function which generally manifest in early childhood and persist throughout life. Systematic study of unselected series of patients identifies a specific cause in approximately 10% of cases of autism and 50% of cases with mental retardation. Recognized causes are heterogeneous, including genetic defects (e.g., chromosome aberrations, single gene mutations, uniparental disomy), environmental insults (e.g., infection, injury, chemical), or some combination of the two influences (multi-factorial causation). The lack of understanding of causation of many other aspects of autism and mental retardation, including identification of the many responsible genes, the pathogenetic mechanisms through which brain development is adversely affected, and the delineation of the clinical (including behavioral) phenotypes of autism and mental retardation syndromes, underscores the need for clinical research on these mental disabilities. The Greenwood Genetic Center operates a small but productive clinical research program related to autism and mental retardation. Strengths of the program include expertise in dysmorphology, clinical genetics, childhood development, pathology, cytogenetics, biochemical genetics, and molecular genetics. Gaps in expertise include neurology and neuropsychology, epidemiology and biostatistics. The overall goal of the proposed infrastructure support project is to enhance the capacity for the Greenwood Genetic Center, in collaboration with the SC Department of Disabilities and Special Needs, and to conduct clinical research on autism and mental retardation. This will be accomplished of a Section of Neurology/Behavioral Science, 2) developing the capacity to acquires and analyze data through establishment of a Section on Epidemiology and Biostatistic, 3) expansion of the range of molecular technologies and trained manpower available for research purposes, and 4) strengthening the administrative structure in support of the research program through development of an Office of Grants/Research Management and a Scientific Advisory Board. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: INTERACTION OF RSK2 WITH 5-HT2A RECEPTOR Principal Investigator & Institution: Sheffler, Douglas J.; Biochemistry; Case Western Reserve University 10900 Euclid Ave Cleveland, Oh 44106 Timing: Fiscal Year 2002; Project Start 01-DEC-2002; Project End 30-NOV-2004 Summary: (provided by applicant): The goal of the proposed research is to verify the interaction of RSK2 with the third intracellular loop of the 5-HT2A receptor that was identified in a yeast-2-hybrid screen. Once this interaction has been established, its functional significance will be determined. Our central hypothesis is that RSK2 interacts with the 5-HT2A receptor, indicating a role in signal transduction. It is likely that this interaction plays a role in the ligand-dependent activation of the MAP Kinase cascade by the 5-HT2A receptor. Inactivating mutations in the RSK2 gene are responsible for the human Coffin-Lowry syndrome. This syndrome is characterized by severe non-specific mental retardation, psychosis, and progressive skeletal deformations. The psychosis that is present in Coffin-Lowry syndrome may prove to be attributed to a disfunction of 5-HT2A signaling. To test this central idea we propose to verify the interaction of the 5HT2A receptor's third intracellular loop and RSK2 via coimmunoprecipitation studies and through confocal microscopy studies. The binding site of RSK2 to the 5-HT2A receptor will be defined via site-directed mutageneis studies in conjunction with 2hybrid analysis. The functional role of the interaction between RSK2 and the 5-HT2A receptor will be defined. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: KARYOTYPE RETARDATION

AND

GENETIC

ANALYSIS

OF

MENTAL

Principal Investigator & Institution: Ward, David C.; Professor; Genetics; Yale University 47 College Street, Suite 203 New Haven, Ct 065208047 Timing: Fiscal Year 2001; Project Start 01-AUG-1998; Project End 31-JUL-2003 Summary: (Adapted from investigator's abstract): The objective of this proposal is to assess the efficacy of new fluorescence in situ hybridization techniques as adjunct diagnostic tests for pervasive developmental delay (PDD) and mental retardation (MR). In addition it is proposed to identify chromosoma loci that may harbor genes important in neural development and cognitive function. The hypothesis is that the screening of patients with PDD and/or MR with chromosome-specific painting probes, telomerespecific probe sets, and microdeletion probe sets is an effective method to identify unknown causes of PDD and MR and to identify genes involved in neurodevelopment or cognitive attributes. The investigator's laboratory currently has the capacity to produc a complete karyotype, including banding, from a signal hybridization using multicolor fluorescence in situ hybridization (M-FISH) and to test for rearrangements of telomeric and subtelomeric regions using a set of telomere-specific probes for all chromosomes except the p arms of acrocentric chromosomes. It is proposed to develop robust screening methods for karyotypic analysis, for monitoring telomere integrity and for assessing microdeletion syndrome regions using multicolor combinatorial labeled probe sets. The development of such screening methods for us in clinical cytogenetic laboratories should result in better diagnosis and prognosis assessment, and, ultimately, in better therapies for these individuals. Specifically, it is proposed to screen 300 individuals with PDD, 300 with MR, and approximately 500 normal control subjects for chromosomal abnormalities using state-of-the-art multiplex hybridization and imaging technology. The relative rates of karyotypic aberrations in these populations and the background rate of karyotypic aberrations in normal individuals will be assessed. The

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relative sensitivity of the new screening methods will be compared with standard cytogenetic methods. It is anticipated that this proposal will permit the investigators to make an estimate of the efficacy of karyotypic screening of patients in these diagnostic groups. In addition, specific probes will be identified that will be made available to other researchers for testing specific translocation breakpoint sites and should permit the future cloning of cDNAs that are associated with pervasive developmental delay or mental retardation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: LABORATORY MODEL OF ESCAPE--MOTIVATED ABERRANT BEHAVIOR Principal Investigator & Institution: Perone, Michael; Psychology; West Virginia University P. O. Box 6845 Morgantown, Wv 265066845 Timing: Fiscal Year 2001; Project Start 01-SEP-2000; Project End 31-JUL-2003 Summary: The proposed research concerns environmental factors that contribute to chronic aberrant behavior in persons with mental retardation. Chronic aberrant behavior such as self- injury, aggression, and property destruction is a major barrier to habilitation and independent living for these person, and it represents a long-standing treatment challenge. Laboratory procedures developed to study basic behavioral processes in animals have shown that prolonged, counterproductive interruptions in behavior (pausing) are generated when relatively rich conditions of positive reinforcement are juxtaposed with lean conditions. Indeed, animals act to escape from these same conditions, even though this action delays or reduces the opportunity for reinforcement, suggesting a functional analogue to aberrant behavior in humans. The proposed research will adapt the procedures from the animal laboratory to study processes potentially operative in escape-maintained aberrant behavior in persons with mental retardation, because functional analysis has established that escape is a primary motive for naturally occurring aberrant behavior in this population. A series of intensive single-subject, steady-state experiments will identify boundary conditions for evoking extended pausing and escape in the performance of standard operant tasks by persons with retardation. The experiments will involve manipulation of reinforcer magnitude, behavioral effort, and discriminative stimuli signaling shifts in reinforcement conditions. The general goal is to develop a laboratory model that can be used for the identification and controlled study of variables that may operate in the natural environment to make otherwise neutral or positive situations aversive. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: LYMPHOCYTIC TARGETS AND BEHAVIOR IN FRAGILE X Principal Investigator & Institution: Kaufmann, Walter E.; Associate Professor; Kennedy Krieger Research Institute, Inc. Baltimore, Md 21205 Timing: Fiscal Year 2003; Project Start 20-FEB-2003; Project End 31-JAN-2005 Summary: (provided by applicant): Fragile X syndrome (FraX), the most prevalent form of inherited mental retardation, is associated with the absence of FMRP, a protein involved in regulation of protein synthesis. Recent microarray studies have identified transcripts that are abnormally regulated in both brains from FMRP knockout mice and lymphoblasts from males with FraX. These FMRP targets include structural and functional proteins that participate in synaptic development and plasticity. In addition, using proteomics techniques, we have demonstrated in FraX lymphocytes abnormal acetylation of several proteins that include the microglial regulatory protein annexin-1

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(Anx-1) and the neuronal cytoskeletal protein a-tubulin. Abnormal Anx-1 expression is also predictive of autistic features in FraX. We hypothesize that specific aspects (e.g, autism, megalencephaly), and variability, of the FraX neurobehavioral phenotype are the consequence of abnormal expression of one or more neural proteins, and that these contributions can be identified by characterizing patterns of abnormal neural protein expression in lymphocytes and their neurobehavioral correlates in males with FraX. We propose to study the patterns of expression of 12 selected FMRP targets in lymphocytes from a well-characterized sample of males with FraX, representing the spectrum of neurobehavioral phenotypic manifestations. In Aim l, we will use a combination of immunochemical techniques to characterize patterns and levels of lymphocytic protein expression in 100 males with FraX and 20 controls. In Aim 2, we will examine the relationships between these molecular variables and selective aspects of the neurobehavioral phenotype (e.g., a-tubulin and IQ) of the FraX subjects. Since blood samples and behavioral data are available on 50 FraX and 10 control subjects, only half of the sample will be recruited over two years. Preliminary data also show the feasibility of studying 4 of the proposed proteins. Because of their innovative approach to examining genotype-phenotype relationships, we believe that the proposed studies are in accordance with the purposes of the Exploratory/Developmental Grant (R21) Program. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MEMORY AND COGNITION IN AGING ADULTS WITH DOWN SYNDROME Principal Investigator & Institution: Devenny, Darlynne A.; Institute for Basic Res in Dev Disabil Developmental Disabilities New York, Ny 10314 Timing: Fiscal Year 2001; Project Start 15-JUN-1998; Project End 31-MAY-2003 Summary: Adults with Down syndrome (DS) experience changes in functioning associated with normal aging precociously, and they are at higher risk for developing Alzheimer's disease than their peers with other forms of mental retardation (MR). The first aim of the proposed project is to characterize specific changes in memory and cognitive processing that occur during normal aging and to identify individual differences in vulnerability to early onset of age-associated changes in adults with DS. This project will focus on the study of working and episodic memory, two systems known to be sensitive to aging processes. Each of the components of a model of working memory (central executive function, visuospatial sketchpad, phonlogical loop; Baddeley, 1986) will be tested with multiple converging measures. The second aim is to distinguish the patterns of change in performance on tests of memory and cognition that are associated with normal aging from changes associated with early dementia of the Alzheimer type. The third aim is to describe the natural history of dementia in adults with MR. Individual performance on tests of memory and cognition will be related to mental status and caregiver informant-based scales of adaptive behavior and dementia. Because the test battery spans a broad range of cognitive domains and difficulty, it will be possible to describe the course of decline from early onset through to advanced stages of dementia. By the end of the proposed study those tests that are most sensitive to early declines in functioning will have been identified and their utility as clinical instruments verified with longitudinal measures. By delineating the changes in specific memory and cognitive processes related to individual differences in normal and abnormal aging in adults with DS, this work will contribute to understanding vulnerability to age-related changes, in general. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MENTAL RETARDATION RESEARCH CENTER Principal Investigator & Institution: Denckla, Martha B.; Director; Kennedy Krieger Research Institute, Inc. Baltimore, Md 21205 Timing: Fiscal Year 2001; Project Start 01-AUG-1988; Project End 31-JUL-2003 Summary: This application for support of years 11 through 15 of the Mental Retardation Research Center (MRRC) at the Kennedy Krieger Institute and John Hopkins University is submitted in response to RFA HD-97-003. The MRRC consists of an administrative core (Core A) and five research cores (Cores B, C, D, E, and F). The Core A (Administrative), provides overall management and organizational support to the Center, operates overall management and organizational support to the Center, operates the educational component (lectures and seminars) and links users to biostatistical services. Core B (Genetics) provides centralized tissue culture, amino acid, organic acid analyses, standard and molecular cytogenetics, specialized molecular genetics (DNA and RNA analyses) and bioinformatics. Core C (Neuroscience) provides synaptic neurochemistry (focusing on histology imaging and high performance liquid chromatography) and lipid biochemistry (including general mass spectrometry services). Core D (Animal Facilities) provides animal (mostly mouse) models by assessing genetically altered mutants or stock strains in maintaining them disease-free. Core E (Neuroimaging) provides for acquisition and quantitative analysis of data derived from functional, morphometric, and spectroscopy MR imaging and positron emission tomography. Core F (Behavior Science) offers training of subjects for cooperation with research protocols, selection and administration of standardized tests (developmental, cognitive and functional), direct observations of behavior, and design of activation paradigms for fMRI. The core units serve 62 projects representing current direct annual NIH support totalling over 14 million dollars. The program addresses 14 of the 21 priority areas set forth in RFA HD-87-003, featuring interdisciplinary collaboration and a theme, brain mechanisms whereby genotypes result in specific cognitive and behavioral phenotypes of mental retardation or developmental disability. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MITOCHONDRIAL ENCEPHALOMYOPATHIES AND MENTAL RETARDATION Principal Investigator & Institution: Dimauro, Salvatore; Professor; Neurology; Columbia University Health Sciences New York, Ny 10032 Timing: Fiscal Year 2001; Project Start 10-FEB-1995; Project End 30-NOV-2004 Summary: The notion that mitochondrial encephalomyopathies are important causes of mental retardation in childhood that has been reinforced in the past four years by numerous studies, including those conducted as part of our Program Project. While our original application focused on disorders associated with point mutations of mtDNA, we will extend our interest to mitochondrial diseases due to mutations in the nuclear genome, specifically to the most common form of Leigh syndrome (LS), due to cytochrome c oxidase (COX) deficiency. We will also build on the experienced gained in the past 4 years to develop therapeutic approaches, at both the clinical and investigative levels. Project 1 (D.C. De Vivo, PI) will continue to characterize the natural history of MELAS and MERRF, correlating cognitive, and behavioral deficits in proband and oligosymptomatic maternal relatives with cerebral energy metabolism assessed by functional MRI and MRSI. Taking advantage of the large cohort of genetically homogeneous patients enrolled in the past four years, it will institute a controlled clinical trial with dichloroacetate (DCA), following brain lactate and neuropsychological

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features. Project 2 (S. DiMauro, PI) will add a molecular dimension to Project 1, by following mutational loads and drifts in heteroplasmy in various tissues from MELAS and MERRF patients, and by verifying the effects of DCA in vitro on cell lines harboring the MELAS mutation. It will extend promising preliminary data on disorders due to mutations in newly-identified mtDNA protein- coding genes. It will screen tissues from about 50 patients with COX- deficient LS for mutations in nuclear "COX-assembly" genes. It will screen tissues from about 50 patients with COX-deficient LS for mutations in nuclear "COX-disorders due to mutations in mtDNA protein-coding genes, and will attempt to devise a pharmacological approach to these disorders directed towards "downshifting" the percentage of mutant mtDNAs. Project 4 (M.M. Davidson, PI) will concentrate on genetic and functional complementation between the MELAS and MERRF mutations in syncytial myotubes and myofibers in culture, and explore the roles of mitochondrial fusion, mitochondrial fission, for the MELAS and MERRF mutations, using immunohistochemical techniques, focusing on the blood-brain proteins in brains from normal children and from children with COX-deficient LS. The Core Unit (S. DiMauro, Director) will provide direction, administration external consultation, and shared equipment/technical service to the project as a whole. Dr. Moraes, Rapin and Shoubridge will constitute the External Advisory Committee. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR ANALYSIS OF FRAGILE X SYNDROME Principal Investigator & Institution: Nelson, David; Associate Professor; Molecular and Human Genetics; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-APR-1992; Project End 31-MAR-2003 Summary: (adapted from the Investigator's abstract). Fragile X syndrome is among the most common human single gene disorders and the leading cause of inherited mental retardation. The most common mutation reduces expression of the FMR1 gene through expansion and methylation of an unstable CGG trinucleotide repeat. Two similar repeats are found nearby on the X chromosome which can expand and become unstable. All three sequences lead to cytogenetically visible fragile sites in their largest forms. Two sites, FRAXA and FRAXE lead to mental retardation associated with the FMR1 and FMR2 genes, respectively. The third, FRAXF, appears to be benign. This project seeks to continue study of these two genes leading to mental retardation. It will continue analysis of functional aspects of FMR1 and proposes to extend these studies to FMR2. It plans to study the roles of FMR1-related genes FXR1 and FXR2. These genes are similar in sequence to the FMR1 gene, and appear to interact with the FMR1 gene product in vivo. All three genes have properties of RNA binding proteins and may be involved in regulating RNA metabolism. The RNA targets of these three proteins may be important to the finding of mental retardation. FMR2 is unrelated in sequence and has features reminiscent of a transcription factor; however, it is highly expressed in portions of the brain associated with learning and memory, explaining the mental retardation seen in individuals lacking the protein. Specific aims are: 1) identification and characterization of mRNAs mis-regulated by absence of or lesions in FMR1 and FXR2; 2) development of mouse models to delineate developmental and temporal requirements for FMR1 in learning and behavior, and to study structure/ function aspects of FMR1.; 3) continued characterization of FMR1 and interacting proteins through two hybrid and mutation screens, 4) targeted disruption of the murine Fxr2 locus and description of the phenotype. If viable homozygotes can be produced, this animal will be crossed with the Fmr1 knockout mouse to determine the effect of the double knockout; 5) characterization of the FMR2 gene defective in FRAXE mental retardation.

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Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR ASPECTS OF MENTAL RETARDATION Principal Investigator & Institution: De Vellis, Jean; Professor; Psychiatry; University of California Los Angeles 10920 Wilshire Blvd., Suite 1200 Los Angeles, Ca 90024 Timing: Fiscal Year 2002; Project Start 30-SEP-1983; Project End 30-NOV-2006 Summary: The goal of this Program Project Grant is to advance our knowledge of the neurobiological mechanisms that give rise to mental retardation and developmental disabilities, using clinically relevant animal model systems. The projects investigate pathophysiological responses at the cellular and molecular levels that are the consequences of genetic mutations and environmental agents. The projects focus on key mechanisms, such as anomalies in cell signaling, immune response, cytokines and cell death that generally contribute to the progression of neurodegenerative disorders. All projects propose to explore factors important in development, repair and regeneration in the CNS processes central to the problem of mental retardation. Project I will investigate the human arginase and arginase deficiency. Project II will investigate mechanisms of glial pathogenesis and regeneration in demyelinating diseases; Project II will investigate neurotrophic and cellular interactions in myelin repair; Project IV will investigate VIP and PACAP action in CNS development and injury. The investigators share: animal and injury models, cell culture systems, cell and molecular biology approaches and transgenic and mouse knockouts. This interactive and multi-disciplinary group uses tools from genetic manipulation to cell transplant to address biological processes. The long-term goal is to build the foundation for therapeutic strategies to restore neurobiological functions in impaired individuals. This program has recently attracted several outstanding and basic scientists. Have laboratories of the UCLA Mental retardation RESEARCH Center in four closely interrelated projects carry out the research effort. Each project draws on the expertise of investigators in the other components of the program project grant. The research effort of the five research teams is greatly facilitated by their close physical location, the sharing of an administrative and shared facilities and the access to excellent services and scientific cores in the UCLA Mental Retardation Research Center. This grant will facilitate the research training of pre- and post-doctoral fellows who wish to direct their career goals in research in mental retardation and developmental disabilities. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: MOLECULAR BIOLOGY OF INTERCELLULAR JUNCTIONS Principal Investigator & Institution: Goodenough, Daniel A.; Professor; Cell Biology; Harvard University (Medical School) Medical School Campus Boston, Ma 02115 Timing: Fiscal Year 2001; Project Start 01-JAN-1975; Project End 31-DEC-2002 Summary: This proposal outlines experiments designed to further understand the molecular structure and function of two classes of intercellular junctions, gap junctions and tight junctions. There are five specific aims. First, the investigators will use Cx37 -/transgenic mice to study the signalling between granulosa and oocyte, and assess the role of cAMP in meiosis. Second, they will use KO Cx37 and KO Cx40 animals to study the role of those connexins on endothelial function. Third, the maternally inherited mRNA coding for Cx38 will be ablated and the developmental consequences to embryos will be studied. Fourth, dominant negative mutants of occludin, ZO1 and ZO2 will be used to study the role of those proteins in the Xenopus embryo. Finally, as a fifth

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specific aim, the possible role of Cx33 in a form of CMTX associated with mental retardation will be studied, and Cx33 KO mice will be generated. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR DEGENERATION

BIOLOGY

OF

SYNDROMIC

RETINAL

Principal Investigator & Institution: Sheffield, Val C.; Professor; Pediatrics; University of Iowa Iowa City, Ia 52242 Timing: Fiscal Year 2001; Project Start 01-AUG-1996; Project End 31-JUL-2002 Summary: (Adapted from applicant's abstract): This is a proposal to identify the molecular defect in Bardet-Biedl syndrome, an autosomal recessive disorder characterized by retinal degeneration, obesity, renal problems and mental retardation. The applicant and coworkers have identified three large unrelated inbred kindreds with Bardet-Biedl syndrome in genetically-isolated Bedouin tribes. The disorder maps to chromosome 16 in one kindred, chromosome 3 in another and chromosome 15 in the third. Specific aim one is to genetically fine-map the Bardet-Biedl syndrome candidate regions on chromosomes 16, 3 and 15. The mapping will be done by analyzing short terminal repeat polymorphisms (STRPs) in these families. The applicant, in conjunction with the Cooperative Human Linkage Center (CHLC), has developed 2000 markers, which can be screened in multiples by PCR. The locus has been mapped to within 20 cM for the chromosome 16q-linked disorder and 10 cM for the chromosomes 3- and 15linked disorders. The applicant estimates that with the available resources, linkage can be narrowed to 1-2 cM for chromosome 16 and <5 cM for chromosomes 3 and 15. Specific aim two is to employ positional cloning strategies to isolate the Bardet-Biedl syndrome gene on chromosome 16q. Closely-linked markers will be used to isolate YACs, which will be assembled into a YAC contig map. The YACs will be used to isolate cosmid clones from the region, and also to identify individual genes by exon trapping. Genes isolated in aim two will be sequenced. Those encoding "developmentally important" proteins such as growth factors, transcriptional factors, and homeotic genes will be priority. They will be studied by looking for mutations in Bardet-Biedl syndrome DNA samples by DGGE and/or SSCP analysis. This will be followed by sequence analysis. Specific aim three is to screen known candidate genes mapping to chromosomes 16, 3 and 15 that have been identified in other laboratories. Genes will be selected on the basis of several criteria: (1) homology to other genes that cause retinal degeneration; (2) developmental regulation; (3) genes expressed in retina and other affected tissues; and (4) genes expected to have pleiotropic effects. Candidate genes will be evaluated by genetic mapping and mutational screening in Bardet-Biedl syndrome patients. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR MECHANISM OF RECURRENT REARRANGEMENTS Principal Investigator & Institution: Morrow, Bernice E.; Associate Professor of Molecular Genetic; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 11-JUN-2001; Project End 31-MAY-2006 Summary: The chromosome 22q11 region is susceptible to rearrangement leading to congenital anomaly disorders associated with mental retardation. Cat eye syndrome (CES), der(22) syndrome and velo-cardio-facial syndrome/DiGeorge (VCFS/DGS), are associated with tetrasomy, trisomy or monosomy, respectively, of part of 22q11. Der(22) syndrome occurs in offspring of normal carriers of the constitutional t(11;22)

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translocation, the only known recurrent non-Robertsonian translocation in humans. We found a low copy repeat that we termed, LCR22, in the vicinity of recurrent chromosome breakpoints for these diseases implicating them in mediating 22q11 rearrangements that lead to congenital anomaly disorders on 22q11. In addition to congenital anomaly disorders, the 22q11 region is susceptible to somatic rearrangement leading to malignant disease. The major goal of this project is to determine the molecular basis of constitutional chromosome 22q11 rearrangements in mental retardation disorders. As our first goal, we propose to determine the mechanism by which the constitutional t(11;22) translocation occurs and understand whether other rearrangements in this interval are mediated by the same mechanism. Second, we will generate a precise map of novel chromosome rearrangement breakpoints within the genomic sequence of chromosome 22q11. One such example of a novel rearrangement is the unbalanced t (1;22) translocation that disrupts chromosomes 1p36 (Project III) and 22q11. We will clone and sequence chromosome breakpoint junctions to understand molecular mechanisms. We will generate a functional assay to examine the role of specific LCR22 motifs in mediating mitotic homologous recombination events. Finally, we will examine the evolutionary origin of LCR22s in primate species to understand the origin and maintenance of the current LCR22 structure. This knowledge will provide tools to be used to identify other architectural features in the human genome that may be susceptible to rearrangements. We believe that the 22qll region serves as a model to understand the molecular basis of chromosome rearrangements associated with mental retardation disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: MOLECULAR MECHANISMS OF NEURONAL MIGRATION Principal Investigator & Institution: Gleeson, Joseph G.; Assistant Professor; Neurosciences; University of California San Diego 9500 Gilman Dr, Dept. 0934 La Jolla, Ca 92093 Timing: Fiscal Year 2002; Project Start 15-JAN-2002; Project End 31-DEC-2006 Summary: (provided by applicant): Almost nothing is known about how neurons migrate, or about the molecular mechanism regulating this migration. Understanding these mechanisms is critical for our understanding of childhood epilepsy and mental retardation, as defects in neuronal migration frequently underlie these disorders. Additionally, one of the major hurdles in neuronal transplantation or regeneration following damage is poor neuronal migration into target areas, which may be overcome through approaches derived from a better understanding of how neurons migrate. One common inherited cause of severe mental retardation and epilepsy in humans is classical lissencephaly, defined by a lack of cortical gyri and sulci formation and due to a failure of neurons to properly migrate. Mutations in either of two genes, doublecortin (DCX) or lissencephalyI (LIS1), leads to severe generalized defects in neuronal migration and produces nearly identical lissencephaly in humans. A mutation in the cdk5 gene in mouse also leads to a defect in neuronal migration that is strikingly similar to human lissencephaly. The central hypothesis of this application is that these common mutant phenotypes suggest that there may be interactions between the encoded proteins. The predicted DCX and LIS1 proteins are entirely novel, suggesting they may help define novel molecular mechanisms of neuronal migration, and both were previously shown to function as microtubuleassociated proteins that are localized around the nucleus. The cdk5 gene is a serinethreonine kinase that phosphorylates some cytoskeletal proteins. However, the role of DCX, LIS1 and cdk5 in neuronal migration is unknown. Additionally, despite the very similar mutant phenotypes, it is untested whether these

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proteins interact directly to mediate their effect or even act in a common pathway. Therefore the Specific Aims of this proposal are to determine whether: 1) There are genetic or physical interactions between DCX and LIS1. 2) DCX and LIS1 function to regulate nuclear movement during neuronal migration. 3) DCX is regulated by cdk5 during neuronal migration. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: NEUROBIOLOGY OF FACIAL EXPRESSION IN AUTISM Principal Investigator & Institution: Bennetto, Loisa; University of Rochester Timing: Fiscal Year 2003; Project Start 01-JUL-2003; Project End 30-JUN-2008 Summary: The proposed research will examine the neurobiological bases of impaired facial movement and emotional expressiveness in autism. Individuals with autism typically have significant impairments in emotional expression, including limited range and inappropriate use of facial expressions to communicate affect and intentions. Research on facial imitation and neurological functioning in autism suggests that impairment in affective facial expression may be due in part to an underlying motor dysfunction. The proposed research will test two competing models of impaired facial motility in autism: dysfunction of brainstem structures that control the musculature of facial expression versus neurological dysfunction at a cortical level. These aims will be examined in two samples: children with high functioning autism compared to typically developing children matched on age, VIQ, gender, and race, and children with low functioning autism similarly matched to children with non-specific mental retardation. Facial motility will be examined through a quantitative analysis of discrete facial movements and the coordination of these movements in affective facial expressions. Nonmeaningful facial expressions will be assessed during imitation of movements that correspond to specific branches of the facial nerve. Affective facial expressions will be measured during spontaneous, elicited, and imitated expressions of six canonical emotions. The communicative value of emotional expressions will also be assessed through qualitative analyses of participants' expressions by na'fve raters. Models of neurological involvement in facial expressiveness will further be examined through a comprehensive and quantitative evaluation of cranial nerve function. Finally, this project will examine several early developmental genes that are important in the formation and function of the brainstem and cerebellum, and thus may influence facial movement abnormalities in autism. By specifying the nature of impaired facial motility in autism, this research will have implications for early intervention and may help in the identification of endophenotypes of this disorder. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: NON-RADIAL CELL MIGRATION IN CNS DEVELOPMENT Principal Investigator & Institution: Golden, Jeffrey A.; Associate Professor of Pathology; Children's Hospital of Philadelphia 34Th St and Civic Ctr Blvd Philadelphia, Pa 19104 Timing: Fiscal Year 2003; Project Start 15-FEB-2003; Project End 31-DEC-2007 Summary: (provided by applicant): Epilepsy, mental retardation and structural anomalies of the brain often have a genetic etiology. Although they affect 3-5% of all children, the underlying pathogeneses for these disorders is poorly understood in most cases. Cell migration is a central component of normal central nervous system (CNS) development and disruptions in this process have been implicated in the development of multiple disorders such as Fukuyama Muscular dystrophy, Miller-Dieker Syndrome, Walker-Warburg Syndrome, and the Muscle-Eye-Brain syndrome to name just a few.

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Two primary patterns of cell migration are recognized during CNS development, radial and non-radial. While the cellular and molecular bases of radial cell migration, long considered the predominant mode of cell migration, have begun to be defined, the mechanisms of guidance for non-radial cell migration remain largely unexplored. Using lineage analysis, we have defined the developmental time and location where non-radial cell migration begins in the chick forebrain. Based on these data we have developed a model to explain the cellular and molecular mechanisms of non-radial cell migration. Our model is based on the hypotheses that cell surface molecules, secreted molecules, and extracellular matrix molecules guide non-radially migrating cells. This proposal will begin to address our hypothesis by 1) directly testing several components of our model, and 2) generate a mammalian model to further study one of the molecules we have identified as a component of non-radial cell migration in the chick. These data will certainly enhance our understanding of normal CNS development. Furthermore, we anticipate the data from these studies will provide insight into the pathogenesis of a variety of inherited and non-inherited conditions that afflict children such as epilepsy, mental retardation and structural malformations of the brain. This may ultimately lead to improvements in the diagnosis, management, and prevention of neurological diseases where abnormal cell migration has a pathogenetic role. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: NOVEL ANTIPSYCHOTICS AND STEREOTYPED MOVEMENT DISORDER Principal Investigator & Institution: Newell, Karl M.; Assistant Professor Of; Kinesiology; Pennsylvania State University-Univ Park 201 Old Main University Park, Pa 16802 Timing: Fiscal Year 2003; Project Start 01-SEP-2003; Project End 30-JUN-2008 Summary: (provided by investigator): The introduction of the novel "atypical" antipsychotic medications (e.g., olanzapine, risperidone) has led to this class of medication becoming the most frequently used medication treatment for behavioral and psychiatric disorders associated with developmental disabilities. The atypical antipsychotics are purported to have both a lower risk of antipsychotic-induced side effects (e.g., dyskinesia) and selective beneficial effects for stereotypy and self-injury in persons with developmental disabilities. There have been few adequate tests to date of these assumptions in persons with developmental disabilities. In our previous work with typical antipsychotic medications, we validated a battery of instrumental tests for measuring antipsychotic- induced effects on motor control, and we characterized the dynamic nature of antipsychotic-induced effects on both aberrant behaviors and abnormal movements as a function of the addition, maintenance, and withdrawal of typical antipsychotic agents. In the proposed study, we will examine in persons with mental retardation the variety of potential antipsychotic-induced effects associated with atypical antipsychotic treatment (dsperidone and olanzapine) using behavioral observations, movement kinematic analyses, motor control tests and adaptive performance tasks. In the proposed study we will conduct an acute double-blind comparison of the atypical antipsychotics risperidone and olanzapine to each other and to placebo. A longterm follow up will also be conducted of the effects of the atypical antipsychotic treatment over a 1-year period in subjects who are clinical responders to acute treatment with either olanzapine or risperidone. The following questions will be addressed: (1) Does treatment with atypical antipsychotic medication significantly reduce the occurrence of stereotyped behaviors and abnormal movements? (2) How does atypical antipsychotic medication affect the movement dynamic properties of

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stereotyped behaviors and abnormal movements? (3) How does atypical antipsychotic medication affect the performance of adaptive, goaloriented actions? And (4) Are there differences between the short and long term effects of atypical antipsychotics on aberrant behavior, abnormal movements, and goal-oriented motor task performance? Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PORCINE SUBMAXILLARY MUCIN AND HUMAN NORRIN Principal Investigator & Institution: Hill, Robert L.; Professor and Chairman; Biochemistry; Duke University Durham, Nc 27706 Timing: Fiscal Year 2001; Project Start 01-SEP-1978; Project End 31-DEC-2002 Summary: The structure/function relationships of two related proteins will be examined. Porcine submaxillary mucin is a glycoprotein containing about 67 percent by weight carbohydrate. Mucins lubricate the respiratory, gastrointestinal and urogenital tracts and protect epithelial cells from dehydration and injury. Much of porcine submaxillary mucin structure has been elucidated, but not all, including how the monomeric mucin molecules assemble to form dimeric and multimeric mucins that are the mature, functional molecules. The half-cystines that form the inter- and intrachain disulfide bonds in dimers and multimers will be determined. Particular attention will be given to the structural motif CGLCG and its role in assembly of multimeric mucin. These studies will utilize site-directed mutagenesis and expression of mutant constructs in animal cells in culture. Similar studies will be performed with norrin, a 133 residue human protein that is defective in Norrie disease, an X-linked, recessive human neurological disorder characterized by blindness, deafness and mental retardation. This protein is very similar structurally to the domain in some mucins that forms disulfidelinked multimers. The half-cystines in norrin that form inter- and intrachain disulfide bonds will be determined by the same methods used with submaxillary mucin. Hopefully, these studies will reveal how the disulfide-rich domains in proteins in which they occur lead to oligomer formation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: POSITIONAL CLONING OF A GENE FOR MENTAL RETARDATION Principal Investigator & Institution: Higgins, Joseph J.; Associate Clinical Professor of Pediatri; Mid-Hudson Family Health Institute 279 Main St, Ste 101 New Paltz, Ny 12561 Timing: Fiscal Year 2001; Project Start 29-JAN-2001; Project End 31-DEC-2004 Summary: Mental retardation (MR) is the most common developmental disability in the United States affecting between 1 % to 3% of the population. Structural brain injury, chromosomal anomalies, malformation syndromes, and teratogens cause MR in some individuals, but often the etiology is unknown. Research efforts to identify the genetic basis of these non-specific, non-syndromic types of MR have been restricted by the lack of informative family pedigrees and the complexity of intellectual traits. The preliminary results of this proposal traverse these limitations by using five nuclear families from a large founder population to establish genetic linkage between an autosomal recessive, non-specific MR phenotype and a disease locus on the subtelomeric region of chromosome 3p (chr 3pter). Based on these findings there are four main reasons to pursue positional cloning efforts to identify the causative gene: First, the terminal ends of human chromosomes are "hot-spots" in the etiopathogenesis of MR. Second, breakpoint mapping in individuals with syndromic MR and cytogenetic deletions on chr 3pter confirms that a gene for MR resides within our linked candidate interval. Third, several positional candidate genes in the region encode for proteins that

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are crucial for normal brain development, neuronal adhesion and molecular signaling. Fourth, a virtual contig of the region estimates that the size of the interval is amenable to physical mapping. The long-term objective of this study is to identify the genes on chr 3pter that cause delayed acquisition of higher cognitive skills. The specific aims are the following: 1). Collect affected sib pairs and families with MR. 2). Refine the minimal critical region (MCR) that contains a gene for MR by identifying key recombinants. 3). Construct a high-resolution physical map (contig) of the MCR. 4). Isolate the genes within the contig and evaluate these candidates for disease-causing mutations. The identification of a gene for general intelligence will advance our understanding of the developmental neurobiology of MR and open avenues for better interventions and effective treatments to reduce its physical, financial and emotional burden. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: PREVENTING SUBSTANCE ABUSE IN SPECIAL EDUCATION STUDENTS Principal Investigator & Institution: Wells, Jennifer J.; Research Scientist; Oregon Center for Applied Science 1839 Garden Ave Eugene, or 97403 Timing: Fiscal Year 2001; Project Start 30-SEP-2001; Project End 31-MAY-2002 Summary: (provided by investigator): The overall goal of this project is to create an interactive program designed to prevent use and abuse of illicit substances among youth with mental retardation and developmental disabilities (IMRDD). Young people with MRDD want to be like, and be accepted by their non-disabled peers. Research has shown that MRDD youth are more apt to use illicit substances in response to social (i.e., peer) pressure than their non-disabled peers. Additionally, approximately half of individuals with MRDD who use substances (including alcohol) may be characterized as substance abusers. Individuals with MRDD that abuse substances suffer the same health-related, social, economic, and emotional consequences as people without MRDD. This project will develop four stand-alone gender-specific CD-ROM substance abuse prevention programs for youth with MRDD at middle school and high school age levels. Within each program, the social and behavioral skills required for (a) recognition of, (b) response to, and (c) retreat from substance use risk situations will be taught via an interactive text-free program that incorporates graphic, animation, and video components. Direct Instruction (DI) methodology will be employed to facilitate mastery of program content for all program users. Forty MRDD high school males in a pre/post test design will evaluate the program. PROPOSED COMMERCIAL APPLICATIONS: This program fills a unique need: Substance abuse prevention for youth with mental retardation and developmental disabilities (MRDD). This product will be attractive to people with MRDD, and anyone serving this population (e.g., schools, health clinics, parents, services for at-risk youth, training centers, vocational and residential services. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: PSYCHOPATHOLOGY IN YOUNG PEOPLE WITH MENTAL RETARDATION Principal Investigator & Institution: Tonge, Bruce J.; Monash University Faculty of Medicine, Nursing & Health Sciences Melbourne, Vic, 3181 Timing: Fiscal Year 2002; Project Start 20-AUG-2002; Project End 31-JUL-2004 Summary: (provided by applicant): Families caring for young people with mental retardation face major burdens of care if the young person also has emotional and behavioural problems. Serious mental health problems are 2-3 times more common in

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young people with mental retardation, which apart from the personal distress and loss opportunity it causes the child and family, is also a major cost to our community. This project is intended to assist young people and their careers by providing new information about the factors contributing to these mental health problems and how they develop over time. The project makes use of the existing internationally unique epidemiological longitudinal study (the Australian Child and Adolescent Development Study ACAD) which has followed a group of young people with mental retardation aged 4-18 for the last eight years, from childhood through adolescence as they move into young adult life. The ACAD study has collected an extensive range of biopsychosocial data on these young people and their families. This project will examine the unique database produced by the ACAD study using state-of-the-art statistical techniques in to order to elucidate the biopsychosocial risk and protective factors relating to the progression and development of mental health problems in this under researched and under serviced population. It is expected that the results of such analyses will directly contribute to the development of appropriate intervention and prevention programs. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: REGULATION OF THE FRAGILE X MENTAL RETARDATION PROTEIN Principal Investigator & Institution: Gabel, Lisa A.; Neuroscience; Brown University Providence, Ri 02912 Timing: Fiscal Year 2003; Project Start 01-AUG-2003; Project End 31-JUL-2005 Summary: (provided by applicant): The overall goal of the proposed experiments is to characterize the mechanisms that regulate the activity-dependent translation and degradation of Fragile X mental retardation protein (FMRP). Fragile X syndrome is the most common form of inherited mental retardation. This disease is caused by transcriptional silencing of the FMR1 gene and thus loss of expression of FMRP. Recent work indicates that FMRP is an RNA-binding protein involved in the regulation of protein synthesis-dependent synaptic plasticity. Furthermore, FMRP itself may be subject to translational regulation by synaptic activation. Preliminary studies from our laboratory indicate that: 1) visual experience induces transient expression of FMRP in the visual cortex of dark reared/light exposed rats; 2) cytoplasmic polyadenylation element and the CPE binding protein may play a role in the translational activation of FMR1 mRNA following visual experience; 3) the ubiquitin system may be involved in the rapid disappearance of FMRP. The translation and subsequent rapid degradation of FMRP could be important factors in regulating protein synthesis-dependent synaptic modification. In the proposed experiments we will further elucidate the cellular and molecular mechanisms that regulate FMRP expression during synaptic plasticity. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: RESEARCH IN PRADER-WILLI SYNDROME AND OBESITY Principal Investigator & Institution: Driscoll, Daniel J.; Hayward Professorship in Genetics Resear; Pediatrics; University of Florida Gainesville, Fl 32611 Timing: Fiscal Year 2001; Project Start 07-JUN-2000; Project End 31-MAY-2005 Summary: (Adapted from the candidate's description) The goals of this translational research project are to genetically and clinically dissert the imprinted genes underlying each component of Prader-Willi syndrome (especially the gene causing obesity), and to explore the potential role of early childhood morbid obesity plays in mental retardation. This will be accomplished by screening for mutations in candidate genes for various

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phenotypic components of PWS; performing psychometric testing on children with early onset morbid obesity and their families; and correlating hormonal and neurotransmitter levels from blood and CSF with the psychometric and mutation analyses. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SEGMENTAL ANEUSOMY BETWEEN BLOCKS OF DUPLICATED DNA Principal Investigator & Institution: Eichler, Evan E.; Assistant Professor; Genetics; Case Western Reserve University 10900 Euclid Ave Cleveland, Oh 44106 Timing: Fiscal Year 2003; Project Start 03-JAN-2003; Project End 31-DEC-2007 Summary: (provided by applicant): One of the major goals of the field of human genetics is to define the relationship between human genotype and phenotype. Much of our assessment of genotypic variation has been focused on small scale, single nucleotide events. Our understanding of the molecular basis of disease, however, has begun to reveal that large-scale differences including micro duplications and micro deletions contribute significantly to childhood disease, disease susceptibility and normal variation in the population. Despite its importance, there has been no systematic study of this form of genotypic variation. The long-term objective of this proposal is to investigate the pattern and nature of this large-scale variation. Our approach will be directed to regions of the genome that contain highly homologous duplicated sequence and therefore have an increased probability of genomic gain and loss. This proposal is a collaborative effort that brings together expertise in genome structure, array comparative genomic hybridization technology and mental retardation. The specific aims of this proposal are (1) to identify and validate all intrachromosomally duplicated regions within the human genome, (2) to develop a set of large-insert clones bracketed by duplicated sequence to be placed on a CGH microarray platform for genome-wide screening, (3) to assess copy number variation within both normal individuals and children with idiopathic mental retardation and (4) to validate the extent, frequency and inheritance pattern of these large structural "polymorphisms". This project aims to address two fundamental questions; what is the nature and frequency of duplication-mediated structural polymorphisms within the human genome? Are there an excess of de novo events among children with mental retardation and congenital birth defects? Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: SEQUENCE TEACHER: BEHAVIORAL TEACHING TECHNOLOGY Principal Investigator & Institution: Mackay, Harry A.; Praxis, Inc. 13 West St Belmont, Ma 02478 Timing: Fiscal Year 2002; Project Start 01-JUN-2002; Project End 30-NOV-2002 Summary: The SBIR application seeks support to develop and study a computer- based product attended primary for teaching children with mental retardation, autism, and other intellectual disabilities. The product addresses a critical skill repertoire involving the production of stimulus sequences. Sequence production is a pre-academic skill relevant to the development of reading, spelling, numeric skills, and concepts, as well as everyday tasks like making a telephone call or operating a vending machine. Research supported by NIH has resulted in the development of techniques that can establish sequencing skills in our target population. These techniques, however, are not readily accessible to parents, professionals and the children who might benefit. This proposal aims to fill that gap by the development of a user-friendly software product for

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computer-assisted instruction. The basic task involves a form of constructed matching to sample: A sample stimulus is presented to the student, who then must select two or more individual stimuli (e.g., letters, numbers) from a pool to construct a multi-element stimulus complex (e.g., letter-by-letter, number-by-number) that is related to computer screen, and the student would then select the individual letters (S, H, etc.) from an array of letters displayed at the bottom of the screen. As each letter is selected, it moves by computer animation to a position below the picture, so that the student "constructs" the world "SHIRT." The project has two major objectives First, we will adapt well-developed methods from laboratory and applied research for use by teachers, other child care professionals, and parents. Second, we will evaluate the product to determine that it can be used effectively in typical educational situations. PROPOSED COMMERCIAL APPLICATIONS: The product being developed and evaluated may have a significant impact on special education practice. As such, the product is potentially marketable to speech/language pathologists, special educators, psychologists, other professionals, and parents. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SIGNAL TRANSDUCTION IN NEURON MIGRATION & AXON GUIDANCE Principal Investigator & Institution: Walsh, Christopher A.; Professor; Beth Israel Deaconess Medical Center St 1005 Boston, Ma 02215 Timing: Fiscal Year 2001; Project Start 15-AUG-2000; Project End 31-MAY-2005 Summary: This Program Project focuses on the signaling mechanisms that control the directed outgrowth of neuronal processes and the directed migration of neuronal cells. Together these two properties are essential to neuronal function, since the precise location of neurons and proper interconnection of their axons and their dendrites underpins all neuronal signaling and plasticity Abnormal patterns of neuronal migration have been implicated in human neurological disorders associated with mental retardation, epilepsy, and dyslexia, and congenital disorders of mental retardation are increasingly recognized as having major disorders of axonal and dendritic connections. Moreover, cell surface signaling and cytoskeletal regulation are implicated in degenerative disease such as Alzheimer's disease. Finally, proper axon guidance and axon outgrowth are essential to any possibility of neuronal repair following CNS injury. Until recently it has been controversial whether axon outgrowth and neuronal migration utilized a similar set of signals or whether they used fundamentally different mechanisms. However, several genes including filamin and disabled have recently been shown to be essential to proper neuronal migration to the cerebral cortex, as well as for normal axon outgrowth. This proposal contains three projects that examine 1] molecular regulation of neuronal migration to the cortex, 2] molecular control of axon outgrowth in flies, and 3] molecular control of axon outgrowth via ephrins and Eph receptors. The three linked proposals produce synergy by a] exploiting complementary strengths of mammalian and invertebrate genetic systems, in some cases by studying the same molecular and processes in different organisms, and b] utilizing a core to provide modern imaging technology that is essential for all the diverse projects regardless of the organism of study. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: SLEEP RETARDATION

ARCHITECTURE:

INDIVIDUALS

WITH

MENTAL

Principal Investigator & Institution: Kennedy, Craig H.; Special Education; Vanderbilt University 3319 West End Ave. Nashville, Tn 372036917 Timing: Fiscal Year 2003; Project Start 01-APR-2003; Project End 31-MAR-2005 Summary: (provided by applicant): Sleep dysfunction produces a range of negative health and psychological effects. The incidence of sleep problems for individuals with mental retardation (MR) has been reported to be three-fold greater than people without MR. However, previous research estimating the prevalence of sleep problems for people with MR have relied primarily on behavioral observation data. Such analyses can only identify gross behavioral changes in sleep states and are unable to identify subtler alterations in brain activity and sleep architecture that, nonetheless, have serious effects on a person's health and life quality. Hence, current estimates of sleep problems in this population may be underestimated. A more precise analysis of sleep and individual sleep stages is needed. The investigators propose to use polysomnography (i.e., EEG, EOG, and EMG) to investigate the sleep architecture of people with MR. Three groups of adults with MR (mild, moderate, and severe/profound) will be studied to assess the presence of altered sleep architectures as a function of level of MR. These groups will be compared to a contrast group of typical adults. In addition, the investigators propose to assess whether the presence of a co-morbid syndrome (i.e., autism) interacts with level of MR in determining the structure of a person's sleep architecture (i.e., autism plus mild MR, autism plus moderate MR, and autism plus severe MR). Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SOCIAL COGNITION IN WILLIAMS SYNDROME Principal Investigator & Institution: Tager-Flusberg, Helen B.; Professor of Anatomy and Neurobiology; Anatomy and Neurobiology; Boston University Medical Campus 715 Albany St, 560 Boston, Ma 02118 Timing: Fiscal Year 2001; Project Start 29-SEP-1995; Project End 31-AUG-2005 Summary: The goals of the proposed research are to systematically investigate social information processing capacities in Williams syndrome (WMS). Earlier work has shown that people with WMS have good face processing skills and language ability. While they are not spared on classic theory of mind tasks, studies we conducted in our ongoing research, during the current award period, suggest that they are better than matched comparison groups in reading mental state information in eyes. We plan to extend this work by investigating foundational capacities in adolescents and adults with WMS to process information in two main channels for social communication: faces and vocal prosody. A series of experiments will be conducted with groups of adolescents and adults with WMS, matched on age, gender, IQ and receptive vocabulary to adolescents and adults with non-specific mental retardation, and to age and gender matched non-retarded controls. The experiments test the hypothesis that people with WMS are relatively spared compared to the matched mentally retarded controls in: (A) Face recognition; we also predict that they process faces using the same holistic representations as normal adolescents and adults; (B) Voice recognition; and the use of prosody in linguistic processing of both word and sentence ambiguity; (C) Attributing social-mental state information to faces; (D) Attributing mental state information to vocal prosody; (E) Recognizing people and attributing mental state information to dynamically presented social stimuli; (F) Expressing affect and empathy to dynamically presented emotionally charged events at physiological, and behavioral (but not

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cognitive) levels. We are especially interested in investigating the relationships between face processing skills and use of linguistic prosody, and parallels in the ability to attribute social or mental state information to faces and prosody -the two primary channels for interpersonal communication and social interaction. Our research also explores these links between faces and vocal prosody in different modalities: in the perception and expression of emotion. These studies will lay the groundwork for future studies that will address how these capacities develop in children with WMS, and the neural bases of these social information processing skills using functional brain imaging methodologies. This research will significantly advance our understanding of the phenotypic characteristics of people WMS, which has important implications for enhancing their everyday lives. Together, this program of research on WMS will provide a unique contribution to theoretical and empirical work in the newly emerging field of social cognitive neuroscience. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SOMATIC CELL GENE THERAPY AND NITROGEN FLUX IN UREA CYCLE PATIENTS Principal Investigator & Institution: Lee, Brendan; Associate Professor; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 01-AUG-2001; Project End 31-JUL-2002 Summary: The group of inborn errors of hepatic metabolism continue to be a prominent cause of mental retardation because of ineffective treatment strategies. Gene replacement therapy offers the theoretic advantage of correcting the basic protein deficiency. However, progress in hepatocyte directed gene therapy has been limited by questions involving pathophysiologic processes, choice of promoter and vector delivery system, route of delivery, host immune clearance, duration of expression, availability or small and large animal disease models, and quantitative measures of clinical efficacy. The three parts of this proposal attempt to address some of these issues using the group of urea cycle defects a model system. The first goal is to better understand the pathophysiologic disturbances in patients with urea cycle defects by correlating genotype and clinical severity with in vivo measurement of nitrogen flux and ureagenesis, while also developing a quantitative measure for future in vivo gene therapeutic interventions in both animal and humans. Flux through the urea cycle pathway will be measured by quantifying the conversion of [15/N-amide]glutamine to [15/N] urea. This flux will be correlated with genetic status (homozygosity, heterozygosity, hemizygosity), nature of mutation (null versus hypomorphic), and clinical severity (neonatal versus later presentation) in affected patients, heterozygous family members, and normal controls. In the second part of the study, the relative safety and efficacy of first generation (E1a deleted) and second generation (E1a/E2a deleted and all coding sequence deleted) adenovirus vectors will be determined after intravenous (i.v.) delivery in animals. In addition, potential avenues permitting long term transgene expression will be investigated. The efficacy of transient immunosuppression for the readministration of viral vectors will be evaluated, and the potential use of mariner transposon elements in mediating transgene integration in a host mammalian genome will be studied. In the third part, the urea cycle disorders, specifically murine and bovine models of citrullinemia, will be used a model systems in applying these basic findings to a clinical setting. The efficacy of the hybrid, ubiquitously active, CAG and liver-specific human albumin promoters will be compared in vivo. These data will form the preclinical basis for designing phase I clinical trials involving gene therapy in urea cycle patients. These results together will

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also be more generally applicable to other inborn errors of hepatocyte metabolism and to the production of extracellular products by hepatocytes. Early and long term biochemical correction would be expected to greatly decrease the great neurologic morbidity associated with these conditions. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: SOMATIC CELL GENETIC STUDIES OF DOWN SYNDROME Principal Investigator & Institution: Patterson, David; President and Senior Fellow; Eleanor Roosevelt Inst for Cancer Res 1899 Gaylord St Denver, Co 80206 Timing: Fiscal Year 2001; Project Start 01-JAN-1993; Project End 31-DEC-2002 Summary: Affecting one in 700 live births, Down Syndrome is the major recognized genetic cause of mental retardation. It is, and expected to remain, a significant medical and social problem. While the neurological and behavioral phenotype of Down Syndrome and its genetic basis is complex, recent advances in technology, resources and knowledge mean that renal and effective progress in understanding Down Syndrome is now attainable. The immediate goal of this program project is the identification and characterization of the genes involved in the neurological, neurophysiological and behavioral phenotype of Down Syndrome. The long term goal is to apply this knowledge to the development of therapeutic interventions to ameliorate the cognitive deficits seen in Down Syndrome. The accomplishment of goals of this nature requires application of a broad spectrum of approaches, spanning molecular biology, mouse genetics and human behavior. Accordingly, this proposal will build upon the large body of information regarding Down Syndrome and human chromosome 21 compiled by this Program over the last fifteen years, and will extend its research focus in a clinically relevant direction. The specific aims of this program include: i)systematic identification and preliminary characterization of essentially all genes on chromosome 21 to determine best candidates for causation of the neurological and cognitive deficits of Down Syndrome; ii) construction and analysis of mouse models, both single gene transgenics and segmental trisomies, to test the roles of specific genes; iii) dissection of the cognitive deficits seen in individuals with Down Syndrome; iv) analysis of the behavior and physiology of relevant mouse mutants to refine understanding of the deficits seen in individuals with Down Syndrome and to define the limits of utility of mouse models; and v) design and testing in mice of new hypotheses and treatment approaches based on information obtained from the molecular, human behavior and mouse genetic data. Building on the past accomplishments and expertise of members of this program project and adding new directions and new areas of expertise makes attainment of these goals eminently feasible. Their success will add greatly to our understanding of neurological development in general and of mental retardation in Down syndrome in particular. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: SPHINGOGLYCOLIPIDS OF NORMAL AND PATHOLOGICAL BRAINS Principal Investigator & Institution: Yu, Robert K.; Director, Institute of Molecular; Biochem and Molecular Biology; Medical College of Georgia 1120 15Th St Augusta, Ga 30912 Timing: Fiscal Year 2001; Project Start 01-JUL-1988; Project End 31-JUL-2003 Summary: The expression of gangliosides in the nervous system is not only cell specific and developmentally regulated, but also closely related to the differentiation state of the cell. These molecules are known to play important modulatory roles in cellular

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recognition, interaction, adhesion, and signal transduction during specific developmental stages. We hypothesize that the temporal and spatial expression of gangliosides in the nervous system is closely related to their metabolism, in particular, biosynthesis. Four specific aims are proposed to test this hypothesis. (1) We will carry out detailed structural studies on many stage-specific gangliosides which play crucial roles in the early development of the nervous system. These structural studies are fundamental for all molecular biological investigationson the biosynthesis of these molecules. (2) Because one of the regulatory mechanisms occurs at the transcriptional level, we will focus on cloning the gene encoding the first sialyltransferase (GM3synthase) that gates the synthesis of gangliosides; this has never been achieved. (3) We will elucidate the promotor sequences of the genes encoding several key regulatory sialyltransferases and to study their structure. Analysis of the transcription factors for ganglioside synthesis has never been achieved, but would be crucial for understanding the cell-specific and development-regulated regulation of the genes in this process. (4) As evidence has been provided that the activities of several glycoslytransferases can be modified post-translationally, e.g., by phosphorylation and N-glycosylation, we will elucidate the role of phosphorylation/dephosphorylation as a mechanism for the fast and reversible regulation of glycosyltransferase activities in response to physiological demands. The role of N-glycosylation will also be explored with respect to the activity and cellular localization/trafficking of these enzymes in the cell, employing the molecular biological knowledge gained above. An understanding of the molecular mechanisms underlying the differential expression of cell surface gangliosides should greatly enrich our knowledge in their functions in normal brain development as well as in neurological disorders that result in mental retardation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: DISORDERS

STEREOTYPIES

IN

CHILDREN

WITH

DEVELOPMENTAL

Principal Investigator & Institution: Unis, Alan S.; Associate Professor & Director; Psychiatry and Behavioral Scis; University of Washington Seattle, Wa 98195 Timing: Fiscal Year 2001; Project Start 01-DEC-1998; Project End 30-NOV-2001 Summary: This project will characterize early rhythmic behaviors from home videotapes of children later diagnosed with autism or mental retardation. It is hypothesized that rhythmic behaviors, which normally occur with high frequency during infancy, are related to later abnormal stereotyped and self-directed behaviors. Stereotypy scores derived from direct observations at 1 and 2 years of age will be compared to similar direct observational measures and global assessments of stereotypy using contemporary measures (e.g. the CARS) obtained at 3-4 years of age. Stereotypy scores in the first and second year of life will also be compared to measures of general cognitive function and performance on specific neurocognitive tests at 3-4 years of age. This study will specifically: (1) characterize the stability of different stereotypic (rhythmic) behaviors at one-year intervals between the ages of one and four years; (2) identify early temporal and topographic characteristics of stereotypic behaviors that are expected to predict the development of later abnormal behaviors; and, (3) determine which features of early stereotypies (intensity, frequency, topography) predict lower cognitive functioning. Stereotyped and self-directed behaviors, seen in many individuals with mental retardation or mental illness, pose serious problems for individual safely, quality of life, and financial costs for care. Despite considerable research aimed toward elimination of these behaviors, the problem continues to be one of the most serious predictors of chronic institutionalization, morbidity and premature death in individuals with

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developmental disorders. There is neither an understanding of the development of stereotypies and self-directed behaviors nor an ability to predict which individuals within these populations will develop these debilitating behaviors. These studies ultimately aim to identify specific early predictors of self-directed behaviors, thus permitting earlier and informed intervention in high- risk child populations with severe developmental disorders. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: TELOMERE INTEGRITY OF DE NOVO TERMINAL DELETIONS Principal Investigator & Institution: Shaffer, Lisa G.; Baylor College of Medicine 1 Baylor Plaza Houston, Tx 77030 Timing: Fiscal Year 2001; Project Start 11-JUN-2001; Project End 31-MAY-2006 Summary: Although about 3% of the World's population are mentally retarded, the etiology of mental retardation of the majority of patients is unknown. Of the known causes, chromosomal abnormalities account for about 20% of cases. An underrecognized cytogenetic abnormality, which contributes to the mentally retarded population, is deletion of the terminal band of the short arm of chromosome 1. Preliminary mapping of the subtelomeric- region of distal 1p has revealed diversity in the ends of the chromosomes with some apparent terminal deletions, some patients of this proposal is to understand the mechanisms involved in generating terminal deletions. We will use deletions of the short arm of chromosome 1 as a model system to explore the following specific aims. Aim 1: To distinguish the various types of rearrangements that comprise a group of cytogenetically terminal deletions. Deletions of 1p36 will be screened with a commercially available set of telomere region-specific fluorescence in situ hybridization (FISH) probes to identify those that are terminal deletions. Deletions of 1p36 will be screened with a commercially available set of telomere region-specific fluorescence in situ hybridization (FISH) probes to identify those that are terminal deletions, interstitial deletions, or derivative chromosomes (products of an unbalanced translocation). Aim 2: To determine the structure of the subtelomeric region of distal 1p. This will define the end of the physical and genetic map of 1p will enable the identification of deletion breakpoints and identify genomic regions that may recombine with 1pter to produce deletion and derivative chromosomes. Aim 3: Identify the mechanisms by which terminal deletions are formed and stabilized. Using reagents from Aim 2, subtelomeric sequences will be defined and deletions screened for retention of the subtelomeric region. Those without subtelomeric regions will be investigated for the de novo synthesis of the telomeric repeat using a variety of techniques. Aim 4: Delineate a DNA sequence that is susceptible to chromosome breakage or recombination that results in terminal deletions of 1p36. Breakpoints will be identified and sequenced for 1p36 deletions. Sequence comparison of breakpoints is likely to identify a common sequence susceptible to breakage or rearrangement. Aim 5: Determine the evolution and organization of orthologous sequences to human 1p36 in higher primates and determine the fidelity of the breakpoint susceptibility locus in the karyotypic evolution of higher primates. Reagents from Aim 2 will be used to interrogate the genomic structure in higher primates. Breakpoint susceptibility sequences will be analyzed in the primate species to understand their organization and role in karyotypic evolution. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: THE ETIOLOGY OF FRAGILE X MENTAL RETARDATION SYNDROME Principal Investigator & Institution: Dolen, Gul; Neuroscience; Brown University Providence, Ri 02912 Timing: Fiscal Year 2002; Project Start 20-MAR-2003; Project End 31-AUG-2007 Summary: (provided by applicant): Fragile X mental retardation syndrome is one of the most common heritable forms of mental retardation in humans. The molecular genetic basis of fragile X syndrome has been identified; mutation of the fragile X mental retardation-1 gene(FMR1) leads to a loss of the protein product, the fragile X mental retardation protein (FMRP). Despite our genetic understanding of fragile X syndrome, the biological function of FMRP remains unknown. The role of FMRP can now be studied using the Fmrl-KO mouse, a transgenic model of fragile X syndrome in which FMRP has been genetically knocked out. Recent work in our lab has used these mice to identify a functional role for FMRP in regulating activity-dependent synaptic plasticity in the brain; FMR1-KO mice exhibit increased long-term depression (LTD) of synaptic strength induced by metabotropic glutamate receptor (mGluR) activation. We hypothesize that a lack of FMRP increases mGluR-dependent protein synthesis and/or long-term depression (LTD) in the brain and might be an underlying cause of fragile X mental retardation. Specifically, we aim to test the possibility that the abnormal dendritic spine formation and increased susceptibility to epileptiform activity associated with fragile X syndrome is a direct consequence of inappropriate mGluR regulation. Through this mechanistic link, we hope to account for the morphological, physiological, and behavioral characteristics of fragile X syndrome and to devise strategies for therapeutic treatments. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: VALPROATE ADOLESCENTS

RESPONSE

IN

AGGRESSIVE

AUTISTIC

Principal Investigator & Institution: Hellings, Jessica A.; Associate Professor; Psychiatry; University of Kansas Medical Center Msn 1039 Kansas City, Ks 66160 Timing: Fiscal Year 2001; Project Start 15-APR-1998; Project End 31-MAR-2003 Summary: (Adapted from Applicant's Abstract): This career (K08) award applies specified funding, 75 percent research time and a 5-year plan to develop the candidate's experience and expertise, in the understudied area of psychopharmacologic research in persons with autism and mental retardation (MR). A primary goal is for the candidate to learn from Dr. Schroeder how behavioral observation and Applied Behavior Analysis can be used in research in this population. By performing a double-blind drug study, with Dr. Schroeder's weekly supervision, regular internal and expert-consultation and attending research didactic courses, the candidate will develop into an independent psychopharmacology researcher with expertise in behavioral observation and intervention techniques. The proposed study aims to test the hypothesis that aggressive autistic adolescents will show a significantly greater response to valproate maintained at blood levels of 75-100 mcg/ml than to placebo (PRO), in a double-blind case-controlled design. The study will also critically assess the safety of valproate in autistic adolescents. The study will be performed over 5 years, on 30 outpatient autistic adolescents aged 10 to 19 years, referred to the University outpatient MR/Autism Clinic for aggression. Study candidates will undergo DSM-IV evaluation, the ADI-R and ADOS, and baseline blood tests. Exclusion criteria will include degenerative CNS disorders, unstable medical illness, seizures in the past 6 months, a history of valproate sensitivity or

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previous liver disease, a history of ovarian cysts in girls, a low platelet count or raised liver transaminases. No other psychoactive maintenance medication will be allowed. Any level of MR (IQ<70) will be permissible. After baseline screening, and a 1-week placebo lead-in, subjects will be randomized to PBO or valproate for 8 weeks. Dosage adjustment according to blood levels drawn at the end of weeks 2 and 4 will be arranged with parents by a child psychiatrist without breaking the blind. The Aberrant Behavior Checklist-Community (ABC-C) irritability subscale will be the primary measure; the Overt Aggression Scale (OAS), ABC-C hyperactivity subscale, Clinical Global Impressions (CGI) problem severity, Self-Injurious Behavior Questionnaire (SIB-Q), and a valproate side effects checklist will be secondary measures. Primary measure scores obtained will be analyzed using a multivariate analysis of variance comparing drug placebo groups pre- and post-treatment. The secondary measures will be analyzed using a multivariate analysis of variance. Study results will be prepared and submitted as manuscripts for publications and presentations. Achievement of goals of the grant will be monitored by Dr. Schroeder weekly, quarterly and annually. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: VARIABLES AFFECTING RESPONSE BETWEEN ABERRANT & PRODUCTIVE BEHAVIORS Principal Investigator & Institution: Saunders, Richard R.; Senior Scientist; University of Kansas Lawrence Lawrence, Ks 66045 Timing: Fiscal Year 2001; Project Start 01-JAN-2001; Project End 31-DEC-2002 Summary: There is no text on file for this abstract. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

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Project Title: VOCABULARY EXPANSION IN SEVERE MENTAL RETARDATION Principal Investigator & Institution: Wilkinson, Krista M.; Eunice Kennedy Shriver Center; Univ of Massachusetts Med Sch Worcester Office of Research Funding Worcester, Ma 01655 Timing: Fiscal Year 2001; Project Start 01-APR-1998; Project End 31-MAR-2003 Summary: In typically-developing children initially slow, one-at-a-time word learning is followed by an apparent explosion in their lexicon. One estimate suggests that children are learning up to nine new words a day. By contrast, for some individuals with severe mental retardation new words enter the lexicon only through slow and deliberate effort. Yet word learning is a foundational component of human development, necessary for advanced linguistic functions and contributing to complex representational skills (e.g., categorization). To the extent that a cognitive disability interferes with word learning, progress in these domains will be correspondingly limited. It is therefore essential to explore in detail the nature of lexical impairments in severe mental retardation. The existence of difficulties in lexical acquisition among individuals with severe mental retardation raises important questions. When rapid vocabulary expansion occurs, what linguistic cognitive processes support that learning? When it does not occur, what skills are deficient or absent? A phenomenon called fast mapping may be of greatest potential relevance for rapid vocabulary expansion. Fast mapping refers to a quick, initial partial understanding of a new word's meaning derived from the context of word use. Fast mapping has been argued to facilitate the vocabulary explosion. This proposed role has received empirical confirmation in typical youngsters and children with Down syndrome. Yet evidence from children with atypical cognition/language has recently challenged the universality of this relation. The Principal Investigator's studies have

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added to the preliminary evidence of selective deficits in fast mapping among individuals with severe mental retardation, William's syndrome, or specific language impairments. The deficits are most often observed when individuals attempt to apply the principle for the purpose of vocabulary expansion. These recent findings oblige a more systematic analysis of the precise role of the principle in learning outcomes in mental retardation. Is fast mapping at risk in individuals with severe mental retardation, potentially limiting their vocabulary expansion? This application proposes a five-year study of vocabulary expansion and delay, focusing specifically on rapid expansion of an extant, but limited, vocabulary. The proposed series of studies will implement methods developed by the Principal Investigator that will enable the systematic analysis of unresolved questions of lexical expansion in severe mental retardation. The project has four specific aims that will be addressed in the course of four studies: 1) To examine the relation of fast mapping, rapid vocabulary expansion, and nonverbal processes, in order to explore the nature of lexical expansion in severe mental retardation; 2) To explore the points of greatest vulnerability for learning through fast mapping by explicitly taxing participants' skills and observing the ways in which breakdowns occur; 3) To extend the analysis from the commonly examined object-word learning to the acquisition of action-related words, to determine whether processes of fast mapping are similar for the two types of form/class categories; 4) To examine the basis underlying children's apparent assumption that new words should and do map to unnamed objects, and whether this assumption is intact among individuals with severe mental retardation. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen •

Project Title: X-LINKED MENTAL RETARDATION-LINKAGE Principal Investigator & Institution: Schwartz, Charles E.; Director; Greenwood Genetic Center 1 Gregor Mendel Circle Greenwood, Sc 29646 Timing: Fiscal Year 2002; Project Start 01-JUL-1990; Project End 30-NOV-2006 Summary: (provided by applicant): A 20 to 30 percent excess of males among the mentally retarded population is well documented. At least half of the excess is likely due to mutations of X-linked genes. An estimated 30-40 loci are associated with nonsyndromic X-linked mental retardation (XLMR) and one hundred thirty are associated with specific XLMR syndromes. The best known of these is the Fragile X syndrome but it accounts for only a third of XLMR families. Because of the X-linked mode of inheritance and current molecular methodologies, these disorders are especially amenable to study. The hypothesis to be tested is that a full understanding of the genetics and pathogenesis of these disorders will lead to improved diagnosis and (ultimately) therapy. The immediate goal of the study is to identify the causative genes and the genetic pathways leading to XLMR. In addition, we will better define the clinical and neurobehavioral phenotypes of these disorders, each of which presents unique aspects about brain development and function. Over the last ten years, 55 large XLMR families and 68 smaller families have been admitted to the study for gene localization, gene testing and neurobehavioral studies. Thirty-two of the families have been localized to a discrete region of the X chromosome. In addition to these families, 2 males with inversions of the X chromosome associated with mental retardation and 3 males with small deletions are available for molecular studies. We have deposited brains from three XLMR study families are in the Miami Brain Bank and will utilize them for both molecular studies and comprehensive histological analysis. Three new investigators, (Srivastava, Inana, and Warren) have joined the study. A variety of appropriate microarray systems, subtractive cDNA and other appropriate methods (including

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maximal utilization of the new human genorne data) will be used to identify and characterize ten to fifteen XLMR genes over the next five years. We will continue to admit five new families and a number of smaller families each year. Neurobehavioral studies will be closely integrated into the clinical evaluation of each of the new large families. More extensive neurobehavioral studies along with MRI morphometric analysis will be conducted in three XLMR entities: Coffin-Lowry, ATRX and AllanHerndon Syndrome. In summary, this represents a unique study that combines a variety of methodologies and disciplines in order to better understand the role of genes on the X chromosome in brain development and function. Website: http://crisp.cit.nih.gov/crisp/Crisp_Query.Generate_Screen

E-Journals: PubMed Central3 PubMed Central (PMC) is a digital archive of life sciences journal literature developed and managed by the National Center for Biotechnology Information (NCBI) at the U.S. National Library of Medicine (NLM).4 Access to this growing archive of e-journals is free and unrestricted.5 To search, go to http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Pmc, and type “mental retardation” (or synonyms) into the search box. This search gives you access to full-text articles. The following is a sample of items found for mental retardation in the PubMed Central database: •

A highly conserved protein family interacting with the fragile X mental retardation protein (FMRP) and displaying selective interactions with FMRP-related proteins FXR1P and FXR2P. by Schenck A, Bardoni B, Moro A, Bagni C, Mandel JL.; 2001 Jul 17; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=37523

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Altered synaptic plasticity in a mouse model of fragile X mental retardation. by Huber KM, Gallagher SM, Warren ST, Bear MF.; 2002 May 28; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=124340

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Casein Kinase II Phosphorylates the Fragile X Mental Retardation Protein and Modulates Its Biological Properties. by Siomi MC, Higashijima K, Ishizuka A, Siomi H.; 2002 Dec; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=139871

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Characterization of dFMR1, a Drosophila melanogaster Homolog of the Fragile X Mental Retardation Protein. by Wan L, Dockendorff TC, Jongens TA, Dreyfuss G.; 2000 Nov 15; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=102159

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Fragile X mental retardation protein is translated near synapses in response to neurotransmitter activation. by Weiler IJ, Irwin SA, Klintsova AY, Spencer CM, Brazelton AD, Miyashiro K, Comery TA, Patel B, Eberwine J, Greenough WT.; 1997 May 13; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=24689

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Adapted from the National Library of Medicine: http://www.pubmedcentral.nih.gov/about/intro.html.

With PubMed Central, NCBI is taking the lead in preservation and maintenance of open access to electronic literature, just as NLM has done for decades with printed biomedical literature. PubMed Central aims to become a world-class library of the digital age. 5 The value of PubMed Central, in addition to its role as an archive, lies in the availability of data from diverse sources stored in a common format in a single repository. Many journals already have online publishing operations, and there is a growing tendency to publish material online only, to the exclusion of print.

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Increased rates of cerebral glucose metabolism in a mouse model of fragile X mental retardation. by Qin M, Kang J, Smith CB.; 2002 Nov 26; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=137789

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The fragile X mental retardation protein binds specifically to its mRNA via a purine quartet motif. by Schaeffer C, Bardoni B, Mandel JL, Ehresmann B, Ehresmann C, Moine H.; 2001 Sep 3; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=125594

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The fragile X mental retardation protein inhibits translation via interacting with mRNA. by Li Z, Zhang Y, Ku L, Wilkinson KD, Warren ST, Feng Y.; 2001 Jun 1; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=55699

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The Gene for a Recessively Inherited Human Childhood Progressive Epilepsy with Mental Retardation Maps to the Distal Short Arm of Chromosome 8. by Tahvanainen E, Ranta S, Hirvasniemi A, Karila E, Leisti J, Sistonen P, Weissenbach J, Lehesjoki A, dela Chapelle A.; 1994 Jul 19; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&rendertype=abstr act&artid=44380

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The novel Rho-GTPase activating gene MEGAP / srGAP3 has a putative role in severe mental retardation. by Endris V, Wogatzky B, Leimer U, Bartsch D, Zatyka M, Latif F, Maher ER, Tariverdian G, Kirsch S, Karch D, Rappold GA.; 2002 Sep 3; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=129341

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Transport of Fragile X Mental Retardation Protein via Granules in Neurites of PC12 Cells. by De Diego Otero Y, Severijnen LA, van Cappellen G, Schrier M, Oostra B, Willemsen R.; 2002 Dec; http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=134063

The National Library of Medicine: PubMed One of the quickest and most comprehensive ways to find academic studies in both English and other languages is to use PubMed, maintained by the National Library of Medicine.6 The advantage of PubMed over previously mentioned sources is that it covers a greater number of domestic and foreign references. It is also free to use. If the publisher has a Web site that offers full text of its journals, PubMed will provide links to that site, as well as to sites offering other related data. User registration, a subscription fee, or some other type of fee may be required to access the full text of articles in some journals. To generate your own bibliography of studies dealing with mental retardation, simply go to the PubMed Web site at http://www.ncbi.nlm.nih.gov/pubmed. Type “mental retardation” (or synonyms) into the search box, and click “Go.” The following is the type of output you can expect from PubMed for mental retardation (hyperlinks lead to article summaries):

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PubMed was developed by the National Center for Biotechnology Information (NCBI) at the National Library of Medicine (NLM) at the National Institutes of Health (NIH). The PubMed database was developed in conjunction with publishers of biomedical literature as a search tool for accessing literature citations and linking to full-text journal articles at Web sites of participating publishers. Publishers that participate in PubMed supply NLM with their citations electronically prior to or at the time of publication.

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A candidate X-linked mental retardation gene is a component of a new family of histone deacetylase-containing complexes. Author(s): Hakimi MA, Dong Y, Lane WS, Speicher DW, Shiekhattar R. Source: The Journal of Biological Chemistry. 2003 February 28; 278(9): 7234-9. Epub 2002 December 18. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12493763&dopt=Abstract

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A Japanese girl with mental retardation, severe microretrognathia, and brachydactyly: another case of the Gurrieri syndrome. Author(s): Nakane T, Tandou T, Mitsui Y, Hayashibe H, Aihara M, Nakazawa S. Source: American Journal of Medical Genetics. 2003 May 1; 118A(4): 398-401. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12687677&dopt=Abstract

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A new autosomal recessive non-progressive congenital cerebellar ataxia associated with mental retardation, optic atrophy, and skin abnormalities (CAMOS) maps to chromosome 15q24-q26 in a large consanguineous Lebanese Druze Family. Author(s): Delague V, Bareil C, Bouvagnet P, Salem N, Chouery E, Loiselet J, Megarbane A, Claustres M. Source: Neurogenetics. 2002 March; 4(1): 23-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12030328&dopt=Abstract

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A not-so-”new” mental retardation syndrome. Author(s): Chodirker BN, Marles SL, Chudley AE. Source: American Journal of Medical Genetics. 2002 July 22; 111(1): 106; Author Reply 107-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12124746&dopt=Abstract

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A syndromic form of X-linked mental retardation: the Coffin-Lowry syndrome. Author(s): Touraine RL, Zeniou M, Hanauer A. Source: European Journal of Pediatrics. 2002 April; 161(4): 179-87. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12014383&dopt=Abstract

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A variant of Fine-Lubinsky syndrome: a Japanese boy with profound deafness, cataracts, mental retardation, and brachycephaly without craniosynostosis. Author(s): Nakane T, Mizobe N, Hayashibe H, Nakazawa S. Source: Clinical Dysmorphology. 2002 July; 11(3): 195-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12072800&dopt=Abstract

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Access to specialty medical care for children with mental retardation, autism, and other special health care needs. Author(s): Krauss MW, Gulley S, Sciegaj M, Wells N. Source: Mental Retardation. 2003 October; 41(5): 329-39. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12962535&dopt=Abstract

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Advances in molecular cytogenetics for the evaluation of mental retardation. Author(s): Xu J, Chen Z. Source: American Journal of Medical Genetics. 2003 February 15; 117C(1): 15-24. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561054&dopt=Abstract

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Advances in understanding of fragile X pathogenesis and FMRP function, and in identification of X linked mental retardation genes. Author(s): Bardoni B, Mandel JL. Source: Current Opinion in Genetics & Development. 2002 June; 12(3): 284-93. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12076671&dopt=Abstract

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AGTR2 mutations in X-linked mental retardation. Author(s): Vervoort VS, Beachem MA, Edwards PS, Ladd S, Miller KE, de Mollerat X, Clarkson K, DuPont B, Schwartz CE, Stevenson RE, Boyd E, Srivastava AK. Source: Science. 2002 June 28; 296(5577): 2401-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12089445&dopt=Abstract

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Alpha-thalassemia/mental retardation syndrome, X-Linked (ATR-X, MIM #301040, ATR-X/XNP/XH2 gene MIM #300032). Author(s): Villard L, Fontes M. Source: European Journal of Human Genetics : Ejhg. 2002 April; 10(4): 223-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12032728&dopt=Abstract

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Altered synaptic plasticity in a mouse model of fragile X mental retardation. Author(s): Huber KM, Gallagher SM, Warren ST, Bear MF. Source: Proceedings of the National Academy of Sciences of the United States of America. 2002 May 28; 99(11): 7746-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12032354&dopt=Abstract

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Analysis of hospital use for injury among individuals with mental retardation. Author(s): Wang D, McDermott S, Sease T. Source: Injury Control and Safety Promotion. 2002 June; 9(2): 107-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12461837&dopt=Abstract

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Angelman syndrome methylation screening of 15q11-q13 in institutionalized individuals with severe mental retardation. Author(s): Aquino NH, Bastos E, Fonseca LC, Llerena JC Jr. Source: Genetic Testing. 2002 Summer; 6(2): 129-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12215253&dopt=Abstract

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Animal models of mental retardation: from gene to cognitive function. Author(s): Branchi I, Bichler Z, Berger-Sweeney J, Ricceri L. Source: Neuroscience and Biobehavioral Reviews. 2003 January-March; 27(1-2): 141-53. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12732230&dopt=Abstract

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Another family with nonspecific X-linked mental retardation (MRX78) maps to Xp11.4-p11.23. Author(s): de Vries BB, Breedveld GJ, Deelen WH, Breuning MH, Niermeijer MF, Heutink P. Source: American Journal of Medical Genetics. 2002 September 1; 111(4): 443-5. Erratum In: Am J Med Genet. 2002 December 15; 113(4): 391. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12210308&dopt=Abstract

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Asperger's syndrome, X-linked mental retardation (MRX23), and chronic vocal tic disorder. Author(s): Searcy E, Burd L, Kerbeshian J, Stenehjem A, Franceschini LA. Source: Journal of Child Neurology. 2000 October; 15(10): 699-702. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11063087&dopt=Abstract

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Attitudes of community-living staff toward the integration of persons with mental retardation in the community. Author(s): Duvdevany I. Source: International Journal of Rehabilitation Research. Internationale Zeitschrift Fur Rehabilitationsforschung. Revue Internationale De Recherches De Readaptation. 2000 December; 23(4): 303-12. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11192566&dopt=Abstract

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Autism and mental retardation: the genetic relationship and contribution. Author(s): el-Hazmi MA. Source: East Mediterr Health J. 2001 May; 7(3): 536-43. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12690777&dopt=Abstract

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Automatic auditory processing and event-related brain potentials in persons with mental retardation. Author(s): Ikeda K, Okuzumi H, Hayashi A, Hashimoto S, Kanno A. Source: Percept Mot Skills. 2000 December; 91(3 Pt 2): 1145-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11219657&dopt=Abstract

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Behavioral aspects of epilepsy in children with mental retardation. Author(s): Caplan R, Austin JK. Source: Mental Retardation and Developmental Disabilities Research Reviews. 2000; 6(4): 293-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11107194&dopt=Abstract

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Behavioral momentum in computer-presented discriminations in individuals with severe mental retardation. Author(s): Dube WW, McIlvane WJ. Source: J Exp Anal Behav. 2001 January; 75(1): 15-23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11256864&dopt=Abstract

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Behavioral phenotypes in four mental retardation syndromes: fetal alcohol syndrome, Prader-Willi syndrome, fragile X syndrome, and tuberosis sclerosis. Author(s): Steinhausen HC, Von Gontard A, Spohr HL, Hauffa BP, Eiholzer U, Backes M, Willms J, Malin Z. Source: American Journal of Medical Genetics. 2002 September 1; 111(4): 381-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12210296&dopt=Abstract

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Benefit-cost analysis of community residential versus institutional services for adults with severe mental retardation and challenging behaviors. Author(s): Knobbe CA, Carey SP, Rhodes L, Horner RH. Source: Am J Ment Retard. 1995 March; 99(5): 533-41. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7779348&dopt=Abstract

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Benzodiazepine behavioral side effects: review and implications for individuals with mental retardation. Author(s): Kalachnik JE, Hanzel TE, Sevenich R, Harder SR. Source: Am J Ment Retard. 2002 September; 107(5): 376-410. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12186578&dopt=Abstract

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Biology of the fragile X mental retardation protein, an RNA-binding protein. Author(s): Khandjian EW. Source: Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. 1999; 77(4): 331-42. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10546896&dopt=Abstract

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Blepharophimosis, minor facial anomalies, genital anomalies, and mental retardation: report of two sibs with a unique syndrome. Author(s): Nowaczyk MJ, Sutcliffe TL. Source: American Journal of Medical Genetics. 1999 November 5; 87(1): 78-81. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10528253&dopt=Abstract

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Body-rocking and other habits of college students and persons with mental retardation. Author(s): Berkson G, Rafaeli-Mor N, Tarnovsky S. Source: Am J Ment Retard. 1999 March; 104(2): 107-16. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10207574&dopt=Abstract

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Body-rocking in college students and persons with mental retardation: characteristics, stability, and collateral behaviors. Author(s): Berkson G, Andriacchi T. Source: Research in Developmental Disabilities. 2000 January-February; 21(1): 13-29. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10750163&dopt=Abstract

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Bone mineral density and muscle strength in young men with mental retardation (with and without Down syndrome). Author(s): Angelopoulou N, Matziari C, Tsimaras V, Sakadamis A, Souftas V, Mandroukas K. Source: Calcified Tissue International. 2000 March; 66(3): 176-80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10666490&dopt=Abstract

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Brachydactyly and mental retardation: an Albright hereditary osteodystrophy-like syndrome localized to 2q37. Author(s): Wilson LC, Leverton K, Oude Luttikhuis ME, Oley CA, Flint J, Wolstenholme J, Duckett DP, Barrow MA, Leonard JV, Read AP, et al. Source: American Journal of Human Genetics. 1995 February; 56(2): 400-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7847374&dopt=Abstract

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Brachytelephalangy with mental retardation, peculiar face and short stature in two sibs. A new MCA/MR syndrome? Author(s): Megarbane A, Abi Moussa M. Source: Genet Couns. 1997; 8(2): 127-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9219011&dopt=Abstract

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Brain dystrophin, neurogenetics and mental retardation. Author(s): Mehler MF. Source: Brain Research. Brain Research Reviews. 2000 April; 32(1): 277-307. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10751678&dopt=Abstract

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Breakthroughs in molecular and cellular mechanisms underlying X-linked mental retardation. Author(s): Chelly J. Source: Human Molecular Genetics. 1999; 8(10): 1833-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10469834&dopt=Abstract

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Brief report: effects of clozapine on self-injurious behavior of two risperidone nonresponders with mental retardation. Author(s): Hammock R, Levine WR, Schroeder SR. Source: Journal of Autism and Developmental Disorders. 2001 February; 31(1): 109-13. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11439749&dopt=Abstract

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Brief report: gaze behavior and theory of mind abilities in individuals with autism, down syndrome, and mental retardation of unknown etiology. Author(s): Yirmiya N, Pilowsky T, Solomonica-Levi D, Shulman C. Source: Journal of Autism and Developmental Disorders. 1999 August; 29(4): 333-41. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10478733&dopt=Abstract

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Brief report: melatonin facilitates sleep in individuals with mental retardation and insomnia. Author(s): Niederhofer H, Staffen W, Mair A, Pittschieler K. Source: Journal of Autism and Developmental Disorders. 2003 August; 33(4): 469-72. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12959427&dopt=Abstract

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Brief report: reliability and validity of instruments for assessing psychotropic medication effects on self-injurious behavior in mental retardation. Author(s): Schroeder SR, Rojahn J, Reese RM. Source: Journal of Autism and Developmental Disorders. 1997 February; 27(1): 89-102. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9018584&dopt=Abstract

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Brothers and sisters of adults with mental retardation: gendered nature of the sibling relationship. Author(s): Orsmond GI, Seltzer MM. Source: Am J Ment Retard. 2000 November; 105(6): 486-508. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11958202&dopt=Abstract

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Burnout among direct-care staff members of facilities for persons with mental retardation in Japan. Author(s): Ito H, Kurita H, Shiiya J. Source: Mental Retardation. 1999 December; 37(6): 477-81. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10635668&dopt=Abstract

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Casein kinase II phosphorylates the fragile X mental retardation protein and modulates its biological properties. Author(s): Siomi MC, Higashijima K, Ishizuka A, Siomi H. Source: Molecular and Cellular Biology. 2002 December; 22(24): 8438-47. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12446764&dopt=Abstract

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Children with both mental retardation and mental illnesses live in our communities and need dental care. Author(s): Waldman HB, Perlman SP. Source: Asdc J Dent Child. 2001 September-December; 68(5-6): 360-5, 302. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11985201&dopt=Abstract

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Choroidal abnormalities and mental retardation in neurofibromatosis type 1. Author(s): Ruggieri M, Polizzi A. Source: Lancet. 2001 January 27; 357(9252): 311-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11214158&dopt=Abstract

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Chronological age, receptive vocabulary, and syntax comprehension in children and adolescents with mental retardation. Author(s): Facon B, Facon-Bollengier T, Grubar JC. Source: Am J Ment Retard. 2002 March; 107(2): 91-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11853526&dopt=Abstract

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Clinical quiz. Rubinstein-Taybi syndrome (synonyms: broad thumbs and great toes, characteristic facies, and mental retardation -- broad thumb-hallux syndrome). Author(s): Kanitakis J, Claudy A. Source: Eur J Dermatol. 2002 January-February; 12(1): 107, 108-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11809612&dopt=Abstract

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Cognitive and behavioral characteristics in 4 affected males of a family with nonspecific X-linked mental retardation and TM4 SF2-gene mutation. Author(s): De Vos B, Frints S, Borghgraef M, Fryns JP. Source: Genet Couns. 2002; 13(2): 191-4. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12150222&dopt=Abstract

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Cognitive correlates of laterality in mental retardation. Author(s): Di Nuovo S, Buono S. Source: Percept Mot Skills. 2003 April; 96(2): 400-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12776820&dopt=Abstract

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Combination of olanzapine and divalproex for agitation in individuals with profound mental retardation. Author(s): Olson DA, Ingram W, Mann JR. Source: Journal of Clinical Psychopharmacology. 2002 October; 22(5): 529-30. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12352281&dopt=Abstract

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Comorbidity of mental retardation and affective disorders. Author(s): Girimaji SC. Source: J Indian Med Assoc. 2000 May; 98(5): 245, 248-9. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11002623&dopt=Abstract

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Compatibility of housemates with mental retardation. Author(s): Wiltz J, Reiss S. Source: Am J Ment Retard. 2003 May; 108(3): 173-80. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12691596&dopt=Abstract

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Complicated hereditary spastic paraplegia with peripheral neuropathy, optic atrophy and mental retardation. Author(s): Miyama S, Arimoto K, Kimiya S, Tomi H. Source: Neuropediatrics. 2000 August; 31(4): 214-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11071149&dopt=Abstract

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Congenital disorders of glycosylation IIa cause growth retardation, mental retardation, and facial dysmorphism. Author(s): Cormier-Daire V, Amiel J, Vuillaumier-Barrot S, Tan J, Durand G, Munnich A, Le Merrer M, Seta N. Source: Journal of Medical Genetics. 2000 November; 37(11): 875-7. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11228641&dopt=Abstract

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Congenital hypoparathyroidism, associated in this case with mental retardation, seizure disorder, and short stature. Author(s): Aldasouqi SA, Alzahrani AH. Source: Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 2002 January-February; 8(1): 77. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11951813&dopt=Abstract

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Congenital muscular dystrophy associated with calf hypertrophy, microcephaly and severe mental retardation in three Italian families: evidence for a novel CMD syndrome. Author(s): Villanova M, Mercuri E, Bertini E, Sabatelli P, Morandi L, Mora M, Sewry C, Brockington M, Brown SC, Ferreiro A, Maraldi NM, Toda T, Guicheney P, Merlini L, Muntoni F. Source: Neuromuscular Disorders : Nmd. 2000 December; 10(8): 541-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11053679&dopt=Abstract

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Congenital muscular dystrophy with adducted thumbs, ptosis, external ophthalmoplegia, mental retardation and cerebellar hypoplasia: a novel form of CMD. Author(s): Voit T, Parano E, Straub V, Schroder JM, Schaper J, Pavone P, Falsaperla R, Pavone L, Herrmann R. Source: Neuromuscular Disorders : Nmd. 2002 October; 12(7-8): 623-30. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12207929&dopt=Abstract

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Cortical dysplasia, temporal atrophy, mental retardation, dysmorphic facies, and partial epilepsy: an EEG and dynamic susceptibility contrast (DSC) MRI study in a new possible genetic syndrome. Author(s): Romigi A, Silvestri C, Orlandi L, Bozzao A, Placidi F, Tombini M, Sperli F, Izzi F, Curatolo P, Marciani MG. Source: The International Journal of Neuroscience. 2003 March; 113(3): 307-14. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12803135&dopt=Abstract

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Cosegregation of T108A Elk-1 with mental retardation. Author(s): Schroer A, Scheer MP, Zacharias S, Schneider S, Ropers HH, Nothwang HG, Chelly J, Hamel B, Fryns JP, Shaw P, Moraine C. Source: American Journal of Medical Genetics. 2000 December 11; 95(4): 404-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11186900&dopt=Abstract

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Craniofacial anomalies, deafness, brachydactyly, short stature, and moderate mental retardation due to a cryptic 6p;11q translocation. Author(s): Megarbane A, Bejjani BA, Shaffer LG, Jambart S, Souraty N, Kashork CD, Le Merrer M. Source: American Journal of Medical Genetics. 2002 February 15; 108(1): 69-74. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11857553&dopt=Abstract

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Cryptic chromosome rearrangements detected by subtelomere assay in patients with mental retardation and dysmorphic features. Author(s): Dawson AJ, Putnam S, Schultz J, Riordan D, Prasad C, Greenberg CR, Chodirker BN, Mhanni AA, Chudley AE. Source: Clinical Genetics. 2002 December; 62(6): 488-94. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12515261&dopt=Abstract

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Cultural diversity: caring for minority children with mental retardation and other disabilities. Author(s): Waldman HB, Swerdloff M, Perlman SP. Source: Asdc J Dent Child. 2001 July-August; 68(4): 280-5, 229. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11862882&dopt=Abstract

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DC-LD. Diagnostic criteria for psychiatric disorders for use with adults with learning disabilities/mental retardation. Author(s): Royal College of Psychiatrists. Source: Occas Pap R Coll Gen Pract. 2001; (48): I-Xvi, 1-128. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11702594&dopt=Abstract

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De novo double translocation 3;13 and 4;8;18 in a patient with mental retardation and skeletal abnormalities. Author(s): Battisti C, Bonaglia MC, Giglio S, Anichini C, Pucci L, Dotti MT, Zuffardi O, Federico A. Source: American Journal of Medical Genetics. 2003 March 15; 117A(3): 207-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12599183&dopt=Abstract

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De novo MECP2 frameshift mutation in a boy with moderate mental retardation, obesity and gynaecomastia. Author(s): Kleefstra T, Yntema HG, Oudakker AR, Romein T, Sistermans E, Nillessen W, van Bokhoven H, de Vries BB, Hamel BC. Source: Clinical Genetics. 2002 May; 61(5): 359-62. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12081720&dopt=Abstract

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De novo pericentric inversion of chromosome 4, inv(4)(p16q12) in a boy with piebaldism and mental retardation. Author(s): Ramadevi AR, Naik U, Dutta U, Srikanth, Prabhakara K. Source: American Journal of Medical Genetics. 2002 November 22; 113(2): 190-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12407711&dopt=Abstract

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Deinstitutionalization of children with mental retardation: what of dental services? Author(s): Waldman HB, Perlman SP. Source: Asdc J Dent Child. 2000 November-December; 67(6): 413-7, 375. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11204065&dopt=Abstract

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Dendritic anomalies in disorders associated with mental retardation. Author(s): Kaufmann WE, Moser HW. Source: Cerebral Cortex (New York, N.Y. : 1991). 2000 October; 10(10): 981-91. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11007549&dopt=Abstract

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Dendritic cytoskeletal protein expression in mental retardation: an immunohistochemical study of the neocortex in Rett syndrome. Author(s): Kaufmann WE, MacDonald SM, Altamura CR. Source: Cerebral Cortex (New York, N.Y. : 1991). 2000 October; 10(10): 992-1004. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11007550&dopt=Abstract

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Dendritic spine structural anomalies in fragile-X mental retardation syndrome. Author(s): Irwin SA, Galvez R, Greenough WT. Source: Cerebral Cortex (New York, N.Y. : 1991). 2000 October; 10(10): 1038-44. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11007554&dopt=Abstract

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Depression, anxiety, and relevant cognitions in persons with mental retardation. Author(s): Glenn E, Bihm EM, Lammers WJ. Source: Journal of Autism and Developmental Disorders. 2003 February; 33(1): 69-76. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12708581&dopt=Abstract

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Detection of changes in naturalistic scenes: comparisons of individuals with and without mental retardation. Author(s): Carlin MT, Soraci SA, Strawbridge CP, Dennis N, Loiselle R, Chechile NA. Source: Am J Ment Retard. 2003 May; 108(3): 181-93. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12691597&dopt=Abstract

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Detection of preventable heritable disorders leading to mental retardation or physical defects: establishment of genetic disease unit; Unit's duties and responsibilities-objection.on religious grounds. Author(s): California. Source: Health Saf Code Annot State Calif Adopt April 7 1939 Calif. 1977; Section 309 As Amended 1977: Unknown. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12041464&dopt=Abstract

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Detection of submicroscopic aberrations in patients with unexplained mental retardation by fluorescence in situ hybridization using multiple subtelomeric probes. Author(s): Fan YS, Zhang Y, Speevak M, Farrell S, Jung JH, Siu VM. Source: Genetics in Medicine : Official Journal of the American College of Medical Genetics. 2001 November-December; 3(6): 416-21. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11715006&dopt=Abstract

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Diagnostic evaluation of developmental delay/mental retardation: An overview. Author(s): Battaglia A, Carey JC. Source: American Journal of Medical Genetics. 2003 February 15; 117C(1): 3-14. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561053&dopt=Abstract

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Differences in cardiovascular disease risk between nondiabetic adults with mental retardation with and without Down syndrome. Author(s): Draheim CC, McCubbin JA, Williams DP. Source: Am J Ment Retard. 2002 May; 107(3): 201-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11966333&dopt=Abstract

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Direct-care workers' attributions of psychopathology in adults with mental retardation. Author(s): Edelstein TM, Glenwick DS. Source: Mental Retardation. 2001 October; 39(5): 368-78. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11710848&dopt=Abstract

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Disruption of the serine/threonine kinase 9 gene causes severe X-linked infantile spasms and mental retardation. Author(s): Kalscheuer VM, Tao J, Donnelly A, Hollway G, Schwinger E, Kubart S, Menzel C, Hoeltzenbein M, Tommerup N, Eyre H, Harbord M, Haan E, Sutherland GR, Ropers HH, Gecz J. Source: American Journal of Human Genetics. 2003 June; 72(6): 1401-11. Epub 2003 May 07. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12736870&dopt=Abstract

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Dissection of an inverted X(p21.3q27.1) chromosome associated with mental retardation. Author(s): Lepretre F, Delannoy V, Froguel P, Vasseur F, Montpellier C. Source: Cytogenetic and Genome Research. 2003; 101(2): 124-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=14610352&dopt=Abstract

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Do adults with mental retardation show pictorial superiority effects in recall and recognition? Author(s): Cherry KE, Applegate H, Reese CM. Source: Research in Developmental Disabilities. 2002 March-April; 23(2): 135-47. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12061751&dopt=Abstract

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Duplication of (2)(q11.1-q13.2) in a boy with mental retardation and cleft lip and palate: another clefting gene locus on proximal 2q? Author(s): Riegel M, Schinzel A. Source: American Journal of Medical Genetics. 2002 July 22; 111(1): 76-80. Review. Erratum In: Am J Med Genet. 2002 December 1; 113(3): 313. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12124740&dopt=Abstract

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Duplication of chromosome region (16)(p11.2 --> p12.1) in a mother and daughter with mild mental retardation. Author(s): Engelen JJ, de Die-Smulders CE, Dirckx R, Verhoeven WM, Tuinier S, Curfs LM, Hamers AJ. Source: American Journal of Medical Genetics. 2002 April 22; 109(2): 149-53. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11977164&dopt=Abstract

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Early recognition of 1-year-old infants with autism spectrum disorder versus mental retardation. Author(s): Osterling JA, Dawson G, Munson JA. Source: Development and Psychopathology. 2002 Spring; 14(2): 239-51. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12030690&dopt=Abstract

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Early risk factors for mental retardation: role of maternal age and maternal education. Author(s): Chapman DA, Scott KG, Mason CA. Source: Am J Ment Retard. 2002 January; 107(1): 46-59. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11806749&dopt=Abstract

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Early-onset schizophrenia in children with mental retardation: diagnostic reliability and stability of clinical features. Author(s): Lee P, Moss S, Friedlander R, Donnelly T, Honer W. Source: Journal of the American Academy of Child and Adolescent Psychiatry. 2003 February; 42(2): 162-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12544175&dopt=Abstract

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ECT and mental retardation: a review and case reports. Author(s): Aziz M, Maixner DF, DeQuardo J, Aldridge A, Tandon R. Source: The Journal of Ect. 2001 June; 17(2): 149-52. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11417928&dopt=Abstract

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ECT in mental retardation: a review. Author(s): van Waarde JA, Stolker JJ, van der Mast RC. Source: The Journal of Ect. 2001 December; 17(4): 236-43. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11731724&dopt=Abstract

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ECT use delayed in the presence of comorbid mental retardation: a review of clinical and ethical issues. Author(s): Little JD, McFarlane J, Ducharme HM. Source: The Journal of Ect. 2002 December; 18(4): 218-22. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12468999&dopt=Abstract

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ECT: use in individuals with mental retardation. Author(s): Friedlander RI, Solomons K. Source: The Journal of Ect. 2002 March; 18(1): 38-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11925520&dopt=Abstract

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Effect of the deficits of fragile X mental retardation protein on cognitive status of fragile x males and females assessed by robust pedigree analysis. Author(s): Loesch DZ, Huggins RM, Bui QM, Epstein JL, Taylor AK, Hagerman RJ. Source: Journal of Developmental and Behavioral Pediatrics : Jdbp. 2002 December; 23(6): 416-23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12476071&dopt=Abstract

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Effectiveness of video feedback and self-management on inappropriate social behavior of youth with mild mental retardation. Author(s): Embregts PJ. Source: Research in Developmental Disabilities. 2000 September-October; 21(5): 409-23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11100803&dopt=Abstract

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Effects of intelligence level and place of residence on the ability of individuals with mental retardation to identify facial expressions. Author(s): Hetzroni O, Oren B. Source: Research in Developmental Disabilities. 2002 November-December; 23(6): 36978. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12426006&dopt=Abstract

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Effects of living room, Snoezelen room, and outdoor activities on stereotypic behavior and engagement by adults with profound mental retardation. Author(s): Cuvo AJ, May ME, Post TM. Source: Research in Developmental Disabilities. 2001 May-June; 22(3): 183-204. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11380058&dopt=Abstract

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Effects of visual arts instruction on the mental health of adults with mental retardation and mental illness. Author(s): Malley SM, Dattilo J, Gast D. Source: Mental Retardation. 2002 August; 40(4): 278-96. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12123392&dopt=Abstract

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Enhancing free-recall rates of individuals with mental retardation. Author(s): Carlin MT, Soraci SA, Dennis NA, Chechile NA, Loiselle RC. Source: Am J Ment Retard. 2001 July; 106(4): 314-26. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11414872&dopt=Abstract

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Enhancing independent time-management skills of individuals with mental retardation using a Palmtop personal computer. Author(s): Davies DK, Stock SE, Wehmeyer ML. Source: Mental Retardation. 2002 October; 40(5): 358-65. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12215071&dopt=Abstract

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Enhancing the emergency department outcomes of patients with mental retardation. Author(s): Sullivan W, Berg JM, Bradley EA, Brooks-Hill RW, Goldfarb CE, Lovering JS, Lunsky Y, Korosy M, Grossman SA, Hutson HR, Anglin D. Source: Annals of Emergency Medicine. 2000 October; 36(4): 399-400. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11020697&dopt=Abstract

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Equally unequal: gender discrimination in the workplace among adults with mental retardation. Author(s): Julius E, Wolfson H, Yalon-Chamovitz S. Source: Work (Reading, Mass.). 2003; 20(3): 205-13. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12775926&dopt=Abstract

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Establishing mental retardation in capital cases: an update. Author(s): Baroff GS. Source: Mental Retardation. 2003 June; 41(3): 198-202. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12737613&dopt=Abstract

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Expanding phenotype of XNP mutations: mild to moderate mental retardation. Author(s): Yntema HG, Poppelaars FA, Derksen E, Oudakker AR, van Roosmalen T, Jacobs A, Obbema H, Brunner HG, Hamel BC, van Bokhoven H. Source: American Journal of Medical Genetics. 2002 July 1; 110(3): 243-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12116232&dopt=Abstract

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Experiencing undesired daily life events, instrumental functioning, social support and well-being of Israeli elderly women: comparison between caregivers/noncaregivers for adult children with mental retardation. Author(s): Rimmerman A, Muraver M. Source: J Women Aging. 2001; 13(2): 57-69. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11569594&dopt=Abstract

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Eyelid myoclonia with absences in three subjects with mental retardation. Author(s): Scuderi C, Musumeci SA, Ferri R, Calabrese G, Elia M. Source: Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 2000 August; 21(4): 247-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11214665&dopt=Abstract

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FACL4, encoding fatty acid-CoA ligase 4, is mutated in nonspecific X-linked mental retardation. Author(s): Meloni I, Muscettola M, Raynaud M, Longo I, Bruttini M, Moizard MP, Gomot M, Chelly J, des Portes V, Fryns JP, Ropers HH, Magi B, Bellan C, Volpi N, Yntema HG, Lewis SE, Schaffer JE, Renieri A. Source: Nature Genetics. 2002 April; 30(4): 436-40. Epub 2002 March 11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11889465&dopt=Abstract

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Familial cryptic translocation with deletion 4q33-->4qter and duplication 7q34-->7qter in brothers with mental retardation, macrocephaly and iris coloboma. Author(s): Moog U, Engelen JJ, van Schrojenstein Lantman-de Valk HM, Driessen SD, Fryns JP. Source: Clinical Dysmorphology. 2003 January; 12(1): 35-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12514363&dopt=Abstract

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Familial paroxysmal exercise-induced dyskinesia, epilepsy, and mental retardation in a family with autosomal dominant inheritance. Author(s): Perniola T, Margari L, de Iaco MG, Presicci A, Ventura P, Ferrannini E, Illiceto G. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2001 July; 16(4): 724-30. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11481699&dopt=Abstract

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Features of autism in Rett syndrome and severe mental retardation. Author(s): Mount RH, Charman T, Hastings RP, Reilly S, Cass H. Source: Journal of Autism and Developmental Disorders. 2003 August; 33(4): 435-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12959422&dopt=Abstract

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FISH characterisation of dynamic mosaicism involving an inv dup(15) in a patient with mental retardation. Author(s): Cockwell AE, Davalos IP, Rivera HR, Crolla JA. Source: American Journal of Medical Genetics. 2001 November 1; 103(4): 289-94. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11746008&dopt=Abstract

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Floppy eyelid syndrome and mental retardation. Author(s): Boulton JE, Sullivan TJ. Source: Ophthalmology. 2000 November; 107(11): 1989-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11054318&dopt=Abstract

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Floppy eyelid syndrome and mental retardation. Author(s): Mack WP. Source: Ophthalmology. 2001 December; 108(12): 2159-60. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11733249&dopt=Abstract

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Fluvoxamine and aggression in mental retardation. Author(s): La Malfa G, Bertelli M, Conte M. Source: Psychiatric Services (Washington, D.C.). 2001 August; 52(8): 1105. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11474064&dopt=Abstract

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Fragile X mental retardation protein in plasticity and disease. Author(s): Todd PK, Malter JS. Source: Journal of Neuroscience Research. 2002 December 1; 70(5): 623-30. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12424729&dopt=Abstract

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Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function. Author(s): Darnell JC, Jensen KB, Jin P, Brown V, Warren ST, Darnell RB. Source: Cell. 2001 November 16; 107(4): 489-99. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11719189&dopt=Abstract

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Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes. Author(s): Feng Y, Gutekunst CA, Eberhart DE, Yi H, Warren ST, Hersch SM. Source: The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 1997 March 1; 17(5): 1539-47. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9030614&dopt=Abstract

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Fragile X mental retardation: misregulation of protein synthesis in the developing brain? Author(s): Feng Y. Source: Microscopy Research and Technique. 2002 May 1; 57(3): 145-7. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12112449&dopt=Abstract

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Fragile XE-associated familial mental retardation protein 2 (FMR2) acts as a potent transcription activator. Author(s): Hillman MA, Gecz J. Source: Journal of Human Genetics. 2001; 46(5): 251-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11355014&dopt=Abstract

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Frequency of fragile X syndrome among Turkish patients with mental retardation of unknown etiology. Author(s): Tuncbilek E, Alikasifoglu M, Boduroglu K, Aktas D, Anar B. Source: American Journal of Medical Genetics. 1999 May 28; 84(3): 202-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10331591&dopt=Abstract

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From fragile X mental retardation protein to Rac1 GTPase: new insights from Fly CYFIP. Author(s): Billuart P, Chelly J. Source: Neuron. 2003 June 19; 38(6): 843-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12818167&dopt=Abstract

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Frontonasal dysplasia, macroblepharon, eyelid colobomas, ear anomalies, macrostomia, mental retardation, and CNS structural anomalies. A new syndrome? Author(s): Guion-Almeida ML, Richieri-Costa A. Source: Clinical Dysmorphology. 1999 January; 8(1): 1-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10327243&dopt=Abstract

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Functional analysis of problem behavior in children with mental retardation. Author(s): Bosch JJ, Ringdahl J. Source: Mcn. the American Journal of Maternal Child Nursing. 2001 NovemberDecember; 26(6): 307-11; Quiz 312. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11725402&dopt=Abstract

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Functional characterisation of MeCP2 mutations found in male patients with X linked mental retardation. Author(s): Kudo S, Nomura Y, Segawa M, Fujita N, Nakao M, Hammer S, Schanen C, Terai I, Tamura M. Source: Journal of Medical Genetics. 2002 February; 39(2): 132-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11836365&dopt=Abstract

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Functional hemizygosity of PAFAH1B3 due to a PAFAH1B3-CLK2 fusion gene in a female with mental retardation, ataxia and atrophy of the brain. Author(s): Nothwang HG, Kim HG, Aoki J, Geisterfer M, Kubart S, Wegner RD, van Moers A, Ashworth LK, Haaf T, Bell J, Arai H, Tommerup N, Ropers HH, Wirth J. Source: Human Molecular Genetics. 2001 April 1; 10(8): 797-806. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11285245&dopt=Abstract

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Further support for using the dementia rating scale in the assessment of neurocognitive functions of individuals with mental retardation. Author(s): McDaniel WF, McLaughlin T. Source: Clin Neuropsychol. 2000 February; 14(1): 72-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10855061&dopt=Abstract

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Gabapentin for behavioral dyscontrol with mental retardation. Author(s): Bozikas V, Bascialla F, Yulis P, Savvidou I, Karavatos A. Source: The American Journal of Psychiatry. 2001 June; 158(6): 965-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11384914&dopt=Abstract

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Gender differences in the use of linguistic devices by youths with mental retardation: a preliminary analysis. Author(s): Wilkinson KM. Source: Am J Ment Retard. 1999 May; 104(3): 227-35. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10349464&dopt=Abstract

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Gene for apparently nonsyndromic X-linked mental retardation (MRX32) maps to an 18-Mb region of Xp21.2-p22. Author(s): Hane B, Stevenson RE, Arena JF, Lubs HA, Simensen RJ, Schwartz CE. Source: American Journal of Medical Genetics. 1999 July 30; 85(3): 271-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10398242&dopt=Abstract

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Gene for nonspecific X-linked mental retardation (MRX 47) is located in Xq22.3-q24. Author(s): des Portes V, Soufir N, Carrie A, Billuart P, Bienvenu T, Vinet MC, Beldjord C, Ponsot G, Kahn A, Boue J, Chelly J. Source: American Journal of Medical Genetics. 1997 October 31; 72(3): 324-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9332663&dopt=Abstract

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Gene therapy in mental retardation: ethical considerations. Author(s): Fletcher JC. Source: Mental Retardation and Developmental Disabilities Research Reviews. 1995; 1: 7-13. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11660293&dopt=Abstract

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General learning disorder: a new designation for mental retardation. Author(s): Baroff GS. Source: Mental Retardation. 1999 February; 37(1): 68-70. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10028822&dopt=Abstract

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Generalized anxiety disorder in adolescents and young adults with mild mental retardation. Author(s): Masi G, Favilla L, Mucci M. Source: Psychiatry. 2000 Spring; 63(1): 54-64. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10855760&dopt=Abstract

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Generalized identity matching of two-dimensional forms by individuals with moderate to profound mental retardation. Author(s): Saunders KJ, Johnston MD, Tompkins BF, Dutcher DL, Williams DC. Source: Am J Ment Retard. 1997 November; 102(3): 285-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9394137&dopt=Abstract

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Genes on the X chromosome are important in undiagnosed mental retardation. Author(s): Partington M, Mowat D, Einfeld S, Tonge B, Turner G. Source: American Journal of Medical Genetics. 2000 May 1; 92(1): 57-61. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10797424&dopt=Abstract

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Genes responsible for nonspecific mental retardation. Author(s): Castellvi-Bel S, Mila M. Source: Molecular Genetics and Metabolism. 2001 February; 72(2): 104-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11161835&dopt=Abstract

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Genetic effects on human cognition: lessons from the study of mental retardation syndromes. Author(s): Nokelainen P, Flint J. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2002 March; 72(3): 287-96. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11861682&dopt=Abstract

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Genetic influences on mild mental retardation: concepts, findings and research implications. Author(s): Rutter M, Simonoff E, Plomin R. Source: Journal of Biosocial Science. 1996 October; 28(4): 509-26. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8973007&dopt=Abstract

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Genetic localisation of mental retardation with spastic diplegia to the pericentromeric region of the X chromosome: X inactivation in female carriers. Author(s): Martinez F, Tomas M, Millan JM, Fernandez A, Palau F, Prieto F. Source: Journal of Medical Genetics. 1998 April; 35(4): 284-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9598720&dopt=Abstract

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Genetics of mental retardation. Author(s): Chiurazzi P, Oostra BA. Source: Current Opinion in Pediatrics. 2000 December; 12(6): 529-35. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11106270&dopt=Abstract

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Genetics, mental retardation, and the forging of new alliances. Author(s): Finucane B, Haas-Givler B, Simon EW. Source: American Journal of Medical Genetics. 2003 February 15; 117C(1): 66-72. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561060&dopt=Abstract

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Genitopatellar syndrome: a new condition comprising absent patellae, scrotal hypoplasia, renal anomalies, facial dysmorphism, and mental retardation. Author(s): Cormier-Daire V, Chauvet ML, Lyonnet S, Briard ML, Munnich A, Le Merrer M. Source: Journal of Medical Genetics. 2000 July; 37(7): 520-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10882755&dopt=Abstract

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Goodness-of-fit ethic for informed consent to research involving adults with mental retardation and developmental disabilities. Author(s): Fisher CB. Source: Mental Retardation and Developmental Disabilities Research Reviews. 2003; 9(1): 27-31. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12587135&dopt=Abstract

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Growth deficiency, mental retardation and unusual facies. Author(s): Steiner CE, Marques AP. Source: Clinical Dysmorphology. 2000 April; 9(2): 155-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10826636&dopt=Abstract

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Guided visual search in individuals with mental retardation. Author(s): Carlin MT, Soraci SA, Dennis NA, Strawbridge C, Chechile NA. Source: Am J Ment Retard. 2002 July; 107(4): 237-51. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12069643&dopt=Abstract

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Gynecological care for women with mental retardation. Author(s): Prevatt B. Source: Journal of Obstetric, Gynecologic, and Neonatal Nursing : Jognn / Naacog. 1998 May-June; 27(3): 251-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9620816&dopt=Abstract

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Harrdiness and social support as predictors of stress in mothers of typical children, children with autism, and children with mental retardation. Author(s): Weiss MJ. Source: Autism : the International Journal of Research and Practice. 2002 March; 6(1): 115-30. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11918107&dopt=Abstract

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Health care decision-making by adults with mental retardation. Author(s): Cea CD, Fisher CB. Source: Mental Retardation. 2003 April; 41(2): 78-87. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12622524&dopt=Abstract

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Hemizygosity of delta-catenin (CTNND2) is associated with severe mental retardation in cri-du-chat syndrome. Author(s): Medina M, Marinescu RC, Overhauser J, Kosik KS. Source: Genomics. 2000 January 15; 63(2): 157-64. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10673328&dopt=Abstract

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Hepatitis C prevalence in persons with mental retardation. Author(s): Merrick J. Source: Public Health Rev. 1998; 26(4): 311-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10641528&dopt=Abstract

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Hereditary cerebellar ataxia with peripheral neuropathy and mental retardation. Author(s): Tachi N, Kozuka N, Ohya K, Chiba S, Sasaki K. Source: European Neurology. 2000; 43(2): 82-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10686465&dopt=Abstract

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Hereditary motor and sensory neuropathy with absence of large myelinated fibers due to absence of large neurons in dorsal root ganglia and anterior horns, clinically associated with deafness, mental retardation, and epilepsy (HMSN-ADM). Author(s): Muller HD, Mugler M, Ramaekers VT, Schroder JM. Source: Journal of the Peripheral Nervous System : Jpns. 2000 September; 5(3): 147-57. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11442171&dopt=Abstract

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Hereditary spastic paraparesis with distal muscle wasting, microcephaly, mental retardation, arachnodactyly and tremors: new entity? Author(s): Farah S, Sabry MA, al-Shubaili AF, Anim JT, Hussain JM, Montaser MA, Sharfuddin KM. Source: Clinical Neurology and Neurosurgery. 1997 February; 99(1): 66-70. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9107473&dopt=Abstract

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Heteroplasmic mitochondrial DNA 3310 mutation in NADH dehydrogenase subunit 1 associated with type 2 diabetes, hypertrophic cardiomyopathy, and mental retardation in a single patient. Author(s): Hattori Y, Nakajima K, Eizawa T, Ehara T, Koyama M, Hirai T, Fukuda Y, Kinoshita M. Source: Diabetes Care. 2003 March; 26(3): 952-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12610069&dopt=Abstract

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High myopia, and mild mental retardation in three brothers. Author(s): Gardiner CA, Woods CG. Source: Clinical Dysmorphology. 2000 January; 9(1): 43-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10649797&dopt=Abstract

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Hirschsprung disease, mental retardation and dysmorphic facial features in five unrelated children. Author(s): Kaariainen H, Wallgren-Pettersson C, Clarke A, Pihko H, Taskinen H, Rintala R. Source: Clinical Dysmorphology. 2001 July; 10(3): 157-63. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11446406&dopt=Abstract

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Hirschsprung disease, mental retardation, characteristic facial features, and mutation in the gene ZFHX1B (SIP1): confirmation of the Mowat-Wilson syndrome. Author(s): Garavelli L, Donadio A, Zanacca C, Banchini G, Della Giustina E, Bertani G, Albertini G, Del Rossi C, Zweier C, Rauch A, Zollino M, Neri G. Source: American Journal of Medical Genetics. 2003 February 1; 116A(4): 385-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12522797&dopt=Abstract

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Hirschsprung disease, microcephaly, mental retardation, and characteristic facial features: delineation of a new syndrome and identification of a locus at chromosome 2q22-q23. Author(s): Mowat DR, Croaker GD, Cass DT, Kerr BA, Chaitow J, Ades LC, Chia NL, Wilson MJ. Source: Journal of Medical Genetics. 1998 August; 35(8): 617-23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9719364&dopt=Abstract

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History of mental retardation: an essay review. Author(s): Brockley JA. Source: History of Psychology. 1999 February; 2(1): 25-36. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11623617&dopt=Abstract

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History of the roles of nurses caring for persons with mental retardation. Author(s): Nehring WM. Source: Nurs Clin North Am. 2003 June; 38(2): 351-72. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12914312&dopt=Abstract

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Homozygosity mapping in a family with microcephaly, mental retardation, and short stature to a Cohen syndrome region on 8q21.3-8q22.1: redefining a clinical entity. Author(s): Horn D, Krebsova A, Kunze J, Reis A. Source: American Journal of Medical Genetics. 2000 June 5; 92(4): 285-92. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10842298&dopt=Abstract

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How microsatellite analysis can be exploited for subtelomeric chromosomal rearrangement analysis in mental retardation. Author(s): Borgione E, Giudice ML, Galesi O, Castiglia L, Failla P, Romano C, Ragusa A, Fichera M. Source: Journal of Medical Genetics. 2001 January; 38(1): E1. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11134240&dopt=Abstract

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Hypermyelinating neuropathy, mental retardation and epilepsy in a case of merosin deficiency. Author(s): Deodato F, Sabatelli M, Ricci E, Mercuri E, Muntoni F, Sewry C, Naom I, Tonali P, Guzzetta F. Source: Neuromuscular Disorders : Nmd. 2002 May; 12(4): 392-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12062258&dopt=Abstract

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Hypomania in a patient with congenital familial hypothyroidism and mild mental retardation. Author(s): Mahendran R. Source: Singapore Med J. 1999 June; 40(6): 425-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10489513&dopt=Abstract

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Identification of a 650 kb duplication at the X chromosome breakpoint in a patient with 46,X,t(X;8)(q28;q12) and non-syndromic mental retardation. Author(s): Cox JJ, Holden ST, Dee S, Burbridge JI, Raymond FL. Source: Journal of Medical Genetics. 2003 March; 40(3): 169-74. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12624134&dopt=Abstract

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Identification of a family with nonspecific mental retardation (MRX79) with the A140V mutation in the MECP2 gene: is there a need for routine screening? Author(s): Winnepenninckx B, Errijgers V, Hayez-Delatte F, Reyniers E, Frank Kooy R. Source: Human Mutation. 2002 October; 20(4): 249-52. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12325019&dopt=Abstract

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Implicit and explicit learning in young adults with mental retardation. Author(s): Atwell JA, Conners FA, Merrill EC. Source: Am J Ment Retard. 2003 January; 108(1): 56-68. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12475366&dopt=Abstract

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Implicit learning in children and adolescents with mental retardation. Author(s): Vinter A, Detable C. Source: Am J Ment Retard. 2003 March; 108(2): 94-107. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12564942&dopt=Abstract

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Inborn errors of metabolism discovered in Asian department of pediatrics and mental retardation research center. Author(s): Zhang C, Xu K, Dave UP, Wang Y, Matsumoto I. Source: J Chromatogr B Biomed Sci Appl. 2000 September 1; 746(1): 41-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11048739&dopt=Abstract

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Increasing independent decision-making skills of women with mental retardation in simulated interpersonal situations of abuse. Author(s): Khemka I. Source: Am J Ment Retard. 2000 September; 105(5): 387-401. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11008847&dopt=Abstract

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Interaction of the transcription factors USF1, USF2, and alpha -Pal/Nrf-1 with the FMR1 promoter. Implications for Fragile X mental retardation syndrome. Author(s): Kumari D, Usdin K. Source: The Journal of Biological Chemistry. 2001 February 9; 276(6): 4357-64. Epub 2000 October 31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11058604&dopt=Abstract

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Interstitial 6q duplication in an adult male without growth delay or severe mental retardation. Author(s): Cappon SL, Duncan AM, Khalifa MM. Source: Medical Science Monitor : International Medical Journal of Experimental and Clinical Research. 2000 May-June; 6(3): 581-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11208374&dopt=Abstract

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Involuntary sterilization of persons with mental retardation: an ethical analysis. Author(s): Diekema DS. Source: Mental Retardation and Developmental Disabilities Research Reviews. 2003; 9(1): 21-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12587134&dopt=Abstract

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Is mental retardation a defect of synapse structure and function? Author(s): Chechlacz M, Gleeson JG. Source: Pediatric Neurology. 2003 July; 29(1): 11-7. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=13679116&dopt=Abstract

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JAMA patient page. Mental retardation. Author(s): Parmet S. Source: Jama : the Journal of the American Medical Association. 2002 September 25; 288(12): 1548. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12360986&dopt=Abstract

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Job innovation for direct caregivers in the care of persons with mental retardation. A system of personal caregiving. Author(s): Boumans NP, van den Berg AA. Source: Scandinavian Journal of Caring Sciences. 2000; 14(4): 216-23. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12035211&dopt=Abstract

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Keene v. Brigham and Women's Hospital, Inc.: on the value of a life with mental retardation. Author(s): Vitello SJ. Source: Mental Retardation. 2003 October; 41(5): 389-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12964898&dopt=Abstract

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Kidney transplantation in recipients with mental retardation: clinical results in a single-center experience. Author(s): Benedetti E, Asolati M, Dunn T, Walczak DA, Papp P, Bartholomew AM, Smith Y, Washington AW, Pollak R. Source: American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation. 1998 March; 31(3): 509-12. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9506689&dopt=Abstract

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Klinefelter syndrome is a common cause for mental retardation of unknown etiology among prepubertal males. Author(s): Khalifa MM, Struthers JL. Source: Clinical Genetics. 2002 January; 61(1): 49-53. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11903356&dopt=Abstract

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Knowledge on sexual abuse and self-protection skills: a study on female Chinese adolescents with mild mental retardation. Author(s): Tang CS, Lee YK. Source: Child Abuse & Neglect. 1999 March; 23(3): 269-79. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10219945&dopt=Abstract

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Late infantile Hirschsprung disease-mental retardation syndrome with a 3-bp deletion in ZFHX1B. Author(s): Yoneda M, Fujita T, Yamada Y, Yamada K, Fujii A, Inagaki T, Nakagawa H, Shimada A, Kishikawa M, Nagaya M, Azuma T, Kuriyama M, Wakamatsu N. Source: Neurology. 2002 November 26; 59(10): 1637-40. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12451214&dopt=Abstract

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Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase. Author(s): Brunetti-Pierri N, Corso G, Rossi M, Ferrari P, Balli F, Rivasi F, Annunziata I, Ballabio A, Russo AD, Andria G, Parenti G. Source: American Journal of Human Genetics. 2002 October; 71(4): 952-8. Epub 2002 August 20. Erratum In: Am J Hum Genet. 2003 August; 73(2): 445. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12189593&dopt=Abstract

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Lessons from the margins, narrating mental retardation: a review essay. Author(s): Biklen D. Source: Mental Retardation. 2000 October; 38(5): 444-56. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11060985&dopt=Abstract

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Level of Activity in Profound/Severe Mental Retardation (LAPMER): a Rasch-derived scale of disability. Author(s): Tesio L, Valsecchi MR, Sala M, Guzzon P, Battaglia MA. Source: J Appl Meas. 2002; 3(1): 50-84. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11997585&dopt=Abstract

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Linkage analysis in Spanish families with nonspecific X-linked mental retardation: Significant linkage at Xq13-q21. Author(s): Badenas C, Castellvi-Bel S, Volpini V, Jimenez D, Sanchez A, Estivill X, Mila M. Source: American Journal of Medical Genetics. 2001 February 1; 98(4): 343-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11170079&dopt=Abstract

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Liver transplantation and mental retardation. Author(s): Van Vlierberghe H, Colle I, Troisi R, de Hemptinne B, De Vos M. Source: Acta Gastroenterol Belg. 2002 April-June; 65(2): 131-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12148436&dopt=Abstract

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Localization of a gene for nonspecific X-linked mental retardation (MRX 76) to Xp22.3-Xp21.3. Author(s): Kleefstra T, Yntema HG, Oudakker AR, de Vries BB, van Bokhoven H, Hamel BC, Poppelaars FA, Ausems MG. Source: American Journal of Medical Genetics. 2002 July 15; 110(4): 410-1. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12116222&dopt=Abstract

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Longitudinal study on the adaptive and challenging behaviors of deinstitutionalized adults with mental retardation. Author(s): Stancliffe RJ, Hayden MF, Larson SA, Lakin KC. Source: Am J Ment Retard. 2002 July; 107(4): 302-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12069649&dopt=Abstract

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Looking backward, looking forward: mental retardation and the question of equality in the new millennium. Author(s): Smith JD. Source: Mental Retardation. 2000 October; 38(5): 457-9. Erratum In: Ment Retard 2000 December; 38(6): 488. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11060986&dopt=Abstract

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Lymphedema-lymphangiectasia-mental retardation (Hennekam) syndrome: a review. Author(s): Van Balkom ID, Alders M, Allanson J, Bellini C, Frank U, De Jong G, Kolbe I, Lacombe D, Rockson S, Rowe P, Wijburg F, Hennekam RC. Source: American Journal of Medical Genetics. 2002 November 1; 112(4): 412-21. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12376947&dopt=Abstract

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Maladaptive behaviors related to adaptive decline in aging adults with mental retardation. Author(s): Urv TK, Zigman WB, Silverman W. Source: Am J Ment Retard. 2003 September; 108(5): 327-39. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12901708&dopt=Abstract

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Mapping of a new locus for autosomal recessive non-syndromic mental retardation in the chromosomal region 19p13.12-p13.2: further genetic heterogeneity. Author(s): Basel-Vanagaite L, Alkelai A, Straussberg R, Magal N, Inbar D, Mahajna M, Shohat M. Source: Journal of Medical Genetics. 2003 October; 40(10): 729-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=14569116&dopt=Abstract

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Mapping to distal Xq28 of nonspecific X-linked mental retardation MRX72: linkage analysis and clinical findings in a three-generation Sardinian family. Author(s): Russo S, Cogliati F, Cavalleri F, Cassitto MG, Giglioli R, Toniolo D, Casari G, Larizza L. Source: American Journal of Medical Genetics. 2000 October 23; 94(5): 376-82. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11050621&dopt=Abstract

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Mechanisms, models, and mental retardation. Author(s): Fisch GS. Source: American Journal of Medical Genetics. 2000 October 23; 94(5): 372-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11050620&dopt=Abstract

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MECP2 mutation in male patients with non-specific X-linked mental retardation. Author(s): Orrico A, Lam C, Galli L, Dotti MT, Hayek G, Tong SF, Poon PM, Zappella M, Federico A, Sorrentino V. Source: Febs Letters. 2000 September 22; 481(3): 285-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11007980&dopt=Abstract

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Mental retardation, sparse hair, facial dysmorphism with a prominent lower lip, and lipodystrophy. A variant example of Nicolaides-Baraitser syndrome? Author(s): Witters I, Fryns JP. Source: Genet Couns. 2003; 14(2): 245-7. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12872821&dopt=Abstract

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Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts unlinked to the LAMA2, FCMD and MEB loci. Author(s): Talim B, Ferreiro A, Cormand B, Vignier N, Oto A, Gogus S, Cila A, Lehesjoki AE, Pihko H, Guicheney P, Topaloglu H. Source: Neuromuscular Disorders : Nmd. 2000 December; 10(8): 548-52. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11053680&dopt=Abstract

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Microcephaly, cutis verticis gyrata of the scalp, retinitis pigmentosa, cataracts, sensorineural deafness, and mental retardation in two brothers. Author(s): Megarbane A, Waked N, Chouery E, Moglabey YB, Saliba N, Mornet E, Serre JL, Slim R. Source: American Journal of Medical Genetics. 2001 January 22; 98(3): 244-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11169562&dopt=Abstract

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Molecular study of the PAK3 and GDI1 genes in nonsyndromic X-linked mental retardation spanish patients. Author(s): Rife M, Mallolas J, Castellvi-Bel S, Badenas C, Jimenez D, Mila M. Source: American Journal of Medical Genetics. 2000 October 23; 94(5): 389-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11050624&dopt=Abstract

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Mutations in ARHGEF6, encoding a guanine nucleotide exchange factor for Rho GTPases, in patients with X-linked mental retardation. Author(s): Kutsche K, Yntema H, Brandt A, Jantke I, Nothwang HG, Orth U, Boavida MG, David D, Chelly J, Fryns JP, Moraine C, Ropers HH, Hamel BC, van Bokhoven H, Gal A. Source: Nature Genetics. 2000 October; 26(2): 247-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11017088&dopt=Abstract

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National survey of sexual behavior and sexual behavior policies in facilities for individuals with mental retardation/developmental disabilities. Author(s): Gust DA, Wang SA, Grot J, Ransom R, Levine WC. Source: Mental Retardation. 2003 October; 41(5): 365-73. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12962532&dopt=Abstract

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Neuro-cognitive and behavioural aspects in non-specific mental retardation. A proposal for phenotyping new XLMR genes. Author(s): Borghgraef M, Sacco S, Gomot M, De Vos B, Jacobs A, Buret V, Desportes V. Source: Genet Couns. 2002; 13(2): 195-8. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12150223&dopt=Abstract

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Neuroimaging studies in the evaluation of developmental delay/mental retardation. Author(s): Battaglia A. Source: American Journal of Medical Genetics. 2003 February 15; 117C(1): 25-30. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561055&dopt=Abstract

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New mental retardation syndrome associated with ocular colobomas, cleft palate, and genital, skeletal, and craniofacial abnormalities. Author(s): Khalifa MM, Cappon S, Soboleski D, Armstrong D. Source: American Journal of Medical Genetics. 2002 January 22; 107(3): 237-42. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11807906&dopt=Abstract

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Nonconvulsive status epilepticus--a possible cause of mental retardation in patients with Lennox-Gastaut syndrome. Author(s): Hoffmann-Riem M, Diener W, Benninger C, Rating D, Unnebrink K, Stephani U, Ernst HP, Korinthenberg R. Source: Neuropediatrics. 2000 August; 31(4): 169-74. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11071139&dopt=Abstract

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Nonsyndromic mental retardation and cryptogenic epilepsy in women with doublecortin gene mutations. Author(s): Guerrini R, Moro F, Andermann E, Hughes E, D'Agostino D, Carrozzo R, Bernasconi A, Flinter F, Parmeggiani L, Volzone A, Parrini E, Mei D, Jarosz JM, Morris RG, Pratt P, Tortorella G, Dubeau F, Andermann F, Dobyns WB, Das S. Source: Annals of Neurology. 2003 July; 54(1): 30-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12838518&dopt=Abstract

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Non-syndromic mental retardation segregating with an apparently balanced t(1;17) reciprocal translocation through three generations. Author(s): Hussain SZ, Evans AL, Ahmed OA, Jones D, McDermot KD, Svennevik EC, Hastings RJ. Source: American Journal of Medical Genetics. 2000 November 13; 95(2): 99-104. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11078557&dopt=Abstract

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Non-syndromic X-linked mental retardation associated with a missense mutation (P312L) in the FGD1 gene. Author(s): Lebel RR, May M, Pouls S, Lubs HA, Stevenson RE, Schwartz CE. Source: Clinical Genetics. 2002 February; 61(2): 139-45. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11940089&dopt=Abstract

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Nonsyndromic X-linked mental retardation: where are the missing mutations? Author(s): Ropers HH, Hoeltzenbein M, Kalscheuer V, Yntema H, Hamel B, Fryns JP, Chelly J, Partington M, Gecz J, Moraine C. Source: Trends in Genetics : Tig. 2003 June; 19(6): 316-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12801724&dopt=Abstract

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Novel mental retardation-epilepsy syndrome linked to Xp21.1-p11.4. Author(s): Hedera P, Alvarado D, Beydoun A, Fink JK. Source: Annals of Neurology. 2002 January; 51(1): 45-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11782983&dopt=Abstract

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Observations on the use of the ICD-10 guide for mental retardation. Author(s): Einfeld SL, Tonge BJ. Source: Journal of Intellectual Disability Research : Jidr. 1999 October; 43 ( Pt 5): 408-12. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10546965&dopt=Abstract

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Oligophrenin 1 (OPHN1) gene mutation causes syndromic X-linked mental retardation with epilepsy, rostral ventricular enlargement and cerebellar hypoplasia. Author(s): Bergmann C, Zerres K, Senderek J, Rudnik-Schoneborn S, Eggermann T, Hausler M, Mull M, Ramaekers VT. Source: Brain; a Journal of Neurology. 2003 July; 126(Pt 7): 1537-44. Epub 2003 May 21. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12805098&dopt=Abstract

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Oligophrenin 1 encodes a rho-GAP protein involved in X-linked mental retardation. Author(s): Billuart P, Bienvenu T, Ronce N, des Portes V, Vinet MC, Zemni R, Carrie A, Beldjord C, Kahn A, Moraine C, Chelly J. Source: Pathologie-Biologie. 1998 November; 46(9): 678. English, French. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9885813&dopt=Abstract

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On the cause of mental retardation in Down syndrome: extrapolation from full and segmental trisomy 16 mouse models. Author(s): Galdzicki Z, Siarey R, Pearce R, Stoll J, Rapoport SI. Source: Brain Research. Brain Research Reviews. 2001 April; 35(2): 115-45. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11336779&dopt=Abstract

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Oral health profile in an institutionalized population of Italian adults with mental retardation. Author(s): Pregliasco F, Ottolina P, Mensi C, Carmagnola D, Giussani F, Abati S, Strohmenger L. Source: Spec Care Dentist. 2001 November-December; 21(6): 227-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11885672&dopt=Abstract

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Orthodontic treatment of a patient with multiple supernumerary teeth and mental retardation. Author(s): Taner T, Uzamis M. Source: J Clin Pediatr Dent. 1999 Spring; 23(3): 195-200. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10686866&dopt=Abstract

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Osteopetrosis, renal tubular acidosis without urinary concentration abnormality, cerebral calcification and severe mental retardation in three Turkish brothers. Author(s): Ocal G, Berberoglu M, Adiyaman P, Cetinkaya E, Ekim M, Aycan Z, Evliyaoglu O. Source: J Pediatr Endocrinol Metab. 2001 November-December; 14(9): 1671-7. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11795660&dopt=Abstract

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Outcome of the routine assessment of patients with mental retardation in a genetics clinic. Author(s): Hunter AG. Source: American Journal of Medical Genetics. 2000 January 3; 90(1): 60-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10602119&dopt=Abstract

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Outcomes of digital X-ray mini-panel examinations for patients having mental retardation and developmental disability. Author(s): Farman AG, Horsley B, Warr E, Ianke JL, Hood H. Source: Dento Maxillo Facial Radiology. 2003 January; 32(1): 15-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12820848&dopt=Abstract

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Overview of the Mental Retardation Developmental Disabilities Research Center (MRDDRC) at Kennedy Krieger Institute (Johns Hopkins University School of Medicine). Author(s): Denckla M. Source: International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience. 2002 June-August; 20(3-5): 335. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12175870&dopt=Abstract

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Participation of women as authors and editors in journals concerned with mental retardation and related topics. Author(s): Porter CL, Christian L, Poling A. Source: Mental Retardation. 2003 February; 41(1): 1-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12597719&dopt=Abstract

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Penis agenesis associated with mental retardation: a case report. Author(s): Kargi E, Akoz T, Tuncel A, Erdogan B, Mungan A. Source: International Urology and Nephrology. 2002; 34(1): 109-11. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12549651&dopt=Abstract

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Poor physical fitness of adolescents with mental retardation at Rajanukul School, Bangkok. Author(s): Chaiwanichsiri D, Sanguanrungsirikul S, Suwannakul W. Source: J Med Assoc Thai. 2000 November; 83(11): 1387-92. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11215871&dopt=Abstract

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Preclinical Cushing's syndrome presenting with isolated adrenocorticotropin (ACTH) deficiency-like manifestations and severe hypoalbuminemia without overt adrenal masses in a patient with Chilaiditi syndrome and mental retardation. Author(s): Ikeda K, Mizuguchi M, Ebisawa T, Yoshida M, Uchida H, Okabe H, Sekita T, Tojo K, Tajima N, Hosoya T. Source: Intern Med. 2003 May; 42(5): 410-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12793711&dopt=Abstract

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Preference for unreliable reinforcement in children with mental retardation: the role of conditioned reinforcement. Author(s): Lalli JS, Mauro BC, Mace FC. Source: J Appl Behav Anal. 2000 Winter; 33(4): 533-44. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11214029&dopt=Abstract

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Progressive cerebellocerebral atrophy: a new syndrome with microcephaly, mental retardation, and spastic quadriplegia. Author(s): Ben-Zeev B, Hoffman C, Lev D, Watemberg N, Malinger G, Brand N, Lerman-Sagie T. Source: Journal of Medical Genetics. 2003 August; 40(8): E96. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12920088&dopt=Abstract

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Psychiatric services for people with mental retardation. Author(s): Chaplin R. Source: Psychiatric Services (Washington, D.C.). 2003 March; 54(3): 403; Author Reply 403. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12610255&dopt=Abstract

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Psychometric properties of the Beck Depression Inventory and the Zung Self Rating Depression Scale in adults with mental retardation. Author(s): Powell R. Source: Mental Retardation. 2003 April; 41(2): 88-95. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12622525&dopt=Abstract

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Psychopharmacology research for individuals with mental retardation: methodological issues and suggestions. Author(s): Matson JL, Bielecki J, Mayville SB, Matson ML. Source: Research in Developmental Disabilities. 2003 May-June; 24(3): 149-57. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12742384&dopt=Abstract

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Psychotherapy with persons who have mental retardation: an evaluation of effectiveness. Author(s): Prout HT, Nowak-Drabik KM. Source: Am J Ment Retard. 2003 March; 108(2): 82-93. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12564941&dopt=Abstract

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Quality of life for young adults with mental retardation during transition. Author(s): Kraemer BR, McIntyre LL, Blacher J. Source: Mental Retardation. 2003 August; 41(4): 250-62. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12862511&dopt=Abstract

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Quality of life of adults with mental retardation/developmental disabilities who live with family. Author(s): Seltzer MM, Krauss MW. Source: Mental Retardation and Developmental Disabilities Research Reviews. 2001; 7(2): 105-14. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11389565&dopt=Abstract

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Quantitative assessments of sensitivity to reinforcement contingencies in mental retardation. Author(s): Dube WV, McIlvane WJ. Source: Am J Ment Retard. 2002 March; 107(2): 136-45. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11853531&dopt=Abstract

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Rare polymorphic variants of the AGTR2 gene in boys with non-specific mental retardation. Author(s): Bienvenu T, Poirier K, Van Esch H, Hamel B, Moraine C, Fryns JP, Ropers HH, Beldjord C, Yntema HG, Chelly J. Source: Journal of Medical Genetics. 2003 May; 40(5): 357-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12746399&dopt=Abstract

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Rate-decreasing effects of the atypical neuroleptic risperidone attenuated by conditions of reinforcement in a woman with mental retardation. Author(s): Yoo JH, Williams DC, Napolitano DA, Peyton RT, Baer DM, Schroeder SR. Source: J Appl Behav Anal. 2003 Summer; 36(2): 245-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12858988&dopt=Abstract

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Recombinative generalization of within-syllable units in nonreading adults with mental retardation. Author(s): Saunders KJ, O'Donnell J, Vaidya M, Williams DC. Source: J Appl Behav Anal. 2003 Spring; 36(1): 95-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12723870&dopt=Abstract

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Representation of international authorship across prominent journals in the field of mental retardation. Author(s): Matson ML, Matso JL, Lott JD, Logan JR. Source: Research in Developmental Disabilities. 2002 July-August; 23(4): 293-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12365853&dopt=Abstract

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Research brief: some data concerning the reporting of participants' gender in the mental retardation literature. Author(s): Porter CL, Christian L, Poling A. Source: Mental Retardation. 2003 April; 41(2): 75-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12622523&dopt=Abstract

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Research in mental retardation: toward an etiologic approach. Author(s): Dykens EM, Hodapp RM. Source: Journal of Child Psychology and Psychiatry, and Allied Disciplines. 2001 January; 42(1): 49-71. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11205624&dopt=Abstract

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Restoration of competency to stand trial: a training program for persons with mental retardation. Author(s): Wall BW, Krupp BH, Guilmette T. Source: J Am Acad Psychiatry Law. 2003; 31(2): 189-201. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12875497&dopt=Abstract

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Rho proteins and the cellular mechanisms of mental retardation. Author(s): Ramakers GJ. Source: American Journal of Medical Genetics. 2000 October 23; 94(5): 367-71. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11050619&dopt=Abstract

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Risk of mental retardation among children born with birth defects. Author(s): Jelliffe-Pawlowski LL, Shaw GM, Nelson V, Harris JA. Source: Archives of Pediatrics & Adolescent Medicine. 2003 June; 157(6): 545-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12796234&dopt=Abstract

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Rose F. Kennedy Center for Research in Mental Retardation and Developmental Disabilities. Author(s): Faber DS. Source: International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience. 2002 June-August; 20(3-5): 36971. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12175876&dopt=Abstract

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Screening adolescents with mental retardation for depression. Author(s): Ailey SH. Source: J Sch Nurs. 2000 February; 16(1): 6-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11033671&dopt=Abstract

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Screening for cryptic chromosomal abnormalities in patients with mental retardation and dysmorphic facial features using telomere FISH probes. Author(s): Hulley BJ, Hummel M, Wenger SL. Source: American Journal of Medical Genetics. 2003 March 15; 117A(3): 302-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12599198&dopt=Abstract

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Self-determination and mental retardation: is there an association with living arrangement and lifestyle satisfaction? Author(s): Duvdevany I, Ben-Zur H, Ambar A. Source: Mental Retardation. 2002 October; 40(5): 379-89. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12215073&dopt=Abstract

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Shaping exhale durations for breath CO detection for men with mild mental retardation. Author(s): Rea J, Williams D. Source: J Appl Behav Anal. 2002 Winter; 35(4): 415-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12555914&dopt=Abstract

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Soles of the Feet: a mindfulness-based self-control intervention for aggression by an individual with mild mental retardation and mental illness. Author(s): Singh NN, Wahler RG, Adkins AD, Myers RE; Mindfulness Research Group. Source: Research in Developmental Disabilities. 2003 May-June; 24(3): 158-69. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12742385&dopt=Abstract

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Some problems in the genetics of X-linked mental retardation. Author(s): Tariverdian G, Vogel F. Source: Cytogenetics and Cell Genetics. 2000; 91(1-4): 278-84. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11173869&dopt=Abstract

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Splicing error in E1alpha pyruvate dehydrogenase mRNA caused by novel intronic mutation responsible for lactic acidosis and mental retardation. Author(s): Mine M, Brivet M, Touati G, Grabowski P, Abitbol M, Marsac C. Source: The Journal of Biological Chemistry. 2003 April 4; 278(14): 11768-72. Epub 2003 January 27. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12551913&dopt=Abstract

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Studies on prenatal infections in children with unknown cause of mental retardation and examination of their mothers. Author(s): Amrei MA, Al-Hamshary AM, Fotoh OA, Abdel-Rahman S. Source: J Egypt Soc Parasitol. 1999; 29(1): 59-67. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561883&dopt=Abstract

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Survey of nursing and medical profile prior to deinstitutionalization of a population with profound mental retardation. Author(s): Kozma C, Mason S. Source: Clinical Nursing Research. 2003 February; 12(1): 8-22; Discussion 23-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12583497&dopt=Abstract

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Syndromic form of X-linked mental retardation with marked hypotonia in early life, severe mental handicap, and difficult adult behavior maps to Xp22. Author(s): Turner G, Gedeon A, Kerr B, Bennett R, Mulley J, Partington M. Source: American Journal of Medical Genetics. 2003 March 15; 117A(3): 245-50. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12599187&dopt=Abstract

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The assessment of mood in adults who have severe or profound mental retardation. Author(s): Ross E, Oliver C. Source: Clinical Psychology Review. 2003 March; 23(2): 225-45. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12573671&dopt=Abstract

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The behavioral profile of severe mental retardation in a genetic mouse model of phenylketonuria. Author(s): Cabib S, Pascucci T, Ventura R, Romano V, Puglisi-Allegra S. Source: Behavior Genetics. 2003 May; 33(3): 301-10. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12837019&dopt=Abstract

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The differentially expressed C21orf5 gene in the medial temporal-lobe system could play a role in mental retardation in Down syndrome and transgenic mice. Author(s): Lopes C, Chettouh Z, Delabar JM, Rachidi M. Source: Biochemical and Biophysical Research Communications. 2003 June 13; 305(4): 915-24. Erratum In: Biochem Biophys Res Commun. 2003 August 1; 307(3): 758. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12767918&dopt=Abstract

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The effect of polyol-combinant saliva stimulants on S. mutans levels in plaque and saliva of patients with mental retardation. Author(s): Makinen KK, Isotupa KP, Kivilompolo T, Makinen PL, Murtomaa S, Petaja J, Toivanen J, Soderling E. Source: Spec Care Dentist. 2002 September-October; 22(5): 187-93. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12580357&dopt=Abstract

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The effects of a vitamin supplement on the pica of a child with severe mental retardation. Author(s): Pace GM, Toyer EA. Source: J Appl Behav Anal. 2000 Winter; 33(4): 619-22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11214036&dopt=Abstract

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The fragile X mental retardation protein binds and regulates a novel class of mRNAs containing U rich target sequences. Author(s): Chen L, Yun SW, Seto J, Liu W, Toth M. Source: Neuroscience. 2003; 120(4): 1005-17. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12927206&dopt=Abstract

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The fragile X mental retardation protein FMRP binds elongation factor 1A mRNA and negatively regulates its translation in vivo. Author(s): Sung YJ, Dolzhanskaya N, Nolin SL, Brown T, Currie JR, Denman RB. Source: The Journal of Biological Chemistry. 2003 May 2; 278(18): 15669-78. Epub 2003 February 19. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12594214&dopt=Abstract

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The N-terminus of the fragile X mental retardation protein contains a novel domain involved in dimerization and RNA binding. Author(s): Adinolfi S, Ramos A, Martin SR, Dal Piaz F, Pucci P, Bardoni B, Mandel JL, Pastore A. Source: Biochemistry. 2003 September 9; 42(35): 10437-44. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12950170&dopt=Abstract

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Toward a uniform standard for mental retardation in death penalty cases. Author(s): Coleman P, Shellow RA. Source: Mental Retardation. 2003 June; 41(3): 203-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12737614&dopt=Abstract

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Translational roots for mental retardation? Author(s): Barinaga M. Source: Science. 2000 October 27; 290(5492): 737. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11184206&dopt=Abstract

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UDPgalactose epimerase in lens and fibroblasts: activity expression in patients with cataracts and mental retardation. Author(s): Shin YS, Korenke GC, Huppke P, Knerr I, Podskarbi T. Source: Journal of Inherited Metabolic Disease. 2000 June; 23(4): 383-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10896300&dopt=Abstract

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Understanding mental retardation in Down's syndrome using trisomy 16 mouse models. Author(s): Galdzicki Z, Siarey RJ. Source: Genes, Brain, and Behavior. 2003 June; 2(3): 167-78. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12931790&dopt=Abstract

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Understanding the siblings of children with mental retardation. Author(s): Waldman HB, Swerdloff M, Perlman SP. Source: Asdc J Dent Child. 2000 September-October; 67(5): 345-9. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11068667&dopt=Abstract

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University of Alabama at Birmingham Mental Retardation Research Center. Author(s): Friedlander MJ. Source: International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience. 2002 June-August; 20(3-5): 135-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12175850&dopt=Abstract

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Unusual chromosomal mosaicism as a cause of mental retardation and congenital malformations in a familial reciprocal translocation carrier, t(17;22)(q24.2;q11.23). Author(s): Dufke A, Mayrhofer H, Enders H, Kaiser P, Leipoldt M. Source: Cytogenetics and Cell Genetics. 2001; 93(3-4): 168-70. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11528107&dopt=Abstract

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Urinary tract infections during pregnancy and mental retardation and developmental delay. Author(s): McDermott S, Callaghan W, Szwejbka L, Mann H, Daguise V. Source: Obstetrics and Gynecology. 2000 July; 96(1): 113-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10862853&dopt=Abstract

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US Supreme Court rules that execution of defendants with mental retardation is unconstitutional. Author(s): Regan J, Alderson A. Source: Tenn Med. 2002 September; 95(9): 379-81. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12222149&dopt=Abstract

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Use of primed in situ labeling (PRINS) for the detection of telomeric deletions associated with mental retardation. Author(s): Bonifacio S, Centrone C, Da Prato L, Scordo MR, Estienne M, Torricelli F. Source: Cytogenetics and Cell Genetics. 2001; 93(1-2): 16-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11474170&dopt=Abstract

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Use of visual tools to report sexual abuse for adults with mental retardation. Author(s): Valenti-Hein D. Source: Mental Retardation. 2002 August; 40(4): 297-303. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12123393&dopt=Abstract

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Utility of the Vineland Adaptive Behavior Scales in diagnosis and research with adults who have mental retardation. Author(s): Beail N. Source: Mental Retardation. 2003 August; 41(4): 286-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12862514&dopt=Abstract

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Validation of the Complete Minnesota Dexterity Test for adults with moderate graded mental retardation in Hong Kong. Author(s): Lee AT, Tsang HW. Source: International Journal of Rehabilitation Research. Internationale Zeitschrift Fur Rehabilitationsforschung. Revue Internationale De Recherches De Readaptation. 2001 June; 24(2): 153-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11421393&dopt=Abstract

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Validity of a modified aerobic fitness test for adults with mental retardation. Author(s): Draheim CC, Laurie NE, McCubbin JA, Perkins JL. Source: Medicine and Science in Sports and Exercise. 1999 December; 31(12): 1849-54. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10613439&dopt=Abstract

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Validity of the Stanford-Binet Intelligence Scale-IV: its use in young adults with mental retardation. Author(s): Nelson WM 3rd, Dacey CM. Source: Mental Retardation. 1999 August; 37(4): 319-25. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10463026&dopt=Abstract

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Van der Woude syndrome with mental retardation: case report. Author(s): Ugwu BT, Momoh JT. Source: East Afr Med J. 2001 February; 78(2): 111-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11682943&dopt=Abstract

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Variability of stereotypic body-rocking in adults with mental retardation. Author(s): Newell KM, Incledon T, Bodfish JW, Sprague RL. Source: Am J Ment Retard. 1999 May; 104(3): 279-88. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10349469&dopt=Abstract

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Variable expression of mental retardation, autism, seizures, and dystonic hand movements in two families with an identical ARX gene mutation. Author(s): Turner G, Partington M, Kerr B, Mangelsdorf M, Gecz J. Source: American Journal of Medical Genetics. 2002 November 1; 112(4): 405-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12376946&dopt=Abstract

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Varieties of repetitive behavior in autism: comparisons to mental retardation. Author(s): Bodfish JW, Symons FJ, Parker DE, Lewis MH. Source: Journal of Autism and Developmental Disorders. 2000 June; 30(3): 237-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11055459&dopt=Abstract

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Ventricular enlargement in adults with profound mental retardation who demonstrate violent/destructive behaviors. Author(s): May PB Jr, DeMarco K, London EB, Thompson R, Mento TL, Buscemi L, Cody R. Source: The Journal of Neuropsychiatry and Clinical Neurosciences. 2001 Winter; 13(1): 96-100. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11207335&dopt=Abstract

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Video-EEG in the diagnosis of paroxysmal events in children with mental retardation and in children with normal intelligence. Author(s): Thirumalai S, Abou-Khalil B, Fakhoury T, Suresh G. Source: Developmental Medicine and Child Neurology. 2001 November; 43(11): 731-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11730145&dopt=Abstract

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Visual preferences of students with profound mental retardation and healthy, fullterm infants. Author(s): Buhrow M, Bradley-Johnson S. Source: Research in Developmental Disabilities. 2003 March-April; 24(2): 83-94. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12623079&dopt=Abstract

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Weight gain in a controlled study of risperidone in children, adolescents and adults with mental retardation and autism. Author(s): Hellings JA, Zarcone JR, Crandall K, Wallace D, Schroeder SR. Source: Journal of Child and Adolescent Psychopharmacology. 2001 Fall; 11(3): 229-38. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11642473&dopt=Abstract

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Weight gain with risperidone among patients with mental retardation: effect of calorie restriction. Author(s): Cohen S, Glazewski R, Khan S, Khan A. Source: The Journal of Clinical Psychiatry. 2001 February; 62(2): 114-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11247096&dopt=Abstract

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Weight, lipids, glucose, and behavioral measures with ziprasidone treatment in a population with mental retardation. Author(s): Cohen S, Fitzgerald B, Okos A, Khan S, Khan A. Source: The Journal of Clinical Psychiatry. 2003 January; 64(1): 60-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12590625&dopt=Abstract

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What about dental care for people with mental retardation? A commentary. Author(s): Waldman HB, Perlman SP. Source: J Am Coll Dent. 2002 Spring; 69(2): 35-8. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12132257&dopt=Abstract

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What individual, provider, and community characteristics predict employment of individuals with mental retardation? Author(s): McDermott S, Martin M, Butkus S. Source: Am J Ment Retard. 1999 July; 104(4): 346-55. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10450461&dopt=Abstract

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White-matter alterations and callosal abnormalities in syndromic patients with mental retardation. Author(s): Gabrielli O, Bruni S, Coppa GV, Carloni I, Polonara G, Regnicolo L, Salvolini S, Salvolini U. Source: Journal of Child Neurology. 2002 March; 17(3): 164-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12026229&dopt=Abstract

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Why and how to assess the aetiological diagnosis of children with intellectual disability/mental retardation and other neurodevelopmental disorders: description of the Finnish approach. Author(s): Wilska ML, Kaski MK. Source: European Journal of Paediatric Neurology : Ejpn : Official Journal of the European Paediatric Neurology Society. 2001; 5(1): 7-13. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11277365&dopt=Abstract

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Why is providing dental care to people with mental retardation and other developmental disabilities such a low priority? Author(s): Waldman HB, Perlman SP. Source: Public Health Reports (Washington, D.C. : 1974). 2002 September-October; 117(5): 435-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12500959&dopt=Abstract

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Word identification in adults with mild mental retardation: does IQ influence reading achievement? Author(s): Cohen D, Riviere JP, Plaza M, Thompson C, Chauvin D, Hambourg N, Lanthier O, Mazet P, Flament M. Source: Brain and Cognition. 2001 June-July; 46(1-2): 69-73. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11527367&dopt=Abstract

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Working with people who have mental retardation. Author(s): Kuehn JL. Source: Psychiatric Services (Washington, D.C.). 2003 June; 54(6): 911. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12773613&dopt=Abstract

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X linked severe mental retardation, craniofacial dysmorphology, epilepsy, ophthalmoplegia, and cerebellar atrophy in a large South African kindred is localised to Xq24-q27. Author(s): Christianson AL, Stevenson RE, van der Meyden CH, Pelser J, Theron FW, van Rensburg PL, Chandler M, Schwartz CE. Source: Journal of Medical Genetics. 1999 October; 36(10): 759-66. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10528855&dopt=Abstract

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X-linked mental retardation and epilepsy: pathogenetic significance of ARX mutations. Author(s): Hirose S, Mitsudome A. Source: Brain & Development. 2003 April; 25(3): 161-5. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12689693&dopt=Abstract

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X-linked mental retardation with fragile X. A pedigree showing transmission by apparently unaffected males and partial expression in female carriers. Author(s): Nielsen KB, Tommerup N, Poulsen H, Mikkelsen M. Source: Human Genetics. 1981; 59(1): 23-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10819017&dopt=Abstract

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X-linked mental retardation with seizures and carrier manifestations is caused by a mutation in the creatine-transporter gene (SLC6A8) located in Xq28. Author(s): Hahn KA, Salomons GS, Tackels-Horne D, Wood TC, Taylor HA, Schroer RJ, Lubs HA, Jakobs C, Olson RL, Holden KR, Stevenson RE, Schwartz CE. Source: American Journal of Human Genetics. 2002 May; 70(5): 1349-56. Epub 2002 March 15. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11898126&dopt=Abstract

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X-linked mental retardation. Author(s): Neri G, Chiurazzi P. Source: Adv Genet. 1999; 41: 55-94. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10494617&dopt=Abstract

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X-linked mental retardation: evidence for a recent mutation in a five-generation family (MRX65) linked to the pericentromeric region. Author(s): Yntema HG, van den Helm B, Knoers NV, Smits AP, van Roosmalen T, Smeets DF, Mariman EC, van der Burgt I, van Bokhoven H, Ropers HH, Kremer H, Hamel BC. Source: American Journal of Medical Genetics. 1999 July 30; 85(3): 305-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10398247&dopt=Abstract

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X-linked mental retardation: vanishing boundaries between non-specific (MRX) and syndromic (MRXS) forms. Author(s): Frints SG, Froyen G, Marynen P, Fryns JP. Source: Clinical Genetics. 2002 December; 62(6): 423-32. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12485186&dopt=Abstract

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X-linked nonspecific mental retardation (MRX) linkage studies in 25 unrelated families: the European XLMR consortium. Author(s): des Portes V, Beldjord C, Chelly J, Hamel B, Kremer H, Smits A, van Bokhoven H, Ropers HH, Claes S, Fryns JP, Ronce N, Gendrot C, Toutain A, Raynaud M, Moraine C. Source: American Journal of Medical Genetics. 1999 July 30; 85(3): 263-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10398240&dopt=Abstract

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X-linked nonspecific mental retardation (MRX16) mapping to distal Xq28: linkage study and neuropsychological data in a large family. Author(s): Gendrot C, Ronce N, Raynaud M, Ayrault AD, Dourlens J, Castelnau P, Muh JP, Chelly J, Moraine C. Source: American Journal of Medical Genetics. 1999 April 23; 83(5): 411-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10232754&dopt=Abstract

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X-linked non-specific mental retardation. Author(s): Toniolo D, D'Adamo P. Source: Current Opinion in Genetics & Development. 2000 June; 10(3): 280-5. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10826988&dopt=Abstract

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Yeast artificial chromosome cloning of the Xq13.3-q21.31 region and fine mapping of a deletion associated with choroideremia and nonspecific mental retardation. Author(s): van der Maarel SM, Scholten IH, Maat-Kievit JA, Huber I, de Kok YJ, de Wijs I, van de Pol TJ, van Bokhoven H, den Dunnen JT, van Ommen GJ, et al. Source: European Journal of Human Genetics : Ejhg. 1995; 3(4): 207-18. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8528669&dopt=Abstract

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You may be treating children with mental retardation and attention deficit hyperactive disorder in your dental practice. Author(s): Waldman HB, Swerdloff M, Perlman SP. Source: Asdc J Dent Child. 2000 July-August; 67(4): 241-5, 231. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10997238&dopt=Abstract

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Young-Simpson syndrome: further delineation of a distinct syndrome with congenital hypothyroidism, congenital heart defects, facial dysmorphism, and mental retardation. Author(s): Masuno M, Imaizumi K, Okada T, Adachi M, Nishimura G, Ishii T, Tachibana K, Kuroki Y. Source: American Journal of Medical Genetics. 1999 May 7; 84(1): 8-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10213038&dopt=Abstract

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CHAPTER 2. NUTRITION AND MENTAL RETARDATION Overview In this chapter, we will show you how to find studies dedicated specifically to nutrition and mental retardation.

Finding Nutrition Studies on Mental Retardation The National Institutes of Health’s Office of Dietary Supplements (ODS) offers a searchable bibliographic database called the IBIDS (International Bibliographic Information on Dietary Supplements; National Institutes of Health, Building 31, Room 1B29, 31 Center Drive, MSC 2086, Bethesda, Maryland 20892-2086, Tel: 301-435-2920, Fax: 301-480-1845, E-mail: [email protected]). The IBIDS contains over 460,000 scientific citations and summaries about dietary supplements and nutrition as well as references to published international, scientific literature on dietary supplements such as vitamins, minerals, and botanicals.7 The IBIDS includes references and citations to both human and animal research studies. As a service of the ODS, access to the IBIDS database is available free of charge at the following Web address: http://ods.od.nih.gov/databases/ibids.html. After entering the search area, you have three choices: (1) IBIDS Consumer Database, (2) Full IBIDS Database, or (3) Peer Reviewed Citations Only. Now that you have selected a database, click on the “Advanced” tab. An advanced search allows you to retrieve up to 100 fully explained references in a comprehensive format. Type “mental retardation” (or synonyms) into the search box, and click “Go.” To narrow the search, you can also select the “Title” field.

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Adapted from http://ods.od.nih.gov. IBIDS is produced by the Office of Dietary Supplements (ODS) at the National Institutes of Health to assist the public, healthcare providers, educators, and researchers in locating credible, scientific information on dietary supplements. IBIDS was developed and will be maintained through an interagency partnership with the Food and Nutrition Information Center of the National Agricultural Library, U.S. Department of Agriculture.

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The following information is typical of that found when using the “Full IBIDS Database” to search for “mental retardation” (or a synonym): •

Activity-dependent regulation of genes implicated in X-linked non-specific mental retardation. Author(s): Neuropharmacology and Department of Morphology, Centre Medical Universitaire, 1 rue Michel Servet, 1211, Geneva 4, Switzerland. [email protected] Source: Boda, B Mas, C Muller, D Neuroscience. 2002; 114(1): 13-7 0306-4522

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Comment on Vadney and Kraushaar's “Effect of switching from Depakene to generic valproic acid on individuals with mental retardation”. Author(s): Wrentham Developmental Center, MA 02093, USA. Source: Alvarez, N Williams, R Ment-Retard. 1998 August; 36(4): 330-1 0047-6765

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Composite acupuncture treatment of mental retardation in children. Author(s): Institute of Acupuncture, China Academy of Traditional Chinese Medicine, Beijing. Source: Tian, L Yuan, S Ba, E Chen, H Zhou, Z J-Tradit-Chin-Med. 1995 March; 15(1): 347 0254-6272

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Effects of switching from Depakene to generic valproic acid on individuals with mental retardation. Author(s): Abilene State School, Texas Department of Mental Health and Mental Retardation 79604, USA. Source: Vadney, V J Kraushaar, K W Ment-Retard. 1997 December; 35(6): 468-72 00476765

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Fragile X-linked mental retardation and the difficulties of reverse genetics. Author(s): Human Molecular Genetics Laboratory, CIML INSERM-CNRS, Marseille, France. Source: Jordan, B R Bioessays. 1991 May; 13(5): 243-51 0265-9247

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Mental retardation, 1990: an overview. Author(s): New York Medical College. Source: Kugel, R B J-Okla-State-Med-Assoc. 1990 October; 83(10): 489-92 0030-1876

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Role of topiramate in adults with intractable epilepsy, mental retardation, and developmental disabilities. Author(s): Department of Neurology, New York Medical College, Westchester Institute for Human Development, Valhalla, NY 10595-1080, USA. [email protected] Source: Singh, Baldev K White Scott, Sheryl Seizure. 2002 January; 11(1): 47-50 1059-1311

•

The effects of a vitamin supplement on the pica of a child with severe mental retardation. Author(s): Kennedy Krieger Institute and Johns Hopkins University School of Medicine, USA. [email protected] Source: Pace, G M Toyer, E A J-Appl-Behav-Anal. 2000 Winter; 33(4): 619-22 0021-8855

•

Weight gain in a controlled study of risperidone in children, adolescents and adults with mental retardation and autism. Author(s): Department of Psychiatry and Behavioral Sciences, University of Kansas Medical Center, Kansas City 66160, USA. [email protected] Source: Hellings, J A Zarcone, J R Crandall, K Wallace, D Schroeder, S R J-ChildAdolesc-Psychopharmacol. 2001 Fall; 11(3): 229-38 1044-5463

Nutrition

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Federal Resources on Nutrition In addition to the IBIDS, the United States Department of Health and Human Services (HHS) and the United States Department of Agriculture (USDA) provide many sources of information on general nutrition and health. Recommended resources include: •

healthfinder®, HHS’s gateway to health information, including diet and nutrition: http://www.healthfinder.gov/scripts/SearchContext.asp?topic=238&page=0

•

The United States Department of Agriculture’s Web site dedicated to nutrition information: www.nutrition.gov

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The Food and Drug Administration’s Web site for federal food safety information: www.foodsafety.gov

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The National Action Plan on Overweight and Obesity sponsored by the United States Surgeon General: http://www.surgeongeneral.gov/topics/obesity/

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The Center for Food Safety and Applied Nutrition has an Internet site sponsored by the Food and Drug Administration and the Department of Health and Human Services: http://vm.cfsan.fda.gov/

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Center for Nutrition Policy and Promotion sponsored by the United States Department of Agriculture: http://www.usda.gov/cnpp/

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Food and Nutrition Information Center, National Agricultural Library sponsored by the United States Department of Agriculture: http://www.nal.usda.gov/fnic/

•

Food and Nutrition Service sponsored by the United States Department of Agriculture: http://www.fns.usda.gov/fns/

Additional Web Resources A number of additional Web sites offer encyclopedic information covering food and nutrition. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=174&layer=&from=subcats

•

Family Village: http://www.familyvillage.wisc.edu/med_nutrition.html

•

Google: http://directory.google.com/Top/Health/Nutrition/

•

Healthnotes: http://www.healthnotes.com/

•

Open Directory Project: http://dmoz.org/Health/Nutrition/

•

Yahoo.com: http://dir.yahoo.com/Health/Nutrition/

•

WebMDHealth: http://my.webmd.com/nutrition

•

WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

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The following is a specific Web list relating to mental retardation; please note that any particular subject below may indicate either a therapeutic use, or a contraindication (potential danger), and does not reflect an official recommendation: •

Minerals Iron Source: Healthnotes, Inc.; www.healthnotes.com

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CHAPTER 3. ALTERNATIVE MEDICINE AND MENTAL RETARDATION Overview In this chapter, we will begin by introducing you to official information sources on complementary and alternative medicine (CAM) relating to mental retardation. At the conclusion of this chapter, we will provide additional sources.

National Center for Complementary and Alternative Medicine The National Center for Complementary and Alternative Medicine (NCCAM) of the National Institutes of Health (http://nccam.nih.gov/) has created a link to the National Library of Medicine’s databases to facilitate research for articles that specifically relate to mental retardation and complementary medicine. To search the database, go to the following Web site: http://www.nlm.nih.gov/nccam/camonpubmed.html. Select “CAM on PubMed.” Enter “mental retardation” (or synonyms) into the search box. Click “Go.” The following references provide information on particular aspects of complementary and alternative medicine that are related to mental retardation: •

“Is it functional?” A question for music therapists who work with the institutionalized mentally retarded. Author(s): Coates P. Source: J Music Ther. 1987 Fall; 24(3): 170-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10317968&dopt=Abstract

•

“What did you say?” Using review of tape-recorded interactions to increase social acknowledgments by trainees in a community-based vocational program. Author(s): Grossi TA, Kimball JW, Heward WL. Source: Research in Developmental Disabilities. 1994 November-December; 15(6): 45772. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7871233&dopt=Abstract

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•

“What will happen to my child when I'm gone?” A support and education group for aging parents as caregivers. Author(s): Mengel MH, Marcus DB, Dunkle RE. Source: The Gerontologist. 1996 December; 36(6): 816-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8990594&dopt=Abstract

•

A comparison of anxiety treatments with adults who have moderate and severe mental retardation. Author(s): Lindsay WR, Baty FJ, Michie AM, Richardson I. Source: Research in Developmental Disabilities. 1989; 10(2): 129-40. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2657899&dopt=Abstract

•

A national profile of self-help/self-advocacy groups of people with mental retardation. Author(s): Browning P, Thorin E, Rhoades C. Source: Mental Retardation. 1984 October; 22(5): 226-30. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=6513787&dopt=Abstract

•

Child life services and persons with mental retardation. Author(s): Kastner T, Friedman DL, O'Brien DR, Albrecht K. Source: Journal of Developmental and Behavioral Pediatrics : Jdbp. 1989 August; 10(4): 198-200. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2527870&dopt=Abstract

•

Competence to confess: measuring understanding and suggestibility of defendants with mental retardation. Author(s): Everington C, Fulero SM. Source: Mental Retardation. 1999 June; 37(3): 212-20. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10473340&dopt=Abstract

•

Composite acupuncture treatment of mental retardation in children. Author(s): Tian L, Yuan S, Ba E, Chen H, Zhou Z. Source: J Tradit Chin Med. 1995 March; 15(1): 34-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7783459&dopt=Abstract

•

Cultural diversity: caring for minority children with mental retardation and other disabilities. Author(s): Waldman HB, Swerdloff M, Perlman SP. Source: Asdc J Dent Child. 2001 July-August; 68(4): 280-5, 229. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11862882&dopt=Abstract

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Drama: a medium to enhance social interaction between students with and without mental retardation. Author(s): Miller H, Rynders JE, Schleien SJ. Source: Mental Retardation. 1993 August; 31(4): 228-33. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8412728&dopt=Abstract

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Drug abuse in persons with mental retardation: a review. Author(s): Christian L, Poling A. Source: Am J Ment Retard. 1997 September; 102(2): 126-36. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9327088&dopt=Abstract

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Effects of serum zinc supplementation on pica behavior of persons with mental retardation. Author(s): Lofts RH, Schroeder SR, Maier RH. Source: Am J Ment Retard. 1990 July; 95(1): 103-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2386628&dopt=Abstract

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Effects of thioridazine and visual screening on stereotypy and social behavior in individuals with mental retardation. Author(s): Singh NN, Landrum TJ, Ellis CR, Donatelli LS. Source: Research in Developmental Disabilities. 1993 May-June; 14(3): 163-77. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8316680&dopt=Abstract

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Evaluation of a sexual abuse prevention program for adults with mental retardation. Author(s): Lumley VA, Miltenberger RG, Long ES, Rapp JT, Roberts JA. Source: J Appl Behav Anal. 1998 Spring; 31(1): 91-101. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9532753&dopt=Abstract

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Evaluation of OAE-recording as a complementary test method for adults with moderate to profound mental retardation. Author(s): Andersson E, Arlinger S, Jacobsson S. Source: Scandinavian Audiology. 2000; 29(2): 120-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10888349&dopt=Abstract

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Evaluation of the dating skills program for improving heterosocial interactions in people with mental retardation. Author(s): Valenti-Hein DC, Yarnold PR, Mueser KT. Source: Behavior Modification. 1994 January; 18(1): 32-46. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8037644&dopt=Abstract

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Evidence for visual imagery deficits in persons with mental retardation. Author(s): Courbois Y.

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Source: Am J Ment Retard. 1996 September; 101(2): 130-48. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8883668&dopt=Abstract •

Eyewitness memory and suggestibility in children with mental retardation. Author(s): Henry LA, Gudjonsson GH. Source: Am J Ment Retard. 1999 November; 104(6): 491-508. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10587731&dopt=Abstract

•

Floppy eyelid syndrome and mental retardation. Author(s): Mack WP. Source: Ophthalmology. 2001 December; 108(12): 2159-60. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11733249&dopt=Abstract

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Identifying the referents of spoken messages: use of context and clarification requests by children with and without mental retardation. Author(s): Abbeduto L, Davies B, Solesby S, Furman L. Source: Am J Ment Retard. 1991 March; 95(5): 551-62. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2043349&dopt=Abstract

•

Imagery and physical practice in the acquisition of gross motor timing of coincidence by adolescents with mild mental retardation. Author(s): Porretta DL, Surburg PR. Source: Percept Mot Skills. 1995 June; 80(3 Pt 2): 1171-83. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7478874&dopt=Abstract

•

Increasing independent decision-making skills of women with mental retardation in simulated interpersonal situations of abuse. Author(s): Khemka I. Source: Am J Ment Retard. 2000 September; 105(5): 387-401. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11008847&dopt=Abstract

•

Mismatch negativity of the color modality during a selective attention task to auditory stimuli in children with mental retardation. Author(s): Horimoto R, Inagaki M, Yano T, Sata Y, Kaga M. Source: Brain & Development. 2002 October; 24(7): 703-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12427518&dopt=Abstract

•

New definition of mental retardation for the American Association of Mental Retardation. Author(s): Fredericks DW, Williams WL.

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Source: Image--The Journal of Nursing Scholarship. 1998; 30(1): 53-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9549942&dopt=Abstract •

New homeopathic medication in rehabilitation of cerebral palsy and mental retardation. Author(s): Oswal GD. Source: The Nursing Journal of India. 1996 November; 87(11): 242-4, 261-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9096487&dopt=Abstract

•

Painting therapy: a Swiss experience for people with mental retardation. Author(s): Carrigan J. Source: American Journal of Art Therapy. 1993 November; 32(2): 53-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10130004&dopt=Abstract

•

Preparation process facilitation of a motor task through imagery practice with adolescents who have mental retardation. Author(s): Surburg PR. Source: Am J Ment Retard. 1991 January; 95(4): 428-34. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2003912&dopt=Abstract

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Reducing excessive vocal loudness in persons with mental retardation through the use of a portable auditory-feedback device. Author(s): Lancioni GE, van Houten K, ten Hoopen G. Source: Journal of Behavior Therapy and Experimental Psychiatry. 1997 June; 28(2): 1238. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9194009&dopt=Abstract

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Review of strategies for treating sleep problems in persons with severe or profound mental retardation or multiple handicaps. Author(s): Lancioni GE, O'Reilly MF, Basili G. Source: Am J Ment Retard. 1999 March; 104(2): 170-86. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=10207580&dopt=Abstract

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Role taking and social competence in autism and mental retardation. Author(s): Oswald DP, Ollendick TH. Source: Journal of Autism and Developmental Disorders. 1989 March; 19(1): 119-27. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=2708295&dopt=Abstract

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Social evaluation of behaviors comprising three social skills and a comparison of the performance of people with and without mental retardation. Author(s): Sherman JA, Sheldon JB, Harchik AE, Edwards K, Quinn JM.

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Source: Am J Ment Retard. 1992 January; 96(4): 419-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=1739455&dopt=Abstract •

Soles of the Feet: a mindfulness-based self-control intervention for aggression by an individual with mild mental retardation and mental illness. Author(s): Singh NN, Wahler RG, Adkins AD, Myers RE; Mindfulness Research Group. Source: Research in Developmental Disabilities. 2003 May-June; 24(3): 158-69. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12742385&dopt=Abstract

•

Studies on prenatal infections in children with unknown cause of mental retardation and examination of their mothers. Author(s): Amrei MA, Al-Hamshary AM, Fotoh OA, Abdel-Rahman S. Source: J Egypt Soc Parasitol. 1999; 29(1): 59-67. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=12561883&dopt=Abstract

•

The effects of a vitamin supplement on the pica of a child with severe mental retardation. Author(s): Pace GM, Toyer EA. Source: J Appl Behav Anal. 2000 Winter; 33(4): 619-22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=11214036&dopt=Abstract

•

The effects of cue control relaxation on adults with severe mental retardation. Author(s): Lindsay WR, Fee M, Michie A, Heap I. Source: Research in Developmental Disabilities. 1994 November-December; 15(6): 42537. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=7871231&dopt=Abstract

•

The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals. Author(s): Eberhart DE, Malter HE, Feng Y, Warren ST. Source: Human Molecular Genetics. 1996 August; 5(8): 1083-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=8842725&dopt=Abstract

•

The treatment of mental retardation in children by tonifying the kidney. Author(s): Xu JM. Source: J Tradit Chin Med. 1985 September; 5(3): 185-6. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=3853648&dopt=Abstract

•

Theory of mind abilities in individuals with autism, Down syndrome, and mental retardation of unknown etiology: the role of age and intelligence. Author(s): Yirmiya N, Solomonica-Levi D, Shulman C, Pilowsky T.

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Source: Journal of Child Psychology and Psychiatry, and Allied Disciplines. 1996 November; 37(8): 1003-14. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ uids=9119934&dopt=Abstract

Additional Web Resources A number of additional Web sites offer encyclopedic information covering CAM and related topics. The following is a representative sample: •

Alternative Medicine Foundation, Inc.: http://www.herbmed.org/

•

AOL: http://search.aol.com/cat.adp?id=169&layer=&from=subcats

•

Chinese Medicine: http://www.newcenturynutrition.com/

•

drkoop.com: http://www.drkoop.com/InteractiveMedicine/IndexC.html

•

Family Village: http://www.familyvillage.wisc.edu/med_altn.htm

•

Google: http://directory.google.com/Top/Health/Alternative/

•

Healthnotes: http://www.healthnotes.com/

•

MedWebPlus: http://medwebplus.com/subject/Alternative_and_Complementary_Medicine

•

Open Directory Project: http://dmoz.org/Health/Alternative/

•

HealthGate: http://www.tnp.com/

•

WebMDHealth: http://my.webmd.com/drugs_and_herbs

•

WholeHealthMD.com: http://www.wholehealthmd.com/reflib/0,1529,00.html

•

Yahoo.com: http://dir.yahoo.com/Health/Alternative_Medicine/

The following is a specific Web list relating to mental retardation; please note that any particular subject below may indicate either a therapeutic use, or a contraindication (potential danger), and does not reflect an official recommendation: •

General Overview Birth Defects Prevention Source: Healthnotes, Inc.; www.healthnotes.com High Homocysteine Source: Healthnotes, Inc.; www.healthnotes.com Hypoparathyroidism Source: Integrative Medicine Communications; www.drkoop.com Muscular Dystrophy Source: Integrative Medicine Communications; www.drkoop.com

122 Mental Retardation

Phenylketonuria Source: Healthnotes, Inc.; www.healthnotes.com Rubella Source: Integrative Medicine Communications; www.drkoop.com •

Herbs and Supplements Betaine Alternative names: Trimethylglycine Source: Integrative Medicine Communications; www.drkoop.com L-tyrosine Source: Healthnotes, Inc.; www.healthnotes.com Melatonin Source: Healthnotes, Inc.; www.healthnotes.com Trimethylglycine Source: Integrative Medicine Communications; www.drkoop.com

General References A good place to find general background information on CAM is the National Library of Medicine. It has prepared within the MEDLINEplus system an information topic page dedicated to complementary and alternative medicine. To access this page, go to the MEDLINEplus site at http://www.nlm.nih.gov/medlineplus/alternativemedicine.html. This Web site provides a general overview of various topics and can lead to a number of general sources.

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CHAPTER 4. DISSERTATIONS ON MENTAL RETARDATION Overview In this chapter, we will give you a bibliography on recent dissertations relating to mental retardation. We will also provide you with information on how to use the Internet to stay current on dissertations. IMPORTANT NOTE: When following the search strategy described below, you may discover non-medical dissertations that use the generic term “mental retardation” (or a synonym) in their titles. To accurately reflect the results that you might find while conducting research on mental retardation, we have not necessarily excluded non-medical dissertations in this bibliography.

Dissertations on Mental Retardation ProQuest Digital Dissertations, the largest archive of academic dissertations available, is located at the following Web address: http://wwwlib.umi.com/dissertations. From this archive, we have compiled the following list covering dissertations devoted to mental retardation. You will see that the information provided includes the dissertation’s title, its author, and the institution with which the author is associated. The following covers recent dissertations found when using this search procedure: •

Efficacy of a Pre-retirement Planning Intervention for Aging Individuals with Mental Retardation (preretirement Planning, Retirement) by Laughlin, Carl Stafford, PhD from The University of Southern Mississippi, 1992, 141 pages http://wwwlib.umi.com/dissertations/fullcit/9239405

•

Efficacy of Supported Employment for Persons with Mental Retardation by Mangan, Troy William, PhD from University of Minnesota, 1997, 86 pages http://wwwlib.umi.com/dissertations/fullcit/9734714

•

Elderly Mothers Caring for an Offspring with Mental Retardation at Home: Mothers without and with Other Offspring (Children) by Keefe, Robert H., PhD from State University of New York at Albany, 1996, 120 pages http://wwwlib.umi.com/dissertations/fullcit/9626704

124 Mental Retardation

•

Eliciting Information about Knowledge Structures in Persons with Mild or Moderate Mental Retardation Through the Use of Dynamic Assessment and Prototype Theory by Zeppuhar, Mary Ellen, EDD from West Virginia University, 1996, 92 pages http://wwwlib.umi.com/dissertations/fullcit/9639457

•

Employer Attitudes and Hiring Behaviors toward Workers with Mental Retardation by Kanter, Joel Phillip, PhD from Boston College, 1987, 169 pages http://wwwlib.umi.com/dissertations/fullcit/8807533

•

Employment Experiences of Women with Mental Retardation: a Qualitative Study by Uhl-Akhavein, Ellen June, EDD from North Carolina State University, 1998, 200 pages http://wwwlib.umi.com/dissertations/fullcit/9826033

•

Ethnicity, Sex and Age As Variables Which Influence the Prevalence of Mild Mental Retardation by Beeman, Frank Robert, PhD from University of California, Riverside, 1988, 180 pages http://wwwlib.umi.com/dissertations/fullcit/8817512

•

Etiological Factors in Mental Retardation of Children from Two Cultures: Implications for Assessment by Foley, Sarah Veronica, PhD from The University of Arizona, 1986, 105 pages http://wwwlib.umi.com/dissertations/fullcit/8702341

•

Evaluation of a Sex Education Workshop for Parents of Adolescents and Young Adults with Mental Retardation by Blachman, Sheila, EDD from Columbia University Teachers College, 1991, 198 pages http://wwwlib.umi.com/dissertations/fullcit/9136356

•

Evaluation of Rehabilitation Short-term Training Programs: the Long-range Effectiveness of Eleven Seminars on Mental Retardation. by Perry, Sherry Ann, PhD from University of Oregon, 1975, 200 pages http://wwwlib.umi.com/dissertations/fullcit/7605197

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Everyday Wisdom in People with Mental Retardation: Role of Experience and Practical Intelligence by Yalon-Chamovitz, Shira Sarit; PhD from The University of Connecticut, 2000, 74 pages http://wwwlib.umi.com/dissertations/fullcit/9963297

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Expected Score Differences in the WISC-R and WISC-III for Special Populations (mental Retardation, Low Income) by Jones, Pamela Eugenia, PhD from The University of Southern Mississippi, 1995, 68 pages http://wwwlib.umi.com/dissertations/fullcit/9606308

•

Exploring the Application of the Positive Approach to Behavior Management for Adults with Mental Retardation by Fitzgibbons, Patrick W., PhD from State University of New York at Buffalo, 1996, 222 pages http://wwwlib.umi.com/dissertations/fullcit/9704874

•

External and Internal Environmental Factors As Acceptable Reasons for Unscheduled Absences As Perceived by Direct-care Staff Within Five Residential Mental Retardation Facilities in Virginia by Goding, Judith G., EDD from Peabody College for Teachers of Vanderbilt University, 1984, 232 pages http://wwwlib.umi.com/dissertations/fullcit/8503631

•

Factors Affecting Decisions Regarding Community Placement of People with Mental Retardation (Deinstitutionalization) by Granfield, James Michael, PhD from The University of Connecticut, 1990, 175 pages http://wwwlib.umi.com/dissertations/fullcit/9119513

Dissertations 125

•

Factors Affecting Program Planning in Community Mental Health and Mental Retardation Centers. by Mc Intyre, William Franklin, PhD from University of Pittsburgh, 1976, 265 pages http://wwwlib.umi.com/dissertations/fullcit/7715219

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Factors Affecting the Type and Frequency of Acting Out Behavior on Elderly Persons with Mental Retardation by Hoch, Eli Bruce; PhD from Fordham University, 2002, 149 pages http://wwwlib.umi.com/dissertations/fullcit/3058894

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Factors Contributing to the Classification of African Americans in Programs Serving Students with Mild Mental Retardation in the State of Florida by Piland, Vicky Carol; PhD from The Florida State University, 2002, 111 pages http://wwwlib.umi.com/dissertations/fullcit/3062907

•

Families of Children with Autism and Families of Children with Mental Retardation: Relationships among Family Needs, Attributes, and Locus-of-control by Brandon, Lori Anne, PhD from The University of North Carolina at Chapel Hill, 1993, 154 pages http://wwwlib.umi.com/dissertations/fullcit/9323998

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Fifth-grade Teachers' Perceptions of the Benefits of Including Students with Mental Retardation in Public Schools in Missouri by Thielbar, Barbara Louise, PhD from University of Missouri - Columbia, 1995, 101 pages http://wwwlib.umi.com/dissertations/fullcit/9705339

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Fragile X Chromosome Associated with Familial Sex-linked Mental Retardation Expression in Fibroblast Culture by Jacky, Peter Bruce; PhD from The University of British Columbia (Canada), 1980 http://wwwlib.umi.com/dissertations/fullcit/NK51707

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From Institution to Community: a Case Study of the Deinstitutionalization of Persons with Mental Retardation (community Placement) by Kane, Victor Lawrence, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1991, 350 pages http://wwwlib.umi.com/dissertations/fullcit/9129522

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From Paradigm to K Mart: an Alternative Approach to Teaching Students with Severe Mental Retardation by Shaw, Dennis George, EDD from The University of North Carolina at Greensboro, 1992, 144 pages http://wwwlib.umi.com/dissertations/fullcit/9303949

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Functional Music Skills of Persons with Mental Retardation (music Skills) by Digiammarino, Marie, EDD from Illinois State University, 1989, 138 pages http://wwwlib.umi.com/dissertations/fullcit/9014745

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Gender Differences in Long-term Postschool Outcomes for Youth with Mild Mental Retardation, Learning Disabilities and No Disabilities: Myth or Reality? by Levine, Phyllis, PhD from University of Washington, 1993, 201 pages http://wwwlib.umi.com/dissertations/fullcit/9401447

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General Case Instructional Strategies for Training Individuals with Moderate Levels of Mental Retardation Tool Usage Skills in the Construction of Pallets (General Case Instruction) by Carson, Karen S., PhD from The Ohio State University, 1991, 255 pages http://wwwlib.umi.com/dissertations/fullcit/9211100

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Generalized Learning As a Function of General Case and Rule Instruction of Adults with Mental Retardation (General Case Instruction, Adult Learners) by Lehman, Laurie R., PhD from New York University, 1992, 200 pages http://wwwlib.umi.com/dissertations/fullcit/9306869

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Habilitation Programming for Offenders with Mental Retardation in Adult State Correctional Systems (Adult Inmates, Men) by Davis, Carol Leytham, PhD from University of Illinois at Chicago, 1996, 152 pages http://wwwlib.umi.com/dissertations/fullcit/9626853

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How Elementary Classroom Teachers Make Instructional Adaptations for Mainstreamed Students with Mental Retardation: a Case Study by Dyer, Ronald E., EDD from Virginia Polytechnic Institute and State University, 1992, 241 pages http://wwwlib.umi.com/dissertations/fullcit/9311572

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How Leisure Beliefs Relate to Attitudes toward the Normalization Principle As Perceived by Service Providers for People with Mental Retardation by Neumayer, Robert John, PhD from The Pennsylvania State University, 1993, 161 pages http://wwwlib.umi.com/dissertations/fullcit/9326912

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Human Service Professionals' Judgments of the Prerequisite Behaviors Needed to Enter a Semi-independent Living Program (Supported Living, Mental Retardation) by Spinella, David Mark, PhD from Boston College, 1991, 181 pages http://wwwlib.umi.com/dissertations/fullcit/9118434

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Hygiene and Motivator Factors (Herzberg) Considered Important by Employees of a State-operated Facility for Adult Individuals with Mental Retardation (F. Herzberg) by O'neal, Kenneth Wayne, Jr.; PhD from The University of Southern Mississippi, 2001, 126 pages http://wwwlib.umi.com/dissertations/fullcit/3013767

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Implementation Analysis of a Public Policy on the Care and Treatment of Persons with Severe Disabilities (Normalization, Mental Retardation) by Donovan, William Clinton, Jr., PhD from Vanderbilt University, 1986, 113 pages http://wwwlib.umi.com/dissertations/fullcit/8626553

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Incidence and Prevalence of Mental Retardation and Other Disabilities in Four Rural Wyoming Counties by Munsey, Cone Johnson, EDD from University of Northern Colorado, 1972, 89 pages http://wwwlib.umi.com/dissertations/fullcit/7223813

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Increasing Choice Opportunities and Reducing Dangerous Behaviors in Children with Serious Emotional Disturbance and Mental Retardation by Pampel, Lynne; EDD from Columbia University Teachers College, 2000, 108 pages http://wwwlib.umi.com/dissertations/fullcit/9976748

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Increasing Independent Interpersonal Decision-making Skills of Women with Mental Retardation in Response to Social-interpersonal Situations Involving Abuse by Khemka, Ishita, PhD from Columbia University, 1997, 332 pages http://wwwlib.umi.com/dissertations/fullcit/9728233

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Increasing the Selection of Non-preferred Vocational Tasks by Three Adult Males with Moderate Mental Retardation by Prompting and Reinforcing Verbal Statements by Denny, R. Kenton, PhD from Vanderbilt University, 1987, 87 pages http://wwwlib.umi.com/dissertations/fullcit/8806971

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A Case Study of Group Home Development for Persons with Mental Retardation: Entry Approaches and Neighborhood Opposition (deinstitutionalization) by Showfety, Michael S., EDD from Virginia Polytechnic Institute and State University, 1986, 180 pages http://wwwlib.umi.com/dissertations/fullcit/8620665

Dissertations 127

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A Comparative Analysis of Directions, Responses, and Consequences Involving Persons with Mental Retardation in Employment Sites and Vocational Training Programs by Gonzalez, Patricia Ann, PhD from University of Illinois at Urbanachampaign, 1989, 211 pages http://wwwlib.umi.com/dissertations/fullcit/9010867

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A Comparative Study of Infants At-risk for Mental Retardation and the Effects of Day Care Intervention on the Development of Communication Skills by O'connell, Joanne Curry, PhD from The University of North Carolina at Chapel Hill, 1980, 155 pages http://wwwlib.umi.com/dissertations/fullcit/8022493

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A Comparative Study of Job Satisfaction of Persons with Disabilities in Sheltered Employment and Competitive Employment (developmental Disabilities, Mental Retardation) by Cavaiuolo, Domenico Julius, PhD from Temple University, 1994, 137 pages http://wwwlib.umi.com/dissertations/fullcit/9527455

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A Comparative Study of Occupational Stress Levels among Selected Rural Alabama Teachers in the Areas of Mental Retardation, Learning Disabilities, and Emotional Conflict by Wiley, Nancy Susan, EDD from The University of Alabama, 1987, 125 pages http://wwwlib.umi.com/dissertations/fullcit/8810953

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A Comparative Study of Social Functioning between Adolescents with a Dual Diagnosis of Emotional Disturbance and Mental Retardation and Adolescents with Only the Latter Diagnosis by Sadowsky, Alan D., Dsw from University of California, Los Angeles, 1982, 124 pages http://wwwlib.umi.com/dissertations/fullcit/8225606

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A Comparative Study of Stereotypical Behavior by Three Profoundly Retarded Individuals in Institutional V. School Settings (mental Retardation) by Morris, Barbara B., PhD from Texas Woman's University, 1993, 88 pages http://wwwlib.umi.com/dissertations/fullcit/9407733

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A Comparative Study of University Students, Professionals, and Community Attitudes toward Mental Retardation. by Goldstein, Karen Eileen Daum, EDD from The University of Alabama, 1978, 140 pages http://wwwlib.umi.com/dissertations/fullcit/7905407

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A Comparison of Adaptive Behavior Ratings: Vineland Adaptive Behavior Scales and Aamd Adaptive Behavior Scale - School Edition (mental Retardation) by Ronka, Carol Steinbach, PhD from University of Cincinnati, 1985, 142 pages http://wwwlib.umi.com/dissertations/fullcit/8518116

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A Comparison of Educational Attitudes, Role Expectations, and Dimensions of Progressivism between Teachers and Pre-service Teachers in Mental Retardation and Elementary Education. by Berghoff, Beth Adrian Katz, PhD from University of Georgia, 1979, 134 pages http://wwwlib.umi.com/dissertations/fullcit/7915479

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A Comparison of in Vivo and On-site Simulation Instruction of Purchasing Skills in High School Students with Mental Retardation and Severe Emotional Disturbance by Saternow, Martha Estelle, PhD from University of Pittsburgh, 1989, 136 pages http://wwwlib.umi.com/dissertations/fullcit/8921419

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A Comparison of Methods for Reducing Staff Absenteeism at a Public Residential Facility (institutions, Mental Retardation, Feedback) by Feroz, Raymond Felix, PhD from University of Pittsburgh, 1983, 179 pages http://wwwlib.umi.com/dissertations/fullcit/8411804

128 Mental Retardation

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A Comparison of Techniques for Increasing Attending to Visual Stimuli in Classrooms for Children with Mental Retardation by Brown, Louis John, PhD from The Florida State University, 1969, 98 pages http://wwwlib.umi.com/dissertations/fullcit/7003816

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A Comparison of the Attitudes of Parents Whose Children Are Diagnosed with Autism and of Parents Whose Children Are Diagnosed with Mental Retardation by Yeager, Mark Horren, PhD from The University of Southern Mississippi, 1998, 86 pages http://wwwlib.umi.com/dissertations/fullcit/9916049

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A Comparison of the Conceptions and Attitudes of Parents of Mentally Retarded Children Concerning the Subgroups of Mental Retardation As Influenced by Socioeconomic Status by Takeguchi, Sumie Lily Ann, EDD from Syracuse University, 1966, 220 pages http://wwwlib.umi.com/dissertations/fullcit/6707121

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A Comparison of the Effectiveness and Efficiency of Behavior Chaining Techniques in the Acquisition of Selected Motor Fitness Skills by Individuals with Severe Mental Retardation by Decker, James T., PhD from The Ohio State University, 1986, 184 pages http://wwwlib.umi.com/dissertations/fullcit/8625207

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A Comparison of the Effects of Praise and Encouragement on the Production Rate of Individuals with Mental Retardation by Pitsounis, Nickolaos D., EDD from Texas Tech University, 1988, 97 pages http://wwwlib.umi.com/dissertations/fullcit/8819420

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A Comparison of the Expectancy of Success and the Probability Learning of Children and Adults with Mental Retardation (decision-making, Judgment) by Paul, Ginger Owens, EDD from Baylor University, 1983, 155 pages http://wwwlib.umi.com/dissertations/fullcit/8404095

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A Comparison of the Level of Knowledge and Attitudes toward Mental Retardation and Community Integration Held by Housemanagers and Community-based Borderline and Mildly Retarded Adults As Related to the Degree of Involvement in Community/home Activities by Shelley, Bonnie Louann, PhD from Peabody College for Teachers of Vanderbilt University, 1980, 147 pages http://wwwlib.umi.com/dissertations/fullcit/8105526

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A Comparison of the Long-term Recognition of Pictures of Places by Adults with and without Mental Retardation (retention Intervals) by Duh, Jengjyh, PhD from University of Kansas, 1991, 76 pages http://wwwlib.umi.com/dissertations/fullcit/9210044

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A Comparison of Two Mental Retardation Junior Fieldwork Experiences by Conner, Doris Jean Mchargue, PhD from The Florida State University, 1972, 90 pages http://wwwlib.umi.com/dissertations/fullcit/7234022

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A Comparison of Two Teaching Strategies and Jensen's Two-factor Theory of Mental Retardation. by Hodge, Wallace Dwight, EDD from West Virginia University, 1977, 95 pages http://wwwlib.umi.com/dissertations/fullcit/7732077

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A Descriptive Analysis of Aggression in Two Samples of Institutionalized Individuals with Mental Retardation by Knight, Catherine Berger, PhD from The University of Wisconsin - Madison, 1987, 216 pages http://wwwlib.umi.com/dissertations/fullcit/8713160

Dissertations 129

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A Descriptive Analysis of Classroom Interactions between Teachers and Students with Mental Retardation or Emotional and Behavioral Disorders by Johnson, Stephen Maynard; PhD from Vanderbilt University, 1999, 164 pages http://wwwlib.umi.com/dissertations/fullcit/9958445

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A Descriptive Study of Flexibility in Communicative Style in a Down's Syndrome Child (language Acquisition, Mental Retardation, Ethnography) by Byrne-jandacek, Anna, EDD from University of Cincinnati, 1984, 179 pages http://wwwlib.umi.com/dissertations/fullcit/8420861

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A Field Study of the Effects of the Athletes for Outreach Program on Communication Competence and Perceived Social Competencies among Individuals with Mental Retardation by Lord, Michal Anne, PhD from The University of Texas at Austin, 1995, 174 pages http://wwwlib.umi.com/dissertations/fullcit/9603902

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A Follow Up Study of High School Students with Mild Mental Retardation: Has the Transition Been Made? (work Study) by Wells, Ronald Otho, EDD from West Virginia University, 1993, 204 pages http://wwwlib.umi.com/dissertations/fullcit/9410233

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A Group Intervention to Assist Older Parents of Adults with Mental Retardation in Permanency Planning by Botsford, Anne L., PhD from State University of New York at Albany, 1997, 151 pages http://wwwlib.umi.com/dissertations/fullcit/9719906

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A History of Teacher Preparation Programs in Mental Retardation in America by Aaronson, Warren Joseph, EDD from Boston University School of Education, 1967, 231 pages http://wwwlib.umi.com/dissertations/fullcit/6907792

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A Longitudinal Analysis of Factors Associated with Challenging Behavior and Psychopharmacological Treatment Practices for Persons with Mental Retardation Following Deinstitutionalization by Cavanaugh, Danise V.; PhD from Rutgers the State University of New Jersey - New Brunswick, 2002, 321 pages http://wwwlib.umi.com/dissertations/fullcit/3055032

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'a Matter of Moral and Social Duty': Benjamin O. Whitten's Tenure As Superintendent of an Institution for Persons with Mental Retardation in South Carolina, 1918-1965 by Haddock, Jean Hook, PhD from University of South Carolina, 1999, 134 pages http://wwwlib.umi.com/dissertations/fullcit/9928308

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A Meta-analysis of Learning and Memory in Mental Retardation by Mattson, Paul Dennis, PhD from University of California, Riverside, 1985, 269 pages http://wwwlib.umi.com/dissertations/fullcit/8520636

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A National Survey of Advisors to Self-advocacy Groups for Individuals with Mental Retardation by Cone, Alicia Ann, PhD from Virginia Commonwealth University, 1997, 351 pages http://wwwlib.umi.com/dissertations/fullcit/9728755

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A National Survey of Community College Accommodation of Students Who Have Mental Retardation (student Accommodation) by Noble, Gayle Lynn, PhD from The Claremont Graduate University, 1992, 179 pages http://wwwlib.umi.com/dissertations/fullcit/9238867

130 Mental Retardation

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A Neuropsychological Model for the Development of the Cognitive Profiles in Mental Retardation Syndromes: Evidence from Down Syndrome and Williams Syndrome by Edgin, Jamie Ogline; PhD from University of Denver, 2003, 91 pages http://wwwlib.umi.com/dissertations/fullcit/3086381

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A Pragmatic Study of Adults with Mental Retardation Conversing with Their Peers and Adults with 'normal Intelligence.' (volumes I and Ii) by Magocsi, Susan Cissel, Dsc from Boston University, the Sargent College of Allied Health Professions, 1981, 644 pages http://wwwlib.umi.com/dissertations/fullcit/8126610

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A Program Evaluation Study on Employment Services for Developmentally Disabled Adults Comparing a Sheltered Workshop Program with a Supported Work Program (performance, Job Satisfaction, Non-sheltered, Mental Retardation, Vocational Rehabilitation) by Lam, Chow Shing, PhD from The University of Wisconsin - Madison, 1985, 193 pages http://wwwlib.umi.com/dissertations/fullcit/8522523

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A Psychometric Validation of Direct and Indirect Attitude Measurements toward People with Mental Retardation in Korea by Byon, Kyong Hee; PhD from The University of Wisconsin - Madison, 2000, 144 pages http://wwwlib.umi.com/dissertations/fullcit/9983734

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A Quantitative Review of the Literature about Children with Severe-multiple Handicaps (mental Retardation, Standard Celeration, Early Childhood, Precision Teaching) by Kyrklund, Sarah Jane, PhD from University of Kansas, 1985, 296 pages http://wwwlib.umi.com/dissertations/fullcit/8608421

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A Radical Perspective on the Development of American Special Education with a Focus on the Concept of 'learning Disabilities' (student Grouping, Mental Retardation, Pupil Segregation, Policy) by Sigmon, Scott B., EDD from Rutgers the State University of New Jersey - New Brunswick, 1985, 176 pages http://wwwlib.umi.com/dissertations/fullcit/8609242

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A Service Delivery System for Persons with Mental Retardation in Thailand by Khumnirdpetch, Patchareewun, EDD from Illinois State University, 1990, 164 pages http://wwwlib.umi.com/dissertations/fullcit/9101117

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A Study of Job Duties and Functions of Teachers and Paraprofessionals in Mental Health/mental Retardation Day Programs, Region Iv, Alabama by Long, Martha Doughtie, EDD from Auburn University, 1984, 139 pages http://wwwlib.umi.com/dissertations/fullcit/8415502

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A Study of Ohio's New Definition of Educable Mental Retardation. by Amorose, Richard Anthony, PhD from The Ohio State University, 1976, 113 pages http://wwwlib.umi.com/dissertations/fullcit/7702335

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A Study of Organizational Reform: an Examination of the Impact of Organizational Structure on Staff Attitudes Within a Residential Setting for Mentally Retarded Persons (organizational Behavior, Staff Development, Mental Retardation, Institutional Change by Meyer, Emily, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1986, 193 pages http://wwwlib.umi.com/dissertations/fullcit/8622393

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A Study of the Difference between Volunteers and Non-volunteers in Friendship Formation Behaviors in Adults with Mental Retardation by Moran, John Michael; PhD from University of Missouri - Columbia, 2001, 136 pages http://wwwlib.umi.com/dissertations/fullcit/3036848

Dissertations 131

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A Study of the Differential Perceptions of Educators toward Children with Mental Retardation Labels. by Medved, Richard Michael, EDD from State University of New York at Albany, 1976, 127 pages http://wwwlib.umi.com/dissertations/fullcit/7619840

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A Study of the Formal Services Needed and Utilized by Aging Parental Caregivers for Themselves and for Their Child with Mental Retardation Living at Home by Tracey, Marianne Elizabeth; EDD from Columbia University Teachers College, 2003, 126 pages http://wwwlib.umi.com/dissertations/fullcit/3091303

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A Study of the Impact of Community Recreation Skill Acquisition on the Social Lives of Adults with Mental Retardation (recreation Skills Training) by Green, Frederick Paul, PhD from University of Minnesota, 1992, 195 pages http://wwwlib.umi.com/dissertations/fullcit/9220259

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A Study of Variables Affecting the Level of Maternal Adjustment to the Child with Mental Retardation (rehabilitation) by Oh, Kil-sung, Rhd from Southern Illinois University at Carbondale, 1993, 192 pages http://wwwlib.umi.com/dissertations/fullcit/9403165

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A Study of X-linked Mental Retardation in British Columbia by Herbst, Diana Shawn; PhD from The University of British Columbia (Canada), 1980 http://wwwlib.umi.com/dissertations/fullcit/NK49961

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A Study to Investigate the Use of Vocational Interest Assessment with Students with Mild Mental Retardation in Selected Senior High Public School Programs by Begun, Wynne H., PhD from University of Kansas, 1985, 145 pages http://wwwlib.umi.com/dissertations/fullcit/8608375

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A Survey of Missouri Residential Placements for Persons with Mental Retardation by Jayne, Donna Grace, PhD from University of Missouri - Columbia, 1994, 182 pages http://wwwlib.umi.com/dissertations/fullcit/9511659

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A Survey of the Graduate and Undergraduate Teacher Preparation Program in Mental Retardation--1966 Through 1969--special Education Department University of Oregon by Madsen, Glenn Alvin, Ded from University of Oregon, 1971, 170 pages http://wwwlib.umi.com/dissertations/fullcit/7208570

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A Systems Analysis of an Historic Mental Retardation Institution: a Case Study of Elwyn Institute - 1852-1970. (volumes I and Ii) by Royfe, Ephrain Henry, EDD from Temple University, 1972, 413 pages http://wwwlib.umi.com/dissertations/fullcit/7220211

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A Total Quality Management Model for Human Service Organizations: the Particular Case of People with Mental Retardation (spanish Text) by Fernandez-villaran Ara, Asuncion; Dr from Universidad De Deusto (spain), 2001, 548 pages http://wwwlib.umi.com/dissertations/fullcit/3036636

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A Visual Attention Study for Developing Learning Cues for Individuals with Severe Mental Retardation by Aveyard, Roger L.; PhD from The University of Nebraska Lincoln, 2001, 195 pages http://wwwlib.umi.com/dissertations/fullcit/3038972

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Acquisition, Transfer and Maintenance of Behavior in Persons with Mental Retardation As a Function of the Developmental Level of the Task Taught by Mcpherson, Kenneth Francis, PhD from University of Georgia, 1989, 78 pages http://wwwlib.umi.com/dissertations/fullcit/9007686

132 Mental Retardation

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Activity Dependent Translation of the Fragile X Mental Retardation Protein and Itsmrna Targets by Todd, Peter Kennedy; PhD from The University of Wisconsin Madison, 2002, 180 pages http://wwwlib.umi.com/dissertations/fullcit/3060471

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Adaptive Behavior Achievement in Adults with Severe Visual Impairment and Mental Retardation with Implications for Planning Specialized Interventions by Dandas, Erine Evangelia Theodorou, PhD from The University of Michigan, 1991, 154 pages http://wwwlib.umi.com/dissertations/fullcit/9135580

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Adaptive Skill Domain Scores As Predictors of Placement Outcome for Citizens of Mississippi with Mental Retardation by Ellis, James Stephen, PhD from The University of Mississippi, 1989, 87 pages http://wwwlib.umi.com/dissertations/fullcit/9005385

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Administrative Initiative in Policy Implementation: Mental Retardation Deinstitutionalization Policy in Texas by Laubacher, Steven, PhD from University of Houston, 1990, 234 pages http://wwwlib.umi.com/dissertations/fullcit/9024580

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African-american Males with Mental Retardation: Friendship Formation and Maintenance in Community-based Employment by Kronick, Nancy Clara, PhD from University of Illinois at Urbana-champaign, 1996, 208 pages http://wwwlib.umi.com/dissertations/fullcit/9712339

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Age and Functional Level of Children with Mental Retardation in Relation to the Mothers' Marital Satisfaction and Marital Instability by Chaney, Lillian Lee, EDD from Memphis State University, 1988, 73 pages http://wwwlib.umi.com/dissertations/fullcit/8911436

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Aging with Mental Retardation: a Growing Concern by Girgis-hanna, Mary Fahim, PhD from The University of Toledo, 1997, 209 pages http://wwwlib.umi.com/dissertations/fullcit/9735258

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An Analysis of Attitudes of Ward Attendants toward Mental Retardation in Four State Residential Institutions for the Mentally Retarded. by Norman, Henry Arthur, PhD from The Ohio State University, 1975, 148 pages http://wwwlib.umi.com/dissertations/fullcit/7519478

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An Analysis of Motivational Variables in Correspondence Training (mental Retardation, Verbal Behavior, Nonverbal Behavior) by Wilson, Philip G., PhD from University of Illinois at Urbana-champaign, 1991, 161 pages http://wwwlib.umi.com/dissertations/fullcit/9211037

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An Analysis of the Relationship between Initial Intervention and Job Success for Individuals with Mental Retardation Placed Through Supported Employment in Pennsylvania between 1986 and 1990 by Berry, Brian Richard, PhD from Temple University, 1993, 142 pages http://wwwlib.umi.com/dissertations/fullcit/9332771

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An Assessment of the Socio-sexual Knowledge of Institutionalized Persons with Mental Retardation (developmental Disability) by Davis, Reginald, PhD from The University of Michigan, 1986, 223 pages http://wwwlib.umi.com/dissertations/fullcit/8612501

Dissertations 133

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An Empirical Analysis of Social Workers in the Field of Mental Retardation by Deweaver, Kevin Leon, PhD from The Florida State University, 1980, 149 pages http://wwwlib.umi.com/dissertations/fullcit/8020351

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An Examination of Inclusive Recreation and Leisure Participation for Children with Trainable Mental Retardation by Modell, Scott Jason, PhD from The Florida State University, 1997, 154 pages http://wwwlib.umi.com/dissertations/fullcit/9735817

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An Experimental Analysis of the Generalization of Banking Skills from the Classroom to Trained and Untrained Bank Settings in the Community (simulation, Functional Skills, Mental Retardation, Curricula) by Bourbeau, Philip Edward, Jr., PhD from University of Oregon, 1984, 116 pages http://wwwlib.umi.com/dissertations/fullcit/8505747

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An Experimental Study to Assess the Effects of a Nonaversive Treatment Program on the Self-injurious Behaviors of Three Persons with Profound Mental Retardation Who Live in an Institution (mental Retardation) by Pitonyak, David Adams, PhD from Virginia Commonwealth University, 1990, 397 pages http://wwwlib.umi.com/dissertations/fullcit/9024419

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An Exploration into the Child Welfare Experience of Parents with Mental Retardation in Illinois by Deyoung, Lori Ann; PhD from University of Illinois at Urbana-champaign, 2000, 154 pages http://wwwlib.umi.com/dissertations/fullcit/9955607

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An Investigation of the Educational Implications of Jensen's Rationale and Techniques for Differentiating between Primary and Cultural Mental Retardation by Wallace, Ben Marshall, PhD from The University of Texas at Austin, 1970, 114 pages http://wwwlib.umi.com/dissertations/fullcit/7202433

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An Investigation of the Influence of Mental Retardation on College Students' Judgments of Social Distance by Dent, Harold Edward, PhD from University of Hawaii, 1966, 107 pages http://wwwlib.umi.com/dissertations/fullcit/6713694

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An Investigation of the Learning Effects of Teaching Social Problem-solving Skills to Adolescents with Mental Retardation by Nave, Gary Ray, PhD from University of Oregon, 1987, 135 pages http://wwwlib.umi.com/dissertations/fullcit/8808698

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An Investigation of the Relationship between Selected Environmental Factors and the Presence of Mild Mental Retardation among Children in Elementary Schools in Iraq by Al-yasiri, Hussein Noori, EDD from University of Northern Colorado, 1982, 161 pages http://wwwlib.umi.com/dissertations/fullcit/8301132

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An Investigation of the Reliability and Validity of the 'enderle-severson Transition Rating Scale' (adult Transition, Learning Disabilities, Emotional Disturbance, Mental Retardation) by Severson, Susan Janine, EDD from The University of North Dakota, 1993, 127 pages http://wwwlib.umi.com/dissertations/fullcit/9408439

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Analysis of Average Per Pupil Expenditures of Ohio's County Boards of Mental Retardation and Developmental Disabilities by Stephens, Ronald K., PhD from The Ohio State University, 1982, 143 pages http://wwwlib.umi.com/dissertations/fullcit/8222184

134 Mental Retardation

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Application of Management Theory and Practice to a Public Mental Retardation Facility by White, Donald D., Jr., PhD from The University of Nebraska - Lincoln, 1971, 222 pages http://wwwlib.umi.com/dissertations/fullcit/7300144

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Applying a Cognitive Strategy in Social Skill Training: Examining the Effectiveness of a Process Model on the Acquisition, Generalization, and Maintenance of Social Behaviors (mental Retardation) by Park, Hyun-sook, PhD from University of California, Berkeley with San Francisco State Univ., 1989, 209 pages http://wwwlib.umi.com/dissertations/fullcit/9006589

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Assessing Mental Health and Mental Retardation Professionals' Knowledge of Mental Illness, Mental Retardation and Mental Illness As It Relates to Persons with Mental Retardation (dual Diagnosis, Mental Health Professionals) by Petruska, Richard James, PhD from The Ohio State University, 1991, 175 pages http://wwwlib.umi.com/dissertations/fullcit/9201736

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Assessing the Satisfaction of Adults with Mental Retardation Living in the Community (quality of Life) by Harner, Cathy J., PhD from University of Illinois at Urbana-champaign, 1991, 190 pages http://wwwlib.umi.com/dissertations/fullcit/9136610

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Assessing the Training and Staff Development Needs of Mental Health/mental Retardation Professionals: a Multi-method Framework (nominal Group Method, Community, Triangulation, Competencies) by Cogswell, Dennis Robert, EDD from Virginia Polytechnic Institute and State University, 1985, 311 pages http://wwwlib.umi.com/dissertations/fullcit/8521303

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Attitude and Attitude Change toward Mental Retardation of Certain Worker and Student Groups As a Function of Related Education and Experience. by White, Walter Andrew, EDD from Boston University School of Education, 1973, 191 pages http://wwwlib.umi.com/dissertations/fullcit/7414239

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Attitudes of High School Seniors toward Individuals with Mental Retardation and Down Syndrome Following Participation in a Social Integration Friendship Program: an Exploratory Study by Glenn, Anthony T.; EDD from Wilmington College (delaware), 2001, 164 pages http://wwwlib.umi.com/dissertations/fullcit/3004825

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Attitudes of High School Students toward People with Moderate Mental Retardation: an Exploratory Study of Integration and Intervention by Marino, Gloria Fusco, EDD from Columbia University Teachers College, 1994, 105 pages http://wwwlib.umi.com/dissertations/fullcit/9511055

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Attitudes of Kentucky Southern Baptist Pastors toward Mental Retardation: Nature and Determinants. by Beane, Allan Lane, PhD from Southern Illinois University at Carbondale, 1977, 121 pages http://wwwlib.umi.com/dissertations/fullcit/7724449

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Attitudes of Michigan Clergymen toward Mental Retardation and toward Education: Their Nature and Determinants by Heater, William Henderson, PhD from Michigan State University, 1967, 220 pages http://wwwlib.umi.com/dissertations/fullcit/6804151

Dissertations 135

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Attitudes of Nursing Students toward Mental Retardation before and after Curricular Experience with Mentally Retarded Children. by Baker, Amanda Sirmon, PhD from University of Florida, 1974, 133 pages http://wwwlib.umi.com/dissertations/fullcit/7519312

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Attitudes of Taiwanese Eighth-grade Students toward Students with Mild Mental Retardation (china) by Liu, Wei-ping; PhD from State University of New York at Albany, 1999, 171 pages http://wwwlib.umi.com/dissertations/fullcit/9940765

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Attitudes of Texas Mexican-americans toward Mental Retardation: a Guttman Facet Analysis by Morin, Kenneth Noah, PhD from Michigan State University, 1969, 279 pages http://wwwlib.umi.com/dissertations/fullcit/7015089

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Attitudes toward Mental Retardation: a Community Study by Kerns, William Alan, PhD from University of Pittsburgh, 1981, 161 pages http://wwwlib.umi.com/dissertations/fullcit/8213209

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Attitudes toward Persons with Mental Retardation: a Study of Los Angeles County Group Home Administrators (california) by Bowie, Kim Coleman; Dpa from University of La Verne, 2001, 139 pages http://wwwlib.umi.com/dissertations/fullcit/3026747

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Attitudes toward Productivity in Mental Retardation Research Centers (research Productivity) by Zamarripa, Edward J., EDD from University of Kansas, 1991, 146 pages http://wwwlib.umi.com/dissertations/fullcit/9210143

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Attorneys' Perceptions of Child Witnesses with Mental Retardation by Platt, Michelle Dawn; Ms from University of Nevada, Las Vegas, 2002, 78 pages http://wwwlib.umi.com/dissertations/fullcit/1411203

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Augmented Input and Recognitory Comprehension in Persons with Mental Retardation by Heller, Kathryn Wolff, PhD from Georgia State University, 1992, 167 pages http://wwwlib.umi.com/dissertations/fullcit/9212353

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Automatic and Effortful Memory Processing by Students with and without Mental Retardation by Thomas, Suzanne B., PhD from University of Florida, 1994, 168 pages http://wwwlib.umi.com/dissertations/fullcit/9607279

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Behaviorally Anchored Rating Scales and Staff Performance in Icfs/mr (intermediate Care Facilities, Mental Retardation) by Hendrikson, Kay Crisafulli, PhD from University of Minnesota, 1995, 139 pages http://wwwlib.umi.com/dissertations/fullcit/9541324

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Bone Mineral Density in Adult Women with Mental Retardation by Felix, Emmanuel S., PhD from Oregon State University, 1993, 171 pages http://wwwlib.umi.com/dissertations/fullcit/9413713

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Career Education for Students with Mild Mental Retardation at the Junior High Level in the Taiwan Area (china) by Chen Fu, Shiou-mei, EDD from Columbia University Teachers College, 1993, 144 pages http://wwwlib.umi.com/dissertations/fullcit/9400540

136 Mental Retardation

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Caregivers' Attitudes toward the Severity of Punishment for Forty-four Misbehaviors in Mental Retardation Institutions by Pokalo, Mariann, PhD from Temple University, 1987, 230 pages http://wwwlib.umi.com/dissertations/fullcit/8711392

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Case Management in Mental Retardation Service Delivery Systems: a View from the Field (deinstitutionalization, Work Environment, Activities) by Middleton, E. Jane, Dsw from University of Pennsylvania, 1985, 255 pages http://wwwlib.umi.com/dissertations/fullcit/8515991

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Characteristics Associated with Current Recreational Patterns of Persons with Mental Retardation by Shannon, Glenna Faye, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1986, 156 pages http://wwwlib.umi.com/dissertations/fullcit/8622395

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Characteristics of Individuals with Handicaps Who Have Been Abused by Caregivers (behavior Disorders, Mental Retardation) by Zirpoli, Thomas James, Jr., PhD from University of Virginia, 1986, 135 pages http://wwwlib.umi.com/dissertations/fullcit/8705704

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Choice-making in Community-based Residences for Adults with Mental Retardation (adult Mental Retardation, Group Homes) by Artesani, A. James, EDD from West Virginia University, 1992, 269 pages http://wwwlib.umi.com/dissertations/fullcit/9322917

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Classroom Behaviors and Training of Service Providers to Students with Severe or Profound Retardation (mental Retardation) by Smith, Gayle V., PhD from University of Denver, 1995, 279 pages http://wwwlib.umi.com/dissertations/fullcit/9543998

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Clinical Judgment Bias in Case Management Decision-making for Older Persons with Mental Retardation (elderly) by Wadsworth, John Steele, PhD from The University of Iowa, 1996, 140 pages http://wwwlib.umi.com/dissertations/fullcit/9629732

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Color Reversal and the Acquisition and Retention of Survival Vocabulary in Spastic and Nonspastic Multi-handicapped Adolescents with Mental Retardation (black on White, White on Black) by Blyden, Amelia Elizabeth, EDD from Columbia University Teachers College, 1984, 157 pages http://wwwlib.umi.com/dissertations/fullcit/8505352

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Communication Skills Training for Staff in Residential Facilities for Persons with Severe Mental Retardation by Gerard, Cheryl B., PhD from University of Oregon, 1991, 165 pages http://wwwlib.umi.com/dissertations/fullcit/9137346

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Community Integration and Community Residences (mental Retardation, Group Homes, Adult, Staffed Apartments, Social Integration) by Goff, Barbara Joan, EDD from Boston University, 1986, 267 pages http://wwwlib.umi.com/dissertations/fullcit/8616125

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Community Mental Retardation Policies: Satisfaction As a Component by Trivedi, Pallavi Kishore, PhD from The University of Texas at Arlington, 1988, 196 pages http://wwwlib.umi.com/dissertations/fullcit/8914120

Dissertations 137

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Community Supports for Older Adults with Mental Retardation and Their Families: a Comparison of States' Family Support Programs by Rockett, Christopher; PhD from University of Massachusetts Boston, 2001, 290 pages http://wwwlib.umi.com/dissertations/fullcit/3032189

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Comparison of Coping Processes between Aging Mothers of Adults with Mental Illness Versus Aging Mothers of Adults with Mental Retardation: a Longitudinal Study by Kim, Hea-won, PhD from The University of Wisconsin - Madison, 1998, 242 pages http://wwwlib.umi.com/dissertations/fullcit/9839355

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Comparison of Selected Piagetian Tasks with the Wechsler Intelligence Scale for Children As Measures of Mental Retardation by Marchi, Jack Ugo, PhD from University of California, Berkeley, 1970, 106 pages http://wwwlib.umi.com/dissertations/fullcit/7115833

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Comparison of Three Techniques for the Assessment of Mental Retardation in the Elementary Schools by Murphy, Marilyn Henry, EDD from University of California, Los Angeles, 1966, 132 pages http://wwwlib.umi.com/dissertations/fullcit/6704480

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Comparisons of Parent-obtained and Teacher-obtained Adaptive Behavior Scores for Handicapped Children (mental Retardation, Learning Disabled, Vineland) by Rainwater-bryant, Brenda Joy, EDD from Memphis State University, 1985, 157 pages http://wwwlib.umi.com/dissertations/fullcit/8519710

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Compliance Is Not Enough: Smoking Policies in State Mental Retardation and Developmental Disabilities Agencies by Minihan, Paula M.; PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 2003, 261 pages http://wwwlib.umi.com/dissertations/fullcit/3074826

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Components of Successful School-to-work Transition Programs for 19-21 Year Old Students with Mental Retardation by White, Janis Bryant, EDD from University of California, Irvine, 1999, 97 pages http://wwwlib.umi.com/dissertations/fullcit/9932115

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Concurrent Validation of the Abbreviated Mccarron-dial System for Students with Mental Retardation and Learning Disabilities by Kimmel, Charlotte Mae, PhD from Texas A&m University, 1988, 163 pages http://wwwlib.umi.com/dissertations/fullcit/8913399

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Context and Competence: Some Situations of Mental Retardation in a Mediated Ethnography of Communication by Sutherland, Donald Robert, PhD from University of California, Los Angeles, 1980, 180 pages http://wwwlib.umi.com/dissertations/fullcit/8016049

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Contributions of Forensic Evaluator Attributes to Recommendations for Competency to Stand Trial for Defendants with Mental Retardation by Love, Peter Francis; PhD from The University of Connecticut, 2002, 71 pages http://wwwlib.umi.com/dissertations/fullcit/3076709

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Controlling Variables for the Emission of Peer Social Conversational Units by Students with Moderate Mental Retardation (conversational Units) by Donley, Corrine Russell, EDD from Columbia University Teachers College, 1990, 124 pages http://wwwlib.umi.com/dissertations/fullcit/9021283

138 Mental Retardation

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Conversation Topic Training for Adults with Mental Retardation and the Effects of the Training on Conversations with Naive Communication Partners by Mattie, Harold Dwight, EDD from University of Nevada, Reno, 1994, 98 pages http://wwwlib.umi.com/dissertations/fullcit/9607980

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Correspondence between Verbalizations and Emission of Related Actions in Persons with Mild-to-moderate Mental Retardation by Baker, Daniel Jeffrey, PhD from University of Minnesota, 1993, 96 pages http://wwwlib.umi.com/dissertations/fullcit/9324663

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Cost Evaluation of Community Residential Alternatives for Developmentally Disabled Adults (cost-effectiveness, Mental Retardation) by Fujiura, Glenn Tsutomu, PhD from University of Illinois at Urbana-champaign, 1985, 117 pages http://wwwlib.umi.com/dissertations/fullcit/8600187

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Curriculum Emphasis for Students with Mild Mental Retardation: a Survey Research of the Current Perspectives of Taiwan's Secondary Special Educators by Su, Hsichuan, PhD from University of Kansas, 1997, 79 pages http://wwwlib.umi.com/dissertations/fullcit/9811581

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Daily Stress Experienced by Adults with Mental Retardation and Significantly Challenging Behavior: the Relationship between Stress, Problem Behavior, Integration, and Stress of Direct Care Providers by Wells, Jennifer Jane, PhD from University of Oregon, 1994, 127 pages http://wwwlib.umi.com/dissertations/fullcit/9509825

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Defining and Measuring Community Inclusion in the Lives of People with Mental Retardation by Sebastian, Margaret Mary; PhD from Michigan State University, 1999, 145 pages http://wwwlib.umi.com/dissertations/fullcit/9948174

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Deinstitutionalization: a Comparison of Community Program Directors' and Superintendents' Attitudes (mental Retardation) by Henning, Dana Beth, EDD from Temple University, 1984, 136 pages http://wwwlib.umi.com/dissertations/fullcit/8410145

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Depressive Symptoms, Demographic Variables, and Marital Satisfaction in Mothers of Children with Mild Mental Retardation by Platt, Leslie Oliver, PhD from University of Georgia, 1993, 140 pages http://wwwlib.umi.com/dissertations/fullcit/9329828

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Design of an Agency to Provide Transition Services to Adult Life for People with Mental Retardation Within a Future Service Network in Bizkaia (spain) (spanish Text) by Martinez Rueda, Ignacio; Dr from Universidad De Deusto (spain), 1998, 573 pages http://wwwlib.umi.com/dissertations/fullcit/3034577

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Developing Instructional Decision Making Behaviors with Preservice Mental Retardation and Physical Handicap Teachers Utilizing an Interest Centered Approach: a Descriptive Study. by Iannaccone, Carmen James, EDD from State University of New York at Buffalo, 1976, 208 pages http://wwwlib.umi.com/dissertations/fullcit/7616314

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Development and Comparison of Person-environment Fit Models for People with Mental Retardation Placed in Supported Employment (french Text) by Chiocchio, Francois; PhD from Universite De Montreal (Canada), 2002, 351 pages http://wwwlib.umi.com/dissertations/fullcit/NQ71114

Dissertations 139

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Development and Training of Referential Communication in Children with Mental Retardation by Brownell, Marni Diane, PhD from The University of Manitoba (Canada), 1991 http://wwwlib.umi.com/dissertations/fullcit/f1381956

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Development of Procedures for the Driver Training of Individuals with Moderate Mental Retardation by Zider, Steven Jeffrey, PhD from University of Illinois at Urbanachampaign, 1979, 106 pages http://wwwlib.umi.com/dissertations/fullcit/8004314

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Differences among Older Mothers Caring at Home for Offspring with Mental Retardation by Use of Day Programming (home Care) by Fullmer, Elise Michelle, PhD from State University of New York at Albany, 1992, 178 pages http://wwwlib.umi.com/dissertations/fullcit/9319704

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Differences in Self-esteem and Depression among Parents of Individuals with Mental Retardation by Graham, Edward William, Dsw from Boston College, 1992, 124 pages http://wwwlib.umi.com/dissertations/fullcit/9232380

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Differential Diagnosis of the Learning Disabled (special Education, Mental Retardation) by John, Delores M., PhD from University of Missouri - Columbia, 1986, 90 pages http://wwwlib.umi.com/dissertations/fullcit/8701381

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Direct-care Staff and Their Supervisors' Perceptions of Job Satisfaction and Job Stress in Group Homes for Individuals with Mental Retardation by Hamilton, Leta C., EDD from Auburn University, 1995, 103 pages http://wwwlib.umi.com/dissertations/fullcit/9525357

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Discrimination of Emotion, Affective Perspective-taking and Empathy of Individuals with Mental Retardation by Moffatt, Courtney Wren, PhD from The University of Wisconsin - Madison, 1990, 171 pages http://wwwlib.umi.com/dissertations/fullcit/9020458

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Disproportionate Representation of African-american Students in Programs for Students with Mild Mental Retardation in the State of Alabama by Jackson, Patricia A., EDD from The University of Alabama, 1997, 158 pages http://wwwlib.umi.com/dissertations/fullcit/9735718

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Dream at Risk: Families of Young Adults with Mental Retardation Transitioning into Adult Services by Whelley, Teresa Anne, EDD from Peabody College for Teachers of Vanderbilt University, 1990, 185 pages http://wwwlib.umi.com/dissertations/fullcit/9113586

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Economics and Mental Retardation by Barnett, William Steven, PhD from The University of Michigan, 1982, 279 pages http://wwwlib.umi.com/dissertations/fullcit/8304437

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Educable Mental Retardation: Changes in Construct, Criteria and Population Parameters (mental Retardation) by Fuller, Chaulmaine Ballard, PhD from University of California, Los Angeles, 1991, 112 pages http://wwwlib.umi.com/dissertations/fullcit/9221867

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Effect of Practice Schedule Variation on the Acquisition, Retention, and Transfer of an Applied Motor Skill by Children with and without Mild Mental Retardation by Sutlive, Vinson H., Iii, PhD from Indiana University, 1996, 186 pages http://wwwlib.umi.com/dissertations/fullcit/9627050

140 Mental Retardation

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Effect of Training Direct Care Staff in Community Group Homes on Concepts Related to Choice for Individuals with Mental Retardation by Seay, Penelope Crews, PhD from The University of Texas at Austin, 1993, 181 pages http://wwwlib.umi.com/dissertations/fullcit/9323546

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Effectiveness of a Progressive Resistance Training Program on Work Productivity and Muscular Strength among Adult Males with Mental Retardation by Parker, Lorenzo; PhD from Michigan State University, 2001, 190 pages http://wwwlib.umi.com/dissertations/fullcit/3036726

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Effectiveness of Behavior Reduction Procedures Used with Individuals Who Are Severely Handicapped: a Meta-analysis (mental Retardation) by Baumtrog, Colleen Sue, PhD from University of Minnesota, 1985, 221 pages http://wwwlib.umi.com/dissertations/fullcit/8606208

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Effects of a Choice Program on the Reduction of Challenging Behaviors of People with Limited Intellectual Functioning (mental Retardation) by Ip, Sau Mei Vitti, PhD from The University of Wisconsin - Madison, 1991, 114 pages http://wwwlib.umi.com/dissertations/fullcit/9124675

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Effects of a Four-step Strategy on the Acquisition, Maintenance, and Generalization of Three Gross Motor Skills by Adolescents with Mild Mental Retardation by Yang, Jin Jin, PhD from The Ohio State University, 1997, 151 pages http://wwwlib.umi.com/dissertations/fullcit/9731751

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Effects of a Parent Training Program Involving Behavior Modification Techniques to Increase the Walking Behavior of Children with Mental Retardation: a Family Systems Approach by Stanton, Kathleen Ann, PhD from University of Virginia, 1995, 278 pages http://wwwlib.umi.com/dissertations/fullcit/9600475

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Effects of Ability Level and Age on Nonverbal Pragmatics in Students with and without Mental Retardation by Bufkin, Linda J., PhD from University of Missouri Columbia, 1988, 111 pages http://wwwlib.umi.com/dissertations/fullcit/8915297

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Effects of Adapted Bicycles Plus Feedback on the Acquisition, Maintenance, and Generalization of Conventional Cycling Skills for Children with Mild Mental Retardation by Burt, Tammy Lee; PhD from The Ohio State University, 2002, 191 pages http://wwwlib.umi.com/dissertations/fullcit/3049000

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Effects of Age, Iq, and Three Types of Song Instruction on Singing Accuracy among Children with Mental Retardation by Kim, Sook Won; PhD from Temple University, 2002, 98 pages http://wwwlib.umi.com/dissertations/fullcit/3057086

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Effects of an Increased Response Latency with or without Instruction in a Systematic Decision-making Technique on Interpersonal Problem-solving in Individuals with Mild Mental Retardation (social Skills Training) by Parker, Kathlyn, PhD from Columbia University, 1996, 192 pages http://wwwlib.umi.com/dissertations/fullcit/9616729

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Effects of Causal Cohesion in Stories on the Reading Comprehension Performance of Children with Mild Mental Retardation, Children with Learning-disabilities and Children without Disabilities by Wolman, Clara, PhD from University of Minnesota, 1990, 119 pages http://wwwlib.umi.com/dissertations/fullcit/9107469

Dissertations 141

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Effects of Icf-mr Program Services on the Adaptive Behavior of Residents of a Stateoperated Facility (mental Retardation) by Mazzagatti, Clare Eileen, PhD from Temple University, 1984, 66 pages http://wwwlib.umi.com/dissertations/fullcit/8509361

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Effects of Money Counting Fluency Training on the Acquisition and Generalization of Money Counting and Purchasing Skills by High School Students with Mental Retardation by Possi, Mwajabu Ali Kachenje Mlacha, PhD from The Ohio State University, 1994, 210 pages http://wwwlib.umi.com/dissertations/fullcit/9505275

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Effects of Reinforcement and Nonreinforcement on Total Task Presentation with Response Prompts of Increasing Assistance in Teaching Persons with Mental Retardation by Gold, Elizabeth Sharon; PhD from University of Missouri - Columbia, 2002, 197 pages http://wwwlib.umi.com/dissertations/fullcit/3052173

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Effects of the Special Olympics World Games on Attitudes of Nonhandicapped Children towards Persons with Mental Retardation by Shriver, Timothy Perry, PhD from The University of Connecticut, 1997, 74 pages http://wwwlib.umi.com/dissertations/fullcit/9730898

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Effects of Visual Arts Instruction on the Mental Health of Adults with Mental Retardation and Mental Illness by Malley, Sharon Mumford, EDD from University of Georgia, 1999, 193 pages http://wwwlib.umi.com/dissertations/fullcit/9929028

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Effects on Quality of Life in Adults with Mental Retardation and Behavior Disorders Following Multifaceted Intervention by Jackson, Terry Leonard, PhD from University of Missouri - Columbia, 1998, 153 pages http://wwwlib.umi.com/dissertations/fullcit/9904849

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Independence Behaviors of Young Children with Moderate to Severe Mental Retardation: Index of Developmental Gain by Abell, Natalie Cicero, PhD from The University of Toledo, 1989, 124 pages http://wwwlib.umi.com/dissertations/fullcit/9007977

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Individual Differences and Mild Mental Retardation: Cognitive Patterning Similarities among Children and Their Parents by Mcinerney, Maurice, PhD from The University of Wisconsin - Madison, 1983, 264 pages http://wwwlib.umi.com/dissertations/fullcit/8316225

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Individuals with Dual Diagnoses of Mental Retardation and Mental Illness in Illinois Community-based Vocational Programs: Services Provided and Staff Training Needs by Thomas, Janet Ressler, Rhd from Southern Illinois University at Carbondale, 1994, 114 pages http://wwwlib.umi.com/dissertations/fullcit/9516045

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Institutional Work and Self-esteem: the Direct-care Worker at a Facility for Clients with Mental Retardation by Abright, William Francis, Jr., PhD from The University of Texas at Austin, 1989, 228 pages http://wwwlib.umi.com/dissertations/fullcit/8920646

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Instructional Methods for Students with Mental Retardation in Tanzania by Mboya, Mary Wanjiku, PhD from University of Alberta (Canada), 1992, 325 pages http://wwwlib.umi.com/dissertations/fullcit/NN73048

142 Mental Retardation

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Integrating Developmentally Disabled Adults into the Community Work Force (social Distance, Mental Retardation) by Browder, Phyllis Meighen, PhD from Boston College, 1986, 302 pages http://wwwlib.umi.com/dissertations/fullcit/8701300

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Job Retention of Persons with Mental Retardation in Supported Employment by Trivelli, Lucy Ursula, PhD from Gallaudet University, 1994, 194 pages http://wwwlib.umi.com/dissertations/fullcit/9429769

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Job Tenure, Job Fit, and Extraversion/introversion in Men with Mild Mental Retardation Who Work in a Sheltered Workshop by Sinnreich, Karen Joy, PhD from New York University, 1995, 173 pages http://wwwlib.umi.com/dissertations/fullcit/9603304

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Kvce: a Kuwaiti Version of the Vineland Adaptive Behavior Scales-classroom Edition (adaptive Behavior, Mental Retardation) by Hamadah, Lulwah Nahabah, PhD from Indiana University, 1993, 179 pages http://wwwlib.umi.com/dissertations/fullcit/9404316

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Leadership Styles of Direct-care Supervisors in a Residential Facility for Persons with Mental Retardation by Egelston, Jeffrey David, EDD from Virginia Polytechnic Institute and State University, 1990, 180 pages http://wwwlib.umi.com/dissertations/fullcit/9028033

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Learning the Hard Way: Women Labeled with Mental Retardation Describe Their 'way of Knowing' by Sternfeld, Susan Lyeth, EDD from Boston University, 1993, 305 pages http://wwwlib.umi.com/dissertations/fullcit/9308143

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Leisure - Recreation Patterns and Social Networks of Young Adults with Moderate to Profound Levels of Mental Retardation by Steffens, Kathleen Mary, PhD from University of Minnesota, 1989, 197 pages http://wwwlib.umi.com/dissertations/fullcit/9019098

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Leisure Recreational Preferences and Practices among Adolescents with and without Mental Retardation by Mcdowell, Linda Lee Goette, PhD from The University of Southern Mississippi, 1995, 221 pages http://wwwlib.umi.com/dissertations/fullcit/9606316

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Level of Acculturation and Adjustment in Puerto Rican Youngsters with and without Siblings with Mental Retardation by Cloud, Nancy Lois, EDD from Columbia University Teachers College, 1993, 282 pages http://wwwlib.umi.com/dissertations/fullcit/9400544

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Life Satisfaction of Elderly Persons with Mental Retardation Living in Care Centers (well-being, Social Work, Aging) by Langford, Jane Edwards, Dsw from The University of Utah, 1985, 234 pages http://wwwlib.umi.com/dissertations/fullcit/8508433

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Management Walking Tours in a Residential Mental Retardation Center (active Treatment) by Woods, Allen Dale, EDD from The University of North Carolina at Chapel Hill, 1985, 69 pages http://wwwlib.umi.com/dissertations/fullcit/8605567

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Maternal Teaching Strategies of Mothers of Preschool Children with Mental Retardation by Powell, Marcene Lee, Dsw from The University of Utah, 1981, 344 pages http://wwwlib.umi.com/dissertations/fullcit/8208124

Dissertations 143

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Memory Performance in Substance Abusing Persons with Mental Retardation (substance Abuse) by Golden, Harriet, EDD from Columbia University Teachers College, 1993, 148 pages http://wwwlib.umi.com/dissertations/fullcit/9400560

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Memory Representation of Motor Skills in Individuals with Profound Mental Retardation by Choi, Seung-oh, PhD from Texas Woman's University, 1996, 293 pages http://wwwlib.umi.com/dissertations/fullcit/9630151

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Mental Retardation and Institutional Treatment in Nineteenth Century England, 1845-1886 by Gelband, Spencer Hugh, PhD from University of Maryland College Park, 1979, 569 pages http://wwwlib.umi.com/dissertations/fullcit/8016711

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Mental Retardation and William Faulkner's Ironic Vision by Herring, Gina, PhD from Auburn University, 1988, 494 pages http://wwwlib.umi.com/dissertations/fullcit/8825771

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Mental Retardation As a Function of Race, Sex and Social Economic Status by Chenault, Jonas, Jr., PhD from Michigan State University, 1970, 108 pages http://wwwlib.umi.com/dissertations/fullcit/7118185

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Mental Retardation Funds, an Analysis of Federal Policy by Braddock, David Lawrence, PhD from The University of Texas at Austin, 1973, 230 pages http://wwwlib.umi.com/dissertations/fullcit/7325980

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Mental Retardation in a Rural New England Area: the Use of Gatekeepers to Identify Retarded Persons in the Community. by Lagay, Bruce W., PhD from Brandeis University, 1973, 271 pages http://wwwlib.umi.com/dissertations/fullcit/7314561

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Mental Retardation Services Providers and the Massachusetts Quest: Characteristics Related to Quality of Life Outcomes by Foley, Susan Marilyn, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1999, 236 pages http://wwwlib.umi.com/dissertations/fullcit/9933826

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Mental Retardation: a Cluster Analytic Approach to Classification by Resavy, Charles Patrick, PhD from The Pennsylvania State University, 1991, 171 pages http://wwwlib.umi.com/dissertations/fullcit/9214257

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Metacognitive Processes and Attributional Training on Memory Tasks in Individuals with Mental Retardation by Kopfer, Paul Michael, PhD from State University of New York at Buffalo, 1993, 164 pages http://wwwlib.umi.com/dissertations/fullcit/9404829

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Mississippi Families' Perceptions of Two Types of Residential Services for Their Family Members with Mental Retardation by Mcinnis, Charles Michael, PhD from The University of Southern Mississippi, 1998, 40 pages http://wwwlib.umi.com/dissertations/fullcit/9901324

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Models of Bureaucratic Behavior: Sustaining Family Caregiving in Ohio's Mental Retardation and Developmental Disabilities Home Care Program by Fisher, Amber L.; Drph from University of Michigan, School of Public Health, 2002, 173 pages http://wwwlib.umi.com/dissertations/fullcit/3059070

144 Mental Retardation

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Modification of Mother-child Interaction Processes in Families at Risk for Mental Retardation by Slater, Mary Alice, PhD from The University of Wisconsin - Madison, 1980, 306 pages http://wwwlib.umi.com/dissertations/fullcit/8025854

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Newspaper Reports on Mental Retardation: Their Implications for Normalization. by Setzer, Grace White, PhD from Southern Illinois University at Carbondale, 1977, 146 pages http://wwwlib.umi.com/dissertations/fullcit/7716640

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Nonhandicapped Peer Tutors with Students with Moderate Mental Retardation: Examination of Changes in Both Tutors and Tutees by Donder, Daniel Jerome, Jr., PhD from University of Illinois at Urbana-champaign, 1986, 97 pages http://wwwlib.umi.com/dissertations/fullcit/8623283

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Nonverbal Communication and Mental Retardation: Comprehension and Expression of Facial Affect among Adults with Developmental Disabilities by Wilczenski, Felicia L., EDD from University of Massachusetts, 1989, 140 pages http://wwwlib.umi.com/dissertations/fullcit/8917423

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Old Order Amish Awareness and Understanding of Mental Retardation: a Religious Subcultural Approach to the Phenomenon by Melton, James Joe, PhD from The Ohio State University, 1970, 373 pages http://wwwlib.umi.com/dissertations/fullcit/7019343

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On the Threshold of Adult Life: Dis-course and Life Course of Mental Retardation in American Culture by Devlieger, Patrick Joseph, PhD from University of Illinois at Urbana-champaign, 1995, 306 pages http://wwwlib.umi.com/dissertations/fullcit/9543568

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Organizational Field Determinants of the Internal Structure of Organizations: a Study of Community Home Programs (pennsylvania, Mental Retardation) by Spencer, Robert Chester, PhD from Temple University, 1994, 348 pages http://wwwlib.umi.com/dissertations/fullcit/9434743

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Organizational Performance of Nonprofit Providers of Mental Retardation Services in a Purchase of Service Economy (massachusetts) by Regan, Paul S., EDD from University of Lowell, 1991, 178 pages http://wwwlib.umi.com/dissertations/fullcit/9217512

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P.d.e.: Foster Parents for Persons with Mental Retardation. Motivation and Role Perceptions, and Their View of the Foster Person (mental Retardation, Motivation) by Duvdevany, Ilana, PhD from The Union Institute, 1992, 114 pages http://wwwlib.umi.com/dissertations/fullcit/9305320

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Parental Adaptation to Mental Retardation (stress, Counseling, Family, Emr, Tmr) by Chiappelli, Francesco, PhD from University of California, Los Angeles, 1986, 265 pages http://wwwlib.umi.com/dissertations/fullcit/8621040

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Parental Caregivers of Adults with Mental Retardation: the Experience of Older Mothers and Fathers by Essex, Elizabeth Anne, PhD from The University of Wisconsin Madison, 1998, 215 pages http://wwwlib.umi.com/dissertations/fullcit/9837038

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Parental Perceptions of Mental Retardation As a Disability: a Case Study of Cali, Colombia by Cuadros, Jose Hermann; EDD from Columbia University Teachers College, 2002, 176 pages http://wwwlib.umi.com/dissertations/fullcit/3067328

Dissertations 145

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Parental Reactions to the Affiliational Stigma of Mental Retardation. by Schmid, Thomas John, PhD from University of Minnesota, 1977, 398 pages http://wwwlib.umi.com/dissertations/fullcit/7719036

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Parents of Children with Mental Retardation Living in Taiwan: an Analysis of Service Needs and Perceived Stress by Mar, Chun-lin, PhD from University of Northern Colorado, 1996, 235 pages http://wwwlib.umi.com/dissertations/fullcit/9722316

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Parents' Perceptions of the Development of Strengths and the Role of Religious Belief in Coping with a Child's Mental Retardation by Riordan, Judy Stump, PhD from The University of Nebraska - Lincoln, 1993, 188 pages http://wwwlib.umi.com/dissertations/fullcit/9331427

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Parents' Perspective Before, During, and after Initial Diagnoses of Children's Mental Retardation (labeling, Handicapped, Diagnostic Evaluations) by Glascoe, Frances Page, PhD from Vanderbilt University, 1986, 302 pages http://wwwlib.umi.com/dissertations/fullcit/8616353

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Participation of Adults with Mental Retardation in a Voluntary Physical Activity Program by Stanish, Heidi Isabel, PhD from Oregon State University, 1999, 85 pages http://wwwlib.umi.com/dissertations/fullcit/9921265

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Pathways to Choice: Organizational Factors Related to Choice for Adults with Mental Retardation and Other Developmental Disabilities by Gasstrom, Lisa L., Dsw from Adelphi University, School of Social Work, 1998, 249 pages http://wwwlib.umi.com/dissertations/fullcit/9828147

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Patterns of Motivational Orientation, Occupational Knowledge, and Vocational Interests As They Relate to Demographic Variables of Workers with Mental Retardation in Israeli Sheltered Workshops (sheltered Workshops) by Friedman, Luba, PhD from Michigan State University, 1989, 196 pages http://wwwlib.umi.com/dissertations/fullcit/9011987

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Perceived Physical and Actual Motor Competence in Korean Children with Mild Mental Retardation: Relationship to Age, Gender, and Parental Physical Activity by Kim, Ji-tae; PhD from Michigan State University, 2003, 174 pages http://wwwlib.umi.com/dissertations/fullcit/3092166

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Perceptions of Functional Communication Needs for Elderly Persons with Mental Retardation by Green, Twhanna Jones, PhD from Howard University, 1992, 289 pages http://wwwlib.umi.com/dissertations/fullcit/9239172

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Perceptions of Parents, Teachers, Special Education Administrators, State Department Personnel, and Special Education Teacher Educators Regarding Ieps with Respect to Pl 94-142 (mental Retardation) by Wilson, Rebecca Mack, PhD from The University of Southern Mississippi, 1985, 106 pages http://wwwlib.umi.com/dissertations/fullcit/8600730

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Perceptions of Persons with Mental Retardation Regarding Inclusion in Death and Dying Rituals by Wagner, Paulette Marie; PhD from The University of North Dakota, 2002, 139 pages http://wwwlib.umi.com/dissertations/fullcit/3058852

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Perceptions of Teachers of Mental Retardation Regarding Their Preparation Program at King Saud University in Saudi Arabia by Althabet, Ibrahim N.; PhD from University of South Florida, 2002, 142 pages http://wwwlib.umi.com/dissertations/fullcit/3071290

146 Mental Retardation

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Perceptual Differences between Administrators of Mental Retardation Training Centers and Professors of Special Education in Virginia Concerning the Importance of Special Education Teacher Competencies. by Johnson, Dallas M., EDD from University of Virginia, 1978, 140 pages http://wwwlib.umi.com/dissertations/fullcit/7916328

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Performing the Community, Telling the Self: Storytelling by Members of a Group Home for Men with Mental Retardation by Croft, Sharon Elizabeth, PhD from The Louisiana State University and Agricultural and Mechanical Col., 1995, 391 pages http://wwwlib.umi.com/dissertations/fullcit/9613416

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Personal Futures Planning: Finding Directions for Change (mental Retardation Services) by Mount, Beth Ann, Dpa from University of Georgia, 1987, 275 pages http://wwwlib.umi.com/dissertations/fullcit/8724642

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Perspectives on the Friendships of a School-aged Child with Mental Retardation in an Inclusive Classroom by Johnson, Danny Evan, PhD from The University of North Carolina at Chapel Hill, 1996, 229 pages http://wwwlib.umi.com/dissertations/fullcit/9715719

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Physical Activity Levels of Students with Mental Retardation and Students without Disabilities by Hodge, Jennifer Faison; PhD from The Ohio State University, 2001, 161 pages http://wwwlib.umi.com/dissertations/fullcit/3011071

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Physicians' Understanding of Mental Retardation and Their Advice to Parents of Mentally Retarded Children. by Connaughton, Mary Carole, PhD from Indiana University, 1974, 240 pages http://wwwlib.umi.com/dissertations/fullcit/7422760

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Physiological Responses of Individuals with Severe/profound Mental Retardation and Multiple Disabilities to Assisted Exercise by Baggett-mcminn, Sheri, EDD from The University of Southern Mississippi, 1995, 125 pages http://wwwlib.umi.com/dissertations/fullcit/9615272

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Planning for Adult Futures: Parents' Self Report of Future Planning and Its Relationship to Family Functioning and Stress with Sons and Daughters Who Are Disabled (Physical Disability, Mental Retardation, Launching) by Brotherson, Mary Jane, PhD from University of Kansas, 1985, 225 pages http://wwwlib.umi.com/dissertations/fullcit/8529066

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Planning Residential Environments with Persons with Mental Retardation by Murray, William James, PhD from The University of Wisconsin - Milwaukee, 1996, 260 pages http://wwwlib.umi.com/dissertations/fullcit/9715462

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Post-secondary School Adjustment Problems of Persons with Mild Mental Retardation As Perceived by Rehabilitation Counselors and Special Education Teachers by Lopera, Egidio E., PhD from The University of Wisconsin - Madison, 1982, 186 pages http://wwwlib.umi.com/dissertations/fullcit/8304957

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Present Practices and Future Directions in Classification, Eligibility Determination, and Service Availability for Mentally Retarded Persons: a Survey of State Mental Retardation Program Directors by Lowitzer, Arthur C., PhD from Vanderbilt University, 1986, 78 pages http://wwwlib.umi.com/dissertations/fullcit/8703884

Dissertations 147

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Prevalence of Mental Retardation among School Populations in Pima County, Arizona. by Jipson, Frederick Jerome, EDD from The University of Arizona, 1975, 114 pages http://wwwlib.umi.com/dissertations/fullcit/7601406

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Primary Caregivers' Current and Future Expectations of Long-term Planning for Their Children with Mental Retardation in Taiwan (china) by Chen, Shu-yu; PhD from The Pennsylvania State University, 2000, 113 pages http://wwwlib.umi.com/dissertations/fullcit/9998307

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Progress in Mental Retardation According to the Content of Textbooks Published between 1960 and 1980 by Nichols, Mentorry Debose, PhD from The University of Oklahoma, 1982, 89 pages http://wwwlib.umi.com/dissertations/fullcit/8215793

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Promoting Choice Behavior among Adults with Mental Retardation: a Program of Staff Training by Staten, Lillian Frankart; PhD from University of Virginia, 2000, 173 pages http://wwwlib.umi.com/dissertations/fullcit/9954436

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Psychological Distress and Parenting by Mothers with Mental Retardation by WaltonAllen, Nicole, PhD from University of Toronto (Canada), 1993, 247 pages http://wwwlib.umi.com/dissertations/fullcit/NN86365

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Public Attitudes toward Community Placement for People with Severe Mental Retardation in Taipei, Taiwan (China) by Tsuang, Mai-feng, PhD from University of Virginia, 1992, 115 pages http://wwwlib.umi.com/dissertations/fullcit/9324886

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Public Law 94-142, Ohio A.s.h.b. 455, Ohio County Boards of Mental Retardation and Developmental Disabilities: Implementation and Compliance--a Field Study by Burkhardt, Douglas Allen, PhD from Kent State University, 1981, 97 pages http://wwwlib.umi.com/dissertations/fullcit/8128484

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Public School Administrators' Perception of the Difficulties in Implementing the Education for All Handicapped Children Act of P.l. 94-142, 1975 (mental Retardation, Special) by Jones-kolb, Donna Omenia, EDD from The University of Akron, 1986, 185 pages http://wwwlib.umi.com/dissertations/fullcit/8613795

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Puerto Rican Mothers of Adults with Mental Retardation: the Impact of Cultural Values on Lifelong Caregiving by Magana, Sandra Marie, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1999, 151 pages http://wwwlib.umi.com/dissertations/fullcit/9920123

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Quality Boardmanship: Analysis and Comparison of Ohio County Boards of Mental Retardation and Developmental Disabilities, Corporate, School, and Third-sector Boards (boards of Directors) by Maynard, Patrick Richard, PhD from Ohio University, 1995, 168 pages http://wwwlib.umi.com/dissertations/fullcit/9522989

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Quality of Life and the Work Environment: the Relationship between Integration in the Work Environment and Quality of Life As Perceived by Individuals with Mental Retardation by Winer, John Joseph; PhD from Yeshiva University, 2000, 178 pages http://wwwlib.umi.com/dissertations/fullcit/9973143

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Quality of Life Assessment in Adults with Mental Retardation in the Basque Autonomic Community (Spain) by Arostegi Barandika, Igone; Dr from Universidad De Deusto (Spain), 1998, 581 pages http://wwwlib.umi.com/dissertations/fullcit/3034790

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Racial Affiliation of African-americans and Caucasian Americans with Mental Retardation in Group Home Placement: Implications for Program Practice by Edwards, Dothel Wilson, Jr., Rhd from Southern Illinois University at Carbondale, 1999, 140 pages http://wwwlib.umi.com/dissertations/fullcit/9925859

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Reasoning about Mental Retardation: a Study of the American Association on Mental Deficiency by Trent, James Wilson, Jr., PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1982, 284 pages http://wwwlib.umi.com/dissertations/fullcit/8222732

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Recovery House: Residential Facility for Persons with Mild Mental Retardation and Substance Dependence (self-esteem) by Bellows, John Thomas, PhD from The Union Institute, 1995, 276 pages http://wwwlib.umi.com/dissertations/fullcit/9608746

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Recreation and Leisure Programming at the High School Level for Individuals with Mild or Moderate Mental Retardation by Nisbet, Rebecca Seal, PhD from The University of Southern Mississippi, 1994, 75 pages http://wwwlib.umi.com/dissertations/fullcit/9508992

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Recreation/leisure Preferences of Senior Citizens with and without Mental Retardation/developmental Disabilities in South Mississippi (mental Retardation, Disabilities) by Glausier, Sheryl Robertson, PhD from The University of Southern Mississippi, 1994, 105 pages http://wwwlib.umi.com/dissertations/fullcit/9508976

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Relating Staff Attitudes to the Development of a Comprehensive Sexual Policy for the Institutionalized Residents with Mental Retardation by Striar, Sharna Lieberman, PhD from The University of Michigan, 1981, 130 pages http://wwwlib.umi.com/dissertations/fullcit/8125209

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Relationship between Depressive Symptomatology and Work Behaviors of Persons with Mild and Moderate Mental Retardation by Webne-behrman, Lisa Ziatz, PhD from The University of Wisconsin - Madison, 1989, 113 pages http://wwwlib.umi.com/dissertations/fullcit/8916452

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Relationship between Quality of Life for Adults with Mental Retardation and Type of Job Placement by Zapata, Patricia Ann; PhD from The University of Texas at Austin, 2000, 137 pages http://wwwlib.umi.com/dissertations/fullcit/9983609

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Relationship of Residence and Employment on the Quality of Life of Individuals with Mild or Moderate Mental Retardation by Song, Jimin, PhD from The University of Texas at Austin, 1997, 156 pages http://wwwlib.umi.com/dissertations/fullcit/9803032

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Relationships between Cognitive Academic Gain and Dependency-independency Characteristics of Learners and the Degree of Learner Freedom in an Individualized University Course in Mental Retardation by Drucker, Howard, PhD from The Florida State University, 1972, 162 pages http://wwwlib.umi.com/dissertations/fullcit/7300187

Dissertations 149

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Residential Arrangement, Level of Social Support and Depressive Symptomatology in Young Adult Males with Mental Retardation (Men) by Flanagan, Kenneth Martin, PhD from The Ohio State University, 1994, 131 pages http://wwwlib.umi.com/dissertations/fullcit/9420955

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Rival Concepts of Mental Retardation in the Evolution of Special Classes for Students Who Are Educable Mentally Retarded (1900-1975) by Connors, Dennis Michael, EDD from Columbia University Teachers College, 1988, 191 pages http://wwwlib.umi.com/dissertations/fullcit/8824357

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Role Perception of Individuals Monitoring Deinstitutionalization Consent Decrees (Mental Retardation) by Zeffiro-Krenisky, Denise Marie, EDD from University of Massachusetts, 1990, 259 pages http://wwwlib.umi.com/dissertations/fullcit/9035423

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Role-analysis of Resource Teachers of Children with Learning Disabilities and Educable Mental Retardation. by McLoughlin, James Alexander, PhD from The University of Arizona, 1973, 213 pages http://wwwlib.umi.com/dissertations/fullcit/7412440

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School Environments for the Profoundly Handicapped: a Study of Teachers' Definitions, Decisions, and Classroom Activity Structures (Mental Retardation, Effective Classrooms, Teacher-training) by McDaid, Janet Litwinowich, PhD from The Claremont Graduate University, 1986, 297 pages http://wwwlib.umi.com/dissertations/fullcit/8607833

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Self-esteem and Job Satisfaction of Adults with Mild Mental Retardation in Sheltered Workshops and Supported Employment Programs (Adult Mental Retardation) by Griffin, David Kenneth, EDD from Florida International University, 1993, 127 pages http://wwwlib.umi.com/dissertations/fullcit/9401930

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Self-monitoring and Its Effects on the Performance of Staff in a Residential Setting (Mental Retardation, Self-management) by Richman, Gina Susan, PhD from The Florida State University, 1986, 108 pages http://wwwlib.umi.com/dissertations/fullcit/8625790

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Settling into a Community Residence: a Case Study of Adults with Mental Retardation (Deinstitutionalization) by Winczer, Alice N., PhD from Temple University, 1993, 128 pages http://wwwlib.umi.com/dissertations/fullcit/9408833

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Sheltered, Supported and Competitive Employment Settings: the Relationship of the Individual Ability of Adults with Mental Retardation to Their Level of Vocational Placement by Rubinsky, Steven J., PhD from New York University, 1989, 159 pages http://wwwlib.umi.com/dissertations/fullcit/8916039

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Short-term Memory of Mildly Retarded and Nonretarded Students under Conditions of Restricted Cognitive Strategy Use (mental Retardation, Special Needs, Cognitive Training, Memory Limitation) by Katims, David Stuart, EDD from Boston University, 1986, 223 pages http://wwwlib.umi.com/dissertations/fullcit/8609279

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Siblings' Joint Leisure Activities and Their Correlates in Children with and without Mental Retardation by Wilkins, Vicki Louise, PhD from The Pennsylvania State University, 1988, 154 pages http://wwwlib.umi.com/dissertations/fullcit/8826839

150 Mental Retardation

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Siblings of the Handicapped: Stigmatization and Self-concept As a Function of Selected Variables (mental Retardation) by Zuch, Elizabeth Berman, PhD from University of Pennsylvania, 1984, 151 pages http://wwwlib.umi.com/dissertations/fullcit/8417379

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Social Change and Social Context: a Case Study of the International League of Societies for Persons with Mental Handicap (ilsmh) (mental Retardation) by Prudent, Ella Susanna, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1990, 296 pages http://wwwlib.umi.com/dissertations/fullcit/9100465

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Social Class and Mental Retardation: Educational, Vocational, and Social Differences of Young Adult Mentally Retarded Males. by Pearson, Dorothy Marie, PhD from The University of Wisconsin - Madison, 1973, 162 pages http://wwwlib.umi.com/dissertations/fullcit/7330339

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Social Interpersonal Problem-solving and Culture: the Effect of General Cognitive Strategy Training among Students with Mild Mental Retardation by Edeh, Ojoma Mary, PhD from Columbia University, 1998, 208 pages http://wwwlib.umi.com/dissertations/fullcit/9838915

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Social Skills Training with Adults with Mental Retardation: an Analysis of the Ability of Adults with Mental Retardation to Perceive and Interpret Non-verbal Facial Expression by Gumpel, Thomas Peter, PhD from University of California, Berkeley with San Francisco State Univ., 1993, 139 pages http://wwwlib.umi.com/dissertations/fullcit/9407855

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Social Workers Who Work with People Who Have Mental Retardation/Developmental Disabilities: a Qualitative Study Exploring Processes of Entry and Continuation in the Field by Russo, Rosalie (rose) J.; DSW from City University of New York, 2002, 340 pages http://wwwlib.umi.com/dissertations/fullcit/3037438

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Socially Shared Expertise in Groups with Mental Retardation by Stough, Laura Michelle, PhD from The University of Texas at Austin, 1993, 244 pages http://wwwlib.umi.com/dissertations/fullcit/9401002

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Special and General Educators' Perceptions of the Relevance of Leader Behavior in Managing Programs for Students with Mental Retardation in Integrated and Nonintegrated Classrooms (Special Educators, Integrated Classrooms) by Frattura Kampschroer, Elise Marie, PhD from The University of Wisconsin - Madison, 1990, 366 pages http://wwwlib.umi.com/dissertations/fullcit/9034834

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Sport Program Effects on Participants with Mental Retardation (special Olympics) by Riggen, Katherine Joyce, PED from Indiana University, 1992, 160 pages http://wwwlib.umi.com/dissertations/fullcit/9314622

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Staff Perceptions of the Quality of Life for Individuals with Mental Retardation Living in an Institutional Setting and for Those Living in a Community Setting by Lassiter, Suzie Murray, PhD from The University of Southern Mississippi, 1998, 106 pages http://wwwlib.umi.com/dissertations/fullcit/9916035

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Staff Training: How to Work with Individuals Who Have Mental Retardation and Autism by Standage, Kristen, EDD from Texas A&M University, 1991, 151 pages http://wwwlib.umi.com/dissertations/fullcit/9133899

Dissertations 151

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State Employment Policies: Assessing the Impacts on People with Significant Disabilities (Employment, Mental Retardation) by Drum, Charles Everet, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1995, 246 pages http://wwwlib.umi.com/dissertations/fullcit/9537806

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Structural Organization of Cognitive Processes in Adults with Mental Retardation (Adult Mental Retardation) by Mclaren, Kevin Patrick, PhD from University of Virginia, 1991, 156 pages http://wwwlib.umi.com/dissertations/fullcit/9223388

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Study of the 1959, 1960, and 1961 Summer On-campus Practicum Courses in Mental Retardation at Teachers College, Columbia University by Zinicola, Silvio Gennaro, EDD from Columbia University, 1968, 183 pages http://wwwlib.umi.com/dissertations/fullcit/6900685

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Supervisors' Performance Ratings Correlated with Selected Personal Characteristics of Attendants in a Mental Retardation Developmental Center (residential, Administration, Personnel) by Frederick, Joseph Bernard, PhD from Bowling Green State University, 1984, 163 pages http://wwwlib.umi.com/dissertations/fullcit/8428081

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Supported Employment As a Vocational Option for Khmers with Mental Retardation Living in a Displaced Persons Camp Along the Thai-cambodian Border (Cambodia) by Brown, Sharan Elizabeth, EDD from University of Washington, 1991, 139 pages http://wwwlib.umi.com/dissertations/fullcit/9203243

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Taking Control Through Self-determination: the Management of Personal Lifestyles by Adults with Mental Retardation by Arnett, Scott Lynn, PhD from The University of Oklahoma, 1997, 211 pages http://wwwlib.umi.com/dissertations/fullcit/9721051

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Teachers' Educational Philosophies and Beliefs about the Nature of Mental Retardation (teachers' Belief) by Bond, Lori Elizabeth, PhD from University of Pennsylvania, 1992, 168 pages http://wwwlib.umi.com/dissertations/fullcit/9235113

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Teachers' Professional Preparation, Experience, and Work Setting As Determinants of Their Knowledge and Attitude toward Mental Retardation and Mainstreaming by Shaw, Rex Frederick, EDD from Columbia University Teachers College, 1980, 109 pages http://wwwlib.umi.com/dissertations/fullcit/8022157

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Teaching Adolescents with Moderate Mental Retardation to Identify Food Pictures/words: a Study of Prompt Dependency by King, Diane, PhD from Lehigh University, 1991, 136 pages http://wwwlib.umi.com/dissertations/fullcit/9207018

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Teaching Archaeological Skills and Knowledge to Adults with Mental Retardation by Gittings, James St. Clair, PhD from The University of Arizona, 1996, 190 pages http://wwwlib.umi.com/dissertations/fullcit/9706154

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Teaching Mental Abacus Calculation to Students with Mental Retardation by Shen, Hong; PhD from University of Illinois at Urbana-Champaign, 1999, 143 pages http://wwwlib.umi.com/dissertations/fullcit/9944996

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Teaching Sight Word Recognition to Young Children with Mild to Moderate Mental Retardation Through Interactive Multimedia by Lee, Yeunjoo; PhD from University of Georgia, 2001, 155 pages http://wwwlib.umi.com/dissertations/fullcit/3025335

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Testicular Volume and Its Correlation with the Cytogenetics of Mental Retardation by Kirkilionis, Angelika J; PhD from The University of Western Ontario (Canada), 1985 http://wwwlib.umi.com/dissertations/fullcit/NL20723

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The Ability of Special Education Teachers of Emr Students to Distinguish between Behavioral Examples Descriptive of Mental Retardation and Behavioral Examples Descriptive of Traditional Black Culture by Ford, Bridgie Alexis, PhD from Purdue University, 1983, 160 pages http://wwwlib.umi.com/dissertations/fullcit/8400353

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The Ability to Identify Facial Expressions of Emotions: Emotion-specific Deficit or Construct of Mental Retardation by Eckert, Stephen Paul; PhD from The University of New Mexico, 1999, 84 pages http://wwwlib.umi.com/dissertations/fullcit/9938974

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The Attitudes and Perceptions of Parents/Guardians, Work Supervisors, and Employers/Coworkers toward People with Moderate, Severe, and Profound Mental Retardation in Integrated and Sheltered Work Environments by Rogan, Patricia M., PhD from The University of Wisconsin - Madison, 1987, 208 pages http://wwwlib.umi.com/dissertations/fullcit/8723354

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The Attitudes of Special Education Professionals and Parents/guardians toward Integrating Students with Mild, Moderate, and Severe/profound Mental Retardation into Regular Education Buildings, Neighborhood Schools, Recesses, Lunches, Assemblies, Nonacadem by Zanella Albright, Kathy A., PhD from The University of Wisconsin - Madison, 1987, 564 pages http://wwwlib.umi.com/dissertations/fullcit/8800393

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The Community Planning and Coordinating Role of the Mental Retardation/developmental Disabilities Area Program Boards of California. by Aikens, Inez McCabe, PhD from United States International University, 1974, 186 pages http://wwwlib.umi.com/dissertations/fullcit/7420501

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The Comparable Effects of Spanish and English Instruction on Sight Word Reading Using a Constant Time Delay and Incidental Teaching Procedures by Hispanic Learners with Mental Retardation by Rohena-Diaz, Elba I., EDD from Lehigh University, 1998, 179 pages http://wwwlib.umi.com/dissertations/fullcit/9814985

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The Development of Self-esteem in Young Adolescents with Mild Mental Retardation by Long, Carol Ann, PhD from University of Missouri - Columbia, 1995, 133 pages http://wwwlib.umi.com/dissertations/fullcit/9705246

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The Effect of a Cognitive-process Approach on Training Students with Moderate Intellectual Disabilities to Request Assistance on Community-based Vocational Instruction Sites (Mental Retardation, Social Skills Training) by Arnold, Susan Elizabeth, PhD from Georgia State University, 1995, 97 pages http://wwwlib.umi.com/dissertations/fullcit/9524235

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The Effect of a Modified Pinball Video Game on Time-on-task and Successful Hitting Percentage in Adolescents with Moderate to Severe Mental Retardation by An, Yi-Ning Michael, PhD from The Ohio State University, 1995, 129 pages http://wwwlib.umi.com/dissertations/fullcit/9612144

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The Effect of Bimodally Cued Instruction into Which Memory Strategy Training Has Been Integrated on Decoding Skill Acquisition, Maintenance, and Generalization in

Dissertations 153

Students with Mild Mental Retardation by Bursky, Lynne, EDD from Columbia University Teachers College, 1995, 251 pages http://wwwlib.umi.com/dissertations/fullcit/9539782 •

The Effect of Differences in Curricula and Experiences on Social Work Student Attitudes and Knowledge about Mental Retardation by Begab, Michael Jay, PhD from The Catholic University of America, 1968, 147 pages http://wwwlib.umi.com/dissertations/fullcit/6909110

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The Effect of Duration on Perceived Exertion during Exercise in Adults with Mental Retardation by Barber, George Daniel, EDD from University of Kentucky, 1995, 124 pages http://wwwlib.umi.com/dissertations/fullcit/9614448

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The Effect of Expectations on the Task Performance of Vocational Trainees with Mental Retardation by Allen, Bradley Paul, PhD from Marquette University, 1988, 112 pages http://wwwlib.umi.com/dissertations/fullcit/8925430

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The Effect of Perceived Mental Retardation on Behavior Implication by Persons of High and Low Distinction from Mentally Retarded Individuals by Crapps, John Maury, PhD from University of Georgia, 1986, 66 pages http://wwwlib.umi.com/dissertations/fullcit/8621630

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The Effect of Problem Content, Semantic Category, and Difficulty in the Solution of Arithmetic Word Problems by Learners with and without Mental Retardation by Friedman, Laura Waterman, PhD from Columbia University, 1991, 102 pages http://wwwlib.umi.com/dissertations/fullcit/9127855

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The Effect of Social Problem-Solving Training for Individuals with Mental Retardation by Crites, Steven Aaron; PhD from Auburn University, 2001, 130 pages http://wwwlib.umi.com/dissertations/fullcit/3016087

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The Effect of Varying Levels of Overlearning on the Acquisition and Retention of the Overhand Throw for Children with and without Mental Retardation by Gillespie, Michael Keith; PhD from The Ohio State University, 1999, 112 pages http://wwwlib.umi.com/dissertations/fullcit/9951658

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The Effect upon Mental Retardation Facility Employee Job Performance of Defining Client Service Job Duties by Specific Behavioral Parameters and Providing Written Performance Feedback by Titus, Sharon Kay, EDD from University of Florida, 1987, 121 pages http://wwwlib.umi.com/dissertations/fullcit/8724973

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The Effectiveness of a Progressive Time Delay Procedure on Teaching Gross Motor Skills to Individuals with Moderate/severe Mental Retardation by Chen, Shihui, PhD from The University of New Mexico, 1997, 118 pages http://wwwlib.umi.com/dissertations/fullcit/9736098

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The Effectiveness of Group Social Skills Training and Residential Staff Facilitated Homework Assignments As a Treatment for Depression in Adults with Mild Mental Retardation by Pondillo, Albert Michael, PhD from Mississippi State University, 1993, 101 pages http://wwwlib.umi.com/dissertations/fullcit/9400502

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The Effectiveness of Leisure Education and Classroom Recreation Participation on Social and Communication Skills, Community Living Skills, and Problem Behaviors

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of Students with Mental Retardation by Stone, Charlsena Faye, PhD from The University of North Carolina at Chapel Hill, 1998, 149 pages http://wwwlib.umi.com/dissertations/fullcit/9914914 •

The Effectiveness of Systematic Densensitization, in Vivo Desensitization, and Modeling in Reducing Dental Anxiety in Patients with Mental Retardation by Tabor, Mary Anne; Psyd from University of San Francisco, 2003, 117 pages http://wwwlib.umi.com/dissertations/fullcit/3090230

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The Effects a Sibling with Mental Retardation Has on the Sibling Relationship by Johnsey, Anita Colleen; EDD from The University of Southern Mississippi, 2000, 124 pages http://wwwlib.umi.com/dissertations/fullcit/3000244

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The Effects of a Differential Reinforcement of Low Rates of Responding Intervention on Behaviors of Adults with Autism during Vocational Training at a Competitive Job Site (severely Handicapped, Mental Retardation, Employment, On-the-job Training, Behavio by McCarthy, Patricia Ann Talbert, EDD from University of South Carolina, 1984, 174 pages http://wwwlib.umi.com/dissertations/fullcit/8508188

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The Effects of a Self-instructional Training Program Taught in a Group Instructional Format on the Acquisition and Maintenance of Vocational Skills of Students with Mental Retardation by Moore, Stephen Clark, PhD from Utah State University, 1989, 167 pages http://wwwlib.umi.com/dissertations/fullcit/9015023

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The Effects of Contingent Reinforcement on the Exercise Compliance of Adults with Mental Retardation during a Bicycle Ergometer Training Routine by Mathieson, James Andrew, PhD from University of Virginia, 1991, 185 pages http://wwwlib.umi.com/dissertations/fullcit/9218735

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The Effects of Educational Placement on Academic Achievement, Social Maturity, and Motivation for Students with Mild Mental Retardation by Emery, Sandra Lynn, PhD from University of Kansas, 1997, 86 pages http://wwwlib.umi.com/dissertations/fullcit/9817092

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The Effects of Instructional Mode on the Skill Acquisition of a Recreation/leisure Task by Elementary Students with Severe Mental Retardation by Burroughs, Edythe Louise, DED from Ball State University, 1992, 292 pages http://wwwlib.umi.com/dissertations/fullcit/9222619

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The Effects of Participation in Selected Dance Activities on Self-efficacy and Movement Skills in Children with Educable Mental Retardation by Newnam, Hollie Marie; PhD from The Florida State University, 2001, 112 pages http://wwwlib.umi.com/dissertations/fullcit/3034059

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The Effects of Peer-delivered Social Skills Training on Social Interaction Skills of High School Students with Mental Retardation by Hall, Stacey Victoria, PhD from Vanderbilt University, 1997, 177 pages http://wwwlib.umi.com/dissertations/fullcit/9815598

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The Effects of Peer-mediated Reinforcement on the Generalization of Task Performance and Social Interactions of Adults with Mental Retardation and Behavior Problems by Ryall, Colleen Anne, EDD from University of Kentucky, 1993, 156 pages http://wwwlib.umi.com/dissertations/fullcit/9402849

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The Effects of Response Variation Training Procedures on the Generalized Performance of a Laundry Folding Task by Persons with Mental Retardation by Manlove, Christine, EDD from The Johns Hopkins University, 1988, 163 pages http://wwwlib.umi.com/dissertations/fullcit/8908130

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The Effects of Selected Leisure Variables on the Independent Leisure Functioning of Adults with Mental Retardation Who Participate in Two North Carolina Community College Compensatory Education Programs by Carter, Sharon Elizabeth, EDD from North Carolina State University, 1995, 145 pages http://wwwlib.umi.com/dissertations/fullcit/9526912

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The Effects of Self-instructional Training in Improving Time-telling Performance of Individuals with Mental Retardation by Kim, Jin-ho, PhD from Vanderbilt University, 1997, 131 pages http://wwwlib.umi.com/dissertations/fullcit/9724969

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The Effects of Simulation Game Play upon Oral Language Development and Internalization of Locus of Control among Mildly Handicapped Adolescents (emotional Disturbance, Mental Retardation, Learning Disabilities) by Kallam, Michael L., PhD from Oklahoma State University, 1984, 88 pages http://wwwlib.umi.com/dissertations/fullcit/8427677

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The Effects of Specific Interventions with Supervisors on Paraprofessional Turnover in Selected Mental Health and Mental Retardation Facilities by Baxter, Nick A., PhD from University of North Texas, 1981, 144 pages http://wwwlib.umi.com/dissertations/fullcit/8128257

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The Effects of Three Testing and Training Modalities on Exercise Independence and Sit-ups Performance among Children with Severe Mental Retardation by Wallstrom, Timothy James, PhD from The Ohio State University, 1993, 245 pages http://wwwlib.umi.com/dissertations/fullcit/9401380

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The Effects of Video-assisted Training on Employment-related Social Skills of Adults with Severe Mental Retardation (retarded Adults) by Morgan, Robert L., PhD from Utah State University, 1991, 187 pages http://wwwlib.umi.com/dissertations/fullcit/9132783

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The Effects of Videotaped Modeling on the Acquisition, Performance, and Generalization of Job-related Social Skills in Adults with Mental Retardation Living in Group Homes by Sundel, Sandra S., PhD from The University of Texas at Arlington, 1990, 326 pages http://wwwlib.umi.com/dissertations/fullcit/9033494

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The Feebleminded Female: an Historical Analysis of Mental Retardation As a Social Definition, 1890-1920. by Bragar, Madeline C., PhD from Syracuse University, 1977, 177 pages http://wwwlib.umi.com/dissertations/fullcit/7813297

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The History of Federal Policy toward Mental Retardation by Moore, Linda Ann, PhD from University of California, Santa Barbara, 1990, 531 pages http://wwwlib.umi.com/dissertations/fullcit/9104517

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The Impact of Parenting Stress and Social Support on Psychological Well-being of Korean Mothers of Children with Mental Retardation by Lim, Hyun Sung; PhD from University of South Carolina, 2002, 159 pages http://wwwlib.umi.com/dissertations/fullcit/3059454

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The Impact of Systems-level Policies and Practices on Integrated Employment Programming for Persons with Mental Retardation and Other Developmental Disabilities by Connolly, Patricia M., PhD from Boston College, 1999, 284 pages http://wwwlib.umi.com/dissertations/fullcit/9928371

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The Incidence of Handicapping Conditions and Special Education and Related Services Provided in State Juvenile Correctional Institutions in Seven Midwestern States (emotionally-disturbed, Speech-impaired, Learning Disabilities, Mental Retardation) by Thompson, Anne Foster, PhD from The University of Wisconsin Madison, 1986, 253 pages http://wwwlib.umi.com/dissertations/fullcit/8605723

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The Incidence of Learning Disabilities and Mental Retardation among Institutionalized Delinquents, Nonconfined Delinquents, and Children in Need of Supervision by Willis, Thomas William, EDD from The University of Alabama, 1979, 183 pages http://wwwlib.umi.com/dissertations/fullcit/8004580

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The Independent Effect of Age Dispersion and Paternal Age on the Incidence Rates of Mental Retardation by Phillips, William Lawrence, EDD from The University of Southern Mississippi, 1987, 75 pages http://wwwlib.umi.com/dissertations/fullcit/8806661

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The Influence of Familial Econodemographic Status, Characteristics of the Retarded Child, and Placement Situation on Parental Attitudes toward Mental Retardation by Sexton, James David, PhD from The University of Tennessee, 1980, 95 pages http://wwwlib.umi.com/dissertations/fullcit/8018495

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The Influence of Organizations on the Practice of Self-determination and the Pursuit of Community-inclusive Opportunities and Experiences for People with Mental Retardation by Visingardi, Richard James; PhD from Michigan State University, 2000, 417 pages http://wwwlib.umi.com/dissertations/fullcit/9972012

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The Influence of Personal and Professional Values upon the Value of Honesty among Superintendents in County Boards of Mental Retardation and Developmental Disabilities by Miller, Bryan Kevin; EDD from Bowling Green State University, 2001, 199 pages http://wwwlib.umi.com/dissertations/fullcit/3038440

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The Influence of Symbol and Learner Factors on the Learnability of Blissymbols by Students with Mental Retardation (Symbol Learnability) by Nail-chiwetalu, Barbara Jean, PhD from Purdue University, 1991, 225 pages http://wwwlib.umi.com/dissertations/fullcit/9215629

•

The Kids across the Hall: How Students with Mental Retardation and Their Nonhandicapped Peers Perceive Each Other by Ippolito, Margaret Mary, EDD from Hofstra University, 1996, 137 pages http://wwwlib.umi.com/dissertations/fullcit/9710281

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The Lives of Women with Mental Retardation: a Multiple-minority Perspective by Olson, Deborah Lynn, PhD from Syracuse University, 1991, 201 pages http://wwwlib.umi.com/dissertations/fullcit/9204541

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•

The Local Church As a Resource for Persons with Mental Retardation Living in the Community: a Study of Emerging Roles and Service Patterns by Nelson, Ellen Ruth, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1983, 394 pages http://wwwlib.umi.com/dissertations/fullcit/8327164

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The Matson and Mayville (M&M) Seizure Scale: an Assessment of Psychological and Environmental Variables Contributing to Seizure Activity in Persons with Mental Retardation by Mayville, Erik Andrew; PhD from Louisiana State University and Agricultural & Mechanical College, 2002, 93 pages http://wwwlib.umi.com/dissertations/fullcit/3063065

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The Meaning of Leisure Experience in the Lives of Adult Male Offenders and Former Offenders with Mental Retardation by Ardovino, Patricia S.; PhD from Indiana University, 1999, 271 pages http://wwwlib.umi.com/dissertations/fullcit/9962691

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The Mother-child Communicative Interactions of Educationally Advantaged Down Syndrome and Normal Children Matched for Auditory Comprehension (language, Speech, Mental Retardation) by Horstmeier, Deanna Smith, PhD from The Ohio State University, 1985, 127 pages http://wwwlib.umi.com/dissertations/fullcit/8603008

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The Nature of Self-injurious Behaviour among Visually Impaired Residents of Mental Retardation Facilities by Rodrigues, Myra Miliza, PhD from University of Toronto (Canada), 1982 http://wwwlib.umi.com/dissertations/fullcit/f3201493

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The Nature of Self-injurious Behaviour among Visually Impaired Residents of Mental Retardation Facilities by Rodrigues, Myra; PhD from University of Toronto (Canada), 1982 http://wwwlib.umi.com/dissertations/fullcit/NK55822

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The New York State Association for Retarded Children and the State's Decisionmaking Process for Mental Retardation by Lerner, Herbert J., PhD from New York University, 1970, 438 pages http://wwwlib.umi.com/dissertations/fullcit/7115406

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The Perspectives of Six Stakeholder Groups on the Challenging Behavior of Individuals with Mental Retardation And/or Autism by Ruef, Michael Benjamin, PhD from University of Kansas, 1997, 592 pages http://wwwlib.umi.com/dissertations/fullcit/9817103

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The Physiological Effects of a 12-week Program of Progressive Low-impact Aerobic Dance on Adults with Mental Retardation by Cluphf, David John; EDD from West Virginia University, 1999, 131 pages http://wwwlib.umi.com/dissertations/fullcit/9967195

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The Preparation of Teachers for the Education of Multihandicapped (Hearing Impairment/Mental Retardation) Children (Mental Retardation) by Snyder-jones, Laura, EDD from Tennessee State University, 1989, 148 pages http://wwwlib.umi.com/dissertations/fullcit/9019543

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The Presence and Importance of Motivator-hygiene Factors As Perceived by Supervisors in Mental Retardation Programs in South Carolina by Davis, Vivian Salley, EDD from South Carolina State University, 1990, 140 pages http://wwwlib.umi.com/dissertations/fullcit/9117233

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The Psychological and Psychosocial Impact of Various Residential Treatment Milieus on Adults with Mental Retardation (psychological Impact) by Vandergriff, David Von, PhD from University of South Carolina, 1991, 164 pages http://wwwlib.umi.com/dissertations/fullcit/9214979

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The Relationship between Locus of Control and Challenging Behavior for Deinstitutionalized Persons with Mental Retardation (locus of Control) by Sesalem, Kamal Salem, PhD from The University of Wisconsin - Madison, 1991, 117 pages http://wwwlib.umi.com/dissertations/fullcit/9126444

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The Relationship between Locus of Control, Residential Setting, and Employment Status Within a Sample of Adults with Mental Retardation by Attanasio, Michael E.; PsyD from Pace University, 2003, 63 pages http://wwwlib.umi.com/dissertations/fullcit/3095126

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The Relationship between Social Competence and Success in the Competitive Work Place for Persons with Mental Retardation: Implications for Job Placement and Training by Butterworth, John, Jr., PhD from The University of Connecticut, 1990, 119 pages http://wwwlib.umi.com/dissertations/fullcit/9107607

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The Relationship between Social Vocational Skills, Adaptive Behavior and Temperament in Adults with Moderate Mental Retardation by Courtney, Audrey Sue, PhD from University of Georgia, 1989, 90 pages http://wwwlib.umi.com/dissertations/fullcit/9003383

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The Relationship of Behavior Ratings of Psychopathology to Vocational Adjustment among School-aged Students with Mild Mental Retardation or Borderline Intelligence by Etu, Paul David, PsyD from State University of New York at Albany, 1990, 99 pages http://wwwlib.umi.com/dissertations/fullcit/9112312

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The Relationship of Certain Characteristics to Social and Physical Integration for Persons with Mental Retardation Living in Group Homes in Region 4 of the Commonwealth of Virginia by Stierer, Cheryl Louise, PhD from Virginia Commonwealth University, 1988, 139 pages http://wwwlib.umi.com/dissertations/fullcit/8907593

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The Relationship of Day Program Participation to Quality of Life for Persons with Mental Retardation Who Have Been Deinstitutionalized into the Community by Lecher, Richard Clinton; PhD from Rutgers the State University of New Jersey - New Brunswick, 2002, 422 pages http://wwwlib.umi.com/dissertations/fullcit/3055070

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The Relationship of Health and Safety of Individuals with Mental Retardation and Developmental Disabilities and the Size of Their Residential Facilities in Ohio by Wallace, Terry Allen, EDD from The University of Tennessee, 1993, 131 pages http://wwwlib.umi.com/dissertations/fullcit/9421694

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The Relationship of Muscular Strength and Balance on Work Performance Measures in High School Students with Mental Retardation by Smail, Karen Mary; PhD from University of Georgia, 2003 http://wwwlib.umi.com/dissertations/fullcit/f130113

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The Relative Effectiveness of Self- and Externally- Administered Reinforcement on the Production Rate of Adolescents with Multiple Impairments, Including Blindness

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and Mental Retardation by Zambone, Alana Maria, PhD from Peabody College for Teachers of Vanderbilt University, 1984, 67 pages http://wwwlib.umi.com/dissertations/fullcit/8419288 •

The Role of Negative Teaching Examples in the Development of Conjunctive Stimulus Control with Learners Experiencing Moderate and Severe Mental Retardation by Albin, Richard William, PhD from University of Oregon, 1986, 136 pages http://wwwlib.umi.com/dissertations/fullcit/8629546

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The Role of Social Awareness in the Employment Success of Young Adults with Mild Mental Retardation by Black, Rhonda Stout, EDD from University of Georgia, 1996, 184 pages http://wwwlib.umi.com/dissertations/fullcit/9636394

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The Role of the Language Sample and Short Term Interventions in the Differential Diagnosis of Mental Retardation. by Blumenfeld, Jane Ann, PhD from The University of New Mexico, 1973, 168 pages http://wwwlib.umi.com/dissertations/fullcit/7420309

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The Self-esteem of Normal Children of Mothers with Mental Retardation: an Exploratory Study by Nichols, Mary Lee, PhD from University of Oregon, 1989, 211 pages http://wwwlib.umi.com/dissertations/fullcit/9010143

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The Social Competence and Work Performance of People with Mental Retardation by Gregory, Susan Patricia, PhD from The University of Connecticut, 1989, 124 pages http://wwwlib.umi.com/dissertations/fullcit/8926484

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The Social Ecology of the Workplace: an Analysis of the Social Interactions of Workers with and without Mental Retardation in Food Service Settings by Ferguson, Brad Ammon, PhD from The University of Utah, 1990, 111 pages http://wwwlib.umi.com/dissertations/fullcit/9030546

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The Social Integration of Students with Mild Mental Retardation and Learning Disabilities into District of Columbia Public High Schools by Langley, Creola Theresa Frazier, EDD from The George Washington University, 1991, 212 pages http://wwwlib.umi.com/dissertations/fullcit/9133011

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The Testimony of a Child Witness with Mental Retardation in a Sexual Abuse Case? to Believe or Not to Believe? by Yozwiak, John Andrew; PhD from University of Kentucky, 2002, 127 pages http://wwwlib.umi.com/dissertations/fullcit/3068729

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The Training Needs of Correctional Officers Who Work with Inmates with Mental Retardation by Guenther, Francis Leonard, PhD from Temple University, 1989, 252 pages http://wwwlib.umi.com/dissertations/fullcit/9022911

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The Transition Status of Youth with Mental Retardation: a National Perspective by Cameto, Renee Lee, PhD from University of California, Berkeley with San Francisco State Univ., 1997, 326 pages http://wwwlib.umi.com/dissertations/fullcit/9803453

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The Transparency of Manual Signs in a Linguistic and an Environmental Nonlinguistic Context (Iconicity, Mental Retardation) by Dunham, Judy Kay, PhD from Purdue University, 1985, 164 pages http://wwwlib.umi.com/dissertations/fullcit/8606533

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The Use of Alternating Treatments Design to Compare Blissymbol - to - Referent Matching Tasks and Photograph - to - Referent Matching Tasks in Individuals with Severe Mental Retardation by Williams, Donna Gilles, EDD from The Johns Hopkins University, 1986, 262 pages http://wwwlib.umi.com/dissertations/fullcit/8707322

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The Use of Best Example Theory in the Acquisition of Social Skills by Adults with Mental Retardation: a Systematic Replication by Beckwith, Ruthie-marie, PhD from Peabody College for Teachers of Vanderbilt University, 1986, 113 pages http://wwwlib.umi.com/dissertations/fullcit/8624207

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The Use of Self-control Techniques to Facilitate Self-determination Skills during Leisure in Adolescents with Mild and Moderate Mental Retardation by Mahon, Michael James, PhD from The University of North Carolina at Chapel Hill, 1992, 167 pages http://wwwlib.umi.com/dissertations/fullcit/9234991

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The Use of Self-instruction with Multiple Exemplars to Teach Generalized Problemsolving to Individuals with Severe Mental Retardation by Hughes, Carolyn, PhD from University of Illinois at Urbana-champaign, 1990, 196 pages http://wwwlib.umi.com/dissertations/fullcit/9026213

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The Use of Vibration to Decrease Self-injurious and Self-stimulatory Behavior in Persons with Severe or Profound Mental Retardation by Wexler, Lawrence J., EDD from The Johns Hopkins University, 1996, 277 pages http://wwwlib.umi.com/dissertations/fullcit/9629517

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The Voices of Parents with Mental Retardation: Supports They Consider Beneficial in Raising Their Own Children by Lozano, Alison Mary; PhD from The University of Utah, 2000, 151 pages http://wwwlib.umi.com/dissertations/fullcit/9991369

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Three Federal Interventions on Behalf of Childbearing Women: the Sheppard-towner Act, Emergency Maternity and Infant Care, and the Maternal and Child Health and Mental Retardation Planning Amendments of 1963. by Mulligan, Joan Elizabeth, PhD from The University of Michigan, 1976, 301 pages http://wwwlib.umi.com/dissertations/fullcit/7619200

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Three Methods of Self-report Regarding Cigarette Use Behavior in Adolescents with Mild to Moderate Mental Retardation by Lohraff, Betty Jane Bultmann, PhD from University of Missouri - Columbia, 1994, 190 pages http://wwwlib.umi.com/dissertations/fullcit/9513677

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Three Studies on the Prevalence of Hyperactivity/impulsivity and Inattention in Children and Adolescents with Mild Mental Retardation (ADHD) by Smith, Kathryn Hope, PhD from University of Georgia, 1994, 109 pages http://wwwlib.umi.com/dissertations/fullcit/9520849

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Time in Her Life: a Down's Woman's Personal Account (mental Retardation, Life History, Down's Syndrome) by Kennann, Kristina E., PhD from University of California, Los Angeles, 1984, 173 pages http://wwwlib.umi.com/dissertations/fullcit/8420197

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Transfer of Training in the Use of Activity-schedules by Adults with Cerebral Palsy and Mental Retardation to Promote Independent Engagement in Daily Activities by Scheur, Lori Beth; PhD from City University of New York, 2002, 128 pages http://wwwlib.umi.com/dissertations/fullcit/3047263

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Transition for Young Adults with Severe Mental Retardation: Parent Expectations, Outcomes, and Family Involvement by Kraemer, Bonnie Rawlings; PhD from University of California, Riverside, 1999, 148 pages http://wwwlib.umi.com/dissertations/fullcit/9944676

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Transition from School to Adulthood Skills Instruction Needed and Received by Persons with Mild Mental Retardation in Taiwan by Niew, Wern-ing, PhD from University of Kansas, 1995, 237 pages http://wwwlib.umi.com/dissertations/fullcit/9623346

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Understanding Communication in Context: an Ethnographic Inquiry of Communication Style among Adults with Mental Retardation by Cascella, Paul William, PhD from The University of Connecticut, 1994, 127 pages http://wwwlib.umi.com/dissertations/fullcit/9525649

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Using Self-management to Improve Study Skills Performance of High School Students with Mental Retardation in General Education Classrooms by Copeland, Susan R.; PhD from Vanderbilt University, 2000, 186 pages http://wwwlib.umi.com/dissertations/fullcit/9970039

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Validating Vocational Competencies for Handicapped Persons (mental Retardation, Severely Handicapped) by Rose, Ernest David, PhD from The University of Utah, 1984, 153 pages http://wwwlib.umi.com/dissertations/fullcit/8505192

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Validation of a Cardiorespiratory Fitness Test for Men with Mental Retardation by Rintala, Pauli (Olavi), PhD from Oregon State University, 1990, 108 pages http://wwwlib.umi.com/dissertations/fullcit/9032690

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Weak Ties, the Disadvantaged, and Breaking Through the Business Barriers: Looking for the Missing Link to a Greater Employment Rate among People with Mental Retardation by Longjohn, Julie Marie; EDD from Oklahoma State University, 2001, 140 pages http://wwwlib.umi.com/dissertations/fullcit/3023938

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Where Is My Home? an Analysis of Movement in the Community Mental Retardation System (Mental Retardation) by Allard, Mary Ann, PhD from Brandeis U., the F. Heller Grad. Sch. for Adv. Stud. in Soc. Wel., 1990, 414 pages http://wwwlib.umi.com/dissertations/fullcit/9100454

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Wording Formats and Biases in Interview Responses by Adults with Mental Retardation by Rubin, Stephen Scott, PhD from University of Illinois at UrbanaChampaign, 1995, 225 pages http://wwwlib.umi.com/dissertations/fullcit/9624475

Keeping Current Ask the medical librarian at your library if it has full and unlimited access to the ProQuest Digital Dissertations database. From the library, you should be able to do more complete searches via http://wwwlib.umi.com/dissertations.

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CHAPTER 5. RETARDATION

CLINICAL

TRIALS

AND

MENTAL

Overview In this chapter, we will show you how to keep informed of the latest clinical trials concerning mental retardation.

Recent Trials on Mental Retardation The following is a list of recent trials dedicated to mental retardation.8 Further information on a trial is available at the Web site indicated. •

Phase II Study of Stereotypes and Mental Retardation: Neurobiological Basis Condition(s): stereotyped behavior; Mental Retardation Study Status: This study is currently recruiting patients. Sponsor(s): National Institute of Child Health and Human Development (NICHD); University of Florida Purpose - Excerpt: Objectives: I. Determine differences between persons with repetitive behavior disorders and matched controls on measures of motor control relevant to basal ganglia pathophysiology. II. Determine the efficacy of bromocriptine, a dopamine agonist, in the treatment of stereotyped behavior and related behavior disorders. III. Determine the efficacy of sertraline hydrochloride, a selective serotonin uptake inhibitor, in the treatment of repetitive behavior disorders. IV. Identify behavioral, environmental, and biological variables with differential drug treatment response. Phase(s): Phase II Study Type: Interventional Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00004300

8

These are listed at www.ClinicalTrials.gov.

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•

Study of Proteus Syndrome and Related Congenital Disorders Condition(s): Growth Disorder; Mental Retardation; Multiple Abnormalies Study Status: This study is currently recruiting patients. Sponsor(s): National Human Genome Research Institute (NHGRI) Purpose - Excerpt: This study will examine rare congenital disorders that involve malformations and abnormal growth. It will focus on patients with Proteus syndrome, whose physical features are characterized by overgrowth, benign tumors of fatty tissue or blood vessels, asymmetric arms or legs, and large feet with very thick soles. The study will explore the genetic and biochemical cause and course of the disease, the changes in symptoms over time, and the effects of the disease on patients. Patients with Proteus syndrome and their parents may be eligible for this study. Parents will be studied, when possible, for comparison of molecular findings. Study candidates will have a medical history and physical examination, including X-rays and possibly other imaging tests, such as computerized tomography (CT), magnetic resonance imaging (MRI) and ultrasound. Other tests and examinations may be done if needed. Those enrolled in the study will have will be interviewed or complete questionnaires, or both, about how their disease affects them. (Parents will be asked about their feelings about having a child with a rare disorder.) Patients will provide a small blood sample for research and may be asked to undergo biopsies from a normal area of skin and from a tumor. Study Type: Observational Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00001403

•

Study of Smith-Lemli-Opitz Syndrome Condition(s): Abnormalities; Inborn Errors of Metabolism; Mental Retardation; Muscle Hypotonia; Smith Lemli Opitz Syndrome Study Status: This study is currently recruiting patients. Sponsor(s): National Institute of Child Health and Human Development (NICHD) Purpose - Excerpt: Smith-Lemli-Opitz Syndrome (SLOS) is a genetic disorder (autosomal recessive) caused by an abnormality in the production of cholesterol. The disorder can occur in both a "mild" or "severe" form. SLOS is associated with multiple birth defects and mental retardation. Some of the birth defects include; abnormal facial features, poor muscle tone, poor growth, shortened life span, and abnormalities of the heart, lungs, brain, gastrointestinal tract, limbs, genitalia, and kidneys. There is no known cure for SLOS but recently patients have been treated with increased amounts of cholesterol in their diet. The cholesterol in a persons diet is unable to correct the abnormalities in the patient's organs, but researchers hope it will improve growth failure and mental retardation. This study was developed to answer questions about the causes and complications of SLOS, as well as the effectiveness of cholesterol treatment. The study will enroll patients diagnosed with SLOS, and their mothers. The objectives of the study will be to address the following questions: 1. What is the prognosis / natural history of the demyelination in the nervous system of patients with SLOS? 2. Do patients with SLOS have other problems concerning the function of their endocrine systems? 3. What are the genetic make-ups of patients with SLOS? 4. Can further studies of cholesterol metabolism and genetic testing, using SLOS fibroblasts, increase the understanding of SLOS?

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Study Type: Observational Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00001721 •

Genetic Aspects of Neurologic and Psychiatric Disorders Condition(s): Attention Deficit Disorder with Hyperactivity; Bipolar Disorder; Mental Disorder Diagnosed in Childhood; Mental Retardation; Schizophrenia Study Status: This study is no longer recruiting patients. Sponsor(s): National Institute of Mental Health (NIMH) Purpose - Excerpt: The purpose of this study is to improve the understanding of the genetic causes of specific neurologic and psychiatric disorders. The study will focus on conditions of mental retardation, childhood onset schizophrenia, attention deficit hyperactivity disorder (ADHD), atypical psychosis of childhood, and bipolar affective disorder. The study addresses the belief that there may be several genes contributing to the illness. Researchers intend to use several molecular genetic techniques in order to identify the areas of chromosomes containing genes responsible for the development of these disorders. Patients will be selected to participate in this study based on an early age of onset of their condition as well as the severity of the illness and the frequency of the illness among family members. Researchers will collect DNA samples from patients as well as affected and unaffected family members of each patient. The DNA samples collected will be analyzed for a variety of genetic abnormalities including; triplet repeat expansions, chromosome rearrangements, and polymorphisms. Study Type: Observational Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00001544

•

Self-Injury: Diagnosis and Treatment Condition(s): Self-Injurious Behavior; Mental Retardation Study Status: This study is no longer recruiting patients. Sponsor(s): National Institute of Child Health and Human Development (NICHD) Purpose - Excerpt: Self-injurious behavior is behavior in which a person hurts or harms himself. This behavior sometimes occurs in people with mental retardation or autism. This study will evaluate self-injurious behavior in people with mental retardation or autism and will test the effectiveness of new treatments. Phase(s): Phase III Study Type: Interventional Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00065936

•

Akathisia (Restless Legs Syndrome) in People with Schizophrenia and Mental Retardation Condition(s): Akathisia Study Status: This study is completed.

166 Mental Retardation

Sponsor(s): National Institute of Child Health and Human Development (NICHD) Purpose - Excerpt: Akathisia is a movement disorder that is often a side effect of certain psychiatric drugs. People with akathisia are unable to sit or keep still, complain of restlessness, fidget, rock from foot to foot, and pace. Akathisia is sometimes called "restless legs syndrome." The drugs that can cause akathisia are most often used to treat patients with schizophrenia or mental retardation (MR). This study will evaluate akathisia in both schizophrenic and MR patients who either have long-term akathisia or who are starting treatment with psychiatric drugs. Phase(s): Phase III Study Type: Observational Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00065286 •

Atypical Neuroleptic Drugs in People with Mental Retardation/Developmental Delay Condition(s): Mental Retardation; Developmental Delay Disorder Study Status: This study is completed. Sponsor(s): National Institute of Child Health and Human Development (NICHD) Purpose - Excerpt: Psychiatric drugs are often used to treat behavioral symptoms of mental retardation/developmental delay (MR/DD). These drugs can cause serious side effects. Newer drugs may have decreased side effects. This study will compare new and old drugs used to treat behavioral symptoms in people with MR/DD. Phase(s): Phase III Study Type: Interventional Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00065273

•

Evaluation of Patients with Unresolved Chromosome Abnormalities Condition(s): Abnormalities; Failure to Thrive; Mental Retardation; Microcephaly Study Status: This study is completed. Sponsor(s): National Human Genome Research Institute (NHGRI) Purpose - Excerpt: The purpose of this research is to study a new way to test for chromosome abnormalities. Chromosomes are strands of DNA (the genetic material in the cell nucleus) that are made up of genes-the units of heredity. Chromosome abnormalities are usually investigated by staining the chromosomes with a dye (Giemsa stain) and examining them under a microscope. This method can detect many duplications and deletions of pieces of chromosomes and is very accurate in diagnosing certain abnormalities. It is not useful, however, for identifying very small abnormalities. This study will evaluate the accuracy of a test method using 24 different dyes for finding small chromosome abnormalities. Children and adults with various chromosome abnormalities may be eligible for this study, including, for example, people with developmental delay or mental retardation, abnormal growth features or growth retardation, and certain behavioral disorders. Participants will be evaluated in the clinic over a 1- to 3-day period, depending on their symptoms. All participants will be examined by a genetics specialist and will have a physical examination and possibly Xrays, computerized tomography (CT) scans, magnetic resonance imaging (MRI),

Clinical Trials 167

ultrasound studies and medical photography. Blood will be drawn for chromosome testing-about 3 tablespoons from adults and 1 to 3 teaspoons from children. When the test results are available, participants will return to the clinic for follow-up evaluation and review of the test findings. The genetic and medical evaluations, along with their implications, will be discussed. Study Type: Observational Contact(s): see Web site below Web Site: http://clinicaltrials.gov/ct/show/NCT00001639

Keeping Current on Clinical Trials The U.S. National Institutes of Health, through the National Library of Medicine, has developed ClinicalTrials.gov to provide current information about clinical research across the broadest number of diseases and conditions. The site was launched in February 2000 and currently contains approximately 5,700 clinical studies in over 59,000 locations worldwide, with most studies being conducted in the United States. ClinicalTrials.gov receives about 2 million hits per month and hosts approximately 5,400 visitors daily. To access this database, simply go to the Web site at http://www.clinicaltrials.gov/ and search by “mental retardation” (or synonyms). While ClinicalTrials.gov is the most comprehensive listing of NIH-supported clinical trials available, not all trials are in the database. The database is updated regularly, so clinical trials are continually being added. The following is a list of specialty databases affiliated with the National Institutes of Health that offer additional information on trials: •

For clinical studies at the Warren Grant Magnuson Clinical Center located in Bethesda, Maryland, visit their Web site: http://clinicalstudies.info.nih.gov/

•

For clinical studies conducted at the Bayview Campus in Baltimore, Maryland, visit their Web site: http://www.jhbmc.jhu.edu/studies/index.html

•

For cancer trials, visit the National Cancer Institute: http://cancertrials.nci.nih.gov/

•

For eye-related trials, visit and search the Web page of the National Eye Institute: http://www.nei.nih.gov/neitrials/index.htm

•

For heart, lung and blood trials, visit the Web page of the National Heart, Lung and Blood Institute: http://www.nhlbi.nih.gov/studies/index.htm

•

For trials on aging, visit and search the Web site of the National Institute on Aging: http://www.grc.nia.nih.gov/studies/index.htm

•

For rare diseases, visit and search the Web site sponsored by the Office of Rare Diseases: http://ord.aspensys.com/asp/resources/rsch_trials.asp

•

For alcoholism, visit the National Institute on Alcohol Abuse and Alcoholism: http://www.niaaa.nih.gov/intramural/Web_dicbr_hp/particip.htm

•

For trials on infectious, immune, and allergic diseases, visit the site of the National Institute of Allergy and Infectious Diseases: http://www.niaid.nih.gov/clintrials/

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•

For trials on arthritis, musculoskeletal and skin diseases, visit newly revised site of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health: http://www.niams.nih.gov/hi/studies/index.htm

•

For hearing-related trials, visit the National Institute on Deafness and Other Communication Disorders: http://www.nidcd.nih.gov/health/clinical/index.htm

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For trials on diseases of the digestive system and kidneys, and diabetes, visit the National Institute of Diabetes and Digestive and Kidney Diseases: http://www.niddk.nih.gov/patient/patient.htm

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For drug abuse trials, visit and search the Web site sponsored by the National Institute on Drug Abuse: http://www.nida.nih.gov/CTN/Index.htm

•

For trials on mental disorders, visit and search the Web site of the National Institute of Mental Health: http://www.nimh.nih.gov/studies/index.cfm

•

For trials on neurological disorders and stroke, visit and search the Web site sponsored by the National Institute of Neurological Disorders and Stroke of the NIH: http://www.ninds.nih.gov/funding/funding_opportunities.htm#Clinical_Trials

169

CHAPTER 6. PATENTS ON MENTAL RETARDATION Overview Patents can be physical innovations (e.g. chemicals, pharmaceuticals, medical equipment) or processes (e.g. treatments or diagnostic procedures). The United States Patent and Trademark Office defines a patent as a grant of a property right to the inventor, issued by the Patent and Trademark Office.9 Patents, therefore, are intellectual property. For the United States, the term of a new patent is 20 years from the date when the patent application was filed. If the inventor wishes to receive economic benefits, it is likely that the invention will become commercially available within 20 years of the initial filing. It is important to understand, therefore, that an inventor’s patent does not indicate that a product or service is or will be commercially available. The patent implies only that the inventor has “the right to exclude others from making, using, offering for sale, or selling” the invention in the United States. While this relates to U.S. patents, similar rules govern foreign patents. In this chapter, we show you how to locate information on patents and their inventors. If you find a patent that is particularly interesting to you, contact the inventor or the assignee for further information. IMPORTANT NOTE: When following the search strategy described below, you may discover non-medical patents that use the generic term “mental retardation” (or a synonym) in their titles. To accurately reflect the results that you might find while conducting research on mental retardation, we have not necessarily excluded non-medical patents in this bibliography.

Patents on Mental Retardation By performing a patent search focusing on mental retardation, you can obtain information such as the title of the invention, the names of the inventor(s), the assignee(s) or the company that owns or controls the patent, a short abstract that summarizes the patent, and a few excerpts from the description of the patent. The abstract of a patent tends to be more technical in nature, while the description is often written for the public. Full patent descriptions contain much more information than is presented here (e.g. claims, references, figures, diagrams, etc.). We will tell you how to obtain this information later in the chapter. 9Adapted

from the United States Patent and Trademark Office: http://www.uspto.gov/web/offices/pac/doc/general/whatis.htm.

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The following is an example of the type of information that you can expect to obtain from a patent search on mental retardation: •

11 cby genomic sequence Inventor(s): Bergsma; Derk (Berwyn, PA), Ellis; Catherine E. (Glassboro, NJ), Halsey; Wendy (Kenneth Square, PA), Sathe; Ganesh (King of Prussia, PA) Assignee(s): SmithKline Beecham Corporation (Philadelphia, PA) Patent Number: 6,362,326 Date filed: December 22, 1998 Abstract: The present invention relates to 11cby, in particular 11cby polypeptides and 11cby polynucleotides, recombinant materials and methods for their production. In another aspect, the invention relates to methods for using such polypeptides and polynucleotides, including the treatment of bacterial, fungal, protozoan and viral infections, particularly infection caused by HIV-1 or HIV-2; pain; cancers; diabetes; obesity; feeding and drinking abnormalities, such as anorexia and bulimia; asthma; Parkinson's disease; both acute and congestive heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; ulcers; allergies; benign prostatic hypertrophy and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia or severe mental retardation, and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome, hereinafter referred to as "the Diseases", among others. In a still further aspect, the invention relates to diagnostic and prognostic assays for detecting diseases associated with inappropriate 11cby activity or levels. A method of performing genetic association studies for searching a disease susceptibility and/or drug response genes comprising using polymorphic markers in 11cby polynucleotides. Excerpt(s): This invention overall relates to the field of human genetics. More particularly, this invention relates to newly identified polypeptides and polynucleotides encoding such polypeptides, to the use of polynucleotides in gene therapy, and to production of such polypeptides and polynucleotides. In another aspect, this invention relates to prognostic and diagnostic methods for human diseases. This invention also relates to a method of performing genetic association studies for searching a disease susceptibility and/or drug response genes comprising using polymorphic markers. Yet in further aspect, the invention relates to transgenic animals, and use of transgenic animals for disease models to screening for therapeutic compounds. The drug discovery process is currently undergoing a fundamental revolution as it embraces `functional genomics`, that is, high throughput genome- or gene-based biology. This approach is rapidly superceding earlier approaches based on `positional cloning`. A phenotype, that is a biological function or genetic disease, would be identified and this would then be tracked back to the responsible gene, based on its genetic map position. Functional genomics relies heavily on the various tools of bioinformatics to identify gene sequences of potential interest from the many molecular biology databases now available. There is a continuing need to identify and characterise further genes and their related polypeptides/proteins, as targets for drug discovery. Web site: http://www.delphion.com/details?pn=US06362326__

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Aqueous suspensions of 9-hydroxyrisperidone fatty acid esters Inventor(s): Borghijs; Herman Karel (Willebroek, BE), Embrechts; Roger Carolus Augusta (Oud-Turnhout, BE), Fran.cedilla.ois; Marc Karel Jozef (Kalmthout, BE), Monbaliu; Johan (Turnhout, BE) Assignee(s): Janssen Pharmaceutica, N.V. (Beerse, BE) Patent Number: 6,077,843 Date filed: November 12, 1998 Abstract: The present invention is concerned with a pharmaceutical composition suitable as a depot formulation for administration via intramuscular or subcutaneous injection, comprising:(1) as an active ingredient a therapeutically effective amount of a 9-hydroxyrisperidone fatty acid ester or a salt, or a stereoisomer or a stereoisomeric mixture thereof and(2) a pharmaceutically acceptable carrier;wherein the pharmaceutically acceptable carrier is water and the active ingredient is suspended therein; and with a process of preparing such a composition. The invention further concerns such a pharmaceutical composition for use as a medicament in the treatment of schizophrenia, non-schizophrenic psychoses, behavioural disturbances associated with neurodegenerative disorders, e.g. in dementia, behavioural disturbances in mental retardation and autism, bipolar mania, depression, anxiety. Excerpt(s): and with a process of preparing such a composition. The invention further involves such a pharmaceutical composition for use as a medicament in the treatment of schizophrenia, non-schizophrenic psychoses, behavioural disturbances associated with neurodegenerative disorders, e.g. in dementia, behavioural disturbances in mental retardation and autism, bipolar mania, depression, anxiety. Risperidone is generic to 3[2-[4-(6-fluoro-1,2-benzisoxazol-3-yl)-1-piperidinyl]ethyl]-6,7,8,9-tetr ahydro-2-methyl4H-pyridol[1,2-a]pyrimidin-4-one. The preparation and pharmacological activity thereof are described in EP-0,196,132 (corresponding to U.S. Pat. No. 4,804,663). Various conventional pharmaceutical dosage forms, including tablets, capsules, drops, suppositories, oral solutions and injectable solutions are exemplified therein. In practice, risperidone is normally administered as the base in a tablet or in a buffered, oral or intramuscular solution. Particular solutions for oral or intramuscular administration are described in WO-96/01652. Risperidone is a highly potent drug having a relatively narrow therapeutic index. It may produce undesirable side effects on overdosage, most notably extra pyramidal syndrome (EPS) and to a lesser extent hypotension (due to peripheral alpha-adrenergic activity). For the purpose of producing an antipsychotic effect in a patient the total daily dose of risperidone ranges from about 2 to about 8 mg; for the alleviation of behavioral disturbances associated with neurodegenerative disorders the total daily dose is usually less and typically ranges from about 0.5 to about 2 mg. Inter-individual differences and co-medication may necessitate dose titrating in patients. Web site: http://www.delphion.com/details?pn=US06077843__

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Diagnostic test for prenatal identification of Down's syndrome and mental retardation and gene therapy therefor Inventor(s): Rubin; Edward M. (Berkeley, CA), Smith; Desmond J. (Oakland, CA) Assignee(s): The Regents of the University of California (Oakland, CA) Patent Number: 6,100,033 Date filed: April 30, 1998 Abstract: A a diagnostic test useful for prenatal identification of Down syndrome and mental retardation. A method for gene therapy for correction and treatment of Down syndrome. DYRK gene involved in the ability to learn. A method for diagnosing Down's syndrome and mental retardation and an assay therefor. A pharmaceutical composition for treatment of Down's syndrome mental retardation. Excerpt(s): This invention concerns a diagnostic test useful for prenatal identification of Down syndrome and mental retardation and a gene therapy for correction and treatment thereof. In particular, this invention concerns identification of DYRK gene involved in the ability to learn. The invention further concerns a method for diagnosing Down's syndrome and mental retardation and an assay therefor, a method for gene therapy of Down's syndrome and a pharmaceutical composition for treatment of Down's syndrome mental retardation. Down syndrome occurs in about one out of every 800 newborns, with the incidence increasing markedly in the offspring of women over 35. Affecting an estimated one million Americans, it is the leading genetic cause of mental retardation and is associated with a shorter than average life expectancy. Other symptoms are heart and intestinal defects, problems with the immune and endocrine systems, and raft of tissue and skeletal deformities. Individuals with Down syndrome carry a complete extra copy of chromosome 21 in all of their cells, giving each cell a total of 47 chromosomes rather than the normal 46. For this reason, the condition is also known as "Trisomy 21". There are, however, rare forms of Down syndrome in which only part of chromosome 21 is present in triplicate. Web site: http://www.delphion.com/details?pn=US06100033__

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Educational organizer Inventor(s): Bergman; Marilyn M. (9 Shirley Rd., Narberth, PA 19072) Assignee(s): none reported Patent Number: 5,879,162 Date filed: October 1, 1996 Abstract: A compensatory assistive device for students with cognitive impairment (including but not limited to traumatic brain injury, stroke, electrocution, anoxia, mental retardation, dementia, amnesia, and learning disabilities) and/or physical disabilities (such as cerebral palsy) is provided via an interactive computer system that provides an easy-to-use, multi-subject, school planner using a graphical user interface configured with particular color associations, pointer travel limitations, simplified option selections and active view-screen limitations. Excerpt(s): The invention pertains to devices that provide cognitively-impaired students with a compensatory assistance apparatus. In particular, the invention pertains to a graphically interactive computer system that allows the student to read and respond to lessons, schedules, etc., while permitting access to an instructor for supervision

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purposes and access to a remotely-located storage/servicing entity via a modem line. Providing the cognitively-impaired student with an easy and fun way of learning and organizing tasks relies on the use of orthosis (the correction of mental or physical distortion) devices rather than prosthesis (replacement of missing body parts) devices. In other words, the learning disabled student is provided with prompts, cues, or other indicia which minimize or simplify a particular mental or physical distortion experienced by the student in order to facilitate the student's response to a particular task without the use of medical devices coupled to the student. Desk-top personal computers provide the ideal technologic opportunity for implementation of an assistive device to enable cognitively or physically disabled students to accomplish an unlimited number of tasks. Examples of this are the software packages provided by Laureate Learning Systems, Inc., of Winooski, Vermont. Laureate Learning Systems, Inc. provide talking software that allows the disabled student to develop abilities, develop language skills, treat aphasia (a total or partial loss of the power of using or understanding words, usually caused by brain damage or injury) and traumatic brain injury, and reading difficulties. Any computer system having a keyboard, mouse and/or touch-screen can support these software packages. Web site: http://www.delphion.com/details?pn=US05879162__ •

HMTF81 human 7-transmembrane receptor Inventor(s): Ames; Robert S. (Havertown, PA), Chambers; Jon (Haslingfield, GB), Ellis; Catherine E. (Glassboro, NJ), Foley; James J. (Radnor, PA), Halsey; Wendy (Kennett Square, PA), Sarau; Henry M. (Harleysville, PA), Sathe; Ganesh M. (King of Prussia, PA) Assignee(s): Smithkline Beecham Corporation (Philadelphia, PA) Patent Number: 6,506,878 Date filed: June 5, 2000 Abstract: HMTMF81 polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing HMTMF81 polypeptides and polynucleotides in the design of protocols for the treatment of infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; pain; cancers; anorexia; bulimia; asthma; Parkinson's disease; acute heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; ulcers; asthma; allergies; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia, severe mental retardation and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome, among others and diagnostic assays for such conditions. Excerpt(s): This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to G-protein coupled receptor, hereinafter referred to as HMTMF81. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. The invention further relates to inhibiting or activating the action of LTD4 and LTC4. It is well established that many medically significant biological processes are mediated by proteins participating in signal transduction pathways that involve Gproteins and/or second messengers, e., cAMP (Lefkowitz, Nature, 1991, 351:353-354). Herein these proteins are referred to as proteins participating in pathways with Gproteins or PPG proteins. Some examples of these proteins include the GPC receptors,

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such as those for adrenergic agents and dopamine (Kobilka, B. K., et al., Proc. Natl Acad. Sci., USA, 1987, 84:46-50; Kobilka, B. K., et al., Science, 1987, 238:650-656; Bunzow, J. R., et al., Nature, 1988, 336:783-787), G-proteins themselves, effector proteins, e.g., phospholipase C, adenyl cyclase, and phosphodiesterase, and actuator proteins, e.g., protein kinase A and protein kinase C (Simon, M. I., et al., Science, 1991, 252:802-8). For example, in one form of signal transduction, the effect of hormone binding is activation of the enzyme, adenylate cyclase, inside the cell. Enzyme activation by hormones is dependent on the presence of the nucleotide GTP. GTP also influences hormone binding. A G-protein connects the hormone receptor to adenylate cyclase. G-protein was shown to exchange GTP for bound GDP when activated by a hormone receptor. The GTP-carrying form then binds to activated adenylate cyclase. Hydrolysis of GTP to GDP, catalyzed by the G-protein itself, returns the G-protein to its basal, inactive form. Thus, the G-protein serves a dual role, as an intermediate that relays the signal from receptor to effector, and as a clock that controls the duration of the signal. Web site: http://www.delphion.com/details?pn=US06506878__ •

Human neurotensin receptor type 2 and splice variants thereof Inventor(s): Bergsma; Derk Jon (Berwyn, PA), Shabon; Usman (Swarthmore, PA) Assignee(s): SmithKline Beecham Corporation (Philadelphia, PA) Patent Number: 6,008,050 Date filed: April 2, 1997 Abstract: Human neurotensin type 2 polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing human neurotensin type 2 polypeptides and polynucleotides in the design of protocols for the treatment of infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; pain; cancers; anorexia; bulimia; asthma; Parkinson's disease; acute heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; ulcers; asthma; allergies; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium dementia, severe mental retardation and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome, among others and diagnostic assays for such conditions. Excerpt(s): This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to a G-protein coupled 7-transrembrane receptor, hereinafter referred to as human neurotensin type 2. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. It is well established that many medically significant biological processes are mediated by proteins participating in signal transduction pathways that involve G-proteins and/or second messengers, e.g., cAMP (Lefkowitz, Nature, 1991, 351:353-354). These proteins are herein referred to as proteins participating in pathways with G-proteins or PPG proteins. Some examples of these proteins include the GPC receptors, such as those for adrenergic agents and doparnine (Kobilka, B. K., et al., Proc. Natl Acad. Sci., USA, 1987, 84:46-50; Kobilka, B. K., et al., Science, 1987, 238:650-656; Bunzow, J. R., et al., Nature, 1988, 336:783-787), Gproteins themselves, effector proteins, e.g., phospholipase C, adenyl cyclase, and phosphodiesterase, and actuator proteins, e.g., protein kinase A and protein kinase C (Simon, M. I., et al., Science, 1991, 252:802-8). For exarnple, in one form of signal

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transduction, the effect of hormone binding is activation of the enzyme, adenylate cyclase, inside the cell. Enzyme activation by hormones is dependent on the presence of the nucleotide GTP. GTP also influences hormone binding. A G-protein connects the hormone receptor to adenylate cyclase. G-protein was shown to exchange GTP for bound GDP when activated by a hormone receptor. The GTP carrying form then binds to activated adenylate cyclase. Hydrolysis of GTP to GDP, catalyzed by the G-protein itself, returns the G-protein to its basal inactive form Thus, the G-protein serves a dual role, as an intermediate that relays the signal from receptor to effector, and as a clock that controls the duration of the signal. Web site: http://www.delphion.com/details?pn=US06008050__ •

Human protein kinases hYAK3-2 Inventor(s): Creasy; Caretha (Erdenheim, PA), Dillon; Susan B. (Chester Springs, PA), Lord; Kenneth A. (Collegeville, PA) Assignee(s): SmithKline Beecham Corporation (Philadelphia, PA) Patent Number: 6,323,318 Date filed: August 10, 1999 Abstract: hYAK3-2 polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing hYAK3-2 polypeptides and polynucleotides in the design of protocols for the treatment of bone loss including osteoporosis; inflammatory diseases such as Adult Respiratory Disease Syndrome (ARDS), Rheumatoid arthritis, Osteoarthritis, Inflammatory Bowel Disease (IBD), psoriasis, dermatitis, asthma, allergies; infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; HIV-associated cachexia and other immunodeficiency disorders; septic shock; pain; injury; cancers including testicular cancer; anorexia; bulimia; neutropenia; cytopenia; anemias, including anemias due to renal insufficiency or to chronic disease, such as autoimmunity or cancer, and drug-induced anemias; polycythemia; myelosuppression; Parkinson's disease; cardiovascular disease including restenosis, atherosclerosis, acute heart failure, myocardial infarction; hypotension; hypertension; urinary retention; angina pectoris; ulcers; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia, severe mental retardation and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome, among others, and diagnostic assays for such conditions. Excerpt(s): This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to a serine/threonine protein kinase, hereinafter referred to as hYAK3-2. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. A number of polypeptide growth factors and hormones mediate their cellular effects through a signal transduction pathway. Transduction of signals from the cell surface receptors for these ligands to intracellular effectors frequently involves phosphorylation or dephosphorylation of specific protein substrates by regulatory protein serine/threonine kinases (PSTK) and phosphatases. Serine/threonine phosphorylation is a major mediator of signal transduction in multicellular organisms. Receptor-bound, membrane-bound and intracellular PSTKs regulate cell proliferation, cell differentiation and signalling processes in many cell types. Aberrant protein

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serine/threonine kinase activity has been implicated or is suspected in a number of pathologies such as rheumatoid arthritis, psoriasis, septic shock, bone loss, many cancers and other proliferative diseases. Accordingly, serine/threonine kinases and the signal transduction pathways which they are part of are potential targets for drug design. Web site: http://www.delphion.com/details?pn=US06323318__ •

Method for treating mental retardation Inventor(s): Bymaster; Franklin P (Brownsburg, IN), Shannon; Harlan E (Carmel, IN) Assignee(s): Eli Lilly and Company (Indianapolis, IN) Patent Number: 6,034,108 Date filed: December 16, 1998 Abstract: The present invention is directed to a method for using 3-(4-hexyloxy-1,2,5thiadiazol-3yl)-1,2,5,6-tetrahydro-1-methylpyridine, i.e., xanomeline, for the treatment of autism and mental retardation. Excerpt(s): This invention provides a method for using 3-(4-hexyloxy-1,2,5-thiadiazol-3yl)-1,2,5,6-tetrahydro-1-methylpyridine, (hereinafter referred as "xanomeline"), for the treatment of autism and mental retardation. Autism and mental retardation are seriously incapacitating conditions for which there has been little available treatment. The patient suffering from autism has gross and sustained impaired reciprocal social interaction. Impairment in communication is marked and sustained as well. The autistic patient suffers from restricted repetitive and stereotyped patterns of behavior, interests, and activies which are non-functional and/or abnormal in either intensity or focus. Further, the juvenile patient younger than three years old displays delays in at least one of the following areas: social interaction, language as used in social communication, or symbolic or imaginative play. The patient suffering from mental retardation has subaverage general intellectual functioning. The onset of such subaverage intellectual functioning is before the age of 18 (eighteen) years. Web site: http://www.delphion.com/details?pn=US06034108__

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Monoclonal and polyclonal antibodies relating to fragile X syndrome Inventor(s): Baker; Elizabeth (Glenelg North, AU), Kremer; Eric J. (Paris, FR), Lynch; Michael (Barcelona, ES), Mandel; Jean-Louis (Schiltigheim, FR), Mulley; John C. (Firle, AU), Nagaraja; Ramaiah (St. Louis, MO), Pritchard; Melanie April (Barcelona, ES), Richards; Robert I. (North Adelaide, AU), Schlessinger; David (St. Louis, MO), Sutherland; Grant R. (Unley Park, AU), Yu; Sui (Stepney, AU) Assignee(s): Washington University (), Women's and Children's Hospital (AU) Patent Number: 6,242,576 Date filed: June 2, 1995 Abstract: The DNA sequence spanning the fragile X site on the X human chromosome has been obtained in purified and isolated form. As fragile X is associated with mental retardation, the availability of a DNA which spans this locus permits diagnosis and treatment of the related mental disorders. Polyclonal and monoclonal antibodies to an

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amino acid sequence encoded by SEQ ID NO:1, a DNA sequence from the Fragile X site, are also disclosed. Excerpt(s): The invention relates generally to genetic diagnosis of humans. In particular, the invention concerns identification of individuals having particular DNA sequences predictive for Fragile X Syndrome. Fragile X Syndrome is the most common form of familial mental retardation and affects about one in 2,500 children. The syndrome is characterized by the presence of a cytogenetically detectable fragile site in band q27.3 near the end of the long arm of the X chromosome which, if not the cause of the disorder, is closely associated with it. The diagnostic molecular genetics of the Fragile X Syndrome has been reviewed by Sutherland, G. R. et al. (Clinical Genet. (1990) 37:2-11). An additional review is found by Nussbaum, R. L. et al (Ann. Rev. Genet. (1986) 20:109145). Identification of the DNA spanning and including the fragile site has been reported by Kremer et al (Am. J. Human Genetics (1991) 49:656-661) and Heitz et al. (Science (1991) 251:1236). Characterization of the fragile site has indicated a particular region of instability within a 5.0 KB EcoRI restriction fragment, with the instability segregating with the Fragile X genotype (Yu et al., Science (1991) 252:1179). The region of instability has further been localized to a 1 KB Pst I fragment containing a p(CCG).sub.n repeat. The Fragile X genotype is characterized by an increased amount of unstable DNA that maps to the repeat (Kremer et al., Science (1991) 252:1711). The availability of the cloned DNA makes possible the use of the DNA as a probe to detect length polymorphism of the P(CCG).sub.n to characterize the genotype of an individual at that locus (Kremer et al., supra), thereby obviating problems with cytogenetic visualization at the fragile site (Webb et al., Prenatal Diagnosis (1989) 9:771-781). Web site: http://www.delphion.com/details?pn=US06242576__ •

Pharmaceutical formulations and methods for treating patients suffering from diseases that cause muscular hypotonia Inventor(s): Bleiweiss; Daniel Gustavo (Laprida 1204 7th floor, (1425), Bleiweiss; Eduardo Samuel (Laprida 1204 7th floor, (1425), Bleiweiss; Herman (Av. Santa Fe 931, (1059) Assignee(s): Bleiweiss; Daniel Gustavo (Buenos Aires, AR), Bleiweiss; Eduardo Samuel (Buenos Aires, AR), Bleiweiss; Herman (Buenos Aires, AR) Patent Number: 5,738,873 Date filed: September 27, 1996 Abstract: Formulations are provided for the treatment of patients suffering from disorders that have in common the appearance of muscular hypotonia as a symptom. These disorders include Alzheimer's type diseases, atrophy of the brain, atrophy of the cerebellum, Fragile X syndrome, mental retardation of unknown causes, multiple anomalies in the chromosomes, deletions in one or more chromosome, and fragility in a chromosome other than the X chromosome. The formulations of the invention contain therapeutically effective amounts of gamma amino butyric acid (GABA), an anti-oxidant (such as ascorbic acid and/or vitamin E), folic acid, nicotinamide, and a lithium salt, all in a pharmaceutically acceptable excipient. Methods for the treatment of these disorders by the administration of such formulations are also provided. Excerpt(s): The present invention relates to the treatment of a variety of disorders which have in common the symptom of muscular hypotonia. More specifically, the invention pertains to the pharmaceutical treatment of such disorders, using a formulation of

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neurotransmitters, antioxidants, vitamins, and a lithium salt. The present invention is directed to the treatment of a variety of seemingly unrelated disorders, for which no fully satisfactory treatment is currently available. One of these is Fragile X syndrome, a chromosomal abnormality which causes mental retardation. Some children, especially girls, can carry the fragile X chromosome without developing any symptoms, which suggests that fragile X syndrome may be amenable to external influences. Some have suggested that this also creates a hope that the retardation might respond to pharmaceutical treatment. Genetics of Neuropsychiatric diseases, Lennart, Ed., Wetterburg 1988, Macmillan Press, Ltd., p. 117. Web site: http://www.delphion.com/details?pn=US05738873__

Patent Applications on Mental Retardation As of December 2000, U.S. patent applications are open to public viewing.10 Applications are patent requests which have yet to be granted. (The process to achieve a patent can take several years.) The following patent applications have been filed since December 2000 relating to mental retardation: •

AQUEOUS SUSPENSIONS OF SUBMICRON 9-HYDROXYRISPERIDONE FATTY ACID ESTERS Inventor(s): BASSTANIE, ESTHER DINA GUIDO; (ZANDHOVEN, BE), DRIES, WILLY MARIA ALBERT CARLO; (MERKSPLAS, BE), FRANCOIS, MARC KAREL JOZEF; (KAPELLEN, BE) Correspondence: Phillip S. Johnson, ESQ.; Johnson & Johnson; One Johnson & Johnson Plaza; New Brunswick; NJ; 08933-7003; US Patent Application Number: 20030064998 Date filed: May 2, 2000 Abstract: The present invention is concerned with a pharmaceutical composition suitable as a depot formulation for administration via intramuscular or subcutaneous injection, comprising:(1) as an active ingredient a therapeutically effective amount of a 9-hydroxy-risperidone fatty acid ester or a salt, or a stereoisomer or a stereoisomeric mixture thereof in submicron form and(2) a pharmaceutically acceptable carrier; wherein the pharmaceutically acceptable carrier is water and the active ingredient is suspended therein:and with a process of preparing such a composition.The invention further concerns such a pharmaceutical composition for use as a medicament in the treatment of psychosis, schizophrenia, schizoaffective disorders, non-schizophrenic psychoses, behavioural disturbances associated with neurodegenerative disorders, e.g. in dementia, behavioural disturbances in mental retardation and autism, Tourette's syndrome, bipolar mania, depression, anxiety. Excerpt(s): and with a process of preparing such a composition. The invention further involves such a pharmaceutical composition for use as a medicament in the treatment of psychosis, schizophrenia, schizoaffective disorders, non-schizophrenic psychoses, behavioural disturbances associated with neurodegenerative disorders, e.g. in dementia, behavioural disturbances in mental retardation and autism, Tourette's syndrome, bipolar mania, depression, anxiety. Risperidone is generic to 3-[2-[4-(6-fluoro-1,2-

10

This has been a common practice outside the United States prior to December 2000.

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benzisoxazol-3-yl)-1-piperidinyl]ethyl]-6,7,8,9-tetrahydro-2-methyl-4H-pyrido[1,2a]pyrimidin- -4-one. The preparation and pharmacological activity thereof are described in EP-0,196,132 (corresponding to U.S. Pat. No. 4,804,663). Various conventional pharmaceutical dosage forms, including tablets, capsules, drops, suppositories, oral solutions and injectable solutions are exemplified therein. In practice, risperidone is normally administered as the base in a tablet or in a buffered, oral or intramuscular solution. Particular solutions for oral or intramuscular administration are described in WO-96/01652. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Bardet-biedl susceptibility gene and uses thereof Inventor(s): Nishimura, Darryl; (Coralville, IA), Sheffield, Val; (Iowa City, IA), Stone, Edwin; (Iowa City, IA) Correspondence: Steven L. Highlander; Fulbright & Jaworski L.L.P.; Suite 2400; 600 Congress Avenue; Austin; TX; 78701; US Patent Application Number: 20020150931 Date filed: December 18, 2001 Abstract: The present invention relates to the identification of a gene, now designated negevin (ngvn), that is involved in the genetic disease Bardet Biedl Syndrome (BBS), which is characterized by such diverse symptoms as obesity, diabetes, hypertension, mental retardation, renal cancer and other abnormalities, retinopathy and hypogonadism. The human NGVN protein disclosed herein is 731 amino acids in length and is coded for by a gene spanning 17 exons. Homologs have been identified in mouse, rat, zebrafish. Methods of use for the gene, for example in diagnosis and therapy of BBS and in drug screening, also are described. Excerpt(s): The present application claims priority to co-pending U.S. Provisional Patent Application Serial No. 60/256,900 filed on Dec. 19.sup.19, 2000 and U.S. Provisional Patent Application Serial No.60/258,949 filed on Dec. 29.sup.29, 2000. The entire text of the above-referenced applications are specifically incorporated herein by reference without disclaimer. The government may own rights in the present invention pursuant to NIH grant number R01-EY-11298. The present invention relates to the fields of genetics and molecular biology. More particular the invention relates to the identification of a gene on human chromosome 16 that is involved in Bardet-Biedl Syndrome (BBS), designated here as negevin (ngvn). Defects in this gene are associated with a variety of clinical symptoms including diabetes, high blood pressure, renal cancer and other defects, retinal degeneration, congenital heart defects, limb deformity and obesity. Bardet-Biedl Syndrome (BBS) is a rare, autosomal recessive disorder characterized by mental retardation, obesity, pigmentary retinopathy, post-axial polydactyly and hypogonadism. A high frequency of renal abnormalities is also associated with this disorder. The mental retardation is often mild. Obesity begins early in infancy, and complications of obesity including diabetes mellitus and hypertension occur later in life. The associated retinal degeneration is usually severe and most patients become blind prior to 20 years of age. A recent report also provides evidence of an increased incidence of renal cell carcinoma (kidney cancer) as well as kidney malformations in BBS subjects. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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cDNA clone MY1 that encodes a novel human 7-transmembrane receptor Inventor(s): Yanagisawa, Masashi; (Dallas, TX) Correspondence: Glaxosmithkline; Corporate Intellectual Property - Uw2220; P.O. Box 1539; King OF Prussia; PA; 19406-0939; US Patent Application Number: 20030083466 Date filed: October 28, 2002 Abstract: MY1 polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing MY1 polypeptides and polynucleotides in the design of protocols for the treatment of infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; pain; cancers; anorexia nervosa; bulimia; cachexia; obesity; diabetes; asthma; Parkinson's disease; acute heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; ulcers; asthma; allergies; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia, severe mental retardation and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome, among others, and diagnostic assays for such conditions. Excerpt(s): This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to G-protein coupled receptor family, hereinafter referred to as MY1. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. It is well established that many medically significant biological processes are mediated by proteins participating in signal transduction pathways that involve G-proteins and/or second messengers, e.g., cAMP (Lefkowitz, Nature, 1991, 351:353-354). Herein, these proteins are referred to as proteins participating in pathways with G-proteins or PPG proteins. Some examples of these proteins include the GPC receptors, such as those for adrenergic agents and dopamine (Kobilka, B. K., et al., Proc. Natl Acad. Sci., USA, 1987, 84:46-50; Kobilka, B. K., et al., Science, 1987, 238:650-656; Bunzow, J. R., et al., Nature, 1988, 336:783-787), G-proteins themselves, effector proteins, e.g., phospholipase C, adenyl cyclase, and phosphodiesterase, and actuator proteins, e.g., protein kinase A and protein kinase C (Simon, M. I., et al., Science, 1991, 252:802-8). For example, in one form of signal transduction, the effect of hormone binding is activation of the enzyme, adenylate cyclase, inside the cell. Enzyme activation by hormones is dependent on the presence of the nucleotide GTP. GTP also influences hormone binding. A G-protein connects the hormone receptor to adenylate cyclase. Gprotein was shown to exchange GTP for bound GDP when activated by a hormone receptor. The GTP-carrying form then binds to activated adenylate cyclase. Hydrolysis of GTP to GDP, catalyzed by the G-protein itself, returns the G-protein to its basal, inactive form. Thus, the G-protein serves a dual role, as an intermediate that relays the signal from receptor to effector, and as a clock that controls the duration of the signal. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Extract Bunkankasaponin A. B. C. D., crude fats, crude protein and sugar from the shell of Xanthoceras sorbifolia Bunge Inventor(s): Guo, Yu; (US), Wang, Songjiang; (Thousand Oaks, CA), Yang, Baizhen; (Shenyang, CN), Zhao, Chongfu; (US) Correspondence: Songjiang Wang; P.O. Box 1692; Thousand Oaks; CA; 91358; US Patent Application Number: 20030082293 Date filed: October 31, 2002 Abstract: It is discovered that Bunkankasaponin A. B. C. D., crude fats, crude proteins and sugar can be extracted from the shell of Xanthoceras sorbifolia Bunge. The shell of X. sorbifolia used to be treated as waste material and not be utilized at all. This invention extracts 15.about.25 percent of Bunkankasaponin A. B. C. D., 2.about.10 percent of crude fats, 15.about.30 percent of crude proteins, 20.about.30 percent of sugar from the shell of X. sorbifolia. These extracts can be used making drugs to treat people with mental retardation and deficiency, etc., and has shown good effectiveness. The extracts can also be used as brain functional foods. The applications of these extracts from the shell of X. sorbifolia will convert the waste into useful materials and produce significant economic and social benefits. Excerpt(s): The invention relates to applications of Xanthoceras sorbifolia Bunge's shell. Xanthoceras sorbifolia Bunge, also named as Xanthoceras sorbifolium, is a kind of woody oil-bearing plant of Xanthoceras Sapindaceae. It originated primarily at loess plateau in North of China, with characters of drought-enduring, cold-resistant, barrenenduring and saline-alkali-enduring. For these features, it has been planted in most provinces of northern China for windbreak and sand fixation. X. sorbifolia's kernel contains about 60 percentage of fat that can be extracted to eatable oil. There is no any report, after researching both foreign and domestic documents, about extracting materials from the shell of X. sorbifolia so far. The shell of X. sorbifolia is still treated as waste material. There is no evidence of the Shell of X. sorbifolia being applied in medical and healthcare purposes. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Extracting materials from the shell of Xanthoceras sorbifolia Bunge and applying the extracted materials to making drugs and functional foods Inventor(s): Wang, Songjiang; (Thousand Oaks, CA), Yang, Baizhen; (Shenyang, CN) Correspondence: Songjiang Wang; P.O. Box 1692; Thousand Oaks; CA; 91358; US Patent Application Number: 20030096030 Date filed: October 31, 2002 Abstract: This invention proposed applications of the extracted materials from shell of Xanthoceras sorbifolia Bunge to making drugs for enhancing brain functions. The extracted materials from the shell of X. sorbifolia include 2.about.10 percent of crude fats, 15.about.20 percent of crude protein, 15.about.25 percent of Bunkankasaponin A. B. C. D., 20.about.30 percent of sugar and 7.about.12 percent of water as well as two kinds of sterols of (3.beta.,5.alpha.,20R,24S)-stigmasta-7, trans-22-dien-3-ol and (3.beta.,5.alpha.,20R,24R)-stigmasta-7-en-3-ol. The applications of this invention shows evidently effective for curing diseases of mental retardation and deficiency, dementia,

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enuresis, and increasing body's ability of resistance to activity of glycosuria. The extracted materials can also be made of functional foods for increasing brain's functions. Excerpt(s): This invention relates to drug and functional food raw materials extracted from the shell of Xanthoceras sorbifolia Bunge. Xanthoceras sorbifolia Bunge, also named as Xanthoceras sorbifolium, is a kind of woody oil-bearing plant of Xanthoceras Sapindaceae. It originated primarily at loess plateau in North of China, with characters of drought-enduring, cold-resistant, barren-enduring and saline-alkali-enduring. For these features, it has been planted in most provinces of northern China for windbreak and sand fixation. X. sorbifolia's kernel tastes pleasant and eatable according of a Chinese ancient book named Provide Disaster Relief of Chinese Materia Medrea. It contains up to 60 percent of high quality, edible oils which riches in 14 kinds of unsaturated fatty acids. X. sorbifolia's kernel can be applied to industry and pharmacy as fined grease, plasticizers, scald plaster, medicine for curing epilepsy etc., and, has been used to cure enuresis. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Method of treating anemia Inventor(s): Creasy, Caretha; (Erdenheim, PA), Dillon, Susan B.; (Chester Springs, PA), Lord, Kenneth A.; (Collegeville, PA) Correspondence: Ratner & Prestia; P.O. Box 980; Valley Forge; PA; 19482-0980; US Patent Application Number: 20030176375 Date filed: May 14, 2001 Abstract: hYAK3-2 polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing hYAK3-2 polypeptides and polynucleotides in the design of protocols for the treatment of bone loss including osteoporosis; inflammatory diseases such as Adult Respiratory Disease Syndrome (ARDS), Rheumatoid arthritis, Osteoarthritis, Inflammatory Bowel Disease (IBD), psoriasis, dermatitis, asthma, allergies; infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; HIV-associated cachexia and other immunodeficiency disorders; septic shock; pain; injury; cancers including testicular cancer; anorexia; bulimia; neutropenia; cytopenia; anemias, including anemias due to renal insufficiency or to chronic disease, such as autoimmunity or cancer, and drug-induced anemias; polycythemia; myelosuppression; Parkinson's disease; cardiovascular disease including restenosis, atherosclerosis, acute heart failure, myocardial infarction; hypotension; hypertension; urinary retention; angina pectoris; ulcers; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia, severe mental retardation and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome., among others, and diagnostic assays for such conditions. Excerpt(s): This application claims priority to U.S. Ser. No. 60/118,045 filed Feb. 1, 1999, which is incorporated by reference in its entirety. This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to a serine/threonine protein kinase, hereinafter referred to as hYAK3-2. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. A number

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of polypeptide growth factors and hormones mediate their cellular effects through a signal transduction pathway. Transduction of signals from the cell surface receptors for these ligands to intracellular effectors frequently involves phosphorylation or dephosphorylation of specific protein substrates by regulatory protein serine/threonine kinases (PSTK) and phosphatases. Serine/threonine phosphorylation is a major mediator of signal transduction in multicellular organisms. Receptor-bound, membranebound and intracellular PSTKs regulate cell proliferation, cell differentiation and signalling processes in many cell types. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Methods and compositions for modulating brain damage Inventor(s): Follett, Pamela L.; (Boxboro, MA), Jensen, Frances E.; (Chestnut Hill, MA), Rosenberg, Paul; (Newton, MA), Volpe, Joseph; (Brookline, MA) Correspondence: Lahive & Cockfield; 28 State Street; Boston; MA; 02109; US Patent Application Number: 20030092730 Date filed: August 5, 2002 Abstract: Methods and compositions for modulating brain damage mediated by nonNMDA ionotropic glutamate receptor antagonists are provided. Methods for inhibiting neuronal cell damage and death in the brain following an injury (e.g., ischemic/hypoxic injury) and methods for inhibiting cell damage and death in conditions such as periventricular leukomalacia, cerebral palsy, mental retardation, and neonatal stroke, are also provided. These methods generally involve treating a subject with a nonNMDA ionotropic glutamate receptor antagonist. Pharmaceutical and packaged formulations that include non-NMDA ionotropic glutamate receptor antagonists are also provided. Excerpt(s): The present application is a Continuation-In-Part application of and claims priority from U.S. Continuation patent application Ser. No. 10/121,892, filed on Apr. 12, 2002, and from U.S. patent application Ser. No. 09/922,564, filed on Aug. 3, 2001. All of the above applications are expressly incorporated by reference. Preterm infants, particularly those of low birth weight and gestational age, often present neurodevelopmental deficits which include global cognitive delay, cerebral palsy, blindness, and deafness. Deficits such as cognitive delay and cerebral palsy may be attributed, at least in part, to hypoxic/ischemic damage in white and/or grey matter of the brain. A common example of white matter injury observed in infants as a complication of premature birth is referred to as periventricular leukomalacia (PVL). PVL is the principal neuropathological correlate of cerebral palsy. The lesion is defined by focal necrosis of the deep periventricular white matter involving all cellular components, combined with a more diffuse white matter injury that appears selective for developing oligodendrocytes (OLs) (Gilles and Averill (1977) Ann. Neurol. 2:49-56; Dambska et al. (1989) J. Child Neurol. 4:291-298; and Rorke (1998) In Pathology of Perinatal Brain Injury New York: Raven). Reduced cerebral myelin is the most prominent subsequent cerebral abnormality observed in premature infants with evidence of PVL in the neonatal period (Paneth et al. (1990) J. Pediatr. 116:975-984; Rorke (1992) Brain Pathol. 2:211-221; Iida et al. (1995) Pediatr. Neurol. 13:296-304; Olsen et al. (1997) Ann. Neurol. 41:754-761; Skranes et al. (1997) Neuropediatrics 28:149-154; and Inder et al. (1999) Ann Neurol. 46:755-760). Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Methods of treating neurological disorders with group I mGluR antagonists Inventor(s): Bear, Mark F.; (Bristol, RI), Huber, Kimberly M.; (Dallas, TX), Warren, Stephen T.; (Atlanta, GA) Correspondence: David E. Brook, ESQ.; Hamilton, Brook, Smith & Reynolds, P.C.; 530 Virginia Road; P.O. Box 9133; Concord; MA; 01742-9133; US Patent Application Number: 20030100539 Date filed: April 2, 2002 Abstract: Compositions and uses of mGluR5 antagonists for the treatment and prevention of neurological disorders, such as Fragile X, autism, mental retardation, schizophrenia and Down's Syndrome, are disclosed. Excerpt(s): This application is based on U.S. Provisional Application No. 60/280,915, filed Apr. 2, 2002, the specification of which is incorporated by reference herein. In the mammalian central nervous system (CNS), the transmission of nerve impulses is controlled by the interaction between a neurotransmitter released by a sending neuron and a surface receptor on a receiving neuron, causing excitation of this receiving neuron. L-Glutamate, the most abundant neurotransmitter in the CNS, mediates the major excitatory pathway in mammals, and is referred to as an excitatory amino acid (EAA). The receptors that respond to glutamate are called excitatory amino acid receptors (EAA receptors). See Watkins & Evans, Annual Reviews in Pharmacology and Toxicology, 21:165 (1981); Monaghan, Bridges, and Cotman, Annual Reviews in Pharmacology and Toxicology, 29:365 (1989); Watkins, Krogsgaard-Larsen, and Honore, Transactions in Pharmaceutical Science, 11:25 (1990). Excitatory amino acid receptors are classified into two general types. Receptors that are directly coupled to the opening of cation channels in the cell membrane of the neurons are termed "ionotropic." This type of receptor has been subdivided into at least three classes, which are defined by the depolarizing actions of the selective agonists N-methyl-D-aspartate (NMDA),.alpha.-amino-3hydroxy-5-methylisoxazole-4-- propionic acid (AMPA), and kainic acid (KA). Five kainate receptors, classified as either high affinity (KA1 and KA2) or low affinity (GluR5, GluR6 and GluR7) kainate receptors have been identified. (Bleakman et al, Molecular Pharmacology, 1996, Vol. 49, No. 4, pp. 581-585). Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

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Myosin IXa and cyclic nucleotide gated channel-15 (CNGC-15) polynucleotides, polypeptides, compositions, methods, and uses thereof Inventor(s): Adams, Arwen E.; (Oakland, CA), Chin, Choi Ying; (Castro Valley, CA), Duhl, David; (Oakland, CA), Gorman, Susan W.; (Santa Monica, CA), Leng, Song; (Castro Valley, CA), Sheffield, Val; (Iowa City, IA), Welch, Juliet; (Kensington, CA) Correspondence: Chiron Corporation; Intellectual Property R338; P.O. Box 8097; Emeryville; CA; 94662-8097; US Patent Application Number: 20020091248 Date filed: May 8, 2001 Abstract: The present invention discloses the amino acid and nucleic acid sequences of a new CNGC and Myosin that map to the region of the human chromosome associated with Bardet-Biedl Syndrome. Cyclic nucleotide gated channels (CNGCs) comprise a

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family of multimeric protein ion channels that open in response to the binding of a cyclic nucleotide to an intracellular domain. The two new proteins, CNGC-15 and Myosin IXa, are useful in the study, diagnosis and treatment of Bardet-Biedl Syndrome and Usher Syndrome. Other indications that can be treated by CNGC-15 and/or Myosin IXa polypeptides, or agonists or antagonists include hearing loss, retinis pigmentosa, obesity, hypogonadism, sterility, polydactyly, brachydactyly, syndactyly, mental retardation, renal abnormalities, hypertension, diabetes and cardiovascular abnormalities.Compositions and methods for expressing cyclic nucleotide gated channel-15 (CNGC-15) and Myosin IXa are provided. The compositions comprise CNGC-15 and Myosin IXa polypeptides and derivatives thereof, nucleotide sequences, expression cassettes, transformed cells and antibodies to these polypeptides. Methods for the expression and detection of CNGC-15 and Myosin IXa nucleotides and polypeptides and compositions for the treatment of these conditions are provided. Excerpt(s): This application claims the benefit of U.S. Provisional Application Serial No. 60/062,858, filed Oct. 15, 1997, U.S. Provisional Application No. 60/062,241, filed Oct. 17, 1997, and U.S. Provisional Application No. 60/068,953, filed Dec. 30, 1997, the contents of which are herein incorporated by reference. This invention relates to the fields of molecular biology and pharmaceutical research. More specifically, this invention relates to the identification and recombinant expression of two new genes, cyclic nucleotide gated channel-15 (CNGC-15) and Myosin IXa. Accordingly, isolation of the human myosin IXa gene will prove useful in the study, diagnosis and treatment of Bardet-Biedl Syndrome, Usher Syndrome and related conditions. Usher Sydrome typel (USH1) is characterized by a profound congenital sensoneural hearing loss, vestibular dysfunction and prepubescent onset retinitis pigmentosa. Family studies indicated that three genes with different chromosomal locations are responsible for USH1; a defect in any one of these genes causes the disease. Of these, the gene USH1B mapped to a region homologous to the murine region containing the mouse deafness mutant shaker-1 which results from mutations in myosin VIIa. Subsequent work confirmed that myosin VIIa is the cause of Usher Sydrome type 1B and localized the myosin VIIa protein to the receptor cells of the inner ear (Hasson et al. (1995) Genomics 36:431-439) and the connecting cilia of photoreceptor cells in the retina (Liu et al. (1997) Cell Motil. Cytoskel. 37:240-252). Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Novel compound Inventor(s): Southan, Christopher D.; (Bishop's Stortford, GB) Correspondence: Ratner & Prestia; PO Box 980; Valley Forge; PA; 19482-0980; US Patent Application Number: 20010016339 Date filed: January 10, 2001 Abstract: Novel pancreatic polypeptides and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Also disclosed are methods for utilizing novel pancreatic polypeptides and polynucleotides in the design of protocols for the treatment of food intake-related disorders such as obesity, anorexia and bulimia; asthma; Parkinson's disease; acute heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; ulcers; asthma; allergies; benign prostatic hypertrophy; and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia,

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severe mental retardation and dyskinesias; cancer and pain, among others, and diagnostic assays for such conditions. Excerpt(s): This invention relates to newly identified polynucleotides, polypeptides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to the neuropeptide family, hereinafter referred to as novel pancreatic polypeptide. The invention also relates to inhibiting or activating the action of such polynucleotides and polypeptides. It is well established that neuropeptides are synthesised by proteolytic processing of a large preprohormone precursor protein. Peptides within preprohormones are typically flanked by pairs of basic residues, for example, Lys-Arg (KR), Arg-Arg (RR), Lys-Lys (KK) or Arg-Lys (RK). Neuropeptides may also undergo post-translational modifications that include disulfide bond formation, glycosylation, COOH-terminal alpha-amidation, phosphorylation and sulphation (V. Y. H. Hook et al., FASEB J, 8: 1269-1278, 1994 and refs therein). It is not uncommon for the preprohormone processing to give rise to more than one neuropeptide, for example, the precursor of human pancreatic polypeptideand pancreatic isopeptide (Boel et al. EMBO J. 3(4) 909-912, 1984). The synthesised neuropeptides exert their biological action by interacting with the membrane protein gene superfamily of G-protein coupled receptors (Lefkowitz, Nature, 1991, 351:353-354). G-protein coupled receptors have been characterized as including these seven conserved hydrophobic stretches of about 20 to 30 amino acids, connecting at least eight divergent hydrophilic loops. The G-protein family of coupled receptors includes dopamine receptors which bind to neuroleptic drugs used for treating psychotic, neurological and other disorders. Other examples of members of this family include, but are not limited to, pancreatic polypeptide, calcitonin, adrenergic, endothelin, cAMP, adenosine, muscarinic, acetylcholine, serotonin, histamine, thrombin, kinin, follicle stimulating hormone, opsins, endothelial differentiation gene-1, rhodopsins, odorant, and cytomegalovirus receptors. Over the past 15 years, nearly 350 therapeutic agents targeting 7 transmembrane (7 TM) receptors or their ligands have been successfully introduced onto the market. Antibiotic activity has also been reported for Neuropeptide Y (NPY) and polypeptide P (PYY) (Vouldoukis et al FEBS Lett, 380(3):237-240, 1996). Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Polypeptide sequences of human EDG-1c Inventor(s): Bergsma, Derk J.; (Berwyn, PA), Chambers, Jonathan K.; (Cambridge, GB), Chan, Winnie; (West Chester, PA), Jensen, Pamela Joy; (Wayne, PA), Johnson, Randall K.; (Ardmore, PA), Khandoudi, Nassirah; (Saint Gregoire, FR), Livi, George P.; (Havertown, PA), Robert, Phillipe; (Saint Gregoire, FR), Stadel, Jeffrey M.; (Wayne, PA), Wilson, Shelagh; (Hertford, GB) Correspondence: Glaxosmithkline; Corporate Intellectual Property - Uw2220; P.O. Box 1539; King OF Prussia; PA; 19406-0939; US Patent Application Number: 20030082743 Date filed: January 12, 2001 Abstract: Human EDG-1c polypeptidees and polynucleotides and methods for producing such polypeptides by recombinant techniques are disclosed. Human EDG-1c is identified as a selective receptor for sphingosine-1-phosphate ("S-1-P") and for dihydro S-1-P. Also disclosed are methods for discovering agonists and antagonists of the interaction between S-1-P and di-hydro S-1-P and their cellular receptor, human EDG-

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1c, which may have utility in the treatment of several human diseases and disorders, including, but not limited to the treatment of infections such as bacterial, fungal, protozoan and viral infections, particularly infections such as bacterial, fungal, protozoan and viral infections, particularly infections caused by HIV-1 or HIV-2; pain; cancers; diabetes, obesity; anorexia; bulimia; asthma; Parkinson's disease; acute heart failure; hypotension; hypertension; urinary retention; osteoporosis; angina pectoris; myocardial infarction; stroke; congestive heart failure; left ventricular hypertrophy; arrythmias; restenosis after coronary artery angioplasty; vascular sclerosis; deleterious fibrosis; atherosclerosis; inflammation; angiogenesis; wound healing; ulcers; asthma; allergies; benign prostatic hypertrophy; migraine; vomiting; psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, depression, delirium, dementia, and severe mental retardation; degenerative diseases, such as neurodegenerative diseases and ischemic stroke; and dyskinesias, such as Huntington's disease or Gilles dela Tourett's syndrome. Excerpt(s): This application claims benefit to the earlier provisional U.S. application Ser. Nos. 60/077,369, filed on Mar. 9, 1998, and 60/087,102, filed on May 28, 1998, the contents of which are incorporated herein by reference in their entirety. This invention relates to newly identified polypeptides and polynucleotides encoded by them and to the use of such polynucleotides and polypeptides, and to their production. More particularly, the polynucleotides and polypeptides of the present invention relate to the G-protein coupled receptors, hereinafter referred to as human EDG-1c receptor. This invention also relates to methods for discovering agonists and antagonists of the interaction between sphingosine 1-phosphate (hereinafter referred to as "S-1-P") and dihydro sphingosine 1-phosphate (also known as sphingoanine 1-phosphate and hereinafter referred to as "di-hydro S-1-P") and their cellular receptor, human EDG-1c receptor. The invention also relates to the use of human EDG-1c polynucleotides and polypeptides in therapy and in identifying compounds which may be agonists, antagonists and/or inhibitors which are potentially useful in therapy, and to production of such polypeptides and polynucleotides. The drug discovery process is currently undergoing a fundamental revolution as it embraces `functional genomics`, that is, high throughput genome- or gene-based biology. This approach is rapidly superseding earlier approaches based on `positional cloning`. A phenotype, that is a biological function or genetic disease, would be identified and this would then be tracked back to the responsible gene, based on its genetic map position. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html •

Regulation of human alpha 1A adrenergic receptor-line G protein-coupled receptor Inventor(s): Ramakrishnan, Shyam; (Brighton, MA) Correspondence: Banner & Witcoff; 1001 G Street N W; Suite 1100; Washington; DC; 20001; US Patent Application Number: 20030187219 Date filed: May 5, 2003 Abstract: Reagents which regulate human.alpha.sub.1aadrenergic receptor-like GPCR and reagents which bind to human.alpha.sub.1a adrenergic receptor-like GPCR gene products can play a role in preventing, ameliorating, or correcting dysfunctions or diseases including, but not limited to, infections such as bacterial, fungal, protozoan, and viral infections, particularly those caused by HIV viruses, pain, obesity cancers, anorexia, bulimia, asthma, Parkinson's diseases, acute heart failure, hypotension,

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hypertension, urinary retention, osteoporosis, angina pectoris, myocardial infarction, ulcers, asthma, allergies, multiple sclerosis, benign prostatic hypertrophy, and psychotic and neurological disorders, including anxiety, schizophrenia, manic depression, delirium, dementia, several mental retardation, and dyskinesias, such as Huntington's disease and Tourett's syndrome. Excerpt(s): The invention relates to the area of G-protein coupled receptors. More particularly, it relates to the area of human.alpha.sub.1a adrenergic receptor-like G protein-coupled receptors and their regulation. Many medically significant biological processes are mediated by signal transduction pathways that involve G-proteins (Lefkowitz, Nature 351, 353-354, 1991). The family of G-protein coupled receptors (GPCR) includes receptors for hormones, neurotransmitters, growth factors, and viruses. Specific examples of GPCRs include receptors for such diverse agents as dopamine, calcitonin, adrenergic hormones, endothelin, cAMP, adenosine, acetylcholine, serotonin, histamine, thrombin, kinin, follicle stimulating hormone, opsins, endothelial differentiation gene-1, rhodopsins, odorants, cytomegalovirus, Gproteins themselves, effector proteins such as phospholipase C, adenyl cyclase, and phosphodiesterase, and actuator proteins such as protein kinase A and protein kinase C. GPCRs possess seven conserved membrane-spanning domains connecting at least eight divergent hydrophilic loops. GPCRs (also known as 7TM receptors) have been characterized as including these seven conserved hydrophobic stretches of about 20 to 30 amino acids, connecting at least eight divergent hydrophilic loops. Most GPCRs have single conserved cysteine residues in each of the first two extracellular loops, which form disulfide bonds that are believed to stabilize functional protein structure. The seven transmembrane regions are designated as TM1, TM2, TM3, TM4, TM5, TM6, and TM7. TM3 has been implicated in signal transduction. Web site: http://appft1.uspto.gov/netahtml/PTO/search-bool.html

Keeping Current In order to stay informed about patents and patent applications dealing with mental retardation, you can access the U.S. Patent Office archive via the Internet at the following Web address: http://www.uspto.gov/patft/index.html. You will see two broad options: (1) Issued Patent, and (2) Published Applications. To see a list of issued patents, perform the following steps: Under “Issued Patents,” click “Quick Search.” Then, type “mental retardation” (or synonyms) into the “Term 1” box. After clicking on the search button, scroll down to see the various patents which have been granted to date on mental retardation. You can also use this procedure to view pending patent applications concerning mental retardation. Simply go back to http://www.uspto.gov/patft/index.html. Select “Quick Search” under “Published Applications.” Then proceed with the steps listed above.

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CHAPTER 7. BOOKS ON MENTAL RETARDATION Overview This chapter provides bibliographic book references relating to mental retardation. In addition to online booksellers such as www.amazon.com and www.bn.com, excellent sources for book titles on mental retardation include the Combined Health Information Database and the National Library of Medicine. Your local medical library also may have these titles available for loan.

Book Summaries: Federal Agencies The Combined Health Information Database collects various book abstracts from a variety of healthcare institutions and federal agencies. To access these summaries, go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. You will need to use the “Detailed Search” option. To find book summaries, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer. For the format option, select “Monograph/Book.” Now type “mental retardation” (or synonyms) into the “For these words:” box. You should check back periodically with this database which is updated every three months. The following is a typical result when searching for books on mental retardation: •

Of Two Minds: A Guide to the Care of People With the Dual Diagnosis of Alzheimer's Disease and Mental Retardation Source: Malden, MA: Alzheimer Support Services Cooperative for Human Services, Inc. 1995. 167 p. Contact: Available from Antonangeli, J.M., Director, Alzheimer Support Services Cooperative for Human Services, Inc. 110 Pleasant Street, Malden, MA 02148. (617) 3244303; FAX (617) 397-9411. PRICE: $19.95. Summary: This guidebook offers practical suggestions and techniques for caregivers of people with the dual diagnosis of Alzheimer's disease (AD) and mental retardation. It provides an overview of AD and its manifestations in people with mental retardation and suggests how to structure physical environments in a home and in a day program. It also suggests strategies for evaluating and optimizing safety, preventing wandering,

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and communicating with persons who have impaired memories. Guidelines are detailed for helping these persons with all activities of daily living. Behavior management strategies are given for dealing with such common problems as depression, inappropriate expressions of sexuality, and catastrophic reactions, and so-called nuisance' behaviors such as pacing. Also highlighted are strategies for medical conditions and engaging persons in failure-free activities. A section on caring for the caregiver discusses educational support, helping housemates understand AD, and helping staff cope with grief and loss. Tables, reading list, selected index, and table guide. •

Closing the Gap: A National Blueprint to Improve the Health of Persons with Mental Retardation: A Report of the Surgeon General Source: Rockville, MD: U.S. Department of Health and Human Services. National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH). 2002. [76 p.]. Contact: Available from National Institute of Child Health and Human Development (NICHD). P.O. Box 3006, Rockville, MD 20847. (800) 370-2943. Fax (301) 984-1473. Email: [email protected]. Website: www.nichd.nih.gov/publications. PRICE: Single copy free. Summary: This report from the United States Surgeon General presents a national blueprint to improve the health of persons with mental retardation (MR). The blueprint identifies problems and solutions proposed by the community of people with MR and those who care about their health. The report suggests multiple action steps that were developed by work groups at the Surgeon General's National Conference on Health Disparities and Mental Retardation, held in December 2001 in Washington, D.C. The action steps are organized under six broad goals: health promotion and community environments, knowledge and understanding, quality of health care, training of health care providers, health care financing, and sources of health care. This blueprint sets forth an agenda for national, State, and local action, in both public and private sectors, to improve the health of individuals with MR and to include them fully in health systems that meet their needs. Realizing the goals of this blueprint calls for partnerships at all levels of public and private endeavor. In addition to detailed information about the core values underlying this health promotion and the goals and action steps themselves, the blueprint includes six appendices: a summary of the plenary session from the conference; a list of participants in the conference; a list of suggested partners in realizing the blueprint; an article on programs and creative strategies to close the gap (including some programs already in place); a summary of the Surgeon General's Listening Session on Health Disparities and Mental Retardation; and a list of the speakers from that listening session.

Book Summaries: Online Booksellers Commercial Internet-based booksellers, such as Amazon.com and Barnes&Noble.com, offer summaries which have been supplied by each title’s publisher. Some summaries also include customer reviews. Your local bookseller may have access to in-house and commercial databases that index all published books (e.g. Books in Print). IMPORTANT NOTE: Online booksellers typically produce search results for medical and non-medical books. When searching for “mental retardation” at online booksellers’ Web sites, you may discover non-medical books that use the generic term “mental retardation” (or a synonym)

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in their titles. The following is indicative of the results you might find when searching for “mental retardation” (sorted alphabetically by title; follow the hyperlink to view more details at Amazon.com): •

"We're People First": The Social and Emotional Lives of Individuals with Mental Retardation by Elaine E. Castles (Author) (1996); ISBN: 0275952436; http://www.amazon.com/exec/obidos/ASIN/0275952436/icongroupinterna

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1994 Making Accreditation Decisions for Organizations Providing Mental Health, Chemical Dependency, and Mental Retardation/Developmental Disabilities (1993); ISBN: 9993943150; http://www.amazon.com/exec/obidos/ASIN/9993943150/icongroupinterna

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1995 Making Accreditation Decisions for Organizations Providing Mental Health, Chemical Dependency, and Mental Retardation/Developmental Disabilities (1995); ISBN: 9995521407; http://www.amazon.com/exec/obidos/ASIN/9995521407/icongroupinterna

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A Family-Centered Approach to People With Mental Retardation (Innovations, No 14) by Linda Leal (1998); ISBN: 0940898594; http://www.amazon.com/exec/obidos/ASIN/0940898594/icongroupinterna

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A History of Nursing in the Field of Mental Retardation and Developmental Disabilities by Wendy M. Nehring, Gary N. Siperstein (1999); ISBN: 0940898683; http://www.amazon.com/exec/obidos/ASIN/0940898683/icongroupinterna

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Aamr 1992 Mental Retardation Definition Classification and System of Support. by Aamr Pubns Ctr (1992); ISBN: 999559479X; http://www.amazon.com/exec/obidos/ASIN/999559479X/icongroupinterna

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Achieving a Beneficial Fitness: A Program and a Philosophy in Mental Retardation (Contemporary Issues in Health (American Association on Mental Retardation), V. 1, No. 1.) by James H., PhD Rimmer, Allen C., MD Crocker (2000); ISBN: 0940898713; http://www.amazon.com/exec/obidos/ASIN/0940898713/icongroupinterna

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Adults With Autism and Mental Retardation: A Life-Span Perspective (Studia Psychologica Upsaliensia, 20) by Bengt Akerstrom (2001); ISBN: 9155449778; http://www.amazon.com/exec/obidos/ASIN/9155449778/icongroupinterna

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Advances in Mental Retardation & Developmental Disabilities by Stephen E. Breuning (Editor) (1988); ISBN: 0892328584; http://www.amazon.com/exec/obidos/ASIN/0892328584/icongroupinterna

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Advances in Mental Retardation and Developmental Disabilities (1991); ISBN: 0892326301; http://www.amazon.com/exec/obidos/ASIN/0892326301/icongroupinterna

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Advances in Mental Retardation and Developmental Disabilities: 1991 by Robert A. Gable (Editor) (1991); ISBN: 1853020753; http://www.amazon.com/exec/obidos/ASIN/1853020753/icongroupinterna

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Advances in Mental Retardation and Developmental Disabilities: A Research Annual by Stephen E. Breuning, et al (1987); ISBN: 0892325437; http://www.amazon.com/exec/obidos/ASIN/0892325437/icongroupinterna

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Age Appropriate Activities for Adults With Profound Mental Retardation: A Collaborative Design by Music Therapy, Occupational Therapy and Speech Pathology by Nina Galerstein, et al (2001); ISBN: 1581060084; http://www.amazon.com/exec/obidos/ASIN/1581060084/icongroupinterna

192 Mental Retardation

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An Introduction to the Nature and Needs of Students With Mild Disabilities: Mild Mental Retardation, Behavior Disorders, and Learning Disabilities by Caroll J. Jones, Carroll J. Jones (1996); ISBN: 0398067120; http://www.amazon.com/exec/obidos/ASIN/0398067120/icongroupinterna

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Assessment of Individuals With Mental Retardation by Ronald L. Taylor (Editor) (1997); ISBN: 1565937082; http://www.amazon.com/exec/obidos/ASIN/1565937082/icongroupinterna

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Autism and Autistic-Like Conditions in Mental Retardation by Dirk W. Kraijer (1997); ISBN: 9026514638; http://www.amazon.com/exec/obidos/ASIN/9026514638/icongroupinterna

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Bridges: A Curriculum for Persons With Mental Retardation: Summer (New Invitation Bridges , Vol 3) by Hilda R. Davis (Editor) (1998); ISBN: 0687719895; http://www.amazon.com/exec/obidos/ASIN/0687719895/icongroupinterna

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Children's Health: Mental Retardation by Thomas C., MD Jefferson (Editor), Tracy Irons-Georges (Editor) (1999); ISBN: 0893569461; http://www.amazon.com/exec/obidos/ASIN/0893569461/icongroupinterna

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Communicative Competence in Persons With Profound Mental Retardation: Intervention Focused on the Social Context (Studia Psychologica Clinica Upsali) by Mats Granlund (1993); ISBN: 9155431186; http://www.amazon.com/exec/obidos/ASIN/9155431186/icongroupinterna

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Confronting the Stigma in Their Lives: Helping People With a Mental Retardation Label by James R. Dudley (1997); ISBN: 0398067384; http://www.amazon.com/exec/obidos/ASIN/0398067384/icongroupinterna

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DC-LD: Diagnostic Criteria for Psychiatric Disorders for Use with Adults with Learning Disabilities/mental Retardation: Occasional Paper OP48 by Royal College of Psychiatrists (2001); ISBN: 1901242617; http://www.amazon.com/exec/obidos/ASIN/1901242617/icongroupinterna

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Differences in Common: Straight Talk on Mental Retardation, Down Syndrome, and Your Life by Marilyn Trainer, Helen Featherstone (2003); ISBN: 0933149409; http://www.amazon.com/exec/obidos/ASIN/0933149409/icongroupinterna

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Dual Diagnosis Bibliography 1995: (Mental Illness and Mental Retardation) by Caroline Mohr (Compiler), Kathryn Bays (Compiler) (1995); ISBN: 1884442137; http://www.amazon.com/exec/obidos/ASIN/1884442137/icongroupinterna

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Efficacy of Special Education and Related Services (Monographs of the American Association on Mental Retardation, 19) by Kenneth A., PhD Kavale, et al (1998); ISBN: 0940898519; http://www.amazon.com/exec/obidos/ASIN/0940898519/icongroupinterna

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Ellis' Handbook of Mental Deficiency, Psychological Theory and Research by William E., Jr MacLean (Editor) (1996); ISBN: 0805814078; http://www.amazon.com/exec/obidos/ASIN/0805814078/icongroupinterna

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Emerging Issues in Family Support (Monographs of the American Association on Mental Retardation, 18) by Valerie J. Bradley (Editor), et al (1992); ISBN: 0940898292; http://www.amazon.com/exec/obidos/ASIN/0940898292/icongroupinterna

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Equal Treatment for People With Mental Retardation: Having and Raising Children by Martha A. Field, Valerie A. Sanchez (2001); ISBN: 0674006976; http://www.amazon.com/exec/obidos/ASIN/0674006976/icongroupinterna

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Families and Mental Retardation: New Directions in Professional Practice by Diane T. Marsh (Author) (1992); ISBN: 0275940144; http://www.amazon.com/exec/obidos/ASIN/0275940144/icongroupinterna

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Foster Family Care for Persons With Mental Retardation (Monographs of the American Association on Mental Retardation, No 17) by Sharon A. Borthwick-Duffy, et al (1992); ISBN: 0940898276; http://www.amazon.com/exec/obidos/ASIN/0940898276/icongroupinterna

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Gay, Lesbian, Bisexual, and Transgender People With Developmental Disabilities and Mental Retardation: Stories of the Rainbow Support Group by John D. Allen (2003); ISBN: 1560233966; http://www.amazon.com/exec/obidos/ASIN/1560233966/icongroupinterna

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Genetics and Mental Retardation Syndromes: A New Look at Behavior and Interventions by Elisabeth M. Dykens, et al (2000); ISBN: 1557664714; http://www.amazon.com/exec/obidos/ASIN/1557664714/icongroupinterna

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Guidelines for Library Services for People With Mental Retardation by American Library Association (1999); ISBN: 0838980007; http://www.amazon.com/exec/obidos/ASIN/0838980007/icongroupinterna

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Handbook for Qualified Mental Retardation Professionals by Art Dykstra (Editor), Thane Dykstra (Editor) (1998); ISBN: 0965374432; http://www.amazon.com/exec/obidos/ASIN/0965374432/icongroupinterna

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Handbook of Challenging Behavior: Mental Health Aspects of Mental Retardation by Steven Reiss (1994); ISBN: 0964259818; http://www.amazon.com/exec/obidos/ASIN/0964259818/icongroupinterna

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Handbook of Mental Retardation and Development by Jacob A. Burack (Author), et al (1998); ISBN: 0521446686; http://www.amazon.com/exec/obidos/ASIN/0521446686/icongroupinterna

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Health Care Financing for Severe Developmental Disabilities (Monographs of the American Association on Mental Retardation, No. 14) by Arnold Birenbaum, et al (1990); ISBN: 0940898241; http://www.amazon.com/exec/obidos/ASIN/0940898241/icongroupinterna

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Idiots: Stories About Mindedness and Mental Retardation by R. Daniel Linneman (2001); ISBN: 0820450170; http://www.amazon.com/exec/obidos/ASIN/0820450170/icongroupinterna

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Inpatient Utilization of Mental Retardation, Mental Health and Substance Abuse Services in California Hospitals in 1984 (Working Paper, No 41) by Mark Juretic, et al (1987); ISBN: 9998285313; http://www.amazon.com/exec/obidos/ASIN/9998285313/icongroupinterna

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International Review of Research in Mental Retardation : Volume 18 by Norman W. Bray (Editor), Epstein (1992); ISBN: 0123662184; http://www.amazon.com/exec/obidos/ASIN/0123662184/icongroupinterna

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International Review of Research in Mental Retardation, Vol. 25 by Laraine Masters Glidden (Editor) (2002); ISBN: 0123662257; http://www.amazon.com/exec/obidos/ASIN/0123662257/icongroupinterna

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Introduction on Mental Retardation by Smith (2002); ISBN: 0155014900; http://www.amazon.com/exec/obidos/ASIN/0155014900/icongroupinterna

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Inventing the Feeble Mind: A History of Mental Retardation in the United States by James W., Jr Trent (1995); ISBN: 0520203577; http://www.amazon.com/exec/obidos/ASIN/0520203577/icongroupinterna

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Issues in the Developmental Approach to Mental Retardation by Robert M. Hodapp (Editor), et al (1990); ISBN: 0521346193; http://www.amazon.com/exec/obidos/ASIN/0521346193/icongroupinterna

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Key Issues in Mental Retardation Research: Proceedings of the Eighth Congress of the International Association for the Scientific Study of Mental De by William I. Fraser (Editor), International Association for The Scient (1990); ISBN: 0415013631; http://www.amazon.com/exec/obidos/ASIN/0415013631/icongroupinterna

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Language in Mental Retardation by Jean A. Rondal, Susan Edwards (1997); ISBN: 1565938127; http://www.amazon.com/exec/obidos/ASIN/1565938127/icongroupinterna

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Leslie's Story: A Book About a Girl With Mental Retardation by Martha McNey (Photographer), Leslie Fish (1996); ISBN: 0822525763; http://www.amazon.com/exec/obidos/ASIN/0822525763/icongroupinterna

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Life Expectancy and Mental Retardation: A Longitudinal Study in a State Residential Facility by Richard K. Eyman (Editor), Roger Eyman (1987); ISBN: 0940898144; http://www.amazon.com/exec/obidos/ASIN/0940898144/icongroupinterna

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Localisation of Genetic Factors for Nonspecific and Syndromic X-linked Mental Retardation (Acta Biomedica Lovaniensia) by S. Claes (1997); ISBN: 906186853X; http://www.amazon.com/exec/obidos/ASIN/906186853X/icongroupinterna

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Manual of Diagnosis and Professional Practice in Mental Retardation by John W. Jacobson (Editor), James A. Mulick (Editor) (1996); ISBN: 1557983410; http://www.amazon.com/exec/obidos/ASIN/1557983410/icongroupinterna

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Meaningful Care: A Multidisciplinary Approach to the Meaning of Care for People With Mental Retardation by Joop Stolk (Editor), et al (2000); ISBN: 0792362918; http://www.amazon.com/exec/obidos/ASIN/0792362918/icongroupinterna

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Mental Deficiency (1986); ISBN: 0029056209; http://www.amazon.com/exec/obidos/ASIN/0029056209/icongroupinterna

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Mental Retardation and Developmental Disabilities by McLaughlin Phillip J. (Editor), Paul Wehman (Editor) (1996); ISBN: 0890796432; http://www.amazon.com/exec/obidos/ASIN/0890796432/icongroupinterna

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Mental Retardation Handbook by Martin N. Levine (1989); ISBN: 0874242304; http://www.amazon.com/exec/obidos/ASIN/0874242304/icongroupinterna

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Mental Retardation in America: A Historical Reader by Steven Noll (Editor), James W. Trent (Editor) (2004); ISBN: 0814782477; http://www.amazon.com/exec/obidos/ASIN/0814782477/icongroupinterna

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Mental Retardation in the 21st Century by Michael L. Wehmeyer (Editor), James R. Patton (Editor) (1999); ISBN: 0890798192; http://www.amazon.com/exec/obidos/ASIN/0890798192/icongroupinterna

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Mental Retardation Programs: How Does Massachusetts Compare? (Pioneer Paper, No. 4) by Edward, Moscovitch (1991); ISBN: 0929930061; http://www.amazon.com/exec/obidos/ASIN/0929930061/icongroupinterna

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Mental Retardation: A Life Cycle Approach by Clifford J. Drew (1992); ISBN: 0675213576; http://www.amazon.com/exec/obidos/ASIN/0675213576/icongroupinterna

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Mental Retardation: A LifeSpan Approach to People with Intellectual Disabilities, Eighth Edition by Clifford J. Drew (Author), Michael L. Hardman (Author) (2003); ISBN: 0131112163; http://www.amazon.com/exec/obidos/ASIN/0131112163/icongroupinterna

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Mental Retardation: Definition, Classification, and Systems of Support (Book+workbook+forms Package) by American Association of Mental Retardati (2002); ISBN: 0940898845; http://www.amazon.com/exec/obidos/ASIN/0940898845/icongroupinterna

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Mental Retardation: Determining Eligibility for Social Security Benefits by Daniel J. Reschly (Editor), et al (2002); ISBN: 0309083230; http://www.amazon.com/exec/obidos/ASIN/0309083230/icongroupinterna

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Mental Retardation: Developing Pharmacotherapies (Progress in Psychiatry, No 32) by John J. Ratey (Editor) (1991); ISBN: 0880484527; http://www.amazon.com/exec/obidos/ASIN/0880484527/icongroupinterna

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Mental Retardation: Nature, Cause, and Management by George S. Baroff, George S. Baroff (1999); ISBN: 1583910018; http://www.amazon.com/exec/obidos/ASIN/1583910018/icongroupinterna

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Mental Retardation: Subject Reference and Research Guide by Ruby M. Starr (1987); ISBN: 0881645575; http://www.amazon.com/exec/obidos/ASIN/0881645575/icongroupinterna

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Mhm: 1993 Accreditation Manual for Mental Health, Chemical Dependency, and Mental Retardation/Developmental Diabilities Services (1992); ISBN: 0866883053; http://www.amazon.com/exec/obidos/ASIN/0866883053/icongroupinterna

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Mhm: 1995 Accrediation Manual for Mental Health, Chemical Dependency, and Mental Retardation/Developmental Disabilities Service (Serial) (1995); ISBN: 0866883681; http://www.amazon.com/exec/obidos/ASIN/0866883681/icongroupinterna

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Milwaukee Project Preventing Mental Retardation in Children at Risk by Howard L. Garber (1988); ISBN: 0940898160; http://www.amazon.com/exec/obidos/ASIN/0940898160/icongroupinterna

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Monitoring and Evaluation of the Quality and Appropriateness of Care: A Mental Retardation and Developmental Disability Services Example (1989); ISBN: 0866882189; http://www.amazon.com/exec/obidos/ASIN/0866882189/icongroupinterna

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Movement Differences and Diversity in Autism-Mental Retardation: Appreciations and Accommodations People With Communications and Behavior Challenges by Anne M. Donnellan, Martha R. Leary (1994); ISBN: 1886928002; http://www.amazon.com/exec/obidos/ASIN/1886928002/icongroupinterna

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New Invitation Bridges: A Curriculum for Persons With Mental Retardation: Winter (New Invitation Bridges , Vol 3) by Hilda R. Davis (Editor) (1997); ISBN: 0687719879; http://www.amazon.com/exec/obidos/ASIN/0687719879/icongroupinterna

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On My Own: The Impact of Semi-Independent Living Programs for Adults With Mental Retardation by Andrew S. Halpern, et al (1986); ISBN: 0933716567; http://www.amazon.com/exec/obidos/ASIN/0933716567/icongroupinterna

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Parent Training and Developmental Disabilities (Monographs of the American Association on Mental Retardation, Vol 13) by Bruce L. Baker, et al (1989); ISBN: 0940898225; http://www.amazon.com/exec/obidos/ASIN/0940898225/icongroupinterna

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Parents for Children, Children for Parents: The Adoption Alternative (Monographs of the American Association on Mental Retardation, 11) by Laraine Masters Glidden (1989); ISBN: 0940898209; http://www.amazon.com/exec/obidos/ASIN/0940898209/icongroupinterna

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Participatory Evaluation Strategy for Special Education and Rehabilitation (Monographs of the American Association on Mental Retardation) by Darrell R. Lewis, et al (2000); ISBN: 094089873X; http://www.amazon.com/exec/obidos/ASIN/094089873X/icongroupinterna

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Personality and Motivational Differences in Persons With Mental Retardation (The Lea Series on Special Education and Disability) by Harvey N. Switzky (Editor), Harvey L. Switzky (Editor) (2001); ISBN: 080582569X; http://www.amazon.com/exec/obidos/ASIN/080582569X/icongroupinterna

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Personality Development in Individuals with Mental Retardation by Edward Zigler (Editor), Dianne Bennett-Gates (Editor) (1999); ISBN: 0521639638; http://www.amazon.com/exec/obidos/ASIN/0521639638/icongroupinterna

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Physical and Motor Development in Mental Retardation (Medicine and Sport Science, Vol 40) by Adri Vermeer (Editor), et al (1995); ISBN: 3805561180; http://www.amazon.com/exec/obidos/ASIN/3805561180/icongroupinterna

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Positional Cloning of X-Linked Mental Retardation Genes (Acta Biomedica Lovaniensia, 244) by Jun Lin (2001); ISBN: 9058671682; http://www.amazon.com/exec/obidos/ASIN/9058671682/icongroupinterna

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Questions of Destiny: Mental Retardation and Curative Education by Carlo, Pietzner (1996); ISBN: 0880102640; http://www.amazon.com/exec/obidos/ASIN/0880102640/icongroupinterna

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Retardacion Mental: Definicion, Clasificacionk, Y Sistemas De Apoyo: Mental Retardation by Ruth A. Luckasson, Margaret M. Seiter (1994); ISBN: 0940898357; http://www.amazon.com/exec/obidos/ASIN/0940898357/icongroupinterna

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Robert Guthrie--The Pku Story: Crusade Against Mental Retardation by Jean Holt Koch, L. I. Woolf (Preface) (1997); ISBN: 0932727913; http://www.amazon.com/exec/obidos/ASIN/0932727913/icongroupinterna

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Staff Recruitment and Retention: Study Results and Intervention Strategies (Monographs of the American Association on Mental Retardation) by Sheryl Ann Larson, et al (1998); ISBN: 094089856X; http://www.amazon.com/exec/obidos/ASIN/094089856X/icongroupinterna

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Teaching Students with Mental Retardation by Vivienne C. Riches, et al (1997); ISBN: 1557662584; http://www.amazon.com/exec/obidos/ASIN/1557662584/icongroupinterna

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Teaching Students With Mental Retardation: Providing Access to the General Curriculum by Michael L. Wehmeyer (Editor), et al (2001); ISBN: 1557665281; http://www.amazon.com/exec/obidos/ASIN/1557665281/icongroupinterna

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The Americans With Disabilities Act and the Emerging Workforce: Employment of People With Mental Retardation by Peter David Blanck, David L. Braddock (1998); ISBN: 0940898527; http://www.amazon.com/exec/obidos/ASIN/0940898527/icongroupinterna

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The Genetics of Mental Retardation: Biomedical, Psychosocial and Ethical Issues by Esther K. Hicks (Editor), et al (1988); ISBN: 155608062X; http://www.amazon.com/exec/obidos/ASIN/155608062X/icongroupinterna

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The Kennedy Family and the History of Mental Retardation by Edward Shorter (2000); ISBN: 1566397839; http://www.amazon.com/exec/obidos/ASIN/1566397839/icongroupinterna

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The Mental Retardation and Developmental Disability Treatment Planner by Arthur E. Jongsma Jr., et al (2000); ISBN: 0471382531; http://www.amazon.com/exec/obidos/ASIN/0471382531/icongroupinterna

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The Problem of Mental Deficiency: Eugenics, Democracy, and Social Policy in Britain C.1870-1959 (Oxford Historical Monographs) by Mathew Thomson (1998); ISBN: 0198206925; http://www.amazon.com/exec/obidos/ASIN/0198206925/icongroupinterna

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The Rights of People With Mental Disabilities: The Authoritative Aclu Guide to the Rights of People With Mental Illness and Mental Retardation (American Civil Liberties Union Handbook (Paper)) by Robert M. Levy, et al (1996); ISBN: 080931990X; http://www.amazon.com/exec/obidos/ASIN/080931990X/icongroupinterna

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The Use of Projective Techniques With Persons With Mental Retardation: A Guide for Assessment Instrument Selection by Paul E. Panek (1997); ISBN: 0398067562; http://www.amazon.com/exec/obidos/ASIN/0398067562/icongroupinterna

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Total Quality Management in Mental Health and Mental Retardation (Contemporary Issues in Administration, V. 1, No. 1) by Gary V. Sluyter (Editor) (1999); ISBN: 0940898675; http://www.amazon.com/exec/obidos/ASIN/0940898675/icongroupinterna

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Toxic Substances and Mental Retardation: Neurobehavioral Toxicology and Teratology (Monographs of the American Association of Mental Deficiency, 8) by Stephen R. Schroeder (Editor) (1987); ISBN: 0940898152; http://www.amazon.com/exec/obidos/ASIN/0940898152/icongroupinterna

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Twenty-Two Years: Causes and Consequences of Mental Retardation by Stephen A. Richardson, Helene Koller (1997); ISBN: 0674212975; http://www.amazon.com/exec/obidos/ASIN/0674212975/icongroupinterna

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Understanding Mental Retardation by Edward Zigler (Author), Robert M. Hodapp (Author) (1986); ISBN: 0521318785; http://www.amazon.com/exec/obidos/ASIN/0521318785/icongroupinterna

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Virginia Mental Health, Mental Retardation, Substance Abuse and Related Laws of Virginia Annotated (1997); ISBN: 1558346783; http://www.amazon.com/exec/obidos/ASIN/1558346783/icongroupinterna

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Vulnerable Populations: Sexual Abuse Treatment for Children, Adult Survivors, Offenders and Persons with Mental Retardation, Vol. 2 by Suzanne M. Sgroi (Editor)

198 Mental Retardation

(1989); ISBN: 0669209430; http://www.amazon.com/exec/obidos/ASIN/0669209430/icongroupinterna •

What Christians Believe: Confirmation Studies for Persons with Mental Retardation and Developmental Disabilities by George C. Anderson, et al (1999); ISBN: 157895035X; http://www.amazon.com/exec/obidos/ASIN/157895035X/icongroupinterna

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Within Our Reach: Behavior Prevention and Intervention Strategies for Learners With Mental Retardation and Autism by Melissa M. Jones (1998); ISBN: 0865863547; http://www.amazon.com/exec/obidos/ASIN/0865863547/icongroupinterna

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X-Linked Mental Retardation by Charles E. Schwartz (Editor), et al (1999); ISBN: 0195129814; http://www.amazon.com/exec/obidos/ASIN/0195129814/icongroupinterna

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X-Linked Mental Retardation 3 (1988); ISBN: 0845142623; http://www.amazon.com/exec/obidos/ASIN/0845142623/icongroupinterna

The National Library of Medicine Book Index The National Library of Medicine at the National Institutes of Health has a massive database of books published on healthcare and biomedicine. Go to the following Internet site, http://locatorplus.gov/, and then select “Search LOCATORplus.” Once you are in the search area, simply type “mental retardation” (or synonyms) into the search box, and select “books only.” From there, results can be sorted by publication date, author, or relevance. The following was recently catalogued by the National Library of Medicine:11 •

A report of the Governor's Advisory Committee on Mental Retardation, October 8, 1962. Author: Minnesota. Governor's Advisory Committee on Mental Retardation.; Year: 1965; [St. Paul, 1962]

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Action in mental retardation; at Psychiatric Centre, North Ryde, 2d and 3d November, 1963. Author: Conference on Mental Retardation (Australia) 2d, Sydney, 1963.; Year: 1960; [Sydney] Dept. of Public Health, New South Wales [1964]

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Fiscal year 1965 appropriations for mental retardation programs of the Dept. of Health, Education, and Welfare. Author: United States. Dept. of Health, Education, and Welfare. Secretary's Committee on Mental Retardation.; Year: 1964; Washington, 1964

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Implementation of mental retardation programs; report to the President. Author: United States. Dept. of Health, Education, and Welfare. Secretary's Committee on Mental Retardation.; Year: 1964; Washington, 1964

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Medical aspects of mental retardation. Author: Carter, Charles H. (Charles Herschel),; Year: 1961; Springfield, Ill., Thomas [c1965]

11

In addition to LOCATORPlus, in collaboration with authors and publishers, the National Center for Biotechnology Information (NCBI) is currently adapting biomedical books for the Web. The books may be accessed in two ways: (1) by searching directly using any search term or phrase (in the same way as the bibliographic database PubMed), or (2) by following the links to PubMed abstracts. Each PubMed abstract has a "Books" button that displays a facsimile of the abstract in which some phrases are hypertext links. These phrases are also found in the books available at NCBI. Click on hyperlinked results in the list of books in which the phrase is found. Currently, the majority of the links are between the books and PubMed. In the future, more links will be created between the books and other types of information, such as gene and protein sequences and macromolecular structures. See http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Books.

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•

Mental retardation activities of the U. S. Department of Health, Education, and Welfare. Author: United States. Dept. of Health, Education, and Welfare. Secretary's Committee on Mental Retardation.; Year: 1965; Washington, 1965

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Mental retardation and the law; a survey of California laws affecting the mentally retarded, comp. by Kay Werdegar. Author: California. Study Commission on Mental Retardation.; Year: 1964; [Sacramento, 1964]

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Mental retardation: a family study [by] Elizabeth W. Reed and Sheldon C. Reed. Author: Reed, Elizabeth W.; Year: 1963; Philadelphia, Saunders, 1965

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Mental retardation; a handbook for the primary physician. Manuscript prepared by Cathy Covert. Report of the American Medical Association Conference on mental retardation, Chicago, April 9 to 11, 1964. Author: American Medical Association.; Year: 1964; [Chicago, c1965]

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Public health nursing in a mental retardation program, by Ita K. McDermott in collaboration with Carolyn F. Grimm and Eleanor R. Speer. Author: McDermott, Ita K.; Year: 1965; New York, National League for Nursing, 1960

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Summary of selected financial assistance programs in mental retardation of the Department of Health, Education, and Welfare. Author: United States. Dept. of Health, Education, and Welfare. Secretary's Committee on Mental Retardation.; Year: 1957; [Washington] 1964

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Terminology and concepts in mental retardation [by] Joel R. Davitz [et al.]. Author: Davitz, Joel R. (Joel Robert); Year: 1963; New York, Bureau of Publications, Teachers College, Columbia Univ., 1964

Chapters on Mental Retardation In order to find chapters that specifically relate to mental retardation, an excellent source of abstracts is the Combined Health Information Database. You will need to limit your search to book chapters and mental retardation using the “Detailed Search” option. Go to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find book chapters, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Book Chapter.” Type “mental retardation” (or synonyms) into the “For these words:” box. The following is a typical result when searching for book chapters on mental retardation: •

Why Is Providing Dental Care to People with Mental Retardation and Developmental Disabilities Such a Low Priority? Source: in Fenton, S.J.; Perlman, S.; Turner, H., eds. Oral Healthcare for People with Special Needs: Guidelines for Comprehensive Care. River Edge, NJ: Exceptional Parent, Psy-Ed Corp. 2003. p. 12-14, 46. Contact: Available as part of a monograph from Exceptional Parent, Psy-Ed Corp. 65 East Route 4, River Edge, NJ 07661. (800) EPARENT or (800) 372-7368. E-mail: [email protected]. Website: www.eparent.com. PRICE: Contact publisher. Summary: As a result of developments since the mid 1950s, the care of individuals with mental retardation and other development disabilities have increased this population's dependency for dental and other health services on community practitioners. This article is from a monograph that offers guidelines for the comprehensive oral health care for people with special needs. The monograph is designed to help oral health care

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providers embrace more fully all the members of their communities, while being respectful of a variety of special needs. In this article, the authors consider the reasons why providing dental care to people with mental retardation and other developmental disabilities is such a low priority. The authors provide statistics and other information to describe how health care delivery systems and how dental student and practitioner circumstances have changed over time. Topics include the number of dental students, the number of dental practitioners, student debt and employment, and third party economics. The authors do not offer any conclusions or solutions, merely present the current situation as they perceive it. 5 tables. 27 references. •

Children with Mental Retardation/Developmental Disabilities: Do Physicians Ever Consider Needed Dental Care? Source: in Fenton, S.J.; Perlman, S.; Turner, H., eds. Oral Healthcare for People with Special Needs: Guidelines for Comprehensive Care. River Edge, NJ: Exceptional Parent, Psy-Ed Corp. 2003. p. 8-11, 46. Contact: Available as part of a monograph from Exceptional Parent, Psy-Ed Corp. 65 East Route 4, River Edge, NJ 07661. (800) EPARENT or (800) 372-7368. E-mail: [email protected]. Website: www.eparent.com. PRICE: Contact publisher. Summary: As with general medical care, when children and adults with mental retardation and other developmental disabilities were residents of state institutions, they were, to some extent, assured of dental services from state employees. Deinstitutionalization, with resulting community group homes and family residences, has resulted in a breakdown in the continuity of services, especially dental services. This article is from a monograph that offers guidelines for the comprehensive oral health care for people with special needs. The monograph is designed to help oral health care providers embrace more fully all the members of their communities, while being respectful of a variety of special needs. In this article, the authors consider the typical oral health needs of children with mental retardation and other developmental disabilities and encourage physicians to refer children to dental care providers. Topics include baby bottle tooth decay (early childhood caries), prescription medicine-induced dental decay, altered salivary flow and tooth decay, malocclusions, fractured and nonvital teeth, soft tissue complications, and bruxism (grinding of the teeth). The authors stress that, as with all forms of medical and dental services, early intervention is preferable to extensive services at some later date. 13 references.

•

Toilet Training People With Mental Retardation Source: in Jeter, K.F.; Faller, N.; Norton, C., eds. Nursing for Continence. Orlando, FL: W.B. Saunders Company. 1990. p. 199-207. Contact: Available from W.B. Saunders Company. 6277 Sea Harbor Drive, Orlando, FL 32887. (800) 545-2522. Fax (800) 874-6418. PRICE: $35.50 plus shipping. ISBN: 0721628923. Summary: Most people with mental retardation, whatever their degree of impairment, have the potential to attain some degree of continence. This chapter, from a nursing textbook about the diagnosis, treatment, and management of incontinence, discusses toilet training people with mental retardation. Four sections cover training methods, baseline observation, the training program, and containment methods for those persons unresponsive to continence training. Intervention programs are aimed at maximizing each individual's potential, whether to enable eventual community placement or to improve their quality of life in the institution. 1 figure. 1 table. 9 references.

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Dental Health Among Individuals with Mental Retardation Source: in Horwitz, S.M.; Kerker, B. D.; Owens, P.L.; Zigler, E. Health Status and Needs of Individuals with Mental Retardation. New Haven, Connecticut: Department of Epidemiology and Public Health, Yale University School of Medicine. 2000. p.119-175. Contact: Available from Department of Epidemiology and Public Health, Yale University School of Medicine. 60 College Street, P.O. Box 208034, New Haven, Connecticut 06520-8034551. (203)785-6283. Fax: (203)785-6287. Website: http://info.med.yale.edu/eph/html/eph_contacts.html PRICE: Single copy free, plus shipping and handling. Summary: Poor oral health can have dramatic effects on an individual's quality of life. This chapter on dental health among individuals with mental retardation is from a monograph on the general health status and needs of this population. Besides dental caries (cavities) and tooth loss, other oral health concerns include gingivitis (inflammation of the gums) and other periodontal diseases (loss of connective and bone tissues that support the teeth). People with mental retardation (MR) have poorer overall oral health and oral hygiene compared with the general population. The oral health and hygiene of people with MR is associated with severity of MR, etiology (cause) of MR, residential arrangements, and age of the individual. The authors note that although there are inconsistent findings on the prevalence of dental caries among people with MR compared with the general population, the majority of evidence suggests that persons with MR have more untreated caries than those in the general population. Given that treatment of caries is a prevalent and accepted part of good health behavior for much of the world, this lack of treatment, even in developed countries, suggests problems in access to dental services. The authors conclude that efforts to improve the oral hygiene of people with mild MR may be a particularly effective intervention. 59 references.

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Nutrition Concerns for Individuals with Mental Retardation and Other Developmental Disabilities Source: in Fenton, S.J.; Perlman, S.; Turner, H., eds. Oral Health Care for People with Special Needs: Guidelines for Comprehensive Care. River Edge, NJ: Exceptional Parent, Psy-Ed Corp. 2003. p. 28,46. Contact: Available as part of a monograph from Exceptional Parent, Psy-Ed Corp. 65 East Route 4, River Edge, NJ 07661. (800) EPARENT or (800) 372-7368. E-mail: [email protected]. Website: www.eparent.com. PRICE: Contact publisher. Summary: This brief article is from a monograph that offers guidelines for comprehensive oral health care for people with special needs. The monograph is designed to help oral health care providers embrace more fully all the members of their communities, while being respectful of a variety of special needs. In this article, the author considers nutrition concerns for individuals with mental retardation and other developmental disabilities. Topics include the risks of poor nutrition, prevention of early childhood caries (ECC, cavities), the goal of nutrition services, and general nutrition guidance. One table highlights the nutrition problems that are common to selected disabilities, including cerebral palsy, epilepsy, muscular dystrophy, melomeningocele, Down syndrome, Prader-Willi syndrome, mental retardation, and autism. 1 table. 4 references.

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Williams Syndrome: Hypercalcemia, Supravalvular Aortic Stenosis, Elfin Facies, and Mental Retardation Syndrome Source: in Plumridge, D., et al., eds. Student with a Genetic Disorder: Educational Implications for Special Education Teachers and for Physical Therapists, Occupational Therapists, and Speech Pathologists. Springfield, IL: Charles C Thomas Publisher. 1993. p. 171-177. Contact: Available from Charles C Thomas Publisher. 2600 South First Street, Springfield, IL 62794-9265. (212) 789-8980. Fax (217) 789-9130. PRICE: $75.95 plus shipping and handling (cloth); $39.95 plus shipping and handling (paper). ISBN: 0398058393. Summary: Williams syndrome is a multisystem disorder that includes hypercalcemia, supravulvular aortic stenosis, characteristic facial features, distinctive behavioral characteristics, and mental retardation. This chapter on Williams syndrome is from a text for special education teachers, physical therapists, occupational therapists, and speech pathologists on the educational implications of genetic disorders. Topics covered include the physical and characteristic features of the disorder, the genetics of the disorder, the cognitive and behavior profiles, the educational implications, physical therapy, occupational therapy, hearing and speech considerations, psychosocial issues, and prognosis. 1 figure. 8 references.

Directories In addition to the references and resources discussed earlier in this chapter, a number of directories relating to mental retardation have been published that consolidate information across various sources. The Combined Health Information Database lists the following, which you may wish to consult in your local medical library:12 •

Directory of Dental Services in Residential Facilities for the Mentally Retarded and Mentally Ill Source: Chicago, IL: Academy of Dentistry for People with Disabilities. 1993. 89 p. Contact: Available from Academy of Dentistry for People with Disabilities. 211 East Chicago Avenue, Chicago, IL 60611. (312) 440-2660. PRICE: $25.00 for members of the Academy and members of Special Care Dentistry; $45.00 for nonmembers, plus shipping and handling. Summary: Deinstitutionalization has had a profound impact over the past decade on dental services provided to persons with mental retardation and mental illness (MH/MR) who still reside in institutions. In order to assess the extent of this change, and to help plan for dental services for those persons who will continue to be deinstitutionalized, this Directory of Dental Services was compiled. For each state, the following information is listed: state dental director, dental director for MH/MR programs, contact person for dental program directory, additional information and

12

You will need to limit your search to “Directory” and “mental retardation” using the "Detailed Search" option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find directories, use the drop boxes at the bottom of the search page where “You may refine your search by.” For publication date, select “All Years.” Select your preferred language and the format option “Directory.” Type “mental retardation” (or synonyms) into the “For these words:” box. You should check back periodically with this database as it is updated every three months.

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comments, a list of MH/MR facilities, Medicaid coverage for dentistry, and a summary of dental staff/client information. A brief glossary of terms is also included. •

Parent Resources: Agencies, Organizations, Support Groups Source: in DeFeo, A.B., ed. Parent Articles 2. San Antonio, TX: Communication Skill Builders. 1995. p. 213-234. Contact: Available from Communication Skill Builders. Customer Service, 555 Academic Court, San Antonio, TX 78204-2498. (800) 211-8378; Fax (800) 232-1223. PRICE: $55.00 plus shipping and handling. Order Number 076-163-0732. Summary: This appendix section is from a parent education skill builders textbook. The appendix lists agencies, organizations, and support groups that parents might want to contact as they work with developing communication skills in and with their child. National information and advocacy groups are listed, including groups for consumer information, education, financial aid, home care, legal assistance, nonoral communication, orthotics and prosthetics, psychiatry, psychology, rare disorders, rehabilitation, residential placement, self-help, severe disabilities, sibling support, social workers, and telephone usage for persons with disabilities. Also listed are national organizations for specific disabilities and conditions, including acoustic neuroma, autism, birth defects, chronic dizziness and balance disorders, cleft palate and craniofacial disorders, developmental disabilities, Down's syndrome, dyslexia, dystonia, genetic conditions, head injuries, hearing impairments, learning disabilities, mental retardation, neurofibromatosis, neurological disorders, stuttering, Tourette syndrome, and voice disorders and laryngectomies. The address and telephone number for each organization are noted.

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National Information Center for Children and Youth with Disabilities State Resource Sheets Source: Washington, DC: National Information Center for Children and Youth with Disabilities (NICHCY). 1997. 216 p. Contact: National Information Center for Children and Youth with Disabilities (NICHCY). P.O. Box 1492, Washington, DC 20013-1492. Voice/TTY (800) 695-0285 or (202) 884-8200; E-mail: [email protected]; http://www.nichcy.org. PRICE: $10.00 for complete set; copy of state resource sheet is available at no charge. Order Number GR6 (state resource sheet); Number GR7 (complete set of state resource sheets). Summary: This directory lists individual state resources for children and youth with disabilities. Fifty separate chapters list the resources in each state. Included are the contact information for the senators and the governor, the state's department of education, programs for children with disabilities, programs for infants and toddlers with disabilities, the state vocational rehabilitation agency, state mental health representative for children and youth, state mental retardation program, state developmental disabilities planning council, protection and advocacy agency, client assistance program, programs for children with special health care needs, disability organizations, university-affiliated programs, technology-related assistance, parent training information project, parent teacher association, and other disability organizations. For each organization or program listed, the directory provides the director's name, the address, and the telephone numbers.

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CHAPTER 8. MULTIMEDIA ON MENTAL RETARDATION Overview In this chapter, we show you how to keep current on multimedia sources of information on mental retardation. We start with sources that have been summarized by federal agencies, and then show you how to find bibliographic information catalogued by the National Library of Medicine.

Video Recordings An excellent source of multimedia information on mental retardation is the Combined Health Information Database. You will need to limit your search to “Videorecording” and “mental retardation” using the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find video productions, use the drop boxes at the bottom of the search page where “You may refine your search by.” Select the dates and language you prefer, and the format option “Videorecording (videotape, videocassette, etc.).” Type “mental retardation” (or synonyms) into the “For these words:” box. The following is a typical result when searching for video recordings on mental retardation: •

Making Contact: A Strategy to Train Health Care Professionals to Communicate with Adults with Mental Retardation: Dental Version Source: Belleair Bluffs, FL: Suncoast Media, Inc. 1990. (Videocassette and manual). Contact: Available from Suncoast Media, Inc. 2938 West Bay Drive, Suite B, Belleair Bluffs, FL 34640. Voice (800) 899-1008; Fax (813) 587-7942. PRICE: $79.00 plus shipping and handling. Summary: These instructional materials are designed to show how dental care providers can build a productive professional relationship with adult clients with mental retardation. The videotape uses dramatic situations and vignettes to show techniques that can be used with individuals with mental retardation to assist them to recall events, remember instructions, and comprehend questions. The manual presents basic information on mental retardation; information on the cognitive and communicative abilities of persons with mental retardation; a review of common attitudes toward

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individuals with mental retardation; and alternative methods of communication that remove barriers and facilitate effective two-way communication. 28 references. (AA-M).

Bibliography: Multimedia on Mental Retardation The National Library of Medicine is a rich source of information on healthcare-related multimedia productions including slides, computer software, and databases. To access the multimedia database, go to the following Web site: http://locatorplus.gov/. Select “Search LOCATORplus.” Once in the search area, simply type in mental retardation (or synonyms). Then, in the option box provided below the search box, select “Audiovisuals and Computer Files.” From there, you can choose to sort results by publication date, author, or relevance. The following multimedia has been indexed on mental retardation: •

Inborn errors of metabolism associated with mental retardation [slide] Source: College of Nursing, Niagara University, in cooperation with the Lakes Area Regional Medical Program; Year: 1975; Format: Slide; [Buffalo]: Communications in Learning, 1975

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Mental retardation [videorecording] Source: presented by School of Social Work, University of Washington and its Group Child Care Project; Year: 1974; Format: Videorecording; [Seattle]: Vernon E. Bryant, 1974

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Mild mental retardation [videorecording]: what rights, what responsibilities Source: UCLA School of Medicine; Year: 1975; Format: Videorecording; Los Angeles: Univ. of California: [for loan or sale by its Instructional Media Library], 1975

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The Nature of mental retardation [motion picture] Source: Vocational Rehabilitation Administration; produced by the Audio-Visual Department of Parsons State Hospital and Training Center; Year: 1968; Format: Motion picture; [Washington: The Administration; Atlanta: for loan by National Medical Audiovisual Center, 1968]

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The President's Committee on Mental Retardation in action [motion picture] Source: University of Kansas Bureau of Child Research and the Kansas Center for Mental Retardation and Human Development; Year: 1974; Format: Motion picture; Lawrence, Kan.: The Bureau, [1974]

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CHAPTER 9. PERIODICALS AND NEWS ON MENTAL RETARDATION Overview In this chapter, we suggest a number of news sources and present various periodicals that cover mental retardation.

News Services and Press Releases One of the simplest ways of tracking press releases on mental retardation is to search the news wires. In the following sample of sources, we will briefly describe how to access each service. These services only post recent news intended for public viewing. PR Newswire To access the PR Newswire archive, simply go to http://www.prnewswire.com/. Select your country. Type “mental retardation” (or synonyms) into the search box. You will automatically receive information on relevant news releases posted within the last 30 days. The search results are shown by order of relevance. Reuters Health The Reuters’ Medical News and Health eLine databases can be very useful in exploring news archives relating to mental retardation. While some of the listed articles are free to view, others are available for purchase for a nominal fee. To access this archive, go to http://www.reutershealth.com/en/index.html and search by “mental retardation” (or synonyms). The following was recently listed in this archive for mental retardation: •

Gene points to mental retardation therapy Source: Reuters Health eLine Date: November 30, 2001

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Gene may point to mental retardation therapy Source: Reuters Industry Breifing Date: November 30, 2001

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Maternal UTIs linked to increased risk of fetal death, mental retardation Source: Reuters Medical News Date: June 18, 2001

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Very early markers of autism, mental retardation identified Source: Reuters Medical News Date: April 25, 2001

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Mice shed light on girls' mental retardation Source: Reuters Health eLine Date: March 13, 2001

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Maternal urinary tract infections linked to mental retardation in infants Source: Reuters Medical News Date: July 10, 2000

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Levels of certain brain chemicals at birth could signal mental retardation, autism Source: Reuters Medical News Date: May 05, 2000

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New cause of mental retardation identified Source: Reuters Health eLine Date: November 12, 1999

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Chromosomal abnormalities common in children with unexplained mental retardation Source: Reuters Medical News Date: November 12, 1999

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Newly identified IL-1 receptor involved in X-linked mental retardation Source: Reuters Medical News Date: September 07, 1999

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Gene mutation causes mental retardation Source: Reuters Health eLine Date: August 31, 1999

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Newborn screening prevents mental retardation Source: Reuters Health eLine Date: May 10, 1999

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Newborn metabolic screening effective in reducing burden of mental retardation Source: Reuters Medical News Date: May 07, 1999

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Iron deficiency anemia in early childhood linked to mental retardation Source: Reuters Medical News Date: January 21, 1999

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Gene linked to mental retardation Source: Reuters Health eLine Date: September 02, 1998

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Neurotransmission defects account for X-linked nonspecific mental retardation Source: Reuters Medical News Date: June 24, 1998

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Oligophrenin-1 Gene Associated With X-Linked Mental Retardation Source: Reuters Medical News Date: April 30, 1998

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Gene for Mental Retardation? Source: Reuters Health eLine Date: April 30, 1997

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The NIH Within MEDLINEplus, the NIH has made an agreement with the New York Times Syndicate, the AP News Service, and Reuters to deliver news that can be browsed by the public. Search news releases at http://www.nlm.nih.gov/medlineplus/alphanews_a.html. MEDLINEplus allows you to browse across an alphabetical index. Or you can search by date at the following Web page: http://www.nlm.nih.gov/medlineplus/newsbydate.html. Often, news items are indexed by MEDLINEplus within its search engine. Business Wire Business Wire is similar to PR Newswire. To access this archive, simply go to http://www.businesswire.com/. You can scan the news by industry category or company name. Market Wire Market Wire is more focused on technology than the other wires. To browse the latest press releases by topic, such as alternative medicine, biotechnology, fitness, healthcare, legal, nutrition, and pharmaceuticals, access Market Wire’s Medical/Health channel at http://www.marketwire.com/mw/release_index?channel=MedicalHealth. Or simply go to Market Wire’s home page at http://www.marketwire.com/mw/home, type “mental retardation” (or synonyms) into the search box, and click on “Search News.” As this service is technology oriented, you may wish to use it when searching for press releases covering diagnostic procedures or tests. Search Engines Medical news is also available in the news sections of commercial Internet search engines. See the health news page at Yahoo (http://dir.yahoo.com/Health/News_and_Media/), or you can use this Web site’s general news search page at http://news.yahoo.com/. Type in “mental retardation” (or synonyms). If you know the name of a company that is relevant to mental retardation, you can go to any stock trading Web site (such as http://www.etrade.com/) and search for the company name there. News items across various news sources are reported on indicated hyperlinks. Google offers a similar service at http://news.google.com/.

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BBC Covering news from a more European perspective, the British Broadcasting Corporation (BBC) allows the public free access to their news archive located at http://www.bbc.co.uk/. Search by “mental retardation” (or synonyms).

Newsletter Articles Use the Combined Health Information Database, and limit your search criteria to “newsletter articles.” Again, you will need to use the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. Go to the bottom of the search page where “You may refine your search by.” Select the dates and language that you prefer. For the format option, select “Newsletter Article.” Type “mental retardation” (or synonyms) into the “For these words:” box. You should check back periodically with this database as it is updated every three months. The following is a typical result when searching for newsletter articles on mental retardation: •

Exercise Important for Children and Adolescents with Mental Retardation Source: Fit Society Page. p. 4. Fall 2001. Contact: American College of Sports Medicine. P.O. Box 1440, Indianapolis, IN 462061440. Summary: Studies indicate that children and adolescents with mild-to-moderate mental retardation (MR) have very low physical fitness levels when compared to their schoolage peers without disabilities. Their physical fitness is often so poor that it places them at high risk of developing, early in their adult life, diseases such as heart disease, stroke, hypertension, and diabetes. If a doctor examines them and finds no medical problems, children with MR are capable of performing exercises or strenuous activities just like other children. However, less than 15 percent of children and adolescents with MR participate fully in traditional school physical education (PE) programs. Pitetti discusses the barriers that prevent students with MR from participating in regular PE classes and urges parents and guardians to speak out for these children with MR. If a child has no medical limitations, he or she has every right to be active in a PE class. If a child does not have the exercise capacity or motor skills to fully participate in regular PE classes, parents should insist that the school district hire an 'adapted' PE teacher so that children with physical disabilities may participate in a PE class according to their needs.

Academic Periodicals covering Mental Retardation Numerous periodicals are currently indexed within the National Library of Medicine’s PubMed database that are known to publish articles relating to mental retardation. In addition to these sources, you can search for articles covering mental retardation that have been published by any of the periodicals listed in previous chapters. To find the latest studies published, go to http://www.ncbi.nlm.nih.gov/pubmed, type the name of the periodical into the search box, and click “Go.” If you want complete details about the historical contents of a journal, you can also visit the following Web site: http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi. Here, type in the

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name of the journal or its abbreviation, and you will receive an index of published articles. At http://locatorplus.gov/, you can retrieve more indexing information on medical periodicals (e.g. the name of the publisher). Select the button “Search LOCATORplus.” Then type in the name of the journal and select the advanced search option “Journal Title Search.”

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APPENDICES

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APPENDIX A. PHYSICIAN RESOURCES Overview In this chapter, we focus on databases and Internet-based guidelines and information resources created or written for a professional audience.

NIH Guidelines Commonly referred to as “clinical” or “professional” guidelines, the National Institutes of Health publish physician guidelines for the most common diseases. Publications are available at the following by relevant Institute13: •

Office of the Director (OD); guidelines consolidated across agencies available at http://www.nih.gov/health/consumer/conkey.htm

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National Institute of General Medical Sciences (NIGMS); fact sheets available at http://www.nigms.nih.gov/news/facts/

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National Library of Medicine (NLM); extensive encyclopedia (A.D.A.M., Inc.) with guidelines: http://www.nlm.nih.gov/medlineplus/healthtopics.html

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National Cancer Institute (NCI); guidelines available at http://www.cancer.gov/cancerinfo/list.aspx?viewid=5f35036e-5497-4d86-8c2c714a9f7c8d25

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National Eye Institute (NEI); guidelines available at http://www.nei.nih.gov/order/index.htm

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National Heart, Lung, and Blood Institute (NHLBI); guidelines available at http://www.nhlbi.nih.gov/guidelines/index.htm

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National Human Genome Research Institute (NHGRI); research available at http://www.genome.gov/page.cfm?pageID=10000375

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National Institute on Aging (NIA); guidelines available at http://www.nia.nih.gov/health/

13

These publications are typically written by one or more of the various NIH Institutes.

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National Institute on Alcohol Abuse and Alcoholism (NIAAA); guidelines available at http://www.niaaa.nih.gov/publications/publications.htm

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National Institute of Allergy and Infectious Diseases (NIAID); guidelines available at http://www.niaid.nih.gov/publications/

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National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS); fact sheets and guidelines available at http://www.niams.nih.gov/hi/index.htm

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National Institute of Child Health and Human Development (NICHD); guidelines available at http://www.nichd.nih.gov/publications/pubskey.cfm

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National Institute on Deafness and Other Communication Disorders (NIDCD); fact sheets and guidelines at http://www.nidcd.nih.gov/health/

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National Institute of Dental and Craniofacial Research (NIDCR); guidelines available at http://www.nidr.nih.gov/health/

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National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK); guidelines available at http://www.niddk.nih.gov/health/health.htm

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National Institute on Drug Abuse (NIDA); guidelines available at http://www.nida.nih.gov/DrugAbuse.html

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National Institute of Environmental Health Sciences (NIEHS); environmental health information available at http://www.niehs.nih.gov/external/facts.htm

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National Institute of Mental Health (NIMH); guidelines available at http://www.nimh.nih.gov/practitioners/index.cfm

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National Institute of Neurological Disorders and Stroke (NINDS); neurological disorder information pages available at http://www.ninds.nih.gov/health_and_medical/disorder_index.htm

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National Institute of Nursing Research (NINR); publications on selected illnesses at http://www.nih.gov/ninr/news-info/publications.html

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National Institute of Biomedical Imaging and Bioengineering; general information at http://grants.nih.gov/grants/becon/becon_info.htm

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Center for Information Technology (CIT); referrals to other agencies based on keyword searches available at http://kb.nih.gov/www_query_main.asp

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National Center for Complementary and Alternative Medicine (NCCAM); health information available at http://nccam.nih.gov/health/

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National Center for Research Resources (NCRR); various information directories available at http://www.ncrr.nih.gov/publications.asp

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Office of Rare Diseases; various fact sheets available at http://rarediseases.info.nih.gov/html/resources/rep_pubs.html

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Centers for Disease Control and Prevention; various fact sheets on infectious diseases available at http://www.cdc.gov/publications.htm

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NIH Databases In addition to the various Institutes of Health that publish professional guidelines, the NIH has designed a number of databases for professionals.14 Physician-oriented resources provide a wide variety of information related to the biomedical and health sciences, both past and present. The format of these resources varies. Searchable databases, bibliographic citations, full-text articles (when available), archival collections, and images are all available. The following are referenced by the National Library of Medicine:15 •

Bioethics: Access to published literature on the ethical, legal, and public policy issues surrounding healthcare and biomedical research. This information is provided in conjunction with the Kennedy Institute of Ethics located at Georgetown University, Washington, D.C.: http://www.nlm.nih.gov/databases/databases_bioethics.html

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HIV/AIDS Resources: Describes various links and databases dedicated to HIV/AIDS research: http://www.nlm.nih.gov/pubs/factsheets/aidsinfs.html

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NLM Online Exhibitions: Describes “Exhibitions in the History of Medicine”: http://www.nlm.nih.gov/exhibition/exhibition.html. Additional resources for historical scholarship in medicine: http://www.nlm.nih.gov/hmd/hmd.html

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Biotechnology Information: Access to public databases. The National Center for Biotechnology Information conducts research in computational biology, develops software tools for analyzing genome data, and disseminates biomedical information for the better understanding of molecular processes affecting human health and disease: http://www.ncbi.nlm.nih.gov/

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Population Information: The National Library of Medicine provides access to worldwide coverage of population, family planning, and related health issues, including family planning technology and programs, fertility, and population law and policy: http://www.nlm.nih.gov/databases/databases_population.html

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Cancer Information: Access to cancer-oriented databases: http://www.nlm.nih.gov/databases/databases_cancer.html

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Profiles in Science: Offering the archival collections of prominent twentieth-century biomedical scientists to the public through modern digital technology: http://www.profiles.nlm.nih.gov/

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Chemical Information: Provides links to various chemical databases and references: http://sis.nlm.nih.gov/Chem/ChemMain.html

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Clinical Alerts: Reports the release of findings from the NIH-funded clinical trials where such release could significantly affect morbidity and mortality: http://www.nlm.nih.gov/databases/alerts/clinical_alerts.html

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Space Life Sciences: Provides links and information to space-based research (including NASA): http://www.nlm.nih.gov/databases/databases_space.html

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MEDLINE: Bibliographic database covering the fields of medicine, nursing, dentistry, veterinary medicine, the healthcare system, and the pre-clinical sciences: http://www.nlm.nih.gov/databases/databases_medline.html

14

Remember, for the general public, the National Library of Medicine recommends the databases referenced in MEDLINEplus (http://medlineplus.gov/ or http://www.nlm.nih.gov/medlineplus/databases.html). 15 See http://www.nlm.nih.gov/databases/databases.html.

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Toxicology and Environmental Health Information (TOXNET): Databases covering toxicology and environmental health: http://sis.nlm.nih.gov/Tox/ToxMain.html

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Visible Human Interface: Anatomically detailed, three-dimensional representations of normal male and female human bodies: http://www.nlm.nih.gov/research/visible/visible_human.html The Combined Health Information Database

A comprehensive source of information on clinical guidelines written for professionals is the Combined Health Information Database. You will need to limit your search to one of the following: Brochure/Pamphlet, Fact Sheet, or Information Package, and “mental retardation” using the “Detailed Search” option. Go directly to the following hyperlink: http://chid.nih.gov/detail/detail.html. To find associations, use the drop boxes at the bottom of the search page where “You may refine your search by.” For the publication date, select “All Years.” Select your preferred language and the format option “Fact Sheet.” Type “mental retardation” (or synonyms) into the “For these words:” box. The following is a sample result: •

Closing the gap: A national blueprint to improve the health of persons with mental retardation: Report of the Surgeon General's Conference on Health Disparities and Mental Retardation Source: Rockville, MD: U.S. Public Health Service; Washington, DC: for sale by U.S. Government Printing Office. 2002. 53 pp. Contact: Available from U.S. Government Printing Office, P.O. Box 371954, Pittsburgh, PA 15250-7954. Telephone: (202) 512-1656 for public information (D.C. office) or (202) 512-1800 for ordering and publication information (D.C. office) / fax: (202) 512-1293 (public information); (202) 512-2250 (ordering) / e-mail: [email protected] / Web site: http://www.access.gpo.gov/su_docs. Available from the Web site at no charge. Summary: This conference report identifies priority goals and action steps set forth by the mental retardation (MR) community at the national conference held December 5-6, 2001 in Washington, DC. The goals and action steps are described as follows: (1) integrate health promotion into community environments of people with MR; (2) increase knowledge and understanding of health and MR, ensuring that knowledge is made practical and easy to use; (3) improve the quality of health care for people with MR; (4) train health care providers in the care of adults and children with MR; (5) ensure that health care financing produces good health outcomes for adults and children with MR; and (6) increase sources of health care services for adults, adolescents, and children with MR, ensuring that health care is easily accessible for them. The report also includes six appendices covering the conference plenary sessions; participants; a list of potential partners; a description of programs and strategies to close the health disparities gap for those with MR; a summary of the Surgeon General's listening session; and a list of speakers of the listening session.

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Promoting health for individuals with mental retardation: A critical journey barely begun Source: Washington, DC: Special Olympics. 2001. 24 pp.

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Contact: Available from Special Olympics, 1325 G Street, N.W., Suite 500, Washington, DC 20005. Telephone: (202) 628-3630 / fax: (202) 824-0200 / e-mail: [email protected] / Web site: http://www.specialolympics.org. Available at no charge. Summary: This report discusses the health needs of people with mental retardation and makes recommendations for addressing these needs. The report also describes the Special Olympics Healthy Athletes program that provides health assessment, health services, referral services, and health screening for Special Olympics athletes. It discusses screening provided for oral health, vision, hearing and obesity, and training for health professionals who serve people with mental retardation. •

Defining medical necessity: Strategies for promoting access to quality care for persons with developmental disabilities, mental retardation, and other special health care needs Source: [Rockville, MD]: U.S. Maternal and Child Health Bureau. 1999. 34 pp., exec. summ. (4 pp.). Contact: Available from National Maternal and Child Health Clearinghouse, 2070 Chain Bridge Road, Suite 450, Vienna, VA 22182-2536. Telephone: (703) 356-1964 or (888) 4344MCH / fax: (703) 821-2098 / e-mail: [email protected] / Web site: http://www.nmchc.org. Available at no charge. Summary: This report outlines a strategy for defining medical necessity that will promote high quality care for children, youth, and adults with developmental disabilities, mental retardation, serious emotional disorders, or other special health care needs. The report begins with background information including defining the population involved and its service needs. It explains why medical necessity determinations are important and gives key criteria including a recommended approach to defining medical necessity. Appendices include a list of contributors, medical necessity definitions by states and organizations, and an analysis of selected definitions of medical necessity. The executive summary presents a one page synopsis of specifications for defining medical necessity and includes one paragraph each on the problem, the goal, the report, and the audience. [Funded by the Maternal and Child Health Bureau].

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The health status and needs of individuals with mental retardation Source: New Haven, CT: Department of Epidemiology and Public Health, Yale University School of Medicine, and Department of Psychology, Yale University. 2001. 175 pp. Contact: Available from Special Olympics, 1325 G Street, N.W., Suite 500, Washington, DC 20005. Telephone: (202) 628-3630 / fax: (202) 824-0200 / e-mail: [email protected] / Web site: http://www.specialolympics.org. Available at no charge. Summary: This report reviews the definition and prevalence of mental retardation (MR) and follows with an examination of the physical, ocular, mental and dental health needs of individuals with MR. The report also discusses health care services that are available and accessible to this population. Recommendations for improving the health of individuals with MR are presented. References are included in the report.

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The NLM Gateway16 The NLM (National Library of Medicine) Gateway is a Web-based system that lets users search simultaneously in multiple retrieval systems at the U.S. National Library of Medicine (NLM). It allows users of NLM services to initiate searches from one Web interface, providing one-stop searching for many of NLM’s information resources or databases.17 To use the NLM Gateway, simply go to the search site at http://gateway.nlm.nih.gov/gw/Cmd. Type “mental retardation” (or synonyms) into the search box and click “Search.” The results will be presented in a tabular form, indicating the number of references in each database category. Results Summary Category Journal Articles Books / Periodicals / Audio Visual Consumer Health Meeting Abstracts Other Collections Total

Items Found 60585 3078 263 37 31 63994

HSTAT18 HSTAT is a free, Web-based resource that provides access to full-text documents used in healthcare decision-making.19 These documents include clinical practice guidelines, quickreference guides for clinicians, consumer health brochures, evidence reports and technology assessments from the Agency for Healthcare Research and Quality (AHRQ), as well as AHRQ’s Put Prevention Into Practice.20 Simply search by “mental retardation” (or synonyms) at the following Web site: http://text.nlm.nih.gov.

Coffee Break: Tutorials for Biologists21 Coffee Break is a general healthcare site that takes a scientific view of the news and covers recent breakthroughs in biology that may one day assist physicians in developing treatments. Here you will find a collection of short reports on recent biological discoveries. Each report incorporates interactive tutorials that demonstrate how bioinformatics tools are 16

Adapted from NLM: http://gateway.nlm.nih.gov/gw/Cmd?Overview.x.

17

The NLM Gateway is currently being developed by the Lister Hill National Center for Biomedical Communications (LHNCBC) at the National Library of Medicine (NLM) of the National Institutes of Health (NIH). 18 Adapted from HSTAT: http://www.nlm.nih.gov/pubs/factsheets/hstat.html. 19 20

The HSTAT URL is http://hstat.nlm.nih.gov/.

Other important documents in HSTAT include: the National Institutes of Health (NIH) Consensus Conference Reports and Technology Assessment Reports; the HIV/AIDS Treatment Information Service (ATIS) resource documents; the Substance Abuse and Mental Health Services Administration's Center for Substance Abuse Treatment (SAMHSA/CSAT) Treatment Improvement Protocols (TIP) and Center for Substance Abuse Prevention (SAMHSA/CSAP) Prevention Enhancement Protocols System (PEPS); the Public Health Service (PHS) Preventive Services Task Force's Guide to Clinical Preventive Services; the independent, nonfederal Task Force on Community Services’ Guide to Community Preventive Services; and the Health Technology Advisory Committee (HTAC) of the Minnesota Health Care Commission (MHCC) health technology evaluations. 21 Adapted from http://www.ncbi.nlm.nih.gov/Coffeebreak/Archive/FAQ.html.

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used as a part of the research process. Currently, all Coffee Breaks are written by NCBI staff.22 Each report is about 400 words and is usually based on a discovery reported in one or more articles from recently published, peer-reviewed literature.23 This site has new articles every few weeks, so it can be considered an online magazine of sorts. It is intended for general background information. You can access the Coffee Break Web site at the following hyperlink: http://www.ncbi.nlm.nih.gov/Coffeebreak/.

Other Commercial Databases In addition to resources maintained by official agencies, other databases exist that are commercial ventures addressing medical professionals. Here are some examples that may interest you: •

CliniWeb International: Index and table of contents to selected clinical information on the Internet; see http://www.ohsu.edu/cliniweb/.

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Medical World Search: Searches full text from thousands of selected medical sites on the Internet; see http://www.mwsearch.com/.

22

The figure that accompanies each article is frequently supplied by an expert external to NCBI, in which case the source of the figure is cited. The result is an interactive tutorial that tells a biological story. 23 After a brief introduction that sets the work described into a broader context, the report focuses on how a molecular understanding can provide explanations of observed biology and lead to therapies for diseases. Each vignette is accompanied by a figure and hypertext links that lead to a series of pages that interactively show how NCBI tools and resources are used in the research process.

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APPENDIX B. PATIENT RESOURCES Overview Official agencies, as well as federally funded institutions supported by national grants, frequently publish a variety of guidelines written with the patient in mind. These are typically called “Fact Sheets” or “Guidelines.” They can take the form of a brochure, information kit, pamphlet, or flyer. Often they are only a few pages in length. Since new guidelines on mental retardation can appear at any moment and be published by a number of sources, the best approach to finding guidelines is to systematically scan the Internetbased services that post them.

Patient Guideline Sources The remainder of this chapter directs you to sources which either publish or can help you find additional guidelines on topics related to mental retardation. Due to space limitations, these sources are listed in a concise manner. Do not hesitate to consult the following sources by either using the Internet hyperlink provided, or, in cases where the contact information is provided, contacting the publisher or author directly. The National Institutes of Health The NIH gateway to patients is located at http://health.nih.gov/. From this site, you can search across various sources and institutes, a number of which are summarized below. Topic Pages: MEDLINEplus The National Library of Medicine has created a vast and patient-oriented healthcare information portal called MEDLINEplus. Within this Internet-based system are “health topic pages” which list links to available materials relevant to mental retardation. To access this system, log on to http://www.nlm.nih.gov/medlineplus/healthtopics.html. From there you can either search using the alphabetical index or browse by broad topic areas. Recently, MEDLINEplus listed the following when searched for “mental retardation”:

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Other guides Autism http://www.nlm.nih.gov/medlineplus/autism.html Birth Defects http://www.nlm.nih.gov/medlineplus/birthdefects.html Child Mental Health http://www.nlm.nih.gov/medlineplus/childmentalhealth.html Developmental Disabilities http://www.nlm.nih.gov/medlineplus/developmentaldisabilities.html Disabilities http://www.nlm.nih.gov/medlineplus/disabilities.html Learning Disorders http://www.nlm.nih.gov/medlineplus/learningdisorders.html Mental Health http://www.nlm.nih.gov/medlineplus/mentalhealth.html Phenylketonuria http://www.nlm.nih.gov/medlineplus/phenylketonuria.html

Within the health topic page dedicated to mental retardation, the following was listed: •

General/Overviews Developmental Disabilities - An Overview Source: American Academy of Pediatrics http://www.medem.com/MedLB/article_detaillb.cfm?article_ID=ZZZPUU2KGD C&sub_cat=105 JAMA Patient Page: Mental Retardation Source: American Medical Association http://www.medem.com/medlb/article_detaillb.cfm?article_ID=ZZZXSH0HC6D &sub_cat=105 What Is Mental Retardation? Source: National Center on Birth Defects and Developmental Disabilities http://www.cdc.gov/ncbddd/dd/ddmr.htm

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Diagnosis/Symptoms Basics for Parents: Your Child's Evaluation Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/basicpar/bp1txt.htm

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Coping Including Your Child Source: Dept. of Education, Office of Educational Research and Improvement http://www.ed.gov/pubs/parents/Including/title.html

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Individualized Education Plans (IEPs) Source: Nemours Foundation http://kidshealth.org/parent/growth/learning/iep.html •

Specific Conditions/Aspects Communicating with Your Child's School Through Letter Writing Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/parent/pa9txt.htm Developing Your Childs IEP Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/parent/pa12txt.htm Developmental Dyspraxia Source: National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/health_and_medical/disorders/dyspraxia.htm Importance of Physical Activity for Persons with Mental Retardation Source: American Orthopaedic Society for Sports Medicine http://www.sportsmed.org/Publications/mental.htm Intermediate Care Facility for People with Mental Retardation Program (ICF/MR) Source: Centers for Medicare & Medicaid Services http://cms.hhs.gov/medicaid/icfmr/ Living with Fetal Alcohol Syndrome http://www.cdc.gov/ncbddd/factsheets/living_fas.pdf Mental Retardation: Learning How to Help Your Child Source: American Academy of Family Physicians http://familydoctor.org/549.xml Parent Training and Information Centers Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/basicpar/bp3txt.htm Pervasive Developmental Disorders Source: Nemours Foundation http://kidshealth.org/parent/medical/learning/pervasive_develop_disorders.htm l Speech and Language Impairments Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/factshe/fs11txt.htm

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Children Coping with Disaster: Suggestions for Helping Children with Cognitive Disabilities Source: Administration for Children and Families http://www.acf.dhhs.gov/programs/add/Sept11/addcoping.html Kids' Quest on Disability and Health Source: National Center on Birth Defects and Developmental Disabilities http://www.cdc.gov/ncbddd/kids/

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Mainstreaming in Classrooms Source: Nemours Foundation http://kidshealth.org/kid/grow/school_stuff/mainstreaming.html Zigawhat Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/kids/ •

From the National Institutes of Health Multiple Congenital Anomaly/Mental Retardation Syndromes Database Source: National Library of Medicine http://www.nlm.nih.gov/mesh/jablonski/syndrome_title.html Pervasive Developmental Disorders Source: National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/health_and_medical/disorders/pdd.htm

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Law and Policy Benefits for Children with Disabilities Source: Social Security Administration http://www.ssa.gov/pubs/10026.html Closing the Gap: A National Blueprint to Improve the Health of Persons with Mental Retardation Source: National Institute of Child Health and Human Development http://www.nichd.nih.gov/publications/pubs/closingthegap/index.htm Guide to the Individualized Education Program Source: Dept. of Education http://www.ed.gov/parents/needs/speced/iepguide/index.html Questions and Answers about IDEA Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/newsdig/nd21txt.htm Related Services for School-Aged Children with Disabilities Source: National Information Center for Children and Youth with Disabilities http://www.nichcy.org/pubs/newsdig/nd16txt.htm Services in School for Children with Special Needs: What Parents Need to Know Source: American Academy of Child and Adolescent Psychiatry http://www.aacap.org/publications/factsfam/83.htm

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Men Women with Disabilities: Developmental Disabilities Source: National Women's Health Information Center http://www.4woman.gov/wwd/wwd.cfm?page=43

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Organizations Administration on Developmental Disabilities http://www.acf.dhhs.gov/programs/add/

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DisabilityInfo.gov Source: Office of Disability Employment Policy http://disabilityinfo.gov/ National Center on Birth Defects and Developmental Disabilities Source: Centers for Disease Control and Prevention http://www.cdc.gov/ncbddd/default.htm National Center on Physical Activity and Disability http://www.ncpad.org/ National Information Center for Children and Youth with Disabilities http://www.nichcy.org/ National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/ National Organization on Disability http://www.nod.org/ Rehabilitation Services Administration http://www.ed.gov/about/offices/list/osers/rsa/ •

Prevention/Screening Newborn Screening Tests Source: March of Dimes Birth Defects Foundation http://www.marchofdimes.com/pnhec/298_834.asp

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Research Mental Retardation and Developmental Disabilities Branch (MRDD) Source: National Institute of Child Health and Human Development http://www.nichd.nih.gov/about/crmc/mrdd/mrdd.htm Possible Gene for Form of Mental Retardation, Brain Development Identified Source: National Institute of Child Health and Human Development http://www.nih.gov/news/pr/jun2002/nichd-27.htm

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Women Women with Disabilities: Developmental Disabilities Source: National Women's Health Information Center http://www.4woman.gov/wwd/wwd.cfm?page=43

You may also choose to use the search utility provided by MEDLINEplus at the following Web address: http://www.nlm.nih.gov/medlineplus/. Simply type a keyword into the search box and click “Search.” This utility is similar to the NIH search utility, with the exception that it only includes materials that are linked within the MEDLINEplus system (mostly patient-oriented information). It also has the disadvantage of generating unstructured results. We recommend, therefore, that you use this method only if you have a very targeted search.

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The Combined Health Information Database (CHID) CHID Online is a reference tool that maintains a database directory of thousands of journal articles and patient education guidelines on mental retardation. CHID offers summaries that describe the guidelines available, including contact information and pricing. CHID’s general Web site is http://chid.nih.gov/. To search this database, go to http://chid.nih.gov/detail/detail.html. In particular, you can use the advanced search options to look up pamphlets, reports, brochures, and information kits. The following was recently posted in this archive: •

Hearing Changes in Aging People with Mental Retardation Source: Arlington, TX: Arc of the United States. 1999. 8 p. Contact: Available from Arc of the United States. 500 East Border Street, S-300, Arlington, TX 76010. (817) 261-6003. TDD (817) 277-0553. E-mail: [email protected]. Website: TheArc.org. PRICE: $2.00; bulk copies available. Summary: Like others who reach their later years, older people with developmental disabilities are at risk for acquiring a number of age related chronic conditions, including hearing loss (presbycusis). This fact sheet provides answers to frequently asked questions about age related hearing loss, especially for people with developmental disabilities. Topics include how hearing typically changes with age, including presbycusis and tinnitus; the causes of hearing loss; problems with earlier hearing loss, particularly in people with Down syndrome; how hearing losses are identified; hearing tests and where they can be obtained; the typical questions that an audiologist will ask; what to expect during a hearing test; how often hearing should be tested; strategies for coping with a hearing loss; the indications for hearing aids; other assistive listening devices; and the impact of visual impairment in people with hearing loss, and vice versa. The fact sheet includes a brief glossary, a list of agencies that may be of assistance, and a list of related publications (briefly annotated). One sidebar summarizes important information for a caregiver to gather before a hearing evaluation for a person with a developmental disability. 6 references.

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Practice Guidelines for the Clinical Assessment and Care Management of Alzheimer and Other Dementias Among Adults With Mental Retardation. Report of the AAMRIASSID Work Group Source: Albany, NY: New York State Office of Mental Retardation and Developmental Disabilities Bureau of Aging Services. 1995. 17 p. Contact: New York State Office of Mental Retardation and Developmental Disabilities Bureau of Aging Services. 144 Holland Avenue, Albany, NY 12229. (518) 473-7855; FAX (518) 473-0775. PRICE: Free. Summary: These guidelines, developed by an international committee, American Association on Mental Association (AAMR) and International Association for the Scientific Study of Intellectual Disability (IASSID) are intended to provide information about the identification of Alzheimer's disease and other dementias among adults with mental retardation. The guidelines also are designed to provide guidance for stagerelated care management of Alzheimer's disease and suggestions for training and education of caregivers, peers, and clinical and program staff. The guidelines identify the common clinical changes in people with both mental retardation and dementia, and discuss management issues associated with caring for them. The guidelines suggest

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intervention activities in three steps: recognizing changes, conducting assessments and evaluations, and instituting medical and care management. 43 references. •

HIV & AIDS Prevention for People With Mental Retardation: Information for HIV Service Providers Contact: Association for Retarded Citizens of United States, PO Box 1047, Arlington, TX, 76004, (817) 261-6003. Summary: This brochure presents information about Human immunodeficiency virus (HIV) and Acquired immunodeficiency syndrome (AIDS), appropriate for mentally retarded people. It focuses on HIV-prevention activities, such as safer sex education and HIV-antibody counseling and testing. Sexuality and people with mental retardation, as well as sexual abstinence, are discussed. It lists basic concepts of knowledge, attitudes and behaviors which should be communicated and reinforced with people with mental retardation. It also provides guidelines for HIV-service providers to conduct HIVprevention training, HIV-antibody counseling and testing, and other services. It indicates that delivering health-related services to people, regardless of disability, is not only professionally ethical, it is required by the law.

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ARC Facts: HIV/AIDS and Mental Retardation Contact: Association for Retarded Citizens of United States, PO Box 1047, Arlington, TX, 76004, (817) 261-6003. Summary: This fact sheet presents facts about Acquired immunodeficiency syndrome (AIDS) and its relationship to mental retardation. It explains the spectrum of Human immunodeficiency virus (HIV) disease and how the virus is transmitted. It explains that HIV affects people with mental retardation in two ways: First, adults with mental retardation may become victims of sexual exploitation; and second, HIV infection may cause retardation in children. The fact sheet explains the HIV-antibody test and why quarantine procedures would prove ineffective. Methods of HIV prevention are listed, with an emphasis on safer sexual conduct. Various sexual activities are categorized as dangerous, risky, safer, and much safer. The fact sheet lists the sexual rights and responsibilities of mental retardation.

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Assistive Technology for People with Mental Retardation Source: Arlington, TX: The Arc. September 1993. [2 p.]. Contact: Arc. National Headquarters, 500 East Border Street, S-300, Arlington, TX 76010. Voice (817) 261-60003; TTY (817) 277-0553; E-mail: [email protected]; http://TheArc.org/. PRICE: Single copy free (send self-addressed, stamped envelope); $15.00 per 100 copies. Order Number 101-42. Summary: This fact sheet reviews the use of assistive technology by people with mental retardation. Assistive technology is the term used to describe devices that are used by children and adults with mental retardation and other disabilities. The technology is used to compensate for functional limitations and to enhance and increase learning, independence, mobility, communication, environmental control and choice. The fact sheet outlines how assistive technology can benefit people with mental retardation; the uses of assistive technology in the areas of communication, environmental control, mobility, education, activities of daily living, employment, and sports and recreation; questions to ask when considering how or if assistive technology can be of use; barriers

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that may need to be addressed in obtaining assistive technology; and sources of information about assistive technology. 3 references. •

Special Olympics International: Mental Retardation Source: Washington, DC: Special Olympics International. 1993. 1 p. Contact: Available from Special Athletes-Special Smiles. Boston University Goldman School of Graduate Dentistry, Office of External Affairs, 100 East Newton Street, Boston, MA 02118. (617) 638-4891. PRICE: Single copy free in information package. Item Number SOI 2-93. Also available from Special Olympics International. International Headquarters, Public Affairs Office, 1350 New York Avenue, NW, Washington, DC 20005. (202) 628-3630. PRICE: Single copy free. Summary: This fact sheet, produced as part of the Special Olympics program, presents basic information about mental retardation. Topics include a definition of mental retardation, the prevalence of mental retardation, and some basic epidemiological information.

•

Aging with Mental Retardation: Oral Health for Older Individuals with Disabilities Source: Chicago, IL: Rehabilitation Research and Training Center on Aging with Developmental Disabilities. Institute on Disability and Human Development, University of Illinois at Chicago. Silver Spring, MD: The Arc of the United States. 2001. 8 p. Contact: Available from Rehabilitation Research and Training Center on Aging with Developmental Disabilities. Institute on Disability and Human Development, University of Illinois at Chicago. (800) 996-8845. E-mail: [email protected]. Website: www.uic.edu/orgs/rrtcamr. PRICE: $3.00 for printed copy, or view for free on the Web. Summary: This informational oral health fact sheet is designed to provide family members and caregivers of older individuals with disabilities basic information regarding the oral health care needs and requirements of the individuals for whom they care. The authors describe special care dentistry, a term commonly used for addressing the oral health needs of an older person who is medically compromised or an individual with some type of mental, physical, or developmental disability. The authors also discuss common changes in the oral cavity encountered in older adults with disabilities, diagnostic considerations, recommendations for appropriate oral hygiene, choosing a dentist for the care of an individual with disabilities, the prevention of problems and maintenance of the oral cavity, and barriers in treating older adults with disabilities. The authors conclude by reminding readers of the significant relationship between oral health and general health. A list of ten resources for more information is provided at the end of the document. 2 figures. 7 references.

•

Epidemiology of Alzheimer Disease in Mental Retardation. Results and Recommendations from an International Conference. Report of the AAMR/IASSID Workgroup on Epidemiology and Alzheimer's Disease Source: Albany, NY: New York State Office of Mental Retardation and Development Disabilities Bureau of Aging Services. 1995. 16 p. Contact: New York State Office of Mental Retardation and Development Disabilities Bureau of Aging Services. 144 Holland Avenue, Albany, NY 12229. (518) 473-7855; FAX (518) 473-0775. PRICE: Free.

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Summary: This report considers the discussions and recommendations of an epidemiology work group, formed at an international conference convened to discuss Alzheimer's disease among people with mental retardation. Topics include the incidence and prevalence of clinical dementia in this population, risk factors for the development of Alzheimer's disease in adults with mental retardation, and a minimum data set that may be of use for future research on Alzheimer's disease in adults with mental retardation. 1 table, 1 chart, 62 references. The National Guideline Clearinghouse™ The National Guideline Clearinghouse™ offers hundreds of evidence-based clinical practice guidelines published in the United States and other countries. You can search this site located at http://www.guideline.gov/ by using the keyword “mental retardation” (or synonyms). The following was recently posted: •

Practice parameters for the assessment and treatment of children, adolescents, and adults with mental retardation and comorbid mental disorders Source: American Academy of Child and Adolescent Psychiatry - Medical Specialty Society; 1999 June 27; 77 pages http://www.guideline.gov/summary/summary.aspx?doc_id=2140&nbr=1366&a mp;string=mental+AND+retardation Healthfinder™

Healthfinder™ is sponsored by the U.S. Department of Health and Human Services and offers links to hundreds of other sites that contain healthcare information. This Web site is located at http://www.healthfinder.gov. Again, keyword searches can be used to find guidelines. The following was recently found in this database: •

Mental Retardation Source: National Information Center for Children and Youth with Disabilities, U.S. Department of Education http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=3414

•

News Page - American Association on Mental Retardation Summary: Visit this site for current News and events related to this organization's services. Source: American Association on Mental Retardation http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&RecordID=1535 The NIH Search Utility

The NIH search utility allows you to search for documents on over 100 selected Web sites that comprise the NIH-WEB-SPACE. Each of these servers is “crawled” and indexed on an ongoing basis. Your search will produce a list of various documents, all of which will relate in some way to mental retardation. The drawbacks of this approach are that the information

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is not organized by theme and that the references are often a mix of information for professionals and patients. Nevertheless, a large number of the listed Web sites provide useful background information. We can only recommend this route, therefore, for relatively rare or specific disorders, or when using highly targeted searches. To use the NIH search utility, visit the following Web page: http://search.nih.gov/index.html. Additional Web Sources A number of Web sites are available to the public that often link to government sites. These can also point you in the direction of essential information. The following is a representative sample: •

AOL: http://search.aol.com/cat.adp?id=168&layer=&from=subcats

•

Family Village: http://www.familyvillage.wisc.edu/specific.htm

•

Google: http://directory.google.com/Top/Health/Conditions_and_Diseases/

•

Med Help International: http://www.medhelp.org/HealthTopics/A.html

•

Open Directory Project: http://dmoz.org/Health/Conditions_and_Diseases/

•

Yahoo.com: http://dir.yahoo.com/Health/Diseases_and_Conditions/

•

WebMDHealth: http://my.webmd.com/health_topics

Associations and Mental Retardation The following is a list of associations that provide information on and resources relating to mental retardation: •

Center for Mental Retardation Telephone: (216) 621-5858 Toll-free: (800) 899-3039 Fax: (216) 621-0221 Email: [email protected] Web Site: None Background: The Center for Mental Retardation (Edward I. and Fannie L. Baker International Resource Center for Down Syndrome) is a not-for-profit organization dedicated to improving the quality of life for individuals with mental retardation and increasing their role as valued, contributing members of society through the implementation and promotion of services, educational programs, and research. Down Syndrome is a congenital chromosomal disorder characterized by varying degrees of mental retardation, characteristic abnormalities of the head and facial area, malformations of the hands and/or feet, congenital heart defects in approximately 25 percent of affected individuals, and/or other abnormalities. In most individuals with Down Syndrome, all or a portion of chromosome 21 appears three times rather than twice in cells of the body; however, in rare cases, the disorder may occur due to a chromosomal translocation involving chromosome 21 and another chromosome. Established in 1963, the Center for Mental Retardation offers a variety of services and programs including patient advocacy services, parent counseling and support, referral services, and patient and professional education. The Center's educational materials include brochures, pamphlets, and a regular newsletter.

Patient Resources

•

233

RRTC on Aging with Mental Retardation Telephone: (312) 413-1520 Toll-free: (800) 996-8845 Fax: (312) 996-6942 Email: [email protected] Web Site: http://www.uic.edu/orgs/rrtcamr/index.html Background: The Rehabilitation Research and Training Center (RRTC) on Aging with Mental Retardation is a not-for-profit research organization dedicated to promoting independence, productivity, community inclusion, and full citizenship of older adults with mental retardation through a coordinated program of research, training, technical assistance, and dissemination activities. Established in 1988, it is funded by the U.S. Department of Education, Office of Special Education and Rehabilitative Services. The RRTC is a national resource for researchers, planners, providers, self-advocates, families, and students in the field of aging with mental retardation. The RRTC s Clearinghouse on Aging and Developmental Disabilities has a computerized database containing over 2,000 reprints, bibliographies, manuals, and monographs on topics addressing issues of aging with mental retardation. The RRTC also disseminates RRTC research reports, training materials, and other educational materials and publishes a biannual newsletter entitled 'A/DDVANTAGE,' concerning aging and developmental disabilities. Relevant area(s) of interest: Mental Retardation

•

The Arc (a national organization on mental retardation) Telephone: (301) 565-3842 Toll-free: (800) 433-5255 Fax: (301) 565-3843 Email: [email protected] Web Site: http://thearc.org/ Background: The Arc is the largest organization in the United States that is solely devoted to improving the lives of all children and adults with mental retardation. The organization offers support to families affected by mental retardation and fosters research and educational programs on the prevention of mental retardation. The Arc is committed to securing opportunities for all people with mental retardation. To this end, the organization emphasizes personal opportunities for choice in education, housing, employment, and entertainment. The Arc is further committed to reducing the incidence and limiting the consequences of mental retardation through research, advocacy, and mutual support. The Arc provides leadership in the field of mental retardation and develops necessary human and financial resources to attain its goals. In addition, the Arc provides a wide variety of educational materials for parents, teachers, health care professionals, and others, including a regular newsletter, handbooks, instruction packets, reports, booklets, audio-visual aids, posters, and brochures. Many materials are available in Spanish. Relevant area(s) of interest: Mental Retardation

234 Mental Retardation

Finding Associations There are several Internet directories that provide lists of medical associations with information on or resources relating to mental retardation. By consulting all of associations listed in this chapter, you will have nearly exhausted all sources for patient associations concerned with mental retardation. The National Health Information Center (NHIC) The National Health Information Center (NHIC) offers a free referral service to help people find organizations that provide information about mental retardation. For more information, see the NHIC’s Web site at http://www.health.gov/NHIC/ or contact an information specialist by calling 1-800-336-4797. Directory of Health Organizations The Directory of Health Organizations, provided by the National Library of Medicine Specialized Information Services, is a comprehensive source of information on associations. The Directory of Health Organizations database can be accessed via the Internet at http://www.sis.nlm.nih.gov/Dir/DirMain.html. It is composed of two parts: DIRLINE and Health Hotlines. The DIRLINE database comprises some 10,000 records of organizations, research centers, and government institutes and associations that primarily focus on health and biomedicine. To access DIRLINE directly, go to the following Web site: http://dirline.nlm.nih.gov/. Simply type in “mental retardation” (or a synonym), and you will receive information on all relevant organizations listed in the database. Health Hotlines directs you to toll-free numbers to over 300 organizations. You can access this database directly at http://www.sis.nlm.nih.gov/hotlines/. On this page, you are given the option to search by keyword or by browsing the subject list. When you have received your search results, click on the name of the organization for its description and contact information. The Combined Health Information Database Another comprehensive source of information on healthcare associations is the Combined Health Information Database. Using the “Detailed Search” option, you will need to limit your search to “Organizations” and “mental retardation”. Type the following hyperlink into your Web browser: http://chid.nih.gov/detail/detail.html. To find associations, use the drop boxes at the bottom of the search page where “You may refine your search by.” For publication date, select “All Years.” Then, select your preferred language and the format option “Organization Resource Sheet.” Type “mental retardation” (or synonyms) into the “For these words:” box. You should check back periodically with this database since it is updated every three months.

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The National Organization for Rare Disorders, Inc. The National Organization for Rare Disorders, Inc. has prepared a Web site that provides, at no charge, lists of associations organized by health topic. You can access this database at the following Web site: http://www.rarediseases.org/search/orgsearch.html. Type “mental retardation” (or a synonym) into the search box, and click “Submit Query.”

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APPENDIX C. FINDING MEDICAL LIBRARIES Overview In this Appendix, we show you how to quickly find a medical library in your area.

Preparation Your local public library and medical libraries have interlibrary loan programs with the National Library of Medicine (NLM), one of the largest medical collections in the world. According to the NLM, most of the literature in the general and historical collections of the National Library of Medicine is available on interlibrary loan to any library. If you would like to access NLM medical literature, then visit a library in your area that can request the publications for you.24

Finding a Local Medical Library The quickest method to locate medical libraries is to use the Internet-based directory published by the National Network of Libraries of Medicine (NN/LM). This network includes 4626 members and affiliates that provide many services to librarians, health professionals, and the public. To find a library in your area, simply visit http://nnlm.gov/members/adv.html or call 1-800-338-7657.

Medical Libraries in the U.S. and Canada In addition to the NN/LM, the National Library of Medicine (NLM) lists a number of libraries with reference facilities that are open to the public. The following is the NLM’s list and includes hyperlinks to each library’s Web site. These Web pages can provide information on hours of operation and other restrictions. The list below is a small sample of

24

Adapted from the NLM: http://www.nlm.nih.gov/psd/cas/interlibrary.html.

238 Mental Retardation

libraries recommended by the National Library of Medicine (sorted alphabetically by name of the U.S. state or Canadian province where the library is located)25: •

Alabama: Health InfoNet of Jefferson County (Jefferson County Library Cooperative, Lister Hill Library of the Health Sciences), http://www.uab.edu/infonet/

•

Alabama: Richard M. Scrushy Library (American Sports Medicine Institute)

•

Arizona: Samaritan Regional Medical Center: The Learning Center (Samaritan Health System, Phoenix, Arizona), http://www.samaritan.edu/library/bannerlibs.htm

•

California: Kris Kelly Health Information Center (St. Joseph Health System, Humboldt), http://www.humboldt1.com/~kkhic/index.html

•

California: Community Health Library of Los Gatos, http://www.healthlib.org/orgresources.html

•

California: Consumer Health Program and Services (CHIPS) (County of Los Angeles Public Library, Los Angeles County Harbor-UCLA Medical Center Library) - Carson, CA, http://www.colapublib.org/services/chips.html

•

California: Gateway Health Library (Sutter Gould Medical Foundation)

•

California: Health Library (Stanford University Medical Center), http://wwwmed.stanford.edu/healthlibrary/

•

California: Patient Education Resource Center - Health Information and Resources (University of California, San Francisco), http://sfghdean.ucsf.edu/barnett/PERC/default.asp

•

California: Redwood Health Library (Petaluma Health Care District), http://www.phcd.org/rdwdlib.html

•

California: Los Gatos PlaneTree Health Library, http://planetreesanjose.org/

•

California: Sutter Resource Library (Sutter Hospitals Foundation, Sacramento), http://suttermedicalcenter.org/library/

•

California: Health Sciences Libraries (University of California, Davis), http://www.lib.ucdavis.edu/healthsci/

•

California: ValleyCare Health Library & Ryan Comer Cancer Resource Center (ValleyCare Health System, Pleasanton), http://gaelnet.stmarysca.edu/other.libs/gbal/east/vchl.html

•

California: Washington Community Health Resource Library (Fremont), http://www.healthlibrary.org/

•

Colorado: William V. Gervasini Memorial Library (Exempla Healthcare), http://www.saintjosephdenver.org/yourhealth/libraries/

•

Connecticut: Hartford Hospital Health Science Libraries (Hartford Hospital), http://www.harthosp.org/library/

•

Connecticut: Healthnet: Connecticut Consumer Health Information Center (University of Connecticut Health Center, Lyman Maynard Stowe Library), http://library.uchc.edu/departm/hnet/

25

Abstracted from http://www.nlm.nih.gov/medlineplus/libraries.html.

Finding Medical Libraries

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•

Connecticut: Waterbury Hospital Health Center Library (Waterbury Hospital, Waterbury), http://www.waterburyhospital.com/library/consumer.shtml

•

Delaware: Consumer Health Library (Christiana Care Health System, Eugene du Pont Preventive Medicine & Rehabilitation Institute, Wilmington), http://www.christianacare.org/health_guide/health_guide_pmri_health_info.cfm

•

Delaware: Lewis B. Flinn Library (Delaware Academy of Medicine, Wilmington), http://www.delamed.org/chls.html

•

Georgia: Family Resource Library (Medical College of Georgia, Augusta), http://cmc.mcg.edu/kids_families/fam_resources/fam_res_lib/frl.htm

•

Georgia: Health Resource Center (Medical Center of Central Georgia, Macon), http://www.mccg.org/hrc/hrchome.asp

•

Hawaii: Hawaii Medical Library: Consumer Health Information Service (Hawaii Medical Library, Honolulu), http://hml.org/CHIS/

•

Idaho: DeArmond Consumer Health Library (Kootenai Medical Center, Coeur d’Alene), http://www.nicon.org/DeArmond/index.htm

•

Illinois: Health Learning Center of Northwestern Memorial Hospital (Chicago), http://www.nmh.org/health_info/hlc.html

•

Illinois: Medical Library (OSF Saint Francis Medical Center, Peoria), http://www.osfsaintfrancis.org/general/library/

•

Kentucky: Medical Library - Services for Patients, Families, Students & the Public (Central Baptist Hospital, Lexington), http://www.centralbap.com/education/community/library.cfm

•

Kentucky: University of Kentucky - Health Information Library (Chandler Medical Center, Lexington), http://www.mc.uky.edu/PatientEd/

•

Louisiana: Alton Ochsner Medical Foundation Library (Alton Ochsner Medical Foundation, New Orleans), http://www.ochsner.org/library/

•

Louisiana: Louisiana State University Health Sciences Center Medical LibraryShreveport, http://lib-sh.lsuhsc.edu/

•

Maine: Franklin Memorial Hospital Medical Library (Franklin Memorial Hospital, Farmington), http://www.fchn.org/fmh/lib.htm

•

Maine: Gerrish-True Health Sciences Library (Central Maine Medical Center, Lewiston), http://www.cmmc.org/library/library.html

•

Maine: Hadley Parrot Health Science Library (Eastern Maine Healthcare, Bangor), http://www.emh.org/hll/hpl/guide.htm

•

Maine: Maine Medical Center Library (Maine Medical Center, Portland), http://www.mmc.org/library/

•

Maine: Parkview Hospital (Brunswick), http://www.parkviewhospital.org/

•

Maine: Southern Maine Medical Center Health Sciences Library (Southern Maine Medical Center, Biddeford), http://www.smmc.org/services/service.php3?choice=10

•

Maine: Stephens Memorial Hospital’s Health Information Library (Western Maine Health, Norway), http://www.wmhcc.org/Library/

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•

Manitoba, Canada: Consumer & Patient Health Information Service (University of Manitoba Libraries), http://www.umanitoba.ca/libraries/units/health/reference/chis.html

•

Manitoba, Canada: J.W. Crane Memorial Library (Deer Lodge Centre, Winnipeg), http://www.deerlodge.mb.ca/crane_library/about.asp

•

Maryland: Health Information Center at the Wheaton Regional Library (Montgomery County, Dept. of Public Libraries, Wheaton Regional Library), http://www.mont.lib.md.us/healthinfo/hic.asp

•

Massachusetts: Baystate Medical Center Library (Baystate Health System), http://www.baystatehealth.com/1024/

•

Massachusetts: Boston University Medical Center Alumni Medical Library (Boston University Medical Center), http://med-libwww.bu.edu/library/lib.html

•

Massachusetts: Lowell General Hospital Health Sciences Library (Lowell General Hospital, Lowell), http://www.lowellgeneral.org/library/HomePageLinks/WWW.htm

•

Massachusetts: Paul E. Woodard Health Sciences Library (New England Baptist Hospital, Boston), http://www.nebh.org/health_lib.asp

•

Massachusetts: St. Luke’s Hospital Health Sciences Library (St. Luke’s Hospital, Southcoast Health System, New Bedford), http://www.southcoast.org/library/

•

Massachusetts: Treadwell Library Consumer Health Reference Center (Massachusetts General Hospital), http://www.mgh.harvard.edu/library/chrcindex.html

•

Massachusetts: UMass HealthNet (University of Massachusetts Medical School, Worchester), http://healthnet.umassmed.edu/

•

Michigan: Botsford General Hospital Library - Consumer Health (Botsford General Hospital, Library & Internet Services), http://www.botsfordlibrary.org/consumer.htm

•

Michigan: Helen DeRoy Medical Library (Providence Hospital and Medical Centers), http://www.providence-hospital.org/library/

•

Michigan: Marquette General Hospital - Consumer Health Library (Marquette General Hospital, Health Information Center), http://www.mgh.org/center.html

•

Michigan: Patient Education Resouce Center - University of Michigan Cancer Center (University of Michigan Comprehensive Cancer Center, Ann Arbor), http://www.cancer.med.umich.edu/learn/leares.htm

•

Michigan: Sladen Library & Center for Health Information Resources - Consumer Health Information (Detroit), http://www.henryford.com/body.cfm?id=39330

•

Montana: Center for Health Information (St. Patrick Hospital and Health Sciences Center, Missoula)

•

National: Consumer Health Library Directory (Medical Library Association, Consumer and Patient Health Information Section), http://caphis.mlanet.org/directory/index.html

•

National: National Network of Libraries of Medicine (National Library of Medicine) provides library services for health professionals in the United States who do not have access to a medical library, http://nnlm.gov/

•

National: NN/LM List of Libraries Serving the Public (National Network of Libraries of Medicine), http://nnlm.gov/members/

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•

Nevada: Health Science Library, West Charleston Library (Las Vegas-Clark County Library District, Las Vegas), http://www.lvccld.org/special_collections/medical/index.htm

•

New Hampshire: Dartmouth Biomedical Libraries (Dartmouth College Library, Hanover), http://www.dartmouth.edu/~biomed/resources.htmld/conshealth.htmld/

•

New Jersey: Consumer Health Library (Rahway Hospital, Rahway), http://www.rahwayhospital.com/library.htm

•

New Jersey: Dr. Walter Phillips Health Sciences Library (Englewood Hospital and Medical Center, Englewood), http://www.englewoodhospital.com/links/index.htm

•

New Jersey: Meland Foundation (Englewood Hospital and Medical Center, Englewood), http://www.geocities.com/ResearchTriangle/9360/

•

New York: Choices in Health Information (New York Public Library) - NLM Consumer Pilot Project participant, http://www.nypl.org/branch/health/links.html

•

New York: Health Information Center (Upstate Medical University, State University of New York, Syracuse), http://www.upstate.edu/library/hic/

•

New York: Health Sciences Library (Long Island Jewish Medical Center, New Hyde Park), http://www.lij.edu/library/library.html

•

New York: ViaHealth Medical Library (Rochester General Hospital), http://www.nyam.org/library/

•

Ohio: Consumer Health Library (Akron General Medical Center, Medical & Consumer Health Library), http://www.akrongeneral.org/hwlibrary.htm

•

Oklahoma: The Health Information Center at Saint Francis Hospital (Saint Francis Health System, Tulsa), http://www.sfh-tulsa.com/services/healthinfo.asp

•

Oregon: Planetree Health Resource Center (Mid-Columbia Medical Center, The Dalles), http://www.mcmc.net/phrc/

•

Pennsylvania: Community Health Information Library (Milton S. Hershey Medical Center, Hershey), http://www.hmc.psu.edu/commhealth/

•

Pennsylvania: Community Health Resource Library (Geisinger Medical Center, Danville), http://www.geisinger.edu/education/commlib.shtml

•

Pennsylvania: HealthInfo Library (Moses Taylor Hospital, Scranton), http://www.mth.org/healthwellness.html

•

Pennsylvania: Hopwood Library (University of Pittsburgh, Health Sciences Library System, Pittsburgh), http://www.hsls.pitt.edu/guides/chi/hopwood/index_html

•

Pennsylvania: Koop Community Health Information Center (College of Physicians of Philadelphia), http://www.collphyphil.org/kooppg1.shtml

•

Pennsylvania: Learning Resources Center - Medical Library (Susquehanna Health System, Williamsport), http://www.shscares.org/services/lrc/index.asp

•

Pennsylvania: Medical Library (UPMC Health System, Pittsburgh), http://www.upmc.edu/passavant/library.htm

•

Quebec, Canada: Medical Library (Montreal General Hospital), http://www.mghlib.mcgill.ca/

242 Mental Retardation

•

South Dakota: Rapid City Regional Hospital Medical Library (Rapid City Regional Hospital), http://www.rcrh.org/Services/Library/Default.asp

•

Texas: Houston HealthWays (Houston Academy of Medicine-Texas Medical Center Library), http://hhw.library.tmc.edu/

•

Washington: Community Health Library (Kittitas Valley Community Hospital), http://www.kvch.com/

•

Washington: Southwest Washington Medical Center Library (Southwest Washington Medical Center, Vancouver), http://www.swmedicalcenter.com/body.cfm?id=72

243

ONLINE GLOSSARIES The Internet provides access to a number of free-to-use medical dictionaries. The National Library of Medicine has compiled the following list of online dictionaries: •

ADAM Medical Encyclopedia (A.D.A.M., Inc.), comprehensive medical reference: http://www.nlm.nih.gov/medlineplus/encyclopedia.html

•

MedicineNet.com Medical Dictionary (MedicineNet, Inc.): http://www.medterms.com/Script/Main/hp.asp

•

Merriam-Webster Medical Dictionary (Inteli-Health, Inc.): http://www.intelihealth.com/IH/

•

Multilingual Glossary of Technical and Popular Medical Terms in Eight European Languages (European Commission) - Danish, Dutch, English, French, German, Italian, Portuguese, and Spanish: http://allserv.rug.ac.be/~rvdstich/eugloss/welcome.html

•

On-line Medical Dictionary (CancerWEB): http://cancerweb.ncl.ac.uk/omd/

•

Rare Diseases Terms (Office of Rare Diseases): http://ord.aspensys.com/asp/diseases/diseases.asp

•

Technology Glossary (National Library of Medicine) - Health Care Technology: http://www.nlm.nih.gov/nichsr/ta101/ta10108.htm

Beyond these, MEDLINEplus contains a very patient-friendly encyclopedia covering every aspect of medicine (licensed from A.D.A.M., Inc.). The ADAM Medical Encyclopedia can be accessed at http://www.nlm.nih.gov/medlineplus/encyclopedia.html. ADAM is also available on commercial Web sites such as drkoop.com (http://www.drkoop.com/) and Web MD (http://my.webmd.com/adam/asset/adam_disease_articles/a_to_z/a). The NIH suggests the following Web sites in the ADAM Medical Encyclopedia when searching for information on mental retardation: •

Basic Guidelines for Mental Retardation ADHD Web site: http://www.nlm.nih.gov/medlineplus/ency/article/001551.htm Fetal alcohol syndrome Web site: http://www.nlm.nih.gov/medlineplus/ency/article/000911.htm Mental retardation Web site: http://www.nlm.nih.gov/medlineplus/ency/article/001523.htm Prader-Willi syndrome Web site: http://www.nlm.nih.gov/medlineplus/ency/article/001605.htm

•

Diagnostics and Tests for Mental Retardation Cocaine Web site: http://www.nlm.nih.gov/medlineplus/ency/article/003578.htm

244 Mental Retardation

•

Background Topics for Mental Retardation Chromosome Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002327.htm Developmental milestones Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002348.htm Gene Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002371.htm Genetic counseling Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002053.htm Head injury Web site: http://www.nlm.nih.gov/medlineplus/ency/article/000028.htm Incidence Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002387.htm Intrauterine Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002389.htm Lead poisoning Web site: http://www.nlm.nih.gov/medlineplus/ency/article/002473.htm

Online Dictionary Directories The following are additional online directories compiled by the National Library of Medicine, including a number of specialized medical dictionaries: •

Medical Dictionaries: Medical & Biological (World Health Organization): http://www.who.int/hlt/virtuallibrary/English/diction.htm#Medical

•

MEL-Michigan Electronic Library List of Online Health and Medical Dictionaries (Michigan Electronic Library): http://mel.lib.mi.us/health/health-dictionaries.html

•

Patient Education: Glossaries (DMOZ Open Directory Project): http://dmoz.org/Health/Education/Patient_Education/Glossaries/

•

Web of Online Dictionaries (Bucknell University): http://www.yourdictionary.com/diction5.html#medicine

245

MENTAL RETARDATION DICTIONARY The definitions below are derived from official public sources, including the National Institutes of Health [NIH] and the European Union [EU]. 1-phosphate: A drug that halts cell suicide in human white blood cells. [NIH] Abdomen: That portion of the body that lies between the thorax and the pelvis. [NIH] Abdominal: Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs. [NIH] Aberrant: Wandering or deviating from the usual or normal course. [EU] Acceptor: A substance which, while normally not oxidized by oxygen or reduced by hydrogen, can be oxidized or reduced in presence of a substance which is itself undergoing oxidation or reduction. [NIH] Accommodation: Adjustment, especially that of the eye for various distances. [EU] ACE: Angiotensin-coverting enzyme. A drug used to decrease pressure inside blood vessels. [NIH]

Acetylcholine: A neurotransmitter. Acetylcholine in vertebrates is the major transmitter at neuromuscular junctions, autonomic ganglia, parasympathetic effector junctions, a subset of sympathetic effector junctions, and at many sites in the central nervous system. It is generally not used as an administered drug because it is broken down very rapidly by cholinesterases, but it is useful in some ophthalmological applications. [NIH] Acidemia: Increased acidity of blood. [NIH] Acidosis: A pathologic condition resulting from accumulation of acid or depletion of the alkaline reserve (bicarbonate content) in the blood and body tissues, and characterized by an increase in hydrogen ion concentration. [EU] Acoustic: Having to do with sound or hearing. [NIH] Actin: Essential component of the cell skeleton. [NIH] Actinin: A protein factor that regulates the length of R-actin. It is chemically similar, but immunochemically distinguishable from actin. [NIH] Activities of Daily Living: The performance of the basic activities of self care, such as dressing, ambulation, eating, etc., in rehabilitation. [NIH] Acupuncture Analgesia: Analgesia produced by the insertion of acupuncture needles at certain points in the body. These activate the small myelinated nerve fibers in the muscle which transmit impulses to the spinal cord and then activate three centers - the spinal cord, midbrain and pituitary hypothalamus - to produce analgesia. [NIH] Adaptability: Ability to develop some form of tolerance to conditions extremely different from those under which a living organism evolved. [NIH] Adaptation: 1. The adjustment of an organism to its environment, or the process by which it enhances such fitness. 2. The normal ability of the eye to adjust itself to variations in the intensity of light; the adjustment to such variations. 3. The decline in the frequency of firing of a neuron, particularly of a receptor, under conditions of constant stimulation. 4. In dentistry, (a) the proper fitting of a denture, (b) the degree of proximity and interlocking of restorative material to a tooth preparation, (c) the exact adjustment of bands to teeth. 5. In

246 Mental Retardation

microbiology, the adjustment of bacterial physiology to a new environment. [EU] Adenine: A purine base and a fundamental unit of adenine nucleotides. [NIH] Adenosine: A nucleoside that is composed of adenine and d-ribose. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter. [NIH] Adenovirus: A group of viruses that cause respiratory tract and eye infections. Adenoviruses used in gene therapy are altered to carry a specific tumor-fighting gene. [NIH] Adenylate Cyclase: An enzyme of the lyase class that catalyzes the formation of cyclic AMP and pyrophosphate from ATP. EC 4.6.1.1. [NIH] Adjustment: The dynamic process wherein the thoughts, feelings, behavior, and biophysiological mechanisms of the individual continually change to adjust to the environment. [NIH] Adolescence: The period of life beginning with the appearance of secondary sex characteristics and terminating with the cessation of somatic growth. The years usually referred to as adolescence lie between 13 and 18 years of age. [NIH] Adrenal Cortex: The outer layer of the adrenal gland. It secretes mineralocorticoids, androgens, and glucocorticoids. [NIH] Adrenal Glands: Paired glands situated in the retroperitoneal tissues at the superior pole of each kidney. [NIH] Adrenergic: Activated by, characteristic of, or secreting epinephrine or substances with similar activity; the term is applied to those nerve fibres that liberate norepinephrine at a synapse when a nerve impulse passes, i.e., the sympathetic fibres. [EU] Adrenergic Agents: Drugs that act on adrenergic receptors or affect the life cycle of adrenergic transmitters. Included here are adrenergic agonists and antagonists and agents that affect the synthesis, storage, uptake, metabolism, or release of adrenergic transmitters. [NIH]

Adrenoleukodystrophy: A chromosome X-linked disease. [NIH] Adverse Effect: An unwanted side effect of treatment. [NIH] Aerobic: In biochemistry, reactions that need oxygen to happen or happen when oxygen is present. [NIH] Aerosol: A solution of a drug which can be atomized into a fine mist for inhalation therapy. [EU]

Afferent: Concerned with the transmission of neural impulse toward the central part of the nervous system. [NIH] Affinity: 1. Inherent likeness or relationship. 2. A special attraction for a specific element, organ, or structure. 3. Chemical affinity; the force that binds atoms in molecules; the tendency of substances to combine by chemical reaction. 4. The strength of noncovalent chemical binding between two substances as measured by the dissociation constant of the complex. 5. In immunology, a thermodynamic expression of the strength of interaction between a single antigen-binding site and a single antigenic determinant (and thus of the stereochemical compatibility between them), most accurately applied to interactions among simple, uniform antigenic determinants such as haptens. Expressed as the association constant (K litres mole -1), which, owing to the heterogeneity of affinities in a population of antibody molecules of a given specificity, actually represents an average value (mean intrinsic association constant). 6. The reciprocal of the dissociation constant. [EU] Agar: A complex sulfated polymer of galactose units, extracted from Gelidium cartilagineum, Gracilaria confervoides, and related red algae. It is used as a gel in the

Dictionary 247

preparation of solid culture media for microorganisms, as a bulk laxative, in making emulsions, and as a supporting medium for immunodiffusion and immunoelectrophoresis. [NIH]

Age Groups: Persons classified by age from birth (infant, newborn) to octogenarians and older (aged, 80 and over). [NIH] Age of Onset: The age or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual. [NIH] Aged, 80 and Over: A person 80 years of age and older. [NIH] Agenesis: Lack of complete or normal development; congenital absence of an organ or part. [NIH]

Agonist: In anatomy, a prime mover. In pharmacology, a drug that has affinity for and stimulates physiologic activity at cell receptors normally stimulated by naturally occurring substances. [EU] Akathisia: 1. A condition of motor restlessness in which there is a feeling of muscular quivering, an urge to move about constantly, and an inability to sit still, a common extrapyramidal side effect of neuroleptic drugs. 2. An inability to sit down because of intense anxiety at the thought of doing so. [EU] Albumin: 1. Any protein that is soluble in water and moderately concentrated salt solutions and is coagulable by heat. 2. Serum albumin; the major plasma protein (approximately 60 per cent of the total), which is responsible for much of the plasma colloidal osmotic pressure and serves as a transport protein carrying large organic anions, such as fatty acids, bilirubin, and many drugs, and also carrying certain hormones, such as cortisol and thyroxine, when their specific binding globulins are saturated. Albumin is synthesized in the liver. Low serum levels occur in protein malnutrition, active inflammation and serious hepatic and renal disease. [EU] Alexia: The inability to recognize or comprehend written or printed words. [NIH] Algorithms: A procedure consisting of a sequence of algebraic formulas and/or logical steps to calculate or determine a given task. [NIH] Alkaline: Having the reactions of an alkali. [EU] Alkaloid: A member of a large group of chemicals that are made by plants and have nitrogen in them. Some alkaloids have been shown to work against cancer. [NIH] Alleles: Mutually exclusive forms of the same gene, occupying the same locus on homologous chromosomes, and governing the same biochemical and developmental process. [NIH] Allergen: An antigenic substance capable of producing immediate-type hypersensitivity (allergy). [EU] Alternative medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used instead of standard treatments. Alternative medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such as massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Ameliorating: A changeable condition which prevents the consequence of a failure or accident from becoming as bad as it otherwise would. [NIH] Amenorrhea: Absence of menstruation. [NIH] Amine: An organic compound containing nitrogen; any member of a group of chemical compounds formed from ammonia by replacement of one or more of the hydrogen atoms by organic (hydrocarbon) radicals. The amines are distinguished as primary, secondary, and

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tertiary, according to whether one, two, or three hydrogen atoms are replaced. The amines include allylamine, amylamine, ethylamine, methylamine, phenylamine, propylamine, and many other compounds. [EU] Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining protein conformation. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. [NIH] Ammonia: A colorless alkaline gas. It is formed in the body during decomposition of organic materials during a large number of metabolically important reactions. [NIH] Amnesia: Lack or loss of memory; inability to remember past experiences. [EU] Amniotic Fluid: Amniotic cavity fluid which is produced by the amnion and fetal lungs and kidneys. [NIH] Amyloid: A general term for a variety of different proteins that accumulate as extracellular fibrils of 7-10 nm and have common structural features, including a beta-pleated sheet conformation and the ability to bind such dyes as Congo red and thioflavine (Kandel, Schwartz, and Jessel, Principles of Neural Science, 3rd ed). [NIH] Amyloidosis: A group of diseases in which protein is deposited in specific organs (localized amyloidosis) or throughout the body (systemic amyloidosis). Amyloidosis may be either primary (with no known cause) or secondary (caused by another disease, including some types of cancer). Generally, primary amyloidosis affects the nerves, skin, tongue, joints, heart, and liver; secondary amyloidosis often affects the spleen, kidneys, liver, and adrenal glands. [NIH] Anaesthesia: Loss of feeling or sensation. Although the term is used for loss of tactile sensibility, or of any of the other senses, it is applied especially to loss of the sensation of pain, as it is induced to permit performance of surgery or other painful procedures. [EU] Anal: Having to do with the anus, which is the posterior opening of the large bowel. [NIH] Analogous: Resembling or similar in some respects, as in function or appearance, but not in origin or development;. [EU] Analysis of Variance: A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable. [NIH] Anatomical: Pertaining to anatomy, or to the structure of the organism. [EU] Anemia: A reduction in the number of circulating erythrocytes or in the quantity of hemoglobin. [NIH] Anesthesia: A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures. [NIH] Angina: Chest pain that originates in the heart. [NIH] Angina Pectoris: The symptom of paroxysmal pain consequent to myocardial ischemia usually of distinctive character, location and radiation, and provoked by a transient stressful situation during which the oxygen requirements of the myocardium exceed the capacity of

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the coronary circulation to supply it. [NIH] Angiogenesis: Blood vessel formation. Tumor angiogenesis is the growth of blood vessels from surrounding tissue to a solid tumor. This is caused by the release of chemicals by the tumor. [NIH] Angioplasty: Endovascular reconstruction of an artery, which may include the removal of atheromatous plaque and/or the endothelial lining as well as simple dilatation. These are procedures performed by catheterization. When reconstruction of an artery is performed surgically, it is called endarterectomy. [NIH] Animal model: An animal with a disease either the same as or like a disease in humans. Animal models are used to study the development and progression of diseases and to test new treatments before they are given to humans. Animals with transplanted human cancers or other tissues are called xenograft models. [NIH] Anions: Negatively charged atoms, radicals or groups of atoms which travel to the anode or positive pole during electrolysis. [NIH] Anomalies: Birth defects; abnormalities. [NIH] Anorexia: Lack or loss of appetite for food. Appetite is psychologic, dependent on memory and associations. Anorexia can be brought about by unattractive food, surroundings, or company. [NIH] Anorexia Nervosa: The chief symptoms are inability to eat, weight loss, and amenorrhea. [NIH]

Anoxia: Clinical manifestation of respiratory distress consisting of a relatively complete absence of oxygen. [NIH] Antagonism: Interference with, or inhibition of, the growth of a living organism by another living organism, due either to creation of unfavorable conditions (e. g. exhaustion of food supplies) or to production of a specific antibiotic substance (e. g. penicillin). [NIH] Anterior chamber: The space in front of the iris and behind the cornea. [NIH] Antibacterial: A substance that destroys bacteria or suppresses their growth or reproduction. [EU] Antibiotic: A drug used to treat infections caused by bacteria and other microorganisms. [NIH]

Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the antigen that induced their synthesis in cells of the lymphoid series (especially plasma cells), or with an antigen closely related to it. [NIH] Antibody: A type of protein made by certain white blood cells in response to a foreign substance (antigen). Each antibody can bind to only a specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen. [NIH] Anticoagulant: A drug that helps prevent blood clots from forming. Also called a blood thinner. [NIH] Anticonvulsant: An agent that prevents or relieves convulsions. [EU] Antiemetic: An agent that prevents or alleviates nausea and vomiting. Also antinauseant. [EU]

Antigen: Any substance which is capable, under appropriate conditions, of inducing a specific immune response and of reacting with the products of that response, that is, with specific antibody or specifically sensitized T-lymphocytes, or both. Antigens may be soluble

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substances, such as toxins and foreign proteins, or particulate, such as bacteria and tissue cells; however, only the portion of the protein or polysaccharide molecule known as the antigenic determinant (q.v.) combines with antibody or a specific receptor on a lymphocyte. Abbreviated Ag. [EU] Anti-infective: An agent that so acts. [EU] Anti-inflammatory: Having to do with reducing inflammation. [NIH] Antioxidant: A substance that prevents damage caused by free radicals. Free radicals are highly reactive chemicals that often contain oxygen. They are produced when molecules are split to give products that have unpaired electrons. This process is called oxidation. [NIH] Antipsychotic: Effective in the treatment of psychosis. Antipsychotic drugs (called also neuroleptic drugs and major tranquilizers) are a chemically diverse (including phenothiazines, thioxanthenes, butyrophenones, dibenzoxazepines, dibenzodiazepines, and diphenylbutylpiperidines) but pharmacologically similar class of drugs used to treat schizophrenic, paranoid, schizoaffective, and other psychotic disorders; acute delirium and dementia, and manic episodes (during induction of lithium therapy); to control the movement disorders associated with Huntington's chorea, Gilles de la Tourette's syndrome, and ballismus; and to treat intractable hiccups and severe nausea and vomiting. Antipsychotic agents bind to dopamine, histamine, muscarinic cholinergic, a-adrenergic, and serotonin receptors. Blockade of dopaminergic transmission in various areas is thought to be responsible for their major effects : antipsychotic action by blockade in the mesolimbic and mesocortical areas; extrapyramidal side effects (dystonia, akathisia, parkinsonism, and tardive dyskinesia) by blockade in the basal ganglia; and antiemetic effects by blockade in the chemoreceptor trigger zone of the medulla. Sedation and autonomic side effects (orthostatic hypotension, blurred vision, dry mouth, nasal congestion and constipation) are caused by blockade of histamine, cholinergic, and adrenergic receptors. [EU] Antipsychotic Agents: Agents that control agitated psychotic behavior, alleviate acute psychotic states, reduce psychotic symptoms, and exert a quieting effect. They are used in schizophrenia, senile dementia, transient psychosis following surgery or myocardial infarction, etc. These drugs are often referred to as neuroleptics alluding to the tendency to produce neurological side effects, but not all antipsychotics are likely to produce such effects. Many of these drugs may also be effective against nausea, emesis, and pruritus. [NIH] Anxiety: Persistent feeling of dread, apprehension, and impending disaster. [NIH] Apathy: Lack of feeling or emotion; indifference. [EU] Aphasia: A cognitive disorder marked by an impaired ability to comprehend or express language in its written or spoken form. This condition is caused by diseases which affect the language areas of the dominant hemisphere. Clinical features are used to classify the various subtypes of this condition. General categories include receptive, expressive, and mixed forms of aphasia. [NIH] Apoptosis: One of the two mechanisms by which cell death occurs (the other being the pathological process of necrosis). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA (DNA fragmentation) at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth. [NIH] Aqueous: Having to do with water. [NIH] Arachidonic Acid: An unsaturated, essential fatty acid. It is found in animal and human fat

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as well as in the liver, brain, and glandular organs, and is a constituent of animal phosphatides. It is formed by the synthesis from dietary linoleic acid and is a precursor in the biosynthesis of prostaglandins, thromboxanes, and leukotrienes. [NIH] Arginase: A ureahydrolase that catalyzes the hydrolysis of arginine or canavanine to yield L-ORNITHINE and urea. Deficiency of this enzyme causes hyperargininemia. EC 3.5.3.1. [NIH]

Arginine: An essential amino acid that is physiologically active in the L-form. [NIH] Arterial: Pertaining to an artery or to the arteries. [EU] Arteries: The vessels carrying blood away from the heart. [NIH] Arterioles: The smallest divisions of the arteries located between the muscular arteries and the capillaries. [NIH] Ascorbic Acid: A six carbon compound related to glucose. It is found naturally in citrus fruits and many vegetables. Ascorbic acid is an essential nutrient in human diets, and necessary to maintain connective tissue and bone. Its biologically active form, vitamin C, functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant. [NIH] Aseptic: Free from infection or septic material; sterile. [EU] Aspartate: A synthetic amino acid. [NIH] Assay: Determination of the amount of a particular constituent of a mixture, or of the biological or pharmacological potency of a drug. [EU] Astrocytes: The largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the blood brain barrier. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with microglia) respond to injury. Astrocytes have high- affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitter, but their role in signaling (as in many other functions) is not well understood. [NIH] Asymptomatic: Having no signs or symptoms of disease. [NIH] Ataxia: Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharnyx, larnyx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. [NIH] Atrophy: Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. [NIH] Attenuated: Strain with weakened or reduced virulence. [NIH] Atypical: Irregular; not conformable to the type; in microbiology, applied specifically to strains of unusual type. [EU] Audiologist: Study of hearing including treatment of persons with hearing defects. [NIH] Auditory: Pertaining to the sense of hearing. [EU] Authorship: The profession of writing. Also the identity of the writer as the creator of a literary production. [NIH] Autoimmune disease: A condition in which the body recognizes its own tissues as foreign

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and directs an immune response against them. [NIH] Autoimmunity: Process whereby the immune system reacts against the body's own tissues. Autoimmunity may produce or be caused by autoimmune diseases. [NIH] Autonomic: Self-controlling; functionally independent. [EU] Autonomic Nervous System: The enteric, parasympathetic, and sympathetic nervous systems taken together. Generally speaking, the autonomic nervous system regulates the internal environment during both peaceful activity and physical or emotional stress. Autonomic activity is controlled and integrated by the central nervous system, especially the hypothalamus and the solitary nucleus, which receive information relayed from visceral afferents; these and related central and sensory structures are sometimes (but not here) considered to be part of the autonomic nervous system itself. [NIH] Axonal: Condition associated with metabolic derangement of the entire neuron and is manifest by degeneration of the distal portion of the nerve fiber. [NIH] Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body. [NIH] Bacteria: Unicellular prokaryotic microorganisms which generally possess rigid cell walls, multiply by cell division, and exhibit three principal forms: round or coccal, rodlike or bacillary, and spiral or spirochetal. [NIH] Bacterial Physiology: Physiological processes and activities of bacteria. [NIH] Bacteriophage: A virus whose host is a bacterial cell; A virus that exclusively infects bacteria. It generally has a protein coat surrounding the genome (DNA or RNA). One of the coliphages most extensively studied is the lambda phage, which is also one of the most important. [NIH] Bacterium: Microscopic organism which may have a spherical, rod-like, or spiral unicellular or non-cellular body. Bacteria usually reproduce through asexual processes. [NIH] Basal Ganglia: Large subcortical nuclear masses derived from the telencephalon and located in the basal regions of the cerebral hemispheres. [NIH] Basal Ganglia Diseases: Diseases of the basal ganglia including the putamen; globus pallidus; claustrum; amygdala; and caudate nucleus. Dyskinesias (most notably involuntary movements and alterations of the rate of movement) represent the primary clinical manifestations of these disorders. Common etiologies include cerebrovascular disease; neurodegenerative diseases; and craniocerebral trauma. [NIH] Base: In chemistry, the nonacid part of a salt; a substance that combines with acids to form salts; a substance that dissociates to give hydroxide ions in aqueous solutions; a substance whose molecule or ion can combine with a proton (hydrogen ion); a substance capable of donating a pair of electrons (to an acid) for the formation of a coordinate covalent bond. [EU] Base Sequence: The sequence of purines and pyrimidines in nucleic acids and polynucleotides. It is also called nucleotide or nucleoside sequence. [NIH] Basement Membrane: Ubiquitous supportive tissue adjacent to epithelium and around smooth and striated muscle cells. This tissue contains intrinsic macromolecular components such as collagen, laminin, and sulfated proteoglycans. As seen by light microscopy one of its subdivisions is the basal (basement) lamina. [NIH] Behavioral Sciences: Disciplines concerned with the study of human and animal behavior. [NIH]

Behavioral Symptoms: Observable manifestions of impaired psychological functioning. [NIH]

Benign: Not cancerous; does not invade nearby tissue or spread to other parts of the body.

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[NIH]

Benign tumor: A noncancerous growth that does not invade nearby tissue or spread to other parts of the body. [NIH] Beta-pleated: Particular three-dimensional pattern of amyloidoses. [NIH] Bewilderment: Impairment or loss of will power. [NIH] Bilateral: Affecting both the right and left side of body. [NIH] Bile: An emulsifying agent produced in the liver and secreted into the duodenum. Its composition includes bile acids and salts, cholesterol, and electrolytes. It aids digestion of fats in the duodenum. [NIH] Bile Acids: Acids made by the liver that work with bile to break down fats. [NIH] Bile Acids and Salts: Steroid acids and salts. The primary bile acids are derived from cholesterol in the liver and usually conjugated with glycine or taurine. The secondary bile acids are further modified by bacteria in the intestine. They play an important role in the digestion and absorption of fat. They have also been used pharmacologically, especially in the treatment of gallstones. [NIH] Bilirubin: A bile pigment that is a degradation product of heme. [NIH] Biochemical: Relating to biochemistry; characterized by, produced by, or involving chemical reactions in living organisms. [EU] Biogenesis: The origin of life. It includes studies of the potential basis for life in organic compounds but excludes studies of the development of altered forms of life through mutation and natural selection, which is evolution. [NIH] Biological Sciences: All of the divisions of the natural sciences dealing with the various aspects of the phenomena of life and vital processes. The concept includes anatomy and physiology, biochemistry and biophysics, and the biology of animals, plants, and microorganisms. It should be differentiated from biology, one of its subdivisions, concerned specifically with the origin and life processes of living organisms. [NIH] Biological therapy: Treatment to stimulate or restore the ability of the immune system to fight infection and disease. Also used to lessen side effects that may be caused by some cancer treatments. Also known as immunotherapy, biotherapy, or biological response modifier (BRM) therapy. [NIH] Biophysics: The science of physical phenomena and processes in living organisms. [NIH] Biosynthesis: The building up of a chemical compound in the physiologic processes of a living organism. [EU] Biotechnology: Body of knowledge related to the use of organisms, cells or cell-derived constituents for the purpose of developing products which are technically, scientifically and clinically useful. Alteration of biologic function at the molecular level (i.e., genetic engineering) is a central focus; laboratory methods used include transfection and cloning technologies, sequence and structure analysis algorithms, computer databases, and gene and protein structure function analysis and prediction. [NIH] Bipolar Disorder: A major affective disorder marked by severe mood swings (manic or major depressive episodes) and a tendency to remission and recurrence. [NIH] Bladder: The organ that stores urine. [NIH] Blastocyst: The mammalian embryo in the post-morula stage in which a fluid-filled cavity, enclosed primarily by trophoblast, contains an inner cell mass which becomes the embryonic disc. [NIH] Blood Platelets: Non-nucleated disk-shaped cells formed in the megakaryocyte and found

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in the blood of all mammals. They are mainly involved in blood coagulation. [NIH] Blood pressure: The pressure of blood against the walls of a blood vessel or heart chamber. Unless there is reference to another location, such as the pulmonary artery or one of the heart chambers, it refers to the pressure in the systemic arteries, as measured, for example, in the forearm. [NIH] Blood vessel: A tube in the body through which blood circulates. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins. [NIH] Blot: To transfer DNA, RNA, or proteins to an immobilizing matrix such as nitrocellulose. [NIH]

Body Fluids: Liquid components of living organisms. [NIH] Bone Marrow: The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. [NIH] Bone scan: A technique to create images of bones on a computer screen or on film. A small amount of radioactive material is injected into a blood vessel and travels through the bloodstream; it collects in the bones and is detected by a scanner. [NIH] Bowel: The long tube-shaped organ in the abdomen that completes the process of digestion. There is both a small and a large bowel. Also called the intestine. [NIH] Bowel Movement: Body wastes passed through the rectum and anus. [NIH] Bradykinin: A nonapeptide messenger that is enzymatically produced from kallidin in the blood where it is a potent but short-lived agent of arteriolar dilation and increased capillary permeability. Bradykinin is also released from mast cells during asthma attacks, from gut walls as a gastrointestinal vasodilator, from damaged tissues as a pain signal, and may be a neurotransmitter. [NIH] Brain Diseases: Pathologic conditions affecting the brain, which is composed of the intracranial components of the central nervous system. This includes (but is not limited to) the cerebral cortex; intracranial white matter; basal ganglia; thalamus; hypothalamus; brain stem; and cerebellum. [NIH] Brain Neoplasms: Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain. [NIH] Brain Stem: The part of the brain that connects the cerebral hemispheres with the spinal cord. It consists of the mesencephalon, pons, and medulla oblongata. [NIH] Branch: Most commonly used for branches of nerves, but applied also to other structures. [NIH]

Breakdown: A physical, metal, or nervous collapse. [NIH] Bromocriptine: A semisynthetic ergot alkaloid that is a dopamine D2 agonist. It suppresses prolactin secretion and is used to treat amenorrhea, galactorrhea, and female infertility, and has been proposed for Parkinson disease. [NIH] Bronchial: Pertaining to one or more bronchi. [EU] Bruxism: A disorder characterized by grinding and clenching of the teeth. [NIH]

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Bulbar: Pertaining to a bulb; pertaining to or involving the medulla oblongata, as bulbar paralysis. [EU] Bulimia: Episodic binge eating. The episodes may be associated with the fear of not being able to stop eating, depressed mood, or self-deprecating thoughts (binge-eating disorder) and may frequently be terminated by self-induced vomiting (bulimia nervosa). [NIH] Butyric Acid: A four carbon acid, CH3CH2CH2COOH, with an unpleasant odor that occurs in butter and animal fat as the glycerol ester. [NIH] Cachexia: General ill health, malnutrition, and weight loss, usually associated with chronic disease. [NIH] Calcification: Deposits of calcium in the tissues of the breast. Calcification in the breast can be seen on a mammogram, but cannot be detected by touch. There are two types of breast calcification, macrocalcification and microcalcification. Macrocalcifications are large deposits and are usually not related to cancer. Microcalcifications are specks of calcium that may be found in an area of rapidly dividing cells. Many microcalcifications clustered together may be a sign of cancer. [NIH] Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. [NIH] Canonical: A particular nucleotide sequence in which each position represents the base more often found when many actual sequences of a given class of genetic elements are compared. [NIH] Capsules: Hard or soft soluble containers used for the oral administration of medicine. [NIH] Carbohydrate: An aldehyde or ketone derivative of a polyhydric alcohol, particularly of the pentahydric and hexahydric alcohols. They are so named because the hydrogen and oxygen are usually in the proportion to form water, (CH2O)n. The most important carbohydrates are the starches, sugars, celluloses, and gums. They are classified into mono-, di-, tri-, polyand heterosaccharides. [EU] Carbon Dioxide: A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. [NIH] Carcinogenic: Producing carcinoma. [EU] Carcinoma: Cancer that begins in the skin or in tissues that line or cover internal organs. [NIH]

Cardiomyopathy: A general diagnostic term designating primary myocardial disease, often of obscure or unknown etiology. [EU] Cardiovascular: Having to do with the heart and blood vessels. [NIH] Cardiovascular Abnormalities: Congenital structural abnormalities of the cardiovascular system. [NIH] Cardiovascular disease: Any abnormal condition characterized by dysfunction of the heart and blood vessels. CVD includes atherosclerosis (especially coronary heart disease, which can lead to heart attacks), cerebrovascular disease (e.g., stroke), and hypertension (high blood pressure). [NIH] Cardiovascular System: The heart and the blood vessels by which blood is pumped and circulated through the body. [NIH]

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Carotene: The general name for a group of pigments found in green, yellow, and leafy vegetables, and yellow fruits. The pigments are fat-soluble, unsaturated aliphatic hydrocarbons functioning as provitamins and are converted to vitamin A through enzymatic processes in the intestinal wall. [NIH] Case report: A detailed report of the diagnosis, treatment, and follow-up of an individual patient. Case reports also contain some demographic information about the patient (for example, age, gender, ethnic origin). [NIH] Caspase: Enzyme released by the cell at a crucial stage in apoptosis in order to shred all cellular proteins. [NIH] Cataracts: In medicine, an opacity of the crystalline lens of the eye obstructing partially or totally its transmission of light. [NIH] Catecholamine: A group of chemical substances manufactured by the adrenal medulla and secreted during physiological stress. [NIH] Catheterization: Use or insertion of a tubular device into a duct, blood vessel, hollow organ, or body cavity for injecting or withdrawing fluids for diagnostic or therapeutic purposes. It differs from intubation in that the tube here is used to restore or maintain patency in obstructions. [NIH] Caveolae: Endocytic/exocytic cell membrane structures rich in glycosphingolipids, cholesterol, and lipid-anchored membrane proteins that function in endocytosis (potocytosis), transcytosis, and signal transduction. Caveolae assume various shapes from open pits to closed vesicles. Caveolar coats are composed of caveolins. [NIH] Caveolins: The main structural proteins of caveolae. Several distinct genes for caveolins have been identified. [NIH] Cell: The individual unit that makes up all of the tissues of the body. All living things are made up of one or more cells. [NIH] Cell Adhesion: Adherence of cells to surfaces or to other cells. [NIH] Cell Adhesion Molecules: Surface ligands, usually glycoproteins, that mediate cell-to-cell adhesion. Their functions include the assembly and interconnection of various vertebrate systems, as well as maintenance of tissue integration, wound healing, morphogenic movements, cellular migrations, and metastasis. [NIH] Cell Cycle: The complex series of phenomena, occurring between the end of one cell division and the end of the next, by which cellular material is divided between daughter cells. [NIH] Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability. [NIH] Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function which takes place during the development of the embryo and leads to the formation of specialized cells, tissues, and organs. [NIH] Cell Division: The fission of a cell. [NIH] Cell membrane: Cell membrane = plasma membrane. The structure enveloping a cell, enclosing the cytoplasm, and forming a selective permeability barrier; it consists of lipids, proteins, and some carbohydrates, the lipids thought to form a bilayer in which integral proteins are embedded to varying degrees. [EU] Cell Membrane Structures: Structures which are part of the cell membrane or have cell membrane as a major part of their structure. [NIH] Cell proliferation: An increase in the number of cells as a result of cell growth and cell

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division. [NIH] Cell Respiration: The metabolic process of all living cells (animal and plant) in which oxygen is used to provide a source of energy for the cell. [NIH] Cell Survival: The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability. [NIH] Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges. [NIH] Central Nervous System Infections: Pathogenic infections of the brain, spinal cord, and meninges. DNA virus infections; RNA virus infections; bacterial infections; mycoplasma infections; Spirochaetales infections; fungal infections; protozoan infections; helminthiasis; and prion diseases may involve the central nervous system as a primary or secondary process. [NIH] Ceramide: A type of fat produced in the body. It may cause some types of cells to die, and is being studied in cancer treatment. [NIH] Cerebellar: Pertaining to the cerebellum. [EU] Cerebellar Diseases: Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, gait ataxia, and muscle hypotonia. [NIH] Cerebellum: Part of the metencephalon that lies in the posterior cranial fossa behind the brain stem. It is concerned with the coordination of movement. [NIH] Cerebral: Of or pertaining of the cerebrum or the brain. [EU] Cerebral Infarction: The formation of an area of necrosis in the cerebrum caused by an insufficiency of arterial or venous blood flow. Infarcts of the cerebrum are generally classified by hemisphere (i.e., left vs. right), lobe (e.g., frontal lobe infarction), arterial distribution (e.g., infarction, anterior cerebral artery), and etiology (e.g., embolic infarction). [NIH]

Cerebral Palsy: Refers to a motor disability caused by a brain dysfunction. [NIH] Cerebrospinal: Pertaining to the brain and spinal cord. [EU] Cerebrospinal fluid: CSF. The fluid flowing around the brain and spinal cord. Cerebrospinal fluid is produced in the ventricles in the brain. [NIH] Cerebrovascular: Pertaining to the blood vessels of the cerebrum, or brain. [EU] Cerebrum: The largest part of the brain. It is divided into two hemispheres, or halves, called the cerebral hemispheres. The cerebrum controls muscle functions of the body and also controls speech, emotions, reading, writing, and learning. [NIH] Character: In current usage, approximately equivalent to personality. The sum of the relatively fixed personality traits and habitual modes of response of an individual. [NIH] Chemoreceptor: A receptor adapted for excitation by chemical substances, e.g., olfactory and gustatory receptors, or a sense organ, as the carotid body or the aortic (supracardial) bodies, which is sensitive to chemical changes in the blood stream, especially reduced oxygen content, and reflexly increases both respiration and blood pressure. [EU] Child Care: Care of children in the home or institution. [NIH] Chlorhexidine: Disinfectant and topical anti-infective agent used also as mouthwash to prevent oral plaque. [NIH] Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially

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the brain and spinal cord, and in animal fats and oils. [NIH] Cholinergic: Resembling acetylcholine in pharmacological action; stimulated by or releasing acetylcholine or a related compound. [EU] Chondrodysplasia Punctata: A heterogeneous group of bone dysplasias, the common character of which is stippling of the epiphyses in infancy. The group includes a severe autosomal recessive form (Chondrodysplasia punctata, rhizomelic), an autosomal dominant form (Conradi-Hunermann syndrome), and a milder X-linked form. Metabolic defects associated with impaired peroxisomes are present only in the rhizomelic form. [NIH] Chondroitin sulfate: The major glycosaminoglycan (a type of sugar molecule) in cartilage. [NIH]

Chorea: Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as choreatic disorders. Chorea is also a frequent manifestation of basal ganglia diseases. [NIH] Chorioretinitis: Inflammation of the choroid in which the sensory retina becomes edematous and opaque. The inflammatory cells and exudate may burst through the sensory retina to cloud the vitreous body. [NIH] Choroid: The thin, highly vascular membrane covering most of the posterior of the eye between the retina and sclera. [NIH] Chromaffin System: The cells of the body which stain with chromium salts. They occur along the sympathetic nerves, in the adrenal gland, and in various other organs. [NIH] Chromatin: The material of chromosomes. It is a complex of DNA, histones, and nonhistone proteins (chromosomal proteins, non-histone) found within the nucleus of a cell. [NIH] Chromosomal: Pertaining to chromosomes. [EU] Chromosome: Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes. [NIH] Chromosome Aberrations: Deviations from the normal number or structure of chromosomes, not necessarily associated with disease. [NIH] Chromosome Abnormalities: Defects in the structure or number of chromosomes resulting in structural aberrations or manifesting as disease. [NIH] Chromosome Fragility: Susceptibility of chromosomes to breakage and translocation or other aberrations. Chromosome fragile sites are regions that show up in karyotypes as a gap (uncondensed stretch) on the chromatid arm. They are associated with chromosome break sites and other aberrations. A fragile site on the X chromosome is associated with fragile X syndrome. Fragile sites are designated by the letters "FRA" followed by the designation for the specific chromosome and a letter which refers to the different fragile sites on a chromosome (e.g. FRAXA). [NIH] Chronic: A disease or condition that persists or progresses over a long period of time. [NIH] Chronic Disease: Disease or ailment of long duration. [NIH] Circulatory system: The system that contains the heart and the blood vessels and moves blood throughout the body. This system helps tissues get enough oxygen and nutrients, and it helps them get rid of waste products. The lymph system, which connects with the blood system, is often considered part of the circulatory system. [NIH] CIS: Cancer Information Service. The CIS is the National Cancer Institute's link to the public, interpreting and explaining research findings in a clear and understandable manner, and

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providing personalized responses to specific questions about cancer. Access the CIS by calling 1-800-4-CANCER, or by using the Web site at http://cis.nci.nih.gov. [NIH] Citrus: Any tree or shrub of the Rue family or the fruit of these plants. [NIH] Clamp: A u-shaped steel rod used with a pin or wire for skeletal traction in the treatment of certain fractures. [NIH] Clear cell carcinoma: A rare type of tumor of the female genital tract in which the inside of the cells looks clear when viewed under a microscope. [NIH] Cleft Palate: Congenital fissure of the soft and/or hard palate, due to faulty fusion. [NIH] Clinical Medicine: The study and practice of medicine by direct examination of the patient. [NIH]

Clinical study: A research study in which patients receive treatment in a clinic or other medical facility. Reports of clinical studies can contain results for single patients (case reports) or many patients (case series or clinical trials). [NIH] Clinical trial: A research study that tests how well new medical treatments or other interventions work in people. Each study is designed to test new methods of screening, prevention, diagnosis, or treatment of a disease. [NIH] Clone: The term "clone" has acquired a new meaning. It is applied specifically to the bits of inserted foreign DNA in the hybrid molecules of the population. Each inserted segment originally resided in the DNA of a complex genome amid millions of other DNA segment. [NIH]

Cloning: The production of a number of genetically identical individuals; in genetic engineering, a process for the efficient replication of a great number of identical DNA molecules. [NIH] Clozapine: A tricylic dibenzodiazepine, classified as an atypical antipsychotic agent. It binds several types of central nervous system receptors, and displays a unique pharmacological profile. Clozapine is a serotonin antagonist, with strong binding to 5-HT 2A/2C receptor subtype. It also displays strong affinity to several dopaminergic receptors, but shows only weak antagonism at the dopamine D2 receptor, a receptor commonly thought to modulate neuroleptic activity. Agranulocytosis is a major adverse effect associated with administration of this agent. [NIH] Cochlea: The part of the internal ear that is concerned with hearing. It forms the anterior part of the labyrinth, is conical, and is placed almost horizontally anterior to the vestibule. [NIH]

Cochlear: Of or pertaining to the cochlea. [EU] Cochlear Diseases: Diseases of the cochlea, the part of the inner ear that is concerned with hearing. [NIH] Coenzyme: An organic nonprotein molecule, frequently a phosphorylated derivative of a water-soluble vitamin, that binds with the protein molecule (apoenzyme) to form the active enzyme (holoenzyme). [EU] Cofactor: A substance, microorganism or environmental factor that activates or enhances the action of another entity such as a disease-causing agent. [NIH] Cognition: Intellectual or mental process whereby an organism becomes aware of or obtains knowledge. [NIH] Colchicine: A major alkaloid from Colchicum autumnale L. and found also in other Colchicum species. Its primary therapeutic use is in the treatment of gout, but it has been used also in the therapy of familial Mediterranean fever (periodic disease). [NIH]

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Collagen: A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin, connective tissue, and the organic substance of bones and teeth. Different forms of collagen are produced in the body but all consist of three alpha-polypeptide chains arranged in a triple helix. Collagen is differentiated from other fibrous proteins, such as elastin, by the content of proline, hydroxyproline, and hydroxylysine; by the absence of tryptophan; and particularly by the high content of polar groups which are responsible for its swelling properties. [NIH] Collapse: 1. A state of extreme prostration and depression, with failure of circulation. 2. Abnormal falling in of the walls of any part of organ. [EU] Colloidal: Of the nature of a colloid. [EU] Coloboma: Congenital anomaly in which some of the structures of the eye are absent due to incomplete fusion of the fetal intraocular fissure during gestation. [NIH] Combinatorial: A cut-and-paste process that churns out thousands of potentially valuable compounds at once. [NIH] Communication Disorders: Disorders of verbal and nonverbal communication caused by receptive or expressive language disorders, cognitive dysfunction (e.g., mental retardation), psychiatric conditions, and hearing disorders. [NIH] Competency: The capacity of the bacterium to take up DNA from its surroundings. [NIH] Complement: A term originally used to refer to the heat-labile factor in serum that causes immune cytolysis, the lysis of antibody-coated cells, and now referring to the entire functionally related system comprising at least 20 distinct serum proteins that is the effector not only of immune cytolysis but also of other biologic functions. Complement activation occurs by two different sequences, the classic and alternative pathways. The proteins of the classic pathway are termed 'components of complement' and are designated by the symbols C1 through C9. C1 is a calcium-dependent complex of three distinct proteins C1q, C1r and C1s. The proteins of the alternative pathway (collectively referred to as the properdin system) and complement regulatory proteins are known by semisystematic or trivial names. Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case letter suffixes, e.g., C3a. Inactivated fragments may be designated with the suffix 'i', e.g. C3bi. Activated components or complexes with biological activity are designated by a bar over the symbol e.g. C1 or C4b,2a. The classic pathway is activated by the binding of C1 to classic pathway activators, primarily antigen-antibody complexes containing IgM, IgG1, IgG3; C1q binds to a single IgM molecule or two adjacent IgG molecules. The alternative pathway can be activated by IgA immune complexes and also by nonimmunologic materials including bacterial endotoxins, microbial polysaccharides, and cell walls. Activation of the classic pathway triggers an enzymatic cascade involving C1, C4, C2 and C3; activation of the alternative pathway triggers a cascade involving C3 and factors B, D and P. Both result in the cleavage of C5 and the formation of the membrane attack complex. Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. [EU] Complementary and alternative medicine: CAM. Forms of treatment that are used in addition to (complementary) or instead of (alternative) standard treatments. These practices are not considered standard medical approaches. CAM includes dietary supplements, megadose vitamins, herbal preparations, special teas, massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Complementary medicine: Practices not generally recognized by the medical community as standard or conventional medical approaches and used to enhance or complement the standard treatments. Complementary medicine includes the taking of dietary supplements, megadose vitamins, and herbal preparations; the drinking of special teas; and practices such

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as massage therapy, magnet therapy, spiritual healing, and meditation. [NIH] Complementation: The production of a wild-type phenotype when two different mutations are combined in a diploid or a heterokaryon and tested in trans-configuration. [NIH] Computational Biology: A field of biology concerned with the development of techniques for the collection and manipulation of biological data, and the use of such data to make biological discoveries or predictions. This field encompasses all computational methods and theories applicable to molecular biology and areas of computer-based techniques for solving biological problems including manipulation of models and datasets. [NIH] Computed tomography: CT scan. A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computerized tomography and computerized axial tomography (CAT) scan. [NIH] Computer-Assisted Instruction: A self-learning technique, usually online, involving interaction of the student with programmed instructional materials. [NIH] Computerized axial tomography: A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography (CT scan), or computerized tomography. [NIH] Computerized tomography: A series of detailed pictures of areas inside the body, taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computerized axial tomography (CAT) scan and computed tomography (CT scan). [NIH] Conception: The onset of pregnancy, marked by implantation of the blastocyst; the formation of a viable zygote. [EU] Cone: One of the special retinal receptor elements which are presumed to be primarily concerned with perception of light and color stimuli when the eye is adapted to light. [NIH] Confusion: A mental state characterized by bewilderment, emotional disturbance, lack of clear thinking, and perceptual disorientation. [NIH] Congestion: Excessive or abnormal accumulation of blood in a part. [EU] Congestive heart failure: Weakness of the heart muscle that leads to a buildup of fluid in body tissues. [NIH] Conjugated: Acting or operating as if joined; simultaneous. [EU] Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connective Tissue: Tissue that supports and binds other tissues. It consists of connective tissue cells embedded in a large amount of extracellular matrix. [NIH] Connexins: A group of homologous proteins which form the intermembrane channels of gap junctions. The connexins are the products of an identified gene family which has both highly conserved and highly divergent regions. The variety contributes to the wide range of functional properties of gap junctions. [NIH] Consciousness: Sense of awareness of self and of the environment. [NIH] Constipation: Infrequent or difficult evacuation of feces. [NIH] Constitutional: 1. Affecting the whole constitution of the body; not local. 2. Pertaining to the constitution. [EU] Consultation: A deliberation between two or more physicians concerning the diagnosis and the proper method of treatment in a case. [NIH]

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Consumption: Pulmonary tuberculosis. [NIH] Continence: The ability to hold in a bowel movement or urine. [NIH] Contraindications: Any factor or sign that it is unwise to pursue a certain kind of action or treatment, e. g. giving a general anesthetic to a person with pneumonia. [NIH] Controlled clinical trial: A clinical study that includes a comparison (control) group. The comparison group receives a placebo, another treatment, or no treatment at all. [NIH] Controlled study: An experiment or clinical trial that includes a comparison (control) group. [NIH]

Cooperative group: A group of physicians, hospitals, or both formed to treat a large number of persons in the same way so that new treatment can be evaluated quickly. Clinical trials of new cancer treatments often require many more people than a single physician or hospital can care for. [NIH] Coordination: Muscular or motor regulation or the harmonious cooperation of muscles or groups of muscles, in a complex action or series of actions. [NIH] Coronary: Encircling in the manner of a crown; a term applied to vessels; nerves, ligaments, etc. The term usually denotes the arteries that supply the heart muscle and, by extension, a pathologic involvement of them. [EU] Coronary Circulation: The circulation of blood through the coronary vessels of the heart. [NIH]

Coronary heart disease: A type of heart disease caused by narrowing of the coronary arteries that feed the heart, which needs a constant supply of oxygen and nutrients carried by the blood in the coronary arteries. When the coronary arteries become narrowed or clogged by fat and cholesterol deposits and cannot supply enough blood to the heart, CHD results. [NIH] Coronary Thrombosis: Presence of a thrombus in a coronary artery, often causing a myocardial infarction. [NIH] Corpus: The body of the uterus. [NIH] Corpus Callosum: Broad plate of dense myelinated fibers that reciprocally interconnect regions of the cortex in all lobes with corresponding regions of the opposite hemisphere. The corpus callosum is located deep in the longitudinal fissure. [NIH] Cortex: The outer layer of an organ or other body structure, as distinguished from the internal substance. [EU] Cortical: Pertaining to or of the nature of a cortex or bark. [EU] Cortisol: A steroid hormone secreted by the adrenal cortex as part of the body's response to stress. [NIH] Cranial: Pertaining to the cranium, or to the anterior (in animals) or superior (in humans) end of the body. [EU] Craniocerebral Trauma: Traumatic injuries involving the cranium and intracranial structures (i.e., brain; cranial nerves; meninges; and other structures). Injuries may be classified by whether or not the skull is penetrated (i.e., penetrating vs. nonpenetrating) or whether there is an associated hemorrhage. [NIH] Craniofacial Abnormalities: Congenital structural deformities, malformations, or other abnormalities of the cranium and facial bones. [NIH] Creatine: An amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as creatinine in the urine. [NIH]

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Creatinine: A compound that is excreted from the body in urine. Creatinine levels are measured to monitor kidney function. [NIH] Crossing-over: The exchange of corresponding segments between chromatids of homologous chromosomes during meiosia, forming a chiasma. [NIH] Cryofixation: Fixation of a tissue by localized cooling at very low temperature. [NIH] Cryopreservation: Preservation of cells, tissues, organs, or embryos by freezing. In histological preparations, cryopreservation or cryofixation is used to maintain the existing form, structure, and chemical composition of all the constituent elements of the specimens. [NIH]

Cues: Signals for an action; that specific portion of a perceptual field or pattern of stimuli to which a subject has learned to respond. [NIH] Curative: Tending to overcome disease and promote recovery. [EU] Cyclic: Pertaining to or occurring in a cycle or cycles; the term is applied to chemical compounds that contain a ring of atoms in the nucleus. [EU] Cystathionine beta-Synthase: A multifunctional pyridoxal phosphate enzyme. In the second stage of cysteine biosynthesis it catalyzes the reaction of homocysteine with serine to form cystathionine with the elimination of water. Deficiency of this enzyme leads to hyperhomocysteinemia and homocystinuria. EC 4.2.1.22. [NIH] Cysteine: A thiol-containing non-essential amino acid that is oxidized to form cystine. [NIH] Cystine: A covalently linked dimeric nonessential amino acid formed by the oxidation of cysteine. Two molecules of cysteine are joined together by a disulfide bridge to form cystine. [NIH]

Cytochrome: Any electron transfer hemoprotein having a mode of action in which the transfer of a single electron is effected by a reversible valence change of the central iron atom of the heme prosthetic group between the +2 and +3 oxidation states; classified as cytochromes a in which the heme contains a formyl side chain, cytochromes b, which contain protoheme or a closely similar heme that is not covalently bound to the protein, cytochromes c in which protoheme or other heme is covalently bound to the protein, and cytochromes d in which the iron-tetrapyrrole has fewer conjugated double bonds than the hemes have. Well-known cytochromes have been numbered consecutively within groups and are designated by subscripts (beginning with no subscript), e.g. cytochromes c, c1, C2, . New cytochromes are named according to the wavelength in nanometres of the absorption maximum of the a-band of the iron (II) form in pyridine, e.g., c-555. [EU] Cytogenetics: A branch of genetics which deals with the cytological and molecular behavior of genes and chromosomes during cell division. [NIH] Cytokine: Small but highly potent protein that modulates the activity of many cell types, including T and B cells. [NIH] Cytomegalovirus: A genus of the family Herpesviridae, subfamily Betaherpesvirinae, infecting the salivary glands, liver, spleen, lungs, eyes, and other organs, in which they produce characteristically enlarged cells with intranuclear inclusions. Infection with Cytomegalovirus is also seen as an opportunistic infection in AIDS. [NIH] Cytopenia: A reduction in the number of blood cells. [NIH] Cytoplasm: The protoplasm of a cell exclusive of that of the nucleus; it consists of a continuous aqueous solution (cytosol) and the organelles and inclusions suspended in it (phaneroplasm), and is the site of most of the chemical activities of the cell. [EU] Cytoskeletal Proteins: Major constituent of the cytoskeleton found in the cytoplasm of eukaryotic cells. They form a flexible framework for the cell, provide attachment points for

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organelles and formed bodies, and make communication between parts of the cell possible. [NIH]

Cytoskeleton: The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm. [NIH] Cytotoxic: Cell-killing. [NIH] Databases, Bibliographic: Extensive collections, reputedly complete, of references and citations to books, articles, publications, etc., generally on a single subject or specialized subject area. Databases can operate through automated files, libraries, or computer disks. The concept should be differentiated from factual databases which is used for collections of data and facts apart from bibliographic references to them. [NIH] De novo: In cancer, the first occurrence of cancer in the body. [NIH] Deamination: The removal of an amino group (NH2) from a chemical compound. [NIH] Decarboxylation: The removal of a carboxyl group, usually in the form of carbon dioxide, from a chemical compound. [NIH] Degenerative: Undergoing degeneration : tending to degenerate; having the character of or involving degeneration; causing or tending to cause degeneration. [EU] Dehydration: The condition that results from excessive loss of body water. [NIH] Deinstitutionalization: The practice of caring for individuals in the community, rather than in an institutional environment with resultant effects on the individual, the individual's family, the community, and the health care system. [NIH] Deletion: A genetic rearrangement through loss of segments of DNA (chromosomes), bringing sequences, which are normally separated, into close proximity. [NIH] Delirium: (DSM III-R) an acute, reversible organic mental disorder characterized by reduced ability to maintain attention to external stimuli and disorganized thinking as manifested by rambling, irrelevant, or incoherent speech; there are also a reduced level of consciousness, sensory misperceptions, disturbance of the sleep-wakefulness cycle and level of psychomotor activity, disorientation to time, place, or person, and memory impairment. Delirium may be caused by a large number of conditions resulting in derangement of cerebral metabolism, including systemic infection, poisoning, drug intoxication or withdrawal, seizures or head trauma, and metabolic disturbances such as hypoxia, hypoglycaemia, fluid, electrolyte, or acid-base imbalances, or hepatic or renal failure. Called also acute confusional state and acute brain syndrome. [EU] Delusions: A false belief regarding the self or persons or objects outside the self that persists despite the facts, and is not considered tenable by one's associates. [NIH] Dementia: An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness. [NIH] Demyelinating Diseases: Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system. [NIH] Dendrites: Extensions of the nerve cell body. They are short and branched and receive stimuli from other neurons. [NIH] Dendritic: 1. Branched like a tree. 2. Pertaining to or possessing dendrites. [EU] Density: The logarithm to the base 10 of the opacity of an exposed and processed film. [NIH] Dental Care: The total of dental diagnostic, preventive, and restorative services provided to

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meet the needs of a patient (from Illustrated Dictionary of Dentistry, 1982). [NIH] Dental Care for Children: The giving of attention to the special dental needs of children, including the prevention of tooth diseases and instruction in dental hygiene and dental health. The dental care may include the services provided by dental specialists. [NIH] Dental Caries: Localized destruction of the tooth surface initiated by decalcification of the enamel followed by enzymatic lysis of organic structures and leading to cavity formation. If left unchecked, the cavity may penetrate the enamel and dentin and reach the pulp. The three most prominent theories used to explain the etiology of the disase are that acids produced by bacteria lead to decalcification; that micro-organisms destroy the enamel protein; or that keratolytic micro-organisms produce chelates that lead to decalcification. [NIH]

Dental Plaque: A film that attaches to teeth, often causing dental caries and gingivitis. It is composed of mucins, secreted from salivary glands, and microorganisms. [NIH] Dental Staff: Personnel who provide dental service to patients in an organized facility, institution or agency. [NIH] Dentate Gyrus: Gray matter situated above the gyrus hippocampi. It is composed of three layers. The molecular layer is continuous with the hippocampus in the hippocampal fissure. The granular layer consists of closely arranged spherical or oval neurons, called granule cells, whose axons pass through the polymorphic layer ending on the dendrites of pyramidal cells in the hippocampus. [NIH] Dentists: Individuals licensed to practice dentistry. [NIH] Depolarization: The process or act of neutralizing polarity. In neurophysiology, the reversal of the resting potential in excitable cell membranes when stimulated, i.e., the tendency of the cell membrane potential to become positive with respect to the potential outside the cell. [EU] Depressive Disorder: An affective disorder manifested by either a dysphoric mood or loss of interest or pleasure in usual activities. The mood disturbance is prominent and relatively persistent. [NIH] Dermatitis: Any inflammation of the skin. [NIH] DES: Diethylstilbestrol. A synthetic hormone that was prescribed from the early 1940s until 1971 to help women with complications of pregnancy. DES has been linked to an increased risk of clear cell carcinoma of the vagina in daughters of women who used DES. DES may also increase the risk of breast cancer in women who used DES. [NIH] Desensitization: The prevention or reduction of immediate hypersensitivity reactions by administration of graded doses of allergen; called also hyposensitization and immunotherapy. [EU] Developed Countries: Countries that have reached a level of economic achievement through an increase of production, per capita income and consumption, and utilization of natural and human resources. [NIH] Dextroamphetamine: The d-form of amphetamine. It is a central nervous system stimulant and a sympathomimetic. It has also been used in the treatment of narcolepsy and of attention deficit disorders and hyperactivity in children. Dextroamphetamine has multiple mechanisms of action including blocking uptake of adrenergics and dopamine, stimulating release of monamines, and inhibiting monoamine oxidase. It is also a drug of abuse and a psychotomimetic. [NIH] Diabetes Mellitus: A heterogeneous group of disorders that share glucose intolerance in common. [NIH] Diagnostic procedure: A method used to identify a disease. [NIH]

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Diastolic: Of or pertaining to the diastole. [EU] Dichloroacetate: A derivative of acetic acid which increases the activity of pyruvate dehydrogenase and rate of lipogenesis. It is used in organic synthesis, pharmaceuticals, and medicine. [NIH] Digestion: The process of breakdown of food for metabolism and use by the body. [NIH] Digestive system: The organs that take in food and turn it into products that the body can use to stay healthy. Waste products the body cannot use leave the body through bowel movements. The digestive system includes the salivary glands, mouth, esophagus, stomach, liver, pancreas, gallbladder, small and large intestines, and rectum. [NIH] Dilatation: The act of dilating. [NIH] Dilation: A process by which the pupil is temporarily enlarged with special eye drops (mydriatic); allows the eye care specialist to better view the inside of the eye. [NIH] Dilator: A device used to stretch or enlarge an opening. [NIH] Dimerization: The process by which two molecules of the same chemical composition form a condensation product or polymer. [NIH] Diploid: Having two sets of chromosomes. [NIH] Direct: 1. Straight; in a straight line. 2. Performed immediately and without the intervention of subsidiary means. [EU] Discrimination: The act of qualitative and/or quantitative differentiation between two or more stimuli. [NIH] Discrimination Learning: Learning that is manifested in the ability to respond differentially to various stimuli. [NIH] Disease Progression: The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis. [NIH] Disease Susceptibility: A constitution or condition of the body which makes the tissues react in special ways to certain extrinsic stimuli and thus tends to make the individual more than usually susceptible to certain diseases. [NIH] Disorientation: The loss of proper bearings, or a state of mental confusion as to time, place, or identity. [EU] Dissection: Cutting up of an organism for study. [NIH] Dissociation: 1. The act of separating or state of being separated. 2. The separation of a molecule into two or more fragments (atoms, molecules, ions, or free radicals) produced by the absorption of light or thermal energy or by solvation. 3. In psychology, a defense mechanism in which a group of mental processes are segregated from the rest of a person's mental activity in order to avoid emotional distress, as in the dissociative disorders (q.v.), or in which an idea or object is segregated from its emotional significance; in the first sense it is roughly equivalent to splitting, in the second, to isolation. 4. A defect of mental integration in which one or more groups of mental processes become separated off from normal consciousness and, thus separated, function as a unitary whole. [EU] Distal: Remote; farther from any point of reference; opposed to proximal. In dentistry, used to designate a position on the dental arch farther from the median line of the jaw. [EU] Diuretic: A drug that increases the production of urine. [NIH] Dizziness: An imprecise term which may refer to a sense of spatial disorientation, motion of the environment, or lightheadedness. [NIH]

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Dominance: In genetics, the full phenotypic expression of a gene in both heterozygotes and homozygotes. [EU] Dopamine: An endogenous catecholamine and prominent neurotransmitter in several systems of the brain. In the synthesis of catecholamines from tyrosine, it is the immediate precursor to norepinephrine and epinephrine. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of dopaminergic receptor subtypes mediate its action. Dopamine is used pharmacologically for its direct (beta adrenergic agonist) and indirect (adrenergic releasing) sympathomimetic effects including its actions as an inotropic agent and as a renal vasodilator. [NIH] Dorsal: 1. Pertaining to the back or to any dorsum. 2. Denoting a position more toward the back surface than some other object of reference; same as posterior in human anatomy; superior in the anatomy of quadrupeds. [EU] Dorsum: A plate of bone which forms the posterior boundary of the sella turcica. [NIH] Dosage Forms: Completed forms of the pharmaceutical preparation in which prescribed doses of medication are included. They are designed to resist action by gastric fluids, prevent vomiting and nausea, reduce or alleviate the undesirable taste and smells associated with oral administration, achieve a high concentration of drug at target site, or produce a delayed or long-acting drug effect. They include capsules, liniments, ointments, pharmaceutical solutions, powders, tablets, etc. [NIH] Down syndrome: A disorder caused by the presence of an extra chromosome 21 and characterized by mental retardation and distinguishing physical features. [NIH] Drive: A state of internal activity of an organism that is a necessary condition before a given stimulus will elicit a class of responses; e.g., a certain level of hunger (drive) must be present before food will elicit an eating response. [NIH] Drug Design: The molecular designing of drugs for specific purposes (such as DNAbinding, enzyme inhibition, anti-cancer efficacy, etc.) based on knowledge of molecular properties such as activity of functional groups, molecular geometry, and electronic structure, and also on information cataloged on analogous molecules. Drug design is generally computer-assisted molecular modeling and does not include pharmacokinetics, dosage analysis, or drug administration analysis. [NIH] Drug Interactions: The action of a drug that may affect the activity, metabolism, or toxicity of another drug. [NIH] Duct: A tube through which body fluids pass. [NIH] Duodenum: The first part of the small intestine. [NIH] Dyes: Chemical substances that are used to stain and color other materials. The coloring may or may not be permanent. Dyes can also be used as therapeutic agents and test reagents in medicine and scientific research. [NIH] Dynein: A transport protein that normally binds proteins to the microtubule. [NIH] Dynorphins: A class of opioid peptides including dynorphin A, dynorphin B, and smaller fragments of these peptides. Dynorphins prefer kappa-opioid receptors (receptors, opioid, kappa) and have been shown to play a role as central nervous system transmitters. [NIH] Dyskinesia: Impairment of the power of voluntary movement, resulting in fragmentary or incomplete movements. [EU] Dyslexia: Partial alexia in which letters but not words may be read, or in which words may be read but not understood. [NIH] Dysplasia: Cells that look abnormal under a microscope but are not cancer. [NIH]

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Dystonia: Disordered tonicity of muscle. [EU] Dystrophin: A muscle protein localized in surface membranes which is the product of the Duchenne/Becker muscular dystrophy gene. Individuals with Duchenne muscular dystrophy usually lack dystrophin completely while those with Becker muscular dystrophy have dystrophin of an altered size. It shares features with other cytoskeletal proteins such as spectrin and alpha-actinin but the precise function of dystrophin is not clear. One possible role might be to preserve the integrity and alignment of the plasma membrane to the myofibrils during muscle contraction and relaxation. MW 400 kDa. [NIH] Dystrophy: Any disorder arising from defective or faulty nutrition, especially the muscular dystrophies. [EU] Effector: It is often an enzyme that converts an inactive precursor molecule into an active second messenger. [NIH] Effector cell: A cell that performs a specific function in response to a stimulus; usually used to describe cells in the immune system. [NIH] Efficacy: The extent to which a specific intervention, procedure, regimen, or service produces a beneficial result under ideal conditions. Ideally, the determination of efficacy is based on the results of a randomized control trial. [NIH] Elective: Subject to the choice or decision of the patient or physician; applied to procedures that are advantageous to the patient but not urgent. [EU] Electrolyte: A substance that dissociates into ions when fused or in solution, and thus becomes capable of conducting electricity; an ionic solute. [EU] Electrons: Stable elementary particles having the smallest known negative charge, present in all elements; also called negatrons. Positively charged electrons are called positrons. The numbers, energies and arrangement of electrons around atomic nuclei determine the chemical identities of elements. Beams of electrons are called cathode rays or beta rays, the latter being a high-energy biproduct of nuclear decay. [NIH] Electrophoresis: An electrochemical process in which macromolecules or colloidal particles with a net electric charge migrate in a solution under the influence of an electric current. [NIH]

Electrophysiological: Pertaining to electrophysiology, that is a branch of physiology that is concerned with the electric phenomena associated with living bodies and involved in their functional activity. [EU] Embryo: The prenatal stage of mammalian development characterized by rapid morphological changes and the differentiation of basic structures. [NIH] Emesis: Vomiting; an act of vomiting. Also used as a word termination, as in haematemesis. [EU]

Empirical: A treatment based on an assumed diagnosis, prior to receiving confirmatory laboratory test results. [NIH] Emulsion: A preparation of one liquid distributed in small globules throughout the body of a second liquid. The dispersed liquid is the discontinuous phase, and the dispersion medium is the continuous phase. When oil is the dispersed liquid and an aqueous solution is the continuous phase, it is known as an oil-in-water emulsion, whereas when water or aqueous solution is the dispersed phase and oil or oleaginous substance is the continuous phase, it is known as a water-in-oil emulsion. Pharmaceutical emulsions for which official standards have been promulgated include cod liver oil emulsion, cod liver oil emulsion with malt, liquid petrolatum emulsion, and phenolphthalein in liquid petrolatum emulsion. [EU] Enamel: A very hard whitish substance which covers the dentine of the anatomical crown of

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a tooth. [NIH] Encephalopathy: A disorder of the brain that can be caused by disease, injury, drugs, or chemicals. [NIH] Endarterectomy: Surgical excision, performed under general anesthesia, of the atheromatous tunica intima of an artery. When reconstruction of an artery is performed as an endovascular procedure through a catheter, it is called atherectomy. [NIH] Endocrine Glands: Ductless glands that secrete substances which are released directly into the circulation and which influence metabolism and other body functions. [NIH] Endocrine System: The system of glands that release their secretions (hormones) directly into the circulatory system. In addition to the endocrine glands, included are the chromaffin system and the neurosecretory systems. [NIH] Endocytosis: Cellular uptake of extracellular materials within membrane-limited vacuoles or microvesicles. Endosomes play a central role in endocytosis. [NIH] Endorphins: One of the three major groups of endogenous opioid peptides. They are large peptides derived from the pro-opiomelanocortin precursor. The known members of this group are alpha-, beta-, and gamma-endorphin. The term endorphin is also sometimes used to refer to all opioid peptides, but the narrower sense is used here; opioid peptides is used for the broader group. [NIH] Endothelium: A layer of epithelium that lines the heart, blood vessels (endothelium, vascular), lymph vessels (endothelium, lymphatic), and the serous cavities of the body. [NIH] Endothelium-derived: Small molecule that diffuses to the adjacent muscle layer and relaxes it. [NIH] Enkephalin: A natural opiate painkiller, in the hypothalamus. [NIH] Entorhinal Cortex: Cortex where the signals are combined with those from other sensory systems. [NIH] Enuresis: Involuntary discharge of urine after the age at which urinary control should have been achieved; often used alone with specific reference to involuntary discharge of urine occurring during sleep at night (bed-wetting, nocturnal enuresis). [EU] Environmental Health: The science of controlling or modifying those conditions, influences, or forces surrounding man which relate to promoting, establishing, and maintaining health. [NIH]

Enzymatic: Phase where enzyme cuts the precursor protein. [NIH] Enzyme: A protein that speeds up chemical reactions in the body. [NIH] Epidemiological: Relating to, or involving epidemiology. [EU] Epinephrine: The active sympathomimetic hormone from the adrenal medulla in most species. It stimulates both the alpha- and beta- adrenergic systems, causes systemic vasoconstriction and gastrointestinal relaxation, stimulates the heart, and dilates bronchi and cerebral vessels. It is used in asthma and cardiac failure and to delay absorption of local anesthetics. [NIH] Epithelial: Refers to the cells that line the internal and external surfaces of the body. [NIH] Epithelial Cells: Cells that line the inner and outer surfaces of the body. [NIH] Epithelium: One or more layers of epithelial cells, supported by the basal lamina, which covers the inner or outer surfaces of the body. [NIH] Ergot: Cataract due to ergot poisoning caused by eating of rye cereals contaminated by a fungus. [NIH]

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Erythritol: A four-carbon sugar that is found in algae, fungi, and lichens. It is twice as sweet as sucrose and can be used as a coronary vasodilator. [NIH] Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing hemoglobin whose function is to transport oxygen. [NIH] Esophagus: The muscular tube through which food passes from the throat to the stomach. [NIH]

Ether: One of a class of organic compounds in which any two organic radicals are attached directly to a single oxygen atom. [NIH] Eukaryotic Cells: Cells of the higher organisms, containing a true nucleus bounded by a nuclear membrane. [NIH] Evoke: The electric response recorded from the cerebral cortex after stimulation of a peripheral sense organ. [NIH] Excipient: Any more or less inert substance added to a prescription in order to confer a suitable consistency or form to the drug; a vehicle. [EU] Excitation: An act of irritation or stimulation or of responding to a stimulus; the addition of energy, as the excitation of a molecule by absorption of photons. [EU] Excitatory: When cortical neurons are excited, their output increases and each new input they receive while they are still excited raises their output markedly. [NIH] Excitatory Amino Acid Agonists: Drugs that bind to and activate excitatory amino acid receptors. [NIH] Excrete: To get rid of waste from the body. [NIH] Exogenous: Developed or originating outside the organism, as exogenous disease. [EU] Exon: The part of the DNA that encodes the information for the actual amino acid sequence of the protein. In many eucaryotic genes, the coding sequences consist of a series of exons alternating with intron sequences. [NIH] Extensor: A muscle whose contraction tends to straighten a limb; the antagonist of a flexor. [NIH]

Extracellular: Outside a cell or cells. [EU] Extracellular Matrix: A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. [NIH] Extracellular Space: Interstitial space between cells, occupied by fluid as well as amorphous and fibrous substances. [NIH] Extrapyramidal: Outside of the pyramidal tracts. [EU] Eye Infections: Infection, moderate to severe, caused by bacteria, fungi, or viruses, which occurs either on the external surface of the eye or intraocularly with probable inflammation, visual impairment, or blindness. [NIH] Eye Movements: Voluntary or reflex-controlled movements of the eye. [NIH] Facial: Of or pertaining to the face. [EU] Facial Nerve: The 7th cranial nerve. The facial nerve has two parts, the larger motor root which may be called the facial nerve proper, and the smaller intermediate or sensory root. Together they provide efferent innervation to the muscles of facial expression and to the lacrimal and salivary glands, and convey afferent information for taste from the anterior two-thirds of the tongue and for touch from the external ear. [NIH]

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Family Planning: Programs or services designed to assist the family in controlling reproduction by either improving or diminishing fertility. [NIH] Family Relations: Behavioral, psychological, and social relations among various members of the nuclear family and the extended family. [NIH] Fasciculation: A small local contraction of muscles, visible through the skin, representing a spontaneous discharge of a number of fibres innervated by a single motor nerve filament. [EU]

Fat: Total lipids including phospholipids. [NIH] Fathers: Male parents, human or animal. [NIH] Fatigue: The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. [NIH]

Fatty acids: A major component of fats that are used by the body for energy and tissue development. [NIH] Fetal Alcohol Syndrome: A disorder occurring in children born to alcoholic women who continue to drink heavily during pregnancy. Common abnormalities are growth deficiency (prenatal and postnatal), altered morphogenesis, mental deficiency, and characteristic facies - small eyes and flattened nasal bridge. Fine motor dysfunction and tremulousness are observed in the newborn. [NIH] Fetal Death: Death of the young developing in utero. [NIH] Fetus: The developing offspring from 7 to 8 weeks after conception until birth. [NIH] Fibrin: A protein derived from fibrinogen in the presence of thrombin, which forms part of the blood clot. [NIH] Fibrinogen: Plasma glycoprotein clotted by thrombin, composed of a dimer of three nonidentical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products. [NIH] Fibroblasts: Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. [NIH] Fibrosis: Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. [NIH] Fissure: Any cleft or groove, normal or otherwise; especially a deep fold in the cerebral cortex which involves the entire thickness of the brain wall. [EU] Fixation: 1. The act or operation of holding, suturing, or fastening in a fixed position. 2. The condition of being held in a fixed position. 3. In psychiatry, a term with two related but distinct meanings : (1) arrest of development at a particular stage, which like regression (return to an earlier stage), if temporary is a normal reaction to setbacks and difficulties but if protracted or frequent is a cause of developmental failures and emotional problems, and (2) a close and suffocating attachment to another person, especially a childhood figure, such as one's mother or father. Both meanings are derived from psychoanalytic theory and refer to 'fixation' of libidinal energy either in a specific erogenous zone, hence fixation at the oral, anal, or phallic stage, or in a specific object, hence mother or father fixation. 4. The use of a fixative (q.v.) to preserve histological or cytological specimens. 5. In chemistry, the process whereby a substance is removed from the gaseous or solution phase and localized, as in carbon dioxide fixation or nitrogen fixation. 6. In ophthalmology, direction of the gaze so that the visual image of the object falls on the fovea centralis. 7. In film processing, the

272 Mental Retardation

chemical removal of all undeveloped salts of the film emulsion, leaving only the developed silver to form a permanent image. [EU] Fluorescence: The property of emitting radiation while being irradiated. The radiation emitted is usually of longer wavelength than that incident or absorbed, e.g., a substance can be irradiated with invisible radiation and emit visible light. X-ray fluorescence is used in diagnosis. [NIH] Folate: A B-complex vitamin that is being studied as a cancer prevention agent. Also called folic acid. [NIH] Fold: A plication or doubling of various parts of the body. [NIH] Folic Acid: N-(4-(((2-Amino-1,4-dihydro-4-oxo-6-pteridinyl)methyl)amino)benzoyl)-Lglutamic acid. A member of the vitamin B family that stimulates the hematopoietic system. It is present in the liver and kidney and is found in mushrooms, spinach, yeast, green leaves, and grasses. Folic acid is used in the treatment and prevention of folate deficiencies and megaloblastic anemia. [NIH] Forearm: The part between the elbow and the wrist. [NIH] Fossa: A cavity, depression, or pit. [NIH] Fovea: The central part of the macula that provides the sharpest vision. [NIH] Frameshift: A type of mutation which causes out-of-phase transcription of the base sequence; such mutations arise from the addition or delection of nucleotide(s) in numbers other than 3 or multiples of 3. [NIH] Frameshift Mutation: A type of mutation in which a number of nucleotides not divisible by three is deleted from or inserted into a coding sequence, thereby causing an alteration in the reading frame of the entire sequence downstream of the mutation. These mutations may be induced by certain types of mutagens or may occur spontaneously. [NIH] Fungi: A kingdom of eukaryotic, heterotrophic organisms that live as saprobes or parasites, including mushrooms, yeasts, smuts, molds, etc. They reproduce either sexually or asexually, and have life cycles that range from simple to complex. Filamentous fungi refer to those that grow as multicelluar colonies (mushrooms and molds). [NIH] Gallbladder: The pear-shaped organ that sits below the liver. Bile is concentrated and stored in the gallbladder. [NIH] Ganglia: Clusters of multipolar neurons surrounded by a capsule of loosely organized connective tissue located outside the central nervous system. [NIH] Ganglioside: Protein kinase C's inhibitor which reduces ischemia-related brain damage. [NIH]

Gap Junctions: Connections between cells which allow passage of small molecules and electric current. Gap junctions were first described anatomically as regions of close apposition between cells with a narrow (1-2 nm) gap between cell membranes. The variety in the properties of gap junctions is reflected in the number of connexins, the family of proteins which form the junctions. [NIH] Gas: Air that comes from normal breakdown of food. The gases are passed out of the body through the rectum (flatus) or the mouth (burp). [NIH] Gastric: Having to do with the stomach. [NIH] Gastrin: A hormone released after eating. Gastrin causes the stomach to produce more acid. [NIH]

Gastrointestinal: Refers to the stomach and intestines. [NIH] Gastrointestinal tract: The stomach and intestines. [NIH]

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Gelatin: A product formed from skin, white connective tissue, or bone collagen. It is used as a protein food adjuvant, plasma substitute, hemostatic, suspending agent in pharmaceutical preparations, and in the manufacturing of capsules and suppositories. [NIH] Gene: The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein. [NIH]

Gene Deletion: A genetic rearrangement through loss of segments of DNA or RNA, bringing sequences which are normally separated into close proximity. This deletion may be detected using cytogenetic techniques and can also be inferred from the phenotype, indicating a deletion at one specific locus. [NIH] Gene Expression: The phenotypic manifestation of a gene or genes by the processes of gene action. [NIH] Genetic Code: The specifications for how information, stored in nucleic acid sequence (base sequence), is translated into protein sequence (amino acid sequence). The start, stop, and order of amino acids of a protein is specified by consecutive triplets of nucleotides called codons (codon). [NIH] Genetic Counseling: Advising families of the risks involved pertaining to birth defects, in order that they may make an informed decision on current or future pregnancies. [NIH] Genetic Engineering: Directed modification of the gene complement of a living organism by such techniques as altering the DNA, substituting genetic material by means of a virus, transplanting whole nuclei, transplanting cell hybrids, etc. [NIH] Genetic Techniques: Chromosomal, biochemical, intracellular, and other methods used in the study of genetics. [NIH] Genetic testing: Analyzing DNA to look for a genetic alteration that may indicate an increased risk for developing a specific disease or disorder. [NIH] Genetic transcription: The process by which the genetic information encoded in the gene, represented as a linear sequence of deoxyribonucleotides, is copied into an exactly complementary sequence of ribonucleotides known as messenger RNA. [NIH] Genetics: The biological science that deals with the phenomena and mechanisms of heredity. [NIH] Genital: Pertaining to the genitalia. [EU] Genitourinary: Pertaining to the genital and urinary organs; urogenital; urinosexual. [EU] Genomics: The systematic study of the complete DNA sequences (genome) of organisms. [NIH]

Genotype: The genetic constitution of the individual; the characterization of the genes. [NIH] Germ Cells: The reproductive cells in multicellular organisms. [NIH] Gestation: The period of development of the young in viviparous animals, from the time of fertilization of the ovum until birth. [EU] Gestational: Psychosis attributable to or occurring during pregnancy. [NIH] Gestational Age: Age of the conceptus. In humans, this may be assessed by medical history, physical examination, early immunologic pregnancy tests, radiography, ultrasonography, and amniotic fluid analysis. [NIH] Gland: An organ that produces and releases one or more substances for use in the body. Some glands produce fluids that affect tissues or organs. Others produce hormones or participate in blood production. [NIH] Glucose: D-Glucose. A primary source of energy for living organisms. It is naturally

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occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. [NIH] Glucose Intolerance: A pathological state in which the fasting plasma glucose level is less than 140 mg per deciliter and the 30-, 60-, or 90-minute plasma glucose concentration following a glucose tolerance test exceeds 200 mg per deciliter. This condition is seen frequently in diabetes mellitus but also occurs with other diseases. [NIH] Glutamate: Excitatory neurotransmitter of the brain. [NIH] Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid (glutamate) is the most common excitatory neurotransmitter in the central nervous system. [NIH]

Glutamine: A non-essential amino acid present abundantly throught the body and is involved in many metabolic processes. It is synthesized from glutamic acid and ammonia. It is the principal carrier of nitrogen in the body and is an important energy source for many cells. [NIH] Glycerol: A trihydroxy sugar alcohol that is an intermediate in carbohydrate and lipid metabolism. It is used as a solvent, emollient, pharmaceutical agent, and sweetening agent. [NIH]

Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter. [NIH] Glycoprotein: A protein that has sugar molecules attached to it. [NIH] Glycosaminoglycan: A type of long, unbranched polysaccharide molecule. Glycosaminoglycans are major structural components of cartilage and are also found in the cornea of the eye. [NIH] Glycosuria: The presence of glucose in the urine; especially the excretion of an abnormally large amount of sugar (glucose) in the urine, i.e., more than 1 gm. in 24 hours. [EU] Glycosylation: The chemical or biochemical addition of carbohydrate or glycosyl groups to other chemicals, especially peptides or proteins. Glycosyl transferases are used in this biochemical reaction. [NIH] Gonads: The gamete-producing glands, ovary or testis. [NIH] Governing Board: The group in which legal authority is vested for the control of healthrelated institutions and organizations. [NIH] Government Agencies: Administrative units of government responsible for policy making and management of governmental activities in the U.S. and abroad. [NIH] Grade: The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer. [NIH] Graft: Healthy skin, bone, or other tissue taken from one part of the body and used to replace diseased or injured tissue removed from another part of the body. [NIH] Granulocytes: Leukocytes with abundant granules in the cytoplasm. They are divided into three groups: neutrophils, eosinophils, and basophils. [NIH] Grasses: A large family, Gramineae, of narrow-leaved herbaceous monocots. Many grasses produce highly allergenic pollens and are hosts to cattle parasites and toxic fungi. [NIH] Group Homes: Housing for groups of patients, children, or others who need or desire emotional or physical support. They are usually established as planned, single housekeeping units in residential dwellings that provide care and supervision for small groups of residents, who, although unrelated, live together as a family. [NIH]

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Growth: The progressive development of a living being or part of an organism from its earliest stage to maturity. [NIH] Growth factors: Substances made by the body that function to regulate cell division and cell survival. Some growth factors are also produced in the laboratory and used in biological therapy. [NIH] Guanylate Cyclase: An enzyme that catalyzes the conversion of GTP to 3',5'-cyclic GMP and pyrophosphate. It also acts on ITP and dGTP. (From Enzyme Nomenclature, 1992) EC 4.6.1.2. [NIH] Gynaecomastia: Excessive development of the male mammary glands, even to the functional state. [EU] Hammer: The largest of the three ossicles of the ear. [NIH] Handicap: A handicap occurs as a result of disability, but disability does not always constitute a handicap. A handicap may be said to exist when a disability causes a substantial and continuing reduction in a person's capacity to function socially and vocationally. [NIH] Haptens: Small antigenic determinants capable of eliciting an immune response only when coupled to a carrier. Haptens bind to antibodies but by themselves cannot elicit an antibody response. [NIH] Headache: Pain in the cranial region that may occur as an isolated and benign symptom or as a manifestation of a wide variety of conditions including subarachnoid hemorrhage; craniocerebral trauma; central nervous system infections; intracranial hypertension; and other disorders. In general, recurrent headaches that are not associated with a primary disease process are referred to as headache disorders (e.g., migraine). [NIH] Health Behavior: Behaviors expressed by individuals to protect, maintain or promote their health status. For example, proper diet, and appropriate exercise are activities perceived to influence health status. Life style is closely associated with health behavior and factors influencing life style are socioeconomic, educational, and cultural. [NIH] Health Promotion: Encouraging consumer behaviors most likely to optimize health potentials (physical and psychosocial) through health information, preventive programs, and access to medical care. [NIH] Health Services: Services for the diagnosis and treatment of disease and the maintenance of health. [NIH] Health Status: The level of health of the individual, group, or population as subjectively assessed by the individual or by more objective measures. [NIH] Hearing aid: A miniature, portable sound amplifier for persons with impaired hearing, consisting of a microphone, audio amplifier, earphone, and battery. [NIH] Hearing Disorders: Conditions that impair the transmission or perception of auditory impulses and information from the level of the ear to the temporal cortices, including the sensorineural pathways. [NIH] Heart attack: A seizure of weak or abnormal functioning of the heart. [NIH] Heart failure: Loss of pumping ability by the heart, often accompanied by fatigue, breathlessness, and excess fluid accumulation in body tissues. [NIH] Heme: The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins. [NIH] Hemoglobin: One of the fractions of glycosylated hemoglobin A1c. Glycosylated hemoglobin is formed when linkages of glucose and related monosaccharides bind to hemoglobin A and its concentration represents the average blood glucose level over the

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previous several weeks. HbA1c levels are used as a measure of long-term control of plasma glucose (normal, 4 to 6 percent). In controlled diabetes mellitus, the concentration of glycosylated hemoglobin A is within the normal range, but in uncontrolled cases the level may be 3 to 4 times the normal conentration. Generally, complications are substantially lower among patients with Hb levels of 7 percent or less than in patients with HbA1c levels of 9 percent or more. [NIH] Hemolytic: A disease that affects the blood and blood vessels. It destroys red blood cells, cells that cause the blood to clot, and the lining of blood vessels. HUS is often caused by the Escherichia coli bacterium in contaminated food. People with HUS may develop acute renal failure. [NIH] Hemorrhage: Bleeding or escape of blood from a vessel. [NIH] Hemostasis: The process which spontaneously arrests the flow of blood from vessels carrying blood under pressure. It is accomplished by contraction of the vessels, adhesion and aggregation of formed blood elements, and the process of blood or plasma coagulation. [NIH]

Hepatic: Refers to the liver. [NIH] Hepatocyte: A liver cell. [NIH] Hereditary: Of, relating to, or denoting factors that can be transmitted genetically from one generation to another. [NIH] Heredity: 1. The genetic transmission of a particular quality or trait from parent to offspring. 2. The genetic constitution of an individual. [EU] Heterogeneity: The property of one or more samples or populations which implies that they are not identical in respect of some or all of their parameters, e. g. heterogeneity of variance. [NIH]

Hippocampus: A curved elevation of gray matter extending the entire length of the floor of the temporal horn of the lateral ventricle (Dorland, 28th ed). The hippocampus, subiculum, and dentate gyrus constitute the hippocampal formation. Sometimes authors include the entorhinal cortex in the hippocampal formation. [NIH] Histamine: 1H-Imidazole-4-ethanamine. A depressor amine derived by enzymatic decarboxylation of histidine. It is a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter. [NIH] Histidine: An essential amino acid important in a number of metabolic processes. It is required for the production of histamine. [NIH] Histology: The study of tissues and cells under a microscope. [NIH] Histone Deacetylase: Hydrolyzes N-acetyl groups on histones. [NIH] Homeostasis: The processes whereby the internal environment of an organism tends to remain balanced and stable. [NIH] Homeotic: Characterizes genes the mutations of which lead to inappropriate expressions of characteristics normally associated with another part of the organism (homeotic mutants). [NIH]

Homogeneous: Consisting of or composed of similar elements or ingredients; of a uniform quality throughout. [EU] Homologous: Corresponding in structure, position, origin, etc., as (a) the feathers of a bird and the scales of a fish, (b) antigen and its specific antibody, (c) allelic chromosomes. [EU] Homozygotes: An individual having a homozygous gene pair. [NIH] Hormonal: Pertaining to or of the nature of a hormone. [EU]

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Hormone: A substance in the body that regulates certain organs. Hormones such as gastrin help in breaking down food. Some hormones come from cells in the stomach and small intestine. [NIH] Host: Any animal that receives a transplanted graft. [NIH] Housekeeping: The care and management of property. [NIH] Human Development: Continuous sequential changes which occur in the physiological and psychological functions during the individual's life. [NIH] Hybrid: Cross fertilization between two varieties or, more usually, two species of vines, see also crossing. [NIH] Hybridization: The genetic process of crossbreeding to produce a hybrid. Hybrid nucleic acids can be formed by nucleic acid hybridization of DNA and RNA molecules. Protein hybridization allows for hybrid proteins to be formed from polypeptide chains. [NIH] Hydrocephalus: Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial hypertension; headache; lethargy; urinary incontinence; and ataxia (and in infants macrocephaly). This condition may be caused by obstruction of cerebrospinal fluid pathways due to neurologic abnormalities, intracranial hemorrhages; central nervous system infections; brain neoplasms; craniocerebral trauma; and other conditions. Impaired resorption of cerebrospinal fluid from the arachnoid villi results in a communicating form of hydrocephalus. Hydrocephalus ex-vacuo refers to ventricular dilation that occurs as a result of brain substance loss from cerebral infarction and other conditions. [NIH] Hydrogen: The first chemical element in the periodic table. It has the atomic symbol H, atomic number 1, and atomic weight 1. It exists, under normal conditions, as a colorless, odorless, tasteless, diatomic gas. Hydrogen ions are protons. Besides the common H1 isotope, hydrogen exists as the stable isotope deuterium and the unstable, radioactive isotope tritium. [NIH] Hydrolases: Any member of the class of enzymes that catalyze the cleavage of the substrate and the addition of water to the resulting molecules, e.g., esterases, glycosidases (glycoside hydrolases), lipases, nucleotidases, peptidases (peptide hydrolases), and phosphatases (phosphoric monoester hydrolases). EC 3. [NIH] Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water. [NIH] Hydrophilic: Readily absorbing moisture; hygroscopic; having strongly polar groups that readily interact with water. [EU] Hydrophobic: Not readily absorbing water, or being adversely affected by water, as a hydrophobic colloid. [EU] Hyperammonemia: Metabolic disorder characterized by elevated level of ammonia in blood. [NIH] Hypercalcemia: Abnormally high level of calcium in the blood. [NIH] Hyperhomocysteinemia: An inborn error of methionone metabolism which produces an excess of homocysteine in the blood. It is often caused by a deficiency of cystathionine betasynthase and is a risk factor for coronary vascular disease. [NIH] Hypersensitivity: Altered reactivity to an antigen, which can result in pathologic reactions upon subsequent exposure to that particular antigen. [NIH] Hypertension: Persistently high arterial blood pressure. Currently accepted threshold levels are 140 mm Hg systolic and 90 mm Hg diastolic pressure. [NIH]

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Hypertrophic cardiomyopathy: Heart muscle disease that leads to thickening of the heart walls, interfering with the heart's ability to fill with and pump blood. [NIH] Hypertrophy: General increase in bulk of a part or organ, not due to tumor formation, nor to an increase in the number of cells. [NIH] Hypoglycaemia: An abnormally diminished concentration of glucose in the blood, which may lead to tremulousness, cold sweat, piloerection, hypothermia, and headache, accompanied by irritability, confusion, hallucinations, bizarre behaviour, and ultimately, convulsions and coma. [EU] Hypogonadism: Condition resulting from or characterized by abnormally decreased functional activity of the gonads, with retardation of growth and sexual development. [NIH] Hypoplasia: Incomplete development or underdevelopment of an organ or tissue. [EU] Hypotension: Abnormally low blood pressure. [NIH] Hypothalamus: Ventral part of the diencephalon extending from the region of the optic chiasm to the caudal border of the mammillary bodies and forming the inferior and lateral walls of the third ventricle. [NIH] Hypothyroidism: Deficiency of thyroid activity. In adults, it is most common in women and is characterized by decrease in basal metabolic rate, tiredness and lethargy, sensitivity to cold, and menstrual disturbances. If untreated, it progresses to full-blown myxoedema. In infants, severe hypothyroidism leads to cretinism. In juveniles, the manifestations are intermediate, with less severe mental and developmental retardation and only mild symptoms of the adult form. When due to pituitary deficiency of thyrotropin secretion it is called secondary hypothyroidism. [EU] Hypotonia: A condition of diminished tone of the skeletal muscles; diminished resistance of muscles to passive stretching. [EU] Hypoxia: Reduction of oxygen supply to tissue below physiological levels despite adequate perfusion of the tissue by blood. [EU] Id: The part of the personality structure which harbors the unconscious instinctive desires and strivings of the individual. [NIH] Idiopathic: Describes a disease of unknown cause. [NIH] Ileum: The lower end of the small intestine. [NIH] Immune response: The activity of the immune system against foreign substances (antigens). [NIH]

Immune system: The organs, cells, and molecules responsible for the recognition and disposal of foreign ("non-self") material which enters the body. [NIH] Immunodeficiency: The decreased ability of the body to fight infection and disease. [NIH] Immunodeficiency syndrome: The inability of the body to produce an immune response. [NIH]

Immunoglobulin: A protein that acts as an antibody. [NIH] Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents. [NIH] Immunologic: The ability of the antibody-forming system to recall a previous experience with an antigen and to respond to a second exposure with the prompt production of large amounts of antibody. [NIH] Immunology: The study of the body's immune system. [NIH] Immunotherapy: Manipulation of the host's immune system in treatment of disease. It

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includes both active and passive immunization as well as immunosuppressive therapy to prevent graft rejection. [NIH] Impairment: In the context of health experience, an impairment is any loss or abnormality of psychological, physiological, or anatomical structure or function. [NIH] In situ: In the natural or normal place; confined to the site of origin without invasion of neighbouring tissues. [EU] In Situ Hybridization: A technique that localizes specific nucleic acid sequences within intact chromosomes, eukaryotic cells, or bacterial cells through the use of specific nucleic acid-labeled probes. [NIH] In vitro: In the laboratory (outside the body). The opposite of in vivo (in the body). [NIH] In vivo: In the body. The opposite of in vitro (outside the body or in the laboratory). [NIH] Incontinence: Inability to control the flow of urine from the bladder (urinary incontinence) or the escape of stool from the rectum (fecal incontinence). [NIH] Indicative: That indicates; that points out more or less exactly; that reveals fairly clearly. [EU] Induction: The act or process of inducing or causing to occur, especially the production of a specific morphogenetic effect in the developing embryo through the influence of evocators or organizers, or the production of anaesthesia or unconsciousness by use of appropriate agents. [EU] Infancy: The period of complete dependency prior to the acquisition of competence in walking, talking, and self-feeding. [NIH] Infant, Newborn: An infant during the first month after birth. [NIH] Infantile: Pertaining to an infant or to infancy. [EU] Infarction: A pathological process consisting of a sudden insufficient blood supply to an area, which results in necrosis of that area. It is usually caused by a thrombus, an embolus, or a vascular torsion. [NIH] Infection: 1. Invasion and multiplication of microorganisms in body tissues, which may be clinically unapparent or result in local cellular injury due to competitive metabolism, toxins, intracellular replication, or antigen-antibody response. The infection may remain localized, subclinical, and temporary if the body's defensive mechanisms are effective. A local infection may persist and spread by extension to become an acute, subacute, or chronic clinical infection or disease state. A local infection may also become systemic when the microorganisms gain access to the lymphatic or vascular system. 2. An infectious disease. [EU]

Infertility: The diminished or absent ability to conceive or produce an offspring while sterility is the complete inability to conceive or produce an offspring. [NIH] Inflammation: A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. [NIH] Informed Consent: Voluntary authorization, given to the physician by the patient, with full comprehension of the risks involved, for diagnostic or investigative procedures and medical and surgical treatment. [NIH] Ingestion: Taking into the body by mouth [NIH] Inhalation: The drawing of air or other substances into the lungs. [EU] Initiation: Mutation induced by a chemical reactive substance causing cell changes; being a step in a carcinogenic process. [NIH]

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Inner ear: The labyrinth, comprising the vestibule, cochlea, and semicircular canals. [NIH] Innervation: 1. The distribution or supply of nerves to a part. 2. The supply of nervous energy or of nerve stimulus sent to a part. [EU] Inositol: An isomer of glucose that has traditionally been considered to be a B vitamin although it has an uncertain status as a vitamin and a deficiency syndrome has not been identified in man. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1379) Inositol phospholipids are important in signal transduction. [NIH] Inotropic: Affecting the force or energy of muscular contractions. [EU] Insight: The capacity to understand one's own motives, to be aware of one's own psychodynamics, to appreciate the meaning of symbolic behavior. [NIH] Insomnia: Difficulty in going to sleep or getting enough sleep. [NIH] Institutionalization: The caring for individuals in institutions and their adaptation to routines characteristic of the institutional environment, and/or their loss of adaptation to life outside the institution. [NIH] Insulator: Material covering the metal conductor of the lead. It is usually polyurethane or silicone. [NIH] Insulin: A protein hormone secreted by beta cells of the pancreas. Insulin plays a major role in the regulation of glucose metabolism, generally promoting the cellular utilization of glucose. It is also an important regulator of protein and lipid metabolism. Insulin is used as a drug to control insulin-dependent diabetes mellitus. [NIH] Intention tremor: A tremor which arises or which is intensified when a voluntary, coordinated movement is attempted. [EU] Intercellular Junctions: Strictly, and so far as it can be distinguished, the amorphous isotropic layer between adjacent primary walls of cells. [NIH] Intermittent: Occurring at separated intervals; having periods of cessation of activity. [EU] Interstitial: Pertaining to or situated between parts or in the interspaces of a tissue. [EU] Intestinal: Having to do with the intestines. [NIH] Intestine: A long, tube-shaped organ in the abdomen that completes the process of digestion. There is both a large intestine and a small intestine. Also called the bowel. [NIH] Intoxication: Poisoning, the state of being poisoned. [EU] Intracellular: Inside a cell. [NIH] Intracellular Membranes: Membranes of subcellular structures. [NIH] Intracranial Hemorrhages: Bleeding within the intracranial cavity, including hemorrhages in the brain and within the cranial epidural, subdural, and subarachnoid spaces. [NIH] Intracranial Hypertension: Increased pressure within the cranial vault. This may result from several conditions, including hydrocephalus; brain edema; intracranial masses; severe systemic hypertension; pseudotumor cerebri; and other disorders. [NIH] Intracranial Pressure: Pressure within the cranial cavity. It is influenced by brain mass, the circulatory system, CSF dynamics, and skull rigidity. [NIH] Intramuscular: IM. Within or into muscle. [NIH] Intraocular: Within the eye. [EU] Intravenous: IV. Into a vein. [NIH] Intrinsic: Situated entirely within or pertaining exclusively to a part. [EU] Invasive: 1. Having the quality of invasiveness. 2. Involving puncture or incision of the skin

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or insertion of an instrument or foreign material into the body; said of diagnostic techniques. [EU]

Involuntary: Reaction occurring without intention or volition. [NIH] Ion Channels: Gated, ion-selective glycoproteins that traverse membranes. The stimulus for channel gating can be a membrane potential, drug, transmitter, cytoplasmic messenger, or a mechanical deformation. Ion channels which are integral parts of ionotropic neurotransmitter receptors are not included. [NIH] Ions: An atom or group of atoms that have a positive or negative electric charge due to a gain (negative charge) or loss (positive charge) of one or more electrons. Atoms with a positive charge are known as cations; those with a negative charge are anions. [NIH] Iris: The most anterior portion of the uveal layer, separating the anterior chamber from the posterior. It consists of two layers - the stroma and the pigmented epithelium. Color of the iris depends on the amount of melanin in the stroma on reflection from the pigmented epithelium. [NIH] Ischemia: Deficiency of blood in a part, due to functional constriction or actual obstruction of a blood vessel. [EU] Ischemic stroke: A condition in which the blood supply to part of the brain is cut off. Also called "plug-type" strokes. Blocked arteries starve areas of the brain controlling sight, speech, sensation, and movement so that these functions are partially or completely lost. Ischemic stroke is the most common type of stroke, accounting for 80 percent of all strokes. Most ischemic strokes are caused by a blood clot called a thrombus, which blocks blood flow in the arteries feeding the brain, usually the carotid artery in the neck, the major vessel bringing blood to the brain. When it becomes blocked, the risk of stroke is very high. [NIH] Isoprenoid: Molecule that might anchor G protein to the cell membrane as it is hydrophobic. [NIH]

Kainate: Glutamate receptor. [NIH] Kainic Acid: (2S-(2 alpha,3 beta,4 beta))-2-Carboxy-4-(1-methylethenyl)-3-pyrrolidineacetic acid. Ascaricide obtained from the red alga Digenea simplex. It is a potent excitatory amino acid agonist at some types of excitatory amino acid receptors and has been used to discriminate among receptor types. Like many excitatory amino acid agonists it can cause neurotoxicity and has been used experimentally for that purpose. [NIH] Karyotype: The characteristic chromosome complement of an individual, race, or species as defined by their number, size, shape, etc. [NIH] Kb: A measure of the length of DNA fragments, 1 Kb = 1000 base pairs. The largest DNA fragments are up to 50 kilobases long. [NIH] Keratolytic: An agent that promotes keratolysis. [EU] Keto: It consists of 8 carbon atoms and within the endotoxins, it connects poysaccharide and lipid A. [NIH] Kinesin: A microtubule-associated mechanical adenosine triphosphatase, that uses the energy of ATP hydrolysis to move organelles along microtubules toward the plus end of the microtubule. The protein is found in squid axoplasm, optic lobes, and in bovine brain. Bovine kinesin is a heterotetramer composed of two heavy (120 kDa) and two light (62 kDa) chains. EC 3.6.1.-. [NIH] Labyrinth: The internal ear; the essential part of the organ of hearing. It consists of an osseous and a membranous portion. [NIH] Lacrimal: Pertaining to the tears. [EU] Language Development: The gradual expansion in complexity and meaning of symbols and

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sounds as perceived and interpreted by the individual through a maturational and learning process. Stages in development include babbling, cooing, word imitation with cognition, and use of short sentences. [NIH] Language Disorders: Conditions characterized by deficiencies of comprehension or expression of written and spoken forms of language. These include acquired and developmental disorders. [NIH] Large Intestine: The part of the intestine that goes from the cecum to the rectum. The large intestine absorbs water from stool and changes it from a liquid to a solid form. The large intestine is 5 feet long and includes the appendix, cecum, colon, and rectum. Also called colon. [NIH] Latent: Phoria which occurs at one distance or another and which usually has no troublesome effect. [NIH] Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot. [NIH] Laxative: An agent that acts to promote evacuation of the bowel; a cathartic or purgative. [EU]

Learning Disorders: Conditions characterized by a significant discrepancy between an individual's perceived level of intellect and their ability to acquire new language and other cognitive skills. These disorders may result from organic or psychological conditions. Relatively common subtypes include dyslexia, dyscalculia, and dysgraphia. [NIH] Lectin: A complex molecule that has both protein and sugars. Lectins are able to bind to the outside of a cell and cause biochemical changes in it. Lectins are made by both animals and plants. [NIH] Length of Stay: The period of confinement of a patient to a hospital or other health facility. [NIH]

Lens: The transparent, double convex (outward curve on both sides) structure suspended between the aqueous and vitreous; helps to focus light on the retina. [NIH] Lesion: An area of abnormal tissue change. [NIH] Lethal: Deadly, fatal. [EU] Lethargy: Abnormal drowsiness or stupor; a condition of indifference. [EU] Leucine: An essential branched-chain amino acid important for hemoglobin formation. [NIH] Leucocyte: All the white cells of the blood and their precursors (myeloid cell series, lymphoid cell series) but commonly used to indicate granulocytes exclusive of lymphocytes. [NIH]

Leukemia: Cancer of blood-forming tissue. [NIH] Leukotrienes: A family of biologically active compounds derived from arachidonic acid by oxidative metabolism through the 5-lipoxygenase pathway. They participate in host defense reactions and pathophysiological conditions such as immediate hypersensitivity and inflammation. They have potent actions on many essential organs and systems, including the cardiovascular, pulmonary, and central nervous system as well as the gastrointestinal tract and the immune system. [NIH] Library Services: Services offered to the library user. They include reference and circulation. [NIH]

Lichens: Any of a group of plants formed by a mutual combination of an alga and a fungus. [NIH]

Life cycle: The successive stages through which an organism passes from fertilized ovum or

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spore to the fertilized ovum or spore of the next generation. [NIH] Life Expectancy: A figure representing the number of years, based on known statistics, to which any person of a given age may reasonably expect to live. [NIH] Ligament: A band of fibrous tissue that connects bones or cartilages, serving to support and strengthen joints. [EU] Ligands: A RNA simulation method developed by the MIT. [NIH] Ligase: An enzyme that repairs single stranded discontinuities in double-stranded DNA molecules in the cell. Purified DNA ligase is used in gene cloning to join DNA molecules together. [NIH] Linear Models: Statistical models in which the value of a parameter for a given value of a factor is assumed to be equal to a + bx, where a and b are constants. The models predict a linear regression. [NIH] Linkage: The tendency of two or more genes in the same chromosome to remain together from one generation to the next more frequently than expected according to the law of independent assortment. [NIH] Lip: Either of the two fleshy, full-blooded margins of the mouth. [NIH] Lipid: Fat. [NIH] Lipodystrophy: A collection of rare conditions resulting from defective fat metabolism and characterized by atrophy of the subcutaneous fat. They include total, congenital or acquired, partial, abdominal infantile, and localized lipodystrophy. [NIH] Lipoxygenase: An enzyme of the oxidoreductase class that catalyzes reactions between linoleate and other fatty acids and oxygen to form hydroperoxy-fatty acid derivatives. Related enzymes in this class include the arachidonate lipoxygenases, arachidonate 5lipoxygenase, arachidonate 12-lipoxygenase, and arachidonate 15-lipoxygenase. EC 1.13.11.12. [NIH] Lithium: An element in the alkali metals family. It has the atomic symbol Li, atomic number 3, and atomic weight 6.94. Salts of lithium are used in treating manic-depressive disorders. [NIH]

Liver: A large, glandular organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile. [NIH] Liver scan: An image of the liver created on a computer screen or on film. A radioactive substance is injected into a blood vessel and travels through the bloodstream. It collects in the liver, especially in abnormal areas, and can be detected by the scanner. [NIH] Lobe: A portion of an organ such as the liver, lung, breast, or brain. [NIH] Localization: The process of determining or marking the location or site of a lesion or disease. May also refer to the process of keeping a lesion or disease in a specific location or site. [NIH] Localized: Cancer which has not metastasized yet. [NIH] Longitudinal study: Also referred to as a "cohort study" or "prospective study"; the analytic method of epidemiologic study in which subsets of a defined population can be identified who are, have been, or in the future may be exposed or not exposed, or exposed in different degrees, to a factor or factors hypothesized to influence the probability of occurrence of a given disease or other outcome. The main feature of this type of study is to observe large numbers of subjects over an extended time, with comparisons of incidence rates in groups that differ in exposure levels. [NIH] Long-Term Care: Care over an extended period, usually for a chronic condition or disability,

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requiring periodic, intermittent, or continuous care. [NIH] Loop: A wire usually of platinum bent at one end into a small loop (usually 4 mm inside diameter) and used in transferring microorganisms. [NIH] Lymph: The almost colorless fluid that travels through the lymphatic system and carries cells that help fight infection and disease. [NIH] Lymphatic: The tissues and organs, including the bone marrow, spleen, thymus, and lymph nodes, that produce and store cells that fight infection and disease. [NIH] Lymphoblasts: Interferon produced predominantly by leucocyte cells. [NIH] Lymphocyte: A white blood cell. Lymphocytes have a number of roles in the immune system, including the production of antibodies and other substances that fight infection and diseases. [NIH] Lymphocytic: Referring to lymphocytes, a type of white blood cell. [NIH] Lymphoid: Referring to lymphocytes, a type of white blood cell. Also refers to tissue in which lymphocytes develop. [NIH] Lysosomal Storage Diseases: Inborn errors of metabolism characterized by defects in specific lysosomal hydrolases and resulting in intracellular accumulation of unmetabolized substrates. [NIH] Lytic: 1. Pertaining to lysis or to a lysin. 2. Producing lysis. [EU] Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. [NIH] Malformation: A morphologic developmental process. [EU]

defect

resulting

from

an

intrinsically

abnormal

Malignant: Cancerous; a growth with a tendency to invade and destroy nearby tissue and spread to other parts of the body. [NIH] Malnutrition: A condition caused by not eating enough food or not eating a balanced diet. [NIH]

Mammary: Pertaining to the mamma, or breast. [EU] Mammogram: An x-ray of the breast. [NIH] Mania: Excitement of psychotic proportions manifested by mental and physical hyperactivity, disorganization of behaviour, and elevation of mood. [EU] Manic: Affected with mania. [EU] Manic-depressive psychosis: One of a group of psychotic reactions, fundamentally marked by severe mood swings and a tendency to remission and recurrence. [NIH] Manifest: Being the part or aspect of a phenomenon that is directly observable : concretely expressed in behaviour. [EU] Maternal Behavior: The behavior patterns associated with or characteristic of a mother. [NIH]

Medial: Lying near the midsaggital plane of the body; opposed to lateral. [NIH] Mediate: Indirect; accomplished by the aid of an intervening medium. [EU] Mediator: An object or substance by which something is mediated, such as (1) a structure of the nervous system that transmits impulses eliciting a specific response; (2) a chemical substance (transmitter substance) that induces activity in an excitable tissue, such as nerve or muscle; or (3) a substance released from cells as the result of the interaction of antigen

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with antibody or by the action of antigen with a sensitized lymphocyte. [EU] Medicament: A medicinal substance or agent. [EU] MEDLINE: An online database of MEDLARS, the computerized bibliographic Medical Literature Analysis and Retrieval System of the National Library of Medicine. [NIH] Megalencephaly: A condition in which there is an abnormally large, heavy, and usually malfunctioning brain. [NIH] Megaloblastic: A large abnormal red blood cell appearing in the blood in pernicious anaemia. [EU] Meiosis: A special method of cell division, occurring in maturation of the germ cells, by means of which each daughter nucleus receives half the number of chromosomes characteristic of the somatic cells of the species. [NIH] Melanin: The substance that gives the skin its color. [NIH] Melanoblasts: Cell originating from the neural crest that differentiates into a melanocyte. [NIH]

Memantine: Amantadine derivative that has some dopaminergic effects. It has been proposed as an antiparkinson agent. [NIH] Membrane: A very thin layer of tissue that covers a surface. [NIH] Membrane Glycoproteins: Glycoproteins found on the membrane or surface of cells. [NIH] Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors. [NIH] Memory: Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. [NIH] Meninges: The three membranes that cover and protect the brain and spinal cord. [NIH] Menopause: Permanent cessation of menstruation. [NIH] Menstruation: The normal physiologic discharge through the vagina of blood and mucosal tissues from the nonpregnant uterus. [NIH] Mental deficiency: A condition of arrested or incomplete development of mind from inherent causes or induced by disease or injury. [NIH] Mental Disorders: Psychiatric illness or diseases manifested by breakdowns in the adaptational process expressed primarily as abnormalities of thought, feeling, and behavior producing either distress or impairment of function. [NIH] Mental Health: The state wherein the person is well adjusted. [NIH] Mental Processes: Conceptual functions or thinking in all its forms. [NIH] Mental Retardation: Refers to sub-average general intellectual functioning which originated during the developmental period and is associated with impairment in adaptive behavior. [NIH]

Mesolimbic: Inner brain region governing emotion and drives. [NIH] Metabolic disorder: A condition in which normal metabolic processes are disrupted, usually because of a missing enzyme. [NIH] Metabotropic: A glutamate receptor which triggers an increase in production of 2 intracellular messengers: diacylglycerol and inositol 1, 4, 5-triphosphate. [NIH]

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Metastasis: The spread of cancer from one part of the body to another. Tumors formed from cells that have spread are called "secondary tumors" and contain cells that are like those in the original (primary) tumor. The plural is metastases. [NIH] Methylphenidate: A central nervous system stimulant used most commonly in the treatment of attention-deficit disorders in children and for narcolepsy. Its mechanisms appear to be similar to those of dextroamphetamine. [NIH] MI: Myocardial infarction. Gross necrosis of the myocardium as a result of interruption of the blood supply to the area; it is almost always caused by atherosclerosis of the coronary arteries, upon which coronary thrombosis is usually superimposed. [NIH] Microbiology: The study of microorganisms such as fungi, bacteria, algae, archaea, and viruses. [NIH] Microcalcifications: Tiny deposits of calcium in the breast that cannot be felt but can be detected on a mammogram. A cluster of these very small specks of calcium may indicate that cancer is present. [NIH] Microorganism: An organism that can be seen only through a microscope. Microorganisms include bacteria, protozoa, algae, and fungi. Although viruses are not considered living organisms, they are sometimes classified as microorganisms. [NIH] Micro-organism: An organism which cannot be observed with the naked eye; e. g. unicellular animals, lower algae, lower fungi, bacteria. [NIH] Microscopy: The application of microscope magnification to the study of materials that cannot be properly seen by the unaided eye. [NIH] Migration: The systematic movement of genes between populations of the same species, geographic race, or variety. [NIH] Mitochondria: Parts of a cell where aerobic production (also known as cell respiration) takes place. [NIH] Mitochondrial Swelling: Increase in volume of mitochondria due to an influx of fluid; it occurs in hypotonic solutions due to osmotic pressure and in isotonic solutions as a result of altered permeability of the membranes of respiring mitochondria. [NIH] Mitosis: A method of indirect cell division by means of which the two daughter nuclei normally receive identical complements of the number of chromosomes of the somatic cells of the species. [NIH] Mitotic: Cell resulting from mitosis. [NIH] Mobility: Capability of movement, of being moved, or of flowing freely. [EU] Modeling: A treatment procedure whereby the therapist presents the target behavior which the learner is to imitate and make part of his repertoire. [NIH] Modification: A change in an organism, or in a process in an organism, that is acquired from its own activity or environment. [NIH] Molecular: Of, pertaining to, or composed of molecules : a very small mass of matter. [EU] Molecular Motors: Protein based machines that are involved in or cause movement such as the rotary devices (flagellar motor and the F1 ATPase) or the devices whose movement is directed along cytoskeletal filaments (myosin, kinesin and dynein motor families). [NIH] Molecular Structure: The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds. [NIH] Molecule: A chemical made up of two or more atoms. The atoms in a molecule can be the same (an oxygen molecule has two oxygen atoms) or different (a water molecule has two hydrogen atoms and one oxygen atom). Biological molecules, such as proteins and DNA,

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can be made up of many thousands of atoms. [NIH] Monitor: An apparatus which automatically records such physiological signs as respiration, pulse, and blood pressure in an anesthetized patient or one undergoing surgical or other procedures. [NIH] Monoclonal: An antibody produced by culturing a single type of cell. It therefore consists of a single species of immunoglobulin molecules. [NIH] Monoclonal antibodies: Laboratory-produced substances that can locate and bind to cancer cells wherever they are in the body. Many monoclonal antibodies are used in cancer detection or therapy; each one recognizes a different protein on certain cancer cells. Monoclonal antibodies can be used alone, or they can be used to deliver drugs, toxins, or radioactive material directly to a tumor. [NIH] Monosomy: The condition in which one chromosome of a pair is missing. In a normally diploid cell it is represented symbolically as 2N-1. [NIH] Morphogenesis: The development of the form of an organ, part of the body, or organism. [NIH]

Morphological: Relating to the configuration or the structure of live organs. [NIH] Morula: The early embryo at the developmental stage in which the blastomeres, resulting from repeated mitotic divisions of the fertilized ovum, form a compact mass. [NIH] Mosaicism: The occurrence in an individual of two or more cell populations of different chromosomal constitutions, derived from a single zygote, as opposed to chimerism in which the different cell populations are derived from more than one zygote. [NIH] Motility: The ability to move spontaneously. [EU] Motor Activity: The physical activity of an organism as a behavioral phenomenon. [NIH] Motor nerve: An efferent nerve conveying an impulse that excites muscular contraction. [NIH]

Motor Skills: Performance of complex motor acts. [NIH] Movement Disorders: Syndromes which feature dyskinesias as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions. [NIH] Mucins: A secretion containing mucopolysaccharides and protein that is the chief constituent of mucus. [NIH] Mucus: The viscous secretion of mucous membranes. It contains mucin, white blood cells, water, inorganic salts, and exfoliated cells. [NIH] Multiple sclerosis: A disorder of the central nervous system marked by weakness, numbness, a loss of muscle coordination, and problems with vision, speech, and bladder control. Multiple sclerosis is thought to be an autoimmune disease in which the body's immune system destroys myelin. Myelin is a substance that contains both protein and fat (lipid) and serves as a nerve insulator and helps in the transmission of nerve signals. [NIH] Multivariate Analysis: A set of techniques used when variation in several variables has to be studied simultaneously. In statistics, multivariate analysis is interpreted as any analytic method that allows simultaneous study of two or more dependent variables. [NIH] Muscle Contraction: A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments. [NIH] Muscle Fibers: Large single cells, either cylindrical or prismatic in shape, that form the basic unit of muscle tissue. They consist of a soft contractile substance enclosed in a tubular

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sheath. [NIH] Muscular Diseases: Acquired, familial, and congenital disorders of skeletal muscle and smooth muscle. [NIH] Muscular Dystrophies: A general term for a group of inherited disorders which are characterized by progressive degeneration of skeletal muscles. [NIH] Musculature: The muscular apparatus of the body, or of any part of it. [EU] Mutagenesis: Process of generating genetic mutations. It may occur spontaneously or be induced by mutagens. [NIH] Mutagens: Chemical agents that increase the rate of genetic mutation by interfering with the function of nucleic acids. A clastogen is a specific mutagen that causes breaks in chromosomes. [NIH] Myelin: The fatty substance that covers and protects nerves. [NIH] Myelosuppression: A condition in which bone marrow activity is decreased, resulting in fewer red blood cells, white blood cells, and platelets. Myelosuppression is a side effect of some cancer treatments. [NIH] Myocardial infarction: Gross necrosis of the myocardium as a result of interruption of the blood supply to the area; it is almost always caused by atherosclerosis of the coronary arteries, upon which coronary thrombosis is usually superimposed. [NIH] Myocardial Ischemia: A disorder of cardiac function caused by insufficient blood flow to the muscle tissue of the heart. The decreased blood flow may be due to narrowing of the coronary arteries (coronary arteriosclerosis), to obstruction by a thrombus (coronary thrombosis), or less commonly, to diffuse narrowing of arterioles and other small vessels within the heart. Severe interruption of the blood supply to the myocardial tissue may result in necrosis of cardiac muscle (myocardial infarction). [NIH] Myocardium: The muscle tissue of the heart composed of striated, involuntary muscle known as cardiac muscle. [NIH] Myofibrils: Highly organized bundles of actin, myosin, and other proteins in the cytoplasm of skeletal and cardiac muscle cells that contract by a sliding filament mechanism. [NIH] Myopia: That error of refraction in which rays of light entering the eye parallel to the optic axis are brought to a focus in front of the retina, as a result of the eyeball being too long from front to back (axial m.) or of an increased strength in refractive power of the media of the eye (index m.). Called also nearsightedness, because the near point is less distant than it is in emmetropia with an equal amplitude of accommodation. [EU] Myosin: Chief protein in muscle and the main constituent of the thick filaments of muscle fibers. In conjunction with actin, it is responsible for the contraction and relaxation of muscles. [NIH] Myotonic Dystrophy: A condition presenting muscle weakness and wasting which may be progressive. [NIH] Naloxone: A specific opiate antagonist that has no agonist activity. It is a competitive antagonist at mu, delta, and kappa opioid receptors. [NIH] Naltrexone: Derivative of noroxymorphone that is the N-cyclopropylmethyl congener of naloxone. It is a narcotic antagonist that is effective orally, longer lasting and more potent than naloxone, and has been proposed for the treatment of heroin addiction. The FDA has approved naltrexone for the treatment of alcohol dependence. [NIH] Narcolepsy: A condition of unknown cause characterized by a periodic uncontrollable tendency to fall asleep. [NIH]

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Narcotic: 1. Pertaining to or producing narcosis. 2. An agent that produces insensibility or stupor, applied especially to the opioids, i.e. to any natural or synthetic drug that has morphine-like actions. [EU] Natural selection: A part of the evolutionary process resulting in the survival and reproduction of the best adapted individuals. [NIH] Nausea: An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. [NIH] NCI: National Cancer Institute. NCI, part of the National Institutes of Health of the United States Department of Health and Human Services, is the federal government's principal agency for cancer research. NCI conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the NCI Web site at http://cancer.gov. [NIH] Nearsightedness: The common term for myopia. [NIH] Necrosis: A pathological process caused by the progressive degradative action of enzymes that is generally associated with severe cellular trauma. It is characterized by mitochondrial swelling, nuclear flocculation, uncontrolled cell lysis, and ultimately cell death. [NIH] Need: A state of tension or dissatisfaction felt by an individual that impels him to action toward a goal he believes will satisfy the impulse. [NIH] Neocortex: The largest portion of the cerebral cortex. It is composed of neurons arranged in six layers. [NIH] Neonatal: Pertaining to the first four weeks after birth. [EU] Neonatal period: The first 4 weeks after birth. [NIH] Neoplasia: Abnormal and uncontrolled cell growth. [NIH] Neoplasm: A new growth of benign or malignant tissue. [NIH] Neoplastic: Pertaining to or like a neoplasm (= any new and abnormal growth); pertaining to neoplasia (= the formation of a neoplasm). [EU] Nerve: A cordlike structure of nervous tissue that connects parts of the nervous system with other tissues of the body and conveys nervous impulses to, or away from, these tissues. [NIH] Nervous System: The entire nerve apparatus composed of the brain, spinal cord, nerves and ganglia. [NIH] Networks: Pertaining to a nerve or to the nerves, a meshlike structure of interlocking fibers or strands. [NIH] Neural: 1. Pertaining to a nerve or to the nerves. 2. Situated in the region of the spinal axis, as the neutral arch. [EU] Neural Crest: A strip of specialized ectoderm flanking each side of the embryonal neural plate, which after the closure of the neural tube, forms a column of isolated cells along the dorsal aspect of the neural tube. Most of the cranial and all of the spinal sensory ganglion cells arise by differentiation of neural crest cells. [NIH] Neurodegenerative Diseases: Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures. [NIH] Neurogenic: Loss of bladder control caused by damage to the nerves controlling the bladder. [NIH] Neuroleptic: A term coined to refer to the effects on cognition and behaviour of

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antipsychotic drugs, which produce a state of apathy, lack of initiative, and limited range of emotion and in psychotic patients cause a reduction in confusion and agitation and normalization of psychomotor activity. [EU] Neurologic: Having to do with nerves or the nervous system. [NIH] Neurology: A medical specialty concerned with the study of the structures, functions, and diseases of the nervous system. [NIH] Neuroma: A tumor that arises in nerve cells. [NIH] Neuromuscular: Pertaining to muscles and nerves. [EU] Neuromuscular Diseases: A general term encompassing lower motor neuron disease; peripheral nervous system diseases; and certain muscular diseases. Manifestations include muscle weakness; fasciculation; muscle atrophy; spasm; myokymia; muscle hypertonia, myalgias, and musclehypotonia. [NIH] Neuromuscular Junction: The synapse between a neuron and a muscle. [NIH] Neuronal: Pertaining to a neuron or neurons (= conducting cells of the nervous system). [EU] Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the nervous system. [NIH] Neuropathy: A problem in any part of the nervous system except the brain and spinal cord. Neuropathies can be caused by infection, toxic substances, or disease. [NIH] Neuropeptide: A member of a class of protein-like molecules made in the brain. Neuropeptides consist of short chains of amino acids, with some functioning as neurotransmitters and some functioning as hormones. [NIH] Neurophysiology: The scientific discipline concerned with the physiology of the nervous system. [NIH] Neuropsychological Tests: Tests designed to assess neurological function associated with certain behaviors. They are used in diagnosing brain dysfunction or damage and central nervous system disorders or injury. [NIH] Neuropsychology: A branch of psychology which investigates the correlation between experience or behavior and the basic neurophysiological processes. The term neuropsychology stresses the dominant role of the nervous system. It is a more narrowly defined field than physiological psychology or psychophysiology. [NIH] Neuroretinitis: Inflammation of the optic nerve head and adjacent retina. [NIH] Neurosecretory Systems: A system of neurons that has the specialized function to produce and secrete hormones, and that constitutes, in whole or in part, an endocrine organ or system. [NIH] Neurotensin: A biologically active tridecapeptide isolated from the hypothalamus. It has been shown to induce hypotension in the rat, to stimulate contraction of guinea pig ileum and rat uterus, and to cause relaxation of rat duodenum. There is also evidence that it acts as both a peripheral and a central nervous system neurotransmitter. [NIH] Neurotoxicity: The tendency of some treatments to cause damage to the nervous system. [NIH]

Neurotransmitters: Endogenous signaling molecules that alter the behavior of neurons or effector cells. Neurotransmitter is used here in its most general sense, including not only messengers that act directly to regulate ion channels, but also those that act through second messenger systems, and those that act at a distance from their site of release. Included are neuromodulators, neuroregulators, neuromediators, and neurohumors, whether or not

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acting at synapses. [NIH] Neutropenia: An abnormal decrease in the number of neutrophils, a type of white blood cell. [NIH] Neutrophils: Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes. [NIH] Night Blindness: Anomaly of vision in which there is a pronounced inadequacy or complete absence of dark-adaptation. [NIH] Nitric Oxide: A free radical gas produced endogenously by a variety of mammalian cells. It is synthesized from arginine by a complex reaction, catalyzed by nitric oxide synthase. Nitric oxide is endothelium-derived relaxing factor. It is released by the vascular endothelium and mediates the relaxation induced by some vasodilators such as acetylcholine and bradykinin. It also inhibits platelet aggregation, induces disaggregation of aggregated platelets, and inhibits platelet adhesion to the vascular endothelium. Nitric oxide activates cytosolic guanylate cyclase and thus elevates intracellular levels of cyclic GMP. [NIH]

Nitrogen: An element with the atomic symbol N, atomic number 7, and atomic weight 14. Nitrogen exists as a diatomic gas and makes up about 78% of the earth's atmosphere by volume. It is a constituent of proteins and nucleic acids and found in all living cells. [NIH] Nitroglycerin: A highly volatile organic nitrate that acts as a dilator of arterial and venous smooth muscle and is used in the treatment of angina. It provides relief through improvement of the balance between myocardial oxygen supply and demand. Although total coronary blood flow is not increased, there is redistribution of blood flow in the heart when partial occlusion of coronary circulation is effected. [NIH] Nonverbal Communication: Transmission of emotions, ideas, and attitudes between individuals in ways other than the spoken language. [NIH] Norepinephrine: Precursor of epinephrine that is secreted by the adrenal medulla and is a widespread central and autonomic neurotransmitter. Norepinephrine is the principal transmitter of most postganglionic sympathetic fibers and of the diffuse projection system in the brain arising from the locus ceruleus. It is also found in plants and is used pharmacologically as a sympathomimetic. [NIH] Nuclear: A test of the structure, blood flow, and function of the kidneys. The doctor injects a mildly radioactive solution into an arm vein and uses x-rays to monitor its progress through the kidneys. [NIH] Nuclear Family: A family composed of spouses and their children. [NIH] Nuclei: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Nucleic acid: Either of two types of macromolecule (DNA or RNA) formed by polymerization of nucleotides. Nucleic acids are found in all living cells and contain the information (genetic code) for the transfer of genetic information from one generation to the next. [NIH] Nucleic Acid Hybridization: The process whereby two single-stranded polynucleotides form a double-stranded molecule, with hydrogen bonding between the complementary bases in the two strains. [NIH] Nucleus: A body of specialized protoplasm found in nearly all cells and containing the chromosomes. [NIH] Occipital Lobe: Posterior part of the cerebral hemisphere. [NIH]

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Occupational Therapy: The field concerned with utilizing craft or work activities in the rehabilitation of patients. Occupational therapy can also refer to the activities themselves. [NIH]

Ocular: 1. Of, pertaining to, or affecting the eye. 2. Eyepiece. [EU] Ointments: Semisolid preparations used topically for protective emollient effects or as a vehicle for local administration of medications. Ointment bases are various mixtures of fats, waxes, animal and plant oils and solid and liquid hydrocarbons. [NIH] Opacity: Degree of density (area most dense taken for reading). [NIH] Operon: The genetic unit consisting of a feedback system under the control of an operator gene, in which a structural gene transcribes its message in the form of mRNA upon blockade of a repressor produced by a regulator gene. Included here is the attenuator site of bacterial operons where transcription termination is regulated. [NIH] Ophthalmology: A surgical specialty concerned with the structure and function of the eye and the medical and surgical treatment of its defects and diseases. [NIH] Ophthalmoplegia: Paralysis of one or more of the ocular muscles due to disorders of the eye muscles, neuromuscular junction, supporting soft tissue, tendons, or innervation to the muscles. [NIH] Opioid Peptides: The endogenous peptides with opiate-like activity. The three major classes currently recognized are the enkephalins, the dynorphins, and the endorphins. Each of these families derives from different precursors, proenkephalin, prodynorphin, and proopiomelanocortin, respectively. There are also at least three classes of opioid receptors, but the peptide families do not map to the receptors in a simple way. [NIH] Opsin: A visual pigment protein found in the retinal rods. It combines with retinaldehyde to form rhodopsin. [NIH] Optic Atrophy: Atrophy of the optic disk which may be congenital or acquired. This condition indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm, and optic tracts. Glaucoma, ischemia, inflammation, a chronic elevation of intracranial pressure, toxins, optic nerve compression, and inherited conditions are relatively common causes of this condition. [NIH] Optic Chiasm: The X-shaped structure formed by the meeting of the two optic nerves. At the optic chiasm the fibers from the medial part of each retina cross to project to the other side of the brain while the lateral retinal fibers continue on the same side. As a result each half of the brain receives information about the contralateral visual field from both eyes. [NIH]

Optic Disk: The portion of the optic nerve seen in the fundus with the ophthalmoscope. It is formed by the meeting of all the retinal ganglion cell axons as they enter the optic nerve. [NIH]

Optic Nerve: The 2nd cranial nerve. The optic nerve conveys visual information from the retina to the brain. The nerve carries the axons of the retinal ganglion cells which sort at the optic chiasm and continue via the optic tracts to the brain. The largest projection is to the lateral geniculate nuclei; other important targets include the superior colliculi and the suprachiasmatic nuclei. Though known as the second cranial nerve, it is considered part of the central nervous system. [NIH] Oral Health: The optimal state of the mouth and normal functioning of the organs of the mouth without evidence of disease. [NIH] Oral Hygiene: The practice of personal hygiene of the mouth. It includes the maintenance of oral cleanliness, tissue tone, and general preservation of oral health. [NIH]

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Organ Culture: The growth in aseptic culture of plant organs such as roots or shoots, beginning with organ primordia or segments and maintaining the characteristics of the organ. [NIH] Organelles: Specific particles of membrane-bound organized living substances present in eukaryotic cells, such as the mitochondria; the golgi apparatus; endoplasmic reticulum; lysomomes; plastids; and vacuoles. [NIH] Orofacial: Of or relating to the mouth and face. [EU] Orthostatic: Pertaining to or caused by standing erect. [EU] Osmotic: Pertaining to or of the nature of osmosis (= the passage of pure solvent from a solution of lesser to one of greater solute concentration when the two solutions are separated by a membrane which selectively prevents the passage of solute molecules, but is permeable to the solvent). [EU] Ossicles: The hammer, anvil and stirrup, the small bones of the middle ear, which transmit the vibrations from the tympanic membrane to the oval window. [NIH] Osteodystrophy: Defective bone formation. [EU] Osteoporosis: Reduction of bone mass without alteration in the composition of bone, leading to fractures. Primary osteoporosis can be of two major types: postmenopausal osteoporosis and age-related (or senile) osteoporosis. [NIH] Outpatient: A patient who is not an inmate of a hospital but receives diagnosis or treatment in a clinic or dispensary connected with the hospital. [NIH] Ovarian Cysts: General term for cysts and cystic diseases of the ovary. [NIH] Ovary: Either of the paired glands in the female that produce the female germ cells and secrete some of the female sex hormones. [NIH] Overdosage: 1. The administration of an excessive dose. 2. The condition resulting from an excessive dose. [EU] Ovum: A female germ cell extruded from the ovary at ovulation. [NIH] Oxidation: The act of oxidizing or state of being oxidized. Chemically it consists in the increase of positive charges on an atom or the loss of negative charges. Most biological oxidations are accomplished by the removal of a pair of hydrogen atoms (dehydrogenation) from a molecule. Such oxidations must be accompanied by reduction of an acceptor molecule. Univalent o. indicates loss of one electron; divalent o., the loss of two electrons. [EU]

Oxidative metabolism: A chemical process in which oxygen is used to make energy from carbohydrates (sugars). Also known as aerobic respiration, cell respiration, or aerobic metabolism. [NIH] Palate: The structure that forms the roof of the mouth. It consists of the anterior hard palate and the posterior soft palate. [NIH] Palliative: 1. Affording relief, but not cure. 2. An alleviating medicine. [EU] Palsy: Disease of the peripheral nervous system occurring usually after many years of increased lead absorption. [NIH] Pancreas: A mixed exocrine and endocrine gland situated transversely across the posterior abdominal wall in the epigastric and hypochondriac regions. The endocrine portion is comprised of the Islets of Langerhans, while the exocrine portion is a compound acinar gland that secretes digestive enzymes. [NIH] Pancreatic: Having to do with the pancreas. [NIH]

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Pancreatic Polypeptide: A 36-amino acid polypeptide with physiological regulatory functions. It is secreted by pancreatic tissue. Plasma pancreatic polypeptide increases after ingestion of food, with age, and in disease states. A lack of pancreatic polypeptide in the islets of Langerhans has been associated with the obese syndrome in rats and mice. [NIH] Paraffin: A mixture of solid hydrocarbons obtained from petroleum. It has a wide range of uses including as a stiffening agent in ointments, as a lubricant, and as a topical antiinflammatory. It is also commonly used as an embedding material in histology. [NIH] Paralysis: Loss of ability to move all or part of the body. [NIH] Paraplegia: Severe or complete loss of motor function in the lower extremities and lower portions of the trunk. This condition is most often associated with spinal cord diseases, although brain diseases; peripheral nervous system diseases; neuromuscular diseases; and muscular diseases may also cause bilateral leg weakness. [NIH] Parasite: An animal or a plant that lives on or in an organism of another species and gets at least some of its nutrition from that other organism. [NIH] Parasitic: Having to do with or being a parasite. A parasite is an animal or a plant that lives on or in an organism of another species and gets at least some of its nutrients from it. [NIH] Parenchyma: The essential elements of an organ; used in anatomical nomenclature as a general term to designate the functional elements of an organ, as distinguished from its framework, or stroma. [EU] Parkinsonism: A group of neurological disorders characterized by hypokinesia, tremor, and muscular rigidity. [EU] Paroxysmal: Recurring in paroxysms (= spasms or seizures). [EU] Particle: A tiny mass of material. [EU] Patch: A piece of material used to cover or protect a wound, an injured part, etc.: a patch over the eye. [NIH] Pathogenesis: The cellular events and reactions that occur in the development of disease. [NIH]

Pathologic: 1. Indicative of or caused by a morbid condition. 2. Pertaining to pathology (= branch of medicine that treats the essential nature of the disease, especially the structural and functional changes in tissues and organs of the body caused by the disease). [EU] Pathologic Processes: The abnormal mechanisms and forms involved in the dysfunctions of tissues and organs. [NIH] Pathologies: The study of abnormality, especially the study of diseases. [NIH] Pathophysiology: Altered functions in an individual or an organ due to disease. [NIH] Patient Advocacy: Promotion and protection of the rights of patients, frequently through a legal process. [NIH] Patient Compliance: Voluntary cooperation of the patient in following a prescribed regimen. [NIH] Patient Education: The teaching or training of patients concerning their own health needs. [NIH]

Pediatrics: A medical specialty concerned with maintaining health and providing medical care to children from birth to adolescence. [NIH] Pedigree: A record of one's ancestors, offspring, siblings, and their offspring that may be used to determine the pattern of certain genes or disease inheritance within a family. [NIH] Pelvic: Pertaining to the pelvis. [EU]

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Peptide: Any compound consisting of two or more amino acids, the building blocks of proteins. Peptides are combined to make proteins. [NIH] Perception: The ability quickly and accurately to recognize similarities and differences among presented objects, whether these be pairs of words, pairs of number series, or multiple sets of these or other symbols such as geometric figures. [NIH] Perinatal: Pertaining to or occurring in the period shortly before and after birth; variously defined as beginning with completion of the twentieth to twenty-eighth week of gestation and ending 7 to 28 days after birth. [EU] Periodontal disease: Disease involving the supporting structures of the teeth (as the gums and periodontal membranes). [NIH] Peripheral blood: Blood circulating throughout the body. [NIH] Peripheral Nervous System: The nervous system outside of the brain and spinal cord. The peripheral nervous system has autonomic and somatic divisions. The autonomic nervous system includes the enteric, parasympathetic, and sympathetic subdivisions. The somatic nervous system includes the cranial and spinal nerves and their ganglia and the peripheral sensory receptors. [NIH] Peripheral Nervous System Diseases: Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves. [NIH] Peripheral Neuropathy: Nerve damage, usually affecting the feet and legs; causing pain, numbness, or a tingling feeling. Also called "somatic neuropathy" or "distal sensory polyneuropathy." [NIH] Periventricular Leukomalacia: Rare form of epilepsy. [NIH] Peroxisomal Disorders: A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional peroxisomes. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include Zellweger syndrome; infantile Refsum disease; rhizomelic chondrodysplasia (chondrodysplasia punctata, rhizomelic); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and adrenoleukodystrophy (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders. [NIH] Petroleum: Naturally occurring complex liquid hydrocarbons which, after distillation, yield combustible fuels, petrochemicals, and lubricants. [NIH] Phallic: Pertaining to the phallus, or penis. [EU] Pharmaceutical Solutions: Homogeneous liquid preparations that contain one or more chemical substances dissolved, i.e., molecularly dispersed, in a suitable solvent or mixture of mutually miscible solvents. For reasons of their ingredients, method of preparation, or use, they do not fall into another group of products. [NIH] Pharmacokinetics: Dynamic and kinetic mechanisms of exogenous chemical and drug absorption, biotransformation, distribution, release, transport, uptake, and elimination as a function of dosage, and extent and rate of metabolic processes. It includes toxicokinetics, the pharmacokinetic mechanism of the toxic effects of a substance. [NIH] Pharmacologic: Pertaining to pharmacology or to the properties and reactions of drugs. [EU] Phenotype: The outward appearance of the individual. It is the product of interactions between genes and between the genotype and the environment. This includes the killer phenotype, characteristic of yeasts. [NIH]

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Phenylalanine: An aromatic amino acid that is essential in the animal diet. It is a precursor of melanin, dopamine, noradrenalin, and thyroxine. [NIH] Phosphodiesterase: Effector enzyme that regulates the levels of a second messenger, the cyclic GMP. [NIH] Phospholipases: A class of enzymes that catalyze the hydrolysis of phosphoglycerides or glycerophosphatidates. EC 3.1.-. [NIH] Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides; glycerophospholipids) or sphingosine (sphingolipids). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system. [NIH] Phosphorus: A non-metallic element that is found in the blood, muscles, nevers, bones, and teeth, and is a component of adenosine triphosphate (ATP; the primary energy source for the body's cells.) [NIH] Phosphorylates: Attached to a phosphate group. [NIH] Phosphorylation: The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety. [NIH] Photoreceptor: Receptor capable of being activated by light stimuli, as a rod or cone cell of the eye. [NIH] Physical Examination: Systematic and thorough inspection of the patient for physical signs of disease or abnormality. [NIH] Physical Fitness: A state of well-being in which performance is optimal, often as a result of physical conditioning which may be prescribed for disease therapy. [NIH] Physical Therapy: The restoration of function and the prevention of disability following disease or injury with the use of light, heat, cold, water, electricity, ultrasound, and exercise. [NIH]

Physiologic: Having to do with the functions of the body. When used in the phrase "physiologic age," it refers to an age assigned by general health, as opposed to calendar age. [NIH]

Physiology: The science that deals with the life processes and functions of organismus, their cells, tissues, and organs. [NIH] Piebaldism: Autosomal dominant, congenital disorder characterized by localized hypomelanosis of the skin and hair. The most familiar feature is a white forelock presenting in 80 to 90 percent of the patients. The underlying defect is possibly related to the differentiation and migration of melanoblasts, as well as to defective development of the neural crest (neurocristopathy). Piebaldism may be closely related to Waardenburg's syndrome. [NIH] Pigments: Any normal or abnormal coloring matter in plants, animals, or micro-organisms. [NIH]

Pitch: The subjective awareness of the frequency or spectral distribution of a sound. [NIH] Plants: Multicellular, eukaryotic life forms of the kingdom Plantae. They are characterized by a mainly photosynthetic mode of nutrition; essentially unlimited growth at localized regions of cell divisions (meristems); cellulose within cells providing rigidity; the absence of organs of locomotion; absense of nervous and sensory systems; and an alteration of haploid and diploid generations. [NIH] Plaque: A clear zone in a bacterial culture grown on an agar plate caused by localized destruction of bacterial cells by a bacteriophage. The concentration of infective virus in a

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fluid can be estimated by applying the fluid to a culture and counting the number of. [NIH] Plasma: The clear, yellowish, fluid part of the blood that carries the blood cells. The proteins that form blood clots are in plasma. [NIH] Plasma cells: A type of white blood cell that produces antibodies. [NIH] Plasma protein: One of the hundreds of different proteins present in blood plasma, including carrier proteins ( such albumin, transferrin, and haptoglobin), fibrinogen and other coagulation factors, complement components, immunoglobulins, enzyme inhibitors, precursors of substances such as angiotension and bradykinin, and many other types of proteins. [EU] Plasticity: In an individual or a population, the capacity for adaptation: a) through gene changes (genetic plasticity) or b) through internal physiological modifications in response to changes of environment (physiological plasticity). [NIH] Plasticizers: Materials incorporated mechanically in plastics (usually PVC) to increase flexibility, workability or distensibility; due to the non-chemical inclusion, plasticizers leach out from the plastic and are found in body fluids and the general environment. [NIH] Platelet Activation: A series of progressive, overlapping events triggered by exposure of the platelets to subendothelial tissue. These events include shape change, adhesiveness, aggregation, and release reactions. When carried through to completion, these events lead to the formation of a stable hemostatic plug. [NIH] Platelet Aggregation: The attachment of platelets to one another. This clumping together can be induced by a number of agents (e.g., thrombin, collagen) and is part of the mechanism leading to the formation of a thrombus. [NIH] Platelet Count: A count of the number of platelets per unit volume in a sample of venous blood. [NIH] Platelets: A type of blood cell that helps prevent bleeding by causing blood clots to form. Also called thrombocytes. [NIH] Platinum: Platinum. A heavy, soft, whitish metal, resembling tin, atomic number 78, atomic weight 195.09, symbol Pt. (From Dorland, 28th ed) It is used in manufacturing equipment for laboratory and industrial use. It occurs as a black powder (platinum black) and as a spongy substance (spongy platinum) and may have been known in Pliny's time as "alutiae". [NIH]

Point Mutation: A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair. [NIH] Poisoning: A condition or physical state produced by the ingestion, injection or inhalation of, or exposure to a deleterious agent. [NIH] Policy Making: The decision process by which individuals, groups or institutions establish policies pertaining to plans, programs or procedures. [NIH] Polymerase: An enzyme which catalyses the synthesis of DNA using a single DNA strand as a template. The polymerase copies the template in the 5'-3'direction provided that sufficient quantities of free nucleotides, dATP and dTTP are present. [NIH] Polymorphic: Occurring in several or many forms; appearing in different forms at different stages of development. [EU] Polymorphism: The occurrence together of two or more distinct forms in the same population. [NIH] Polypeptide: A peptide which on hydrolysis yields more than two amino acids; called tripeptides, tetrapeptides, etc. according to the number of amino acids contained. [EU]

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Polysaccharide: A type of carbohydrate. It contains sugar molecules that are linked together chemically. [NIH] Posterior: Situated in back of, or in the back part of, or affecting the back or dorsal surface of the body. In lower animals, it refers to the caudal end of the body. [EU] Postmenopausal: Refers to the time after menopause. Menopause is the time in a woman's life when menstrual periods stop permanently; also called "change of life." [NIH] Postnatal: Occurring after birth, with reference to the newborn. [EU] Postsynaptic: Nerve potential generated by an inhibitory hyperpolarizing stimulation. [NIH] Post-synaptic: Nerve potential generated by an inhibitory hyperpolarizing stimulation. [NIH] Post-translational: The cleavage of signal sequence that directs the passage of the protein through a cell or organelle membrane. [NIH] Post-traumatic: Occurring as a result of or after injury. [EU] Postural: Pertaining to posture or position. [EU] Potentiation: An overall effect of two drugs taken together which is greater than the sum of the effects of each drug taken alone. [NIH] Practice Guidelines: Directions or principles presenting current or future rules of policy for the health care practitioner to assist him in patient care decisions regarding diagnosis, therapy, or related clinical circumstances. The guidelines may be developed by government agencies at any level, institutions, professional societies, governing boards, or by the convening of expert panels. The guidelines form a basis for the evaluation of all aspects of health care and delivery. [NIH] Preclinical: Before a disease becomes clinically recognizable. [EU] Precursor: Something that precedes. In biological processes, a substance from which another, usually more active or mature substance is formed. In clinical medicine, a sign or symptom that heralds another. [EU] Pregnancy Tests: Tests to determine whether or not an individual is pregnant. [NIH] Prenatal: Existing or occurring before birth, with reference to the fetus. [EU] Presbycusis: Progressive bilateral loss of hearing that occurs in the aged. Syn: senile deafness. [NIH] Presynaptic: Situated proximal to a synapse, or occurring before the synapse is crossed. [EU] Prevalence: The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. [NIH] Primed In Situ Labeling: A technique that labels specific sequences in whole chromosomes by in situ DNA chain elongation or PCR (polymerase chain reaction). [NIH] Private Sector: That distinct portion of the institutional, industrial, or economic structure of a country that is controlled or owned by non-governmental, private interests. [NIH] Probe: An instrument used in exploring cavities, or in the detection and dilatation of strictures, or in demonstrating the potency of channels; an elongated instrument for exploring or sounding body cavities. [NIH] Programmed Instruction: Instruction in which learners progress at their own rate using workbooks, textbooks, or electromechanical devices that provide information in discrete steps, test learning at each step, and provide immediate feedback about achievement. (ERIC, Thesaurus of ERIC Descriptors, 1996). [NIH] Progression: Increase in the size of a tumor or spread of cancer in the body. [NIH]

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Progressive: Advancing; going forward; going from bad to worse; increasing in scope or severity. [EU] Projection: A defense mechanism, operating unconsciously, whereby that which is emotionally unacceptable in the self is rejected and attributed (projected) to others. [NIH] Prolactin: Pituitary lactogenic hormone. A polypeptide hormone with a molecular weight of about 23,000. It is essential in the induction of lactation in mammals at parturition and is synergistic with estrogen. The hormone also brings about the release of progesterone from lutein cells, which renders the uterine mucosa suited for the embedding of the ovum should fertilization occur. [NIH] Promoter: A chemical substance that increases the activity of a carcinogenic process. [NIH] Promotor: In an operon, a nucleotide sequence located at the operator end which contains all the signals for the correct initiation of genetic transcription by the RNA polymerase holoenzyme and determines the maximal rate of RNA synthesis. [NIH] Prone: Having the front portion of the body downwards. [NIH] Pro-Opiomelanocortin: A precursor protein, MW 30,000, synthesized mainly in the anterior pituitary gland but also found in the hypothalamus, brain, and several peripheral tissues. It incorporates the amino acid sequences of ACTH and beta-lipotropin. These two hormones, in turn, contain the biologically active peptides MSH, corticotropin-like intermediate lobe peptide, alpha-lipotropin, endorphins, and methionine enkephalin. [NIH] Prophase: The first phase of cell division, in which the chromosomes become visible, the nucleus starts to lose its identity, the spindle appears, and the centrioles migrate toward opposite poles. [NIH] Prophylaxis: An attempt to prevent disease. [NIH] Prospective study: An epidemiologic study in which a group of individuals (a cohort), all free of a particular disease and varying in their exposure to a possible risk factor, is followed over a specific amount of time to determine the incidence rates of the disease in the exposed and unexposed groups. [NIH] Prostate: A gland in males that surrounds the neck of the bladder and the urethra. It secretes a substance that liquifies coagulated semen. It is situated in the pelvic cavity behind the lower part of the pubic symphysis, above the deep layer of the triangular ligament, and rests upon the rectum. [NIH] Prosthesis: An artificial replacement of a part of the body. [NIH] Protein C: A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation. [NIH] Protein Conformation: The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. Quaternary protein structure describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain). [NIH] Protein Kinases: A family of enzymes that catalyze the conversion of ATP and a protein to ADP and a phosphoprotein. EC 2.7.1.37. [NIH] Protein S: The vitamin K-dependent cofactor of activated protein C. Together with protein C, it inhibits the action of factors VIIIa and Va. A deficiency in protein S can lead to recurrent venous and arterial thrombosis. [NIH] Proteins: Polymers of amino acids linked by peptide bonds. The specific sequence of amino acids determines the shape and function of the protein. [NIH]

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Proteoglycans: Glycoproteins which have a very high polysaccharide content. [NIH] Proteolytic: 1. Pertaining to, characterized by, or promoting proteolysis. 2. An enzyme that promotes proteolysis (= the splitting of proteins by hydrolysis of the peptide bonds with formation of smaller polypeptides). [EU] Prothrombin: A plasma protein that is the inactive precursor of thrombin. It is converted to thrombin by a prothrombin activator complex consisting of factor Xa, factor V, phospholipid, and calcium ions. Deficiency of prothrombin leads to hypoprothrombinemia. [NIH]

Protocol: The detailed plan for a clinical trial that states the trial's rationale, purpose, drug or vaccine dosages, length of study, routes of administration, who may participate, and other aspects of trial design. [NIH] Proximal: Nearest; closer to any point of reference; opposed to distal. [EU] Pruritus: An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief. [NIH] Psoriasis: A common genetically determined, chronic, inflammatory skin disease characterized by rounded erythematous, dry, scaling patches. The lesions have a predilection for nails, scalp, genitalia, extensor surfaces, and the lumbosacral region. Accelerated epidermopoiesis is considered to be the fundamental pathologic feature in psoriasis. [NIH] Psychiatric: Pertaining to or within the purview of psychiatry. [EU] Psychiatry: The medical science that deals with the origin, diagnosis, prevention, and treatment of mental disorders. [NIH] Psychic: Pertaining to the psyche or to the mind; mental. [EU] Psychogenic: Produced or caused by psychic or mental factors rather than organic factors. [EU]

Psychology: The science dealing with the study of mental processes and behavior in man and animals. [NIH] Psychometric testing: Psychological and mental testing and quantitative analysis of an individual's psychological traits or attitudes or mental processes. [NIH] Psychomotor: Pertaining to motor effects of cerebral or psychic activity. [EU] Psychopathology: The study of significant causes and processes in the development of mental illness. [NIH] Psychophysiology: The study of the physiological basis of human and animal behavior. [NIH]

Psychosis: A mental disorder characterized by gross impairment in reality testing as evidenced by delusions, hallucinations, markedly incoherent speech, or disorganized and agitated behaviour without apparent awareness on the part of the patient of the incomprehensibility of his behaviour; the term is also used in a more general sense to refer to mental disorders in which mental functioning is sufficiently impaired as to interfere grossly with the patient's capacity to meet the ordinary demands of life. Historically, the term has been applied to many conditions, e.g. manic-depressive psychosis, that were first described in psychotic patients, although many patients with the disorder are not judged psychotic. [EU] Psychotropic: Exerting an effect upon the mind; capable of modifying mental activity; usually applied to drugs that effect the mental state. [EU] Ptosis: 1. Prolapse of an organ or part. 2. Drooping of the upper eyelid from paralysis of the

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third nerve or from sympathetic innervation. [EU] Public Health: Branch of medicine concerned with the prevention and control of disease and disability, and the promotion of physical and mental health of the population on the international, national, state, or municipal level. [NIH] Public Policy: A course or method of action selected, usually by a government, from among alternatives to guide and determine present and future decisions. [NIH] Publishing: "The business or profession of the commercial production and issuance of literature" (Webster's 3d). It includes the publisher, publication processes, editing and editors. Production may be by conventional printing methods or by electronic publishing. [NIH]

Pulmonary: Relating to the lungs. [NIH] Pulmonary Artery: The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. [NIH] Pulse: The rhythmical expansion and contraction of an artery produced by waves of pressure caused by the ejection of blood from the left ventricle of the heart as it contracts. [NIH]

Purines: A series of heterocyclic compounds that are variously substituted in nature and are known also as purine bases. They include adenine and guanine, constituents of nucleic acids, as well as many alkaloids such as caffeine and theophylline. Uric acid is the metabolic end product of purine metabolism. [NIH] Pyridoxal: 3-Hydroxy-5-(hydroxymethyl)-2-methyl-4- pyridinecarboxaldehyde. [NIH] Pyridoxal Phosphate: 3-Hydroxy-2-methyl-5-((phosphonooxy)methyl)-4pyridinecarboxaldehyde. An enzyme co-factor vitamin. [NIH] Quadriplegia: Severe or complete loss of motor function in all four limbs which may result from brain diseases; spinal cord diseases; peripheral nervous system diseases; neuromuscular diseases; or rarely muscular diseases. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper brain stem which injures the descending cortico-spinal and cortico-bulbar tracts. [NIH] Quality of Life: A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral and social environment. [NIH] Race: A population within a species which exhibits general similarities within itself, but is both discontinuous and distinct from other populations of that species, though not sufficiently so as to achieve the status of a taxon. [NIH] Radiation: Emission or propagation of electromagnetic energy (waves/rays), or the waves/rays themselves; a stream of electromagnetic particles (electrons, neutrons, protons, alpha particles) or a mixture of these. The most common source is the sun. [NIH] Radioactive: Giving off radiation. [NIH] Radiography: Examination of any part of the body for diagnostic purposes by means of roentgen rays, recording the image on a sensitized surface (such as photographic film). [NIH] Randomized: Describes an experiment or clinical trial in which animal or human subjects are assigned by chance to separate groups that compare different treatments. [NIH] Reality Testing: The individual's objective evaluation of the external world and the ability to differentiate adequately between it and the internal world; considered to be a primary ego function. [NIH]

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Receptor: A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell. [NIH] Receptors, Serotonin: Cell-surface proteins that bind serotonin and trigger intracellular changes which influence the behavior of cells. Several types of serotonin receptors have been recognized which differ in their pharmacology, molecular biology, and mode of action. [NIH] Recombinant: A cell or an individual with a new combination of genes not found together in either parent; usually applied to linked genes. [EU] Recombination: The formation of new combinations of genes as a result of segregation in crosses between genetically different parents; also the rearrangement of linked genes due to crossing-over. [NIH] Rectum: The last 8 to 10 inches of the large intestine. [NIH] Recurrence: The return of a sign, symptom, or disease after a remission. [NIH] Red blood cells: RBCs. Cells that carry oxygen to all parts of the body. Also called erythrocytes. [NIH] Red Nucleus: A pinkish-yellow portion of the midbrain situated in the rostral mesencephalic tegmentum. It receives a large projection from the contralateral half of the cerebellum via the superior cerebellar peduncle and a projection from the ipsilateral motor cortex. [NIH] Refer: To send or direct for treatment, aid, information, de decision. [NIH] Refraction: A test to determine the best eyeglasses or contact lenses to correct a refractive error (myopia, hyperopia, or astigmatism). [NIH] Refractive Power: The ability of an object, such as the eye, to bend light as light passes through it. [NIH] Regeneration: The natural renewal of a structure, as of a lost tissue or part. [EU] Regimen: A treatment plan that specifies the dosage, the schedule, and the duration of treatment. [NIH] Reliability: Used technically, in a statistical sense, of consistency of a test with itself, i. e. the extent to which we can assume that it will yield the same result if repeated a second time. [NIH]

Remission: A decrease in or disappearance of signs and symptoms of cancer. In partial remission, some, but not all, signs and symptoms of cancer have disappeared. In complete remission, all signs and symptoms of cancer have disappeared, although there still may be cancer in the body. [NIH] Renal failure: Progressive renal insufficiency and uremia, due to irreversible and progressive renal glomerular tubular or interstitial disease. [NIH] Renal tubular: A defect in the kidneys that hinders their normal excretion of acids. Failure to excrete acids can lead to weak bones, kidney stones, and poor growth in children. [NIH] Renal tubular acidosis: A rare disorder in which structures in the kidney that filter the blood are impaired, producing using that is more acid than normal. [NIH] Research Support: Financial support of research activities. [NIH] Resident physician: A physician who lives in a hospital and is constantly available, as an intern. [NIH] Residential Facilities: Long-term care facilities which provide supervision and assistance in activities of daily living with medical and nursing services when required. [NIH] Resolving: The ability of the eye or of a lens to make small objects that are close together,

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separately visible; thus revealing the structure of an object. [NIH] Resorption: The loss of substance through physiologic or pathologic means, such as loss of dentin and cementum of a tooth, or of the alveolar process of the mandible or maxilla. [EU] Restless legs: Legs characterized by or showing inability to remain at rest. [EU] Restoration: Broad term applied to any inlay, crown, bridge or complete denture which restores or replaces loss of teeth or oral tissues. [NIH] Retina: The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outer-most layer is pigmented, whereas the inner nine layers are transparent. [NIH] Retinal: 1. Pertaining to the retina. 2. The aldehyde of retinol, derived by the oxidative enzymatic splitting of absorbed dietary carotene, and having vitamin A activity. In the retina, retinal combines with opsins to form visual pigments. One isomer, 11-cis retinal combines with opsin in the rods (scotopsin) to form rhodopsin, or visual purple. Another, all-trans retinal (trans-r.); visual yellow; xanthopsin) results from the bleaching of rhodopsin by light, in which the 11-cis form is converted to the all-trans form. Retinal also combines with opsins in the cones (photopsins) to form the three pigments responsible for colour vision. Called also retinal, and retinene1. [EU] Retinitis: Inflammation of the retina. It is rarely limited to the retina, but is commonly associated with diseases of the choroid (chorioretinitis) and of the optic nerve (neuroretinitis). The disease may be confined to one eye, but since it is generally dependent on a constitutional factor, it is almost always bilateral. It may be acute in course, but as a rule it lasts many weeks or even several months. [NIH] Retinitis Pigmentosa: Hereditary, progressive degeneration of the neuroepithelium of the retina characterized by night blindness and progressive contraction of the visual field. [NIH] Retinol: Vitamin A. It is essential for proper vision and healthy skin and mucous membranes. Retinol is being studied for cancer prevention; it belongs to the family of drugs called retinoids. [NIH] Retinopathy: 1. Retinitis (= inflammation of the retina). 2. Retinosis (= degenerative, noninflammatory condition of the retina). [EU] Rheumatism: A group of disorders marked by inflammation or pain in the connective tissue structures of the body. These structures include bone, cartilage, and fat. [NIH] Rheumatoid: Resembling rheumatism. [EU] Rheumatoid arthritis: A form of arthritis, the cause of which is unknown, although infection, hypersensitivity, hormone imbalance and psychologic stress have been suggested as possible causes. [NIH] Ribose: A pentose active in biological systems usually in its D-form. [NIH] Ribosome: A granule of protein and RNA, synthesized in the nucleolus and found in the cytoplasm of cells. Ribosomes are the main sites of protein synthesis. Messenger RNA attaches to them and there receives molecules of transfer RNA bearing amino acids. [NIH] Risk factor: A habit, trait, condition, or genetic alteration that increases a person's chance of developing a disease. [NIH] Risperidone: A selective blocker of dopamine D2 and serotonin-5-HT-2 receptors that acts as an atypical antipsychotic agent. It has been shown to improve both positive and negative symptoms in the treatment of schizophrenia. [NIH] Rod: A reception for vision, located in the retina. [NIH]

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Rural Population: The inhabitants of rural areas or of small towns classified as rural. [NIH] Saline: A solution of salt and water. [NIH] Saliva: The clear, viscous fluid secreted by the salivary glands and mucous glands of the mouth. It contains mucins, water, organic salts, and ptylin. [NIH] Salivary: The duct that convey saliva to the mouth. [NIH] Salivary glands: Glands in the mouth that produce saliva. [NIH] Scans: Pictures of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body. [NIH] Schizoid: Having qualities resembling those found in greater degree in schizophrenics; a person of schizoid personality. [NIH] Schizophrenia: A mental disorder characterized by a special type of disintegration of the personality. [NIH] Schizotypal Personality Disorder: A personality disorder in which there are oddities of thought (magical thinking, paranoid ideation, suspiciousness), perception (illusions, depersonalization), speech (digressive, vague, overelaborate), and behavior (inappropriate affect in social interactions, frequently social isolation) that are not severe enough to characterize schizophrenia. [NIH] Sclerosis: A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve. [NIH] Screening: Checking for disease when there are no symptoms. [NIH] Second Messenger Systems: Systems in which an intracellular signal is generated in response to an intercellular primary messenger such as a hormone or neurotransmitter. They are intermediate signals in cellular processes such as metabolism, secretion, contraction, phototransduction, and cell growth. Examples of second messenger systems are the adenyl cyclase-cyclic AMP system, the phosphatidylinositol diphosphate-inositol triphosphate system, and the cyclic GMP system. [NIH] Secretion: 1. The process of elaborating a specific product as a result of the activity of a gland; this activity may range from separating a specific substance of the blood to the elaboration of a new chemical substance. 2. Any substance produced by secretion. [EU] Secretory: Secreting; relating to or influencing secretion or the secretions. [NIH] Segmental: Describing or pertaining to a structure which is repeated in similar form in successive segments of an organism, or which is undergoing segmentation. [NIH] Segmentation: The process by which muscles in the intestines move food and wastes through the body. [NIH] Segregation: The separation in meiotic cell division of homologous chromosome pairs and their contained allelomorphic gene pairs. [NIH] Seizures: Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as epilepsy or "seizure disorder." [NIH]

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Self Care: Performance of activities or tasks traditionally performed by professional health care providers. The concept includes care of oneself or one's family and friends. [NIH] Self-Injurious Behavior: Behavior in which persons hurt or harm themselves without the motive of suicide or of sexual deviation. [NIH] Semen: The thick, yellowish-white, viscid fluid secretion of male reproductive organs discharged upon ejaculation. In addition to reproductive organ secretions, it contains spermatozoa and their nutrient plasma. [NIH] Semicircular canal: Three long canals of the bony labyrinth of the ear, forming loops and opening into the vestibule by five openings. [NIH] Semisynthetic: Produced by chemical manipulation of naturally occurring substances. [EU] Senile: Relating or belonging to old age; characteristic of old age; resulting from infirmity of old age. [NIH] Septic: Produced by or due to decomposition by microorganisms; putrefactive. [EU] Sequence Analysis: A multistage process that includes the determination of a sequence (protein, carbohydrate, etc.), its fragmentation and analysis, and the interpretation of the resulting sequence information. [NIH] Sequencing: The determination of the order of nucleotides in a DNA or RNA chain. [NIH] Serine: A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from glycine or threonine. It is involved in the biosynthesis of purines, pyrimidines, and other amino acids. [NIH] Serotonin: A biochemical messenger and regulator, synthesized from the essential amino acid L-tryptophan. In humans it is found primarily in the central nervous system, gastrointestinal tract, and blood platelets. Serotonin mediates several important physiological functions including neurotransmission, gastrointestinal motility, hemostasis, and cardiovascular integrity. Multiple receptor families (receptors, serotonin) explain the broad physiological actions and distribution of this biochemical mediator. [NIH] Sertraline: A selective serotonin uptake inhibitor that is used in the treatment of depression. [NIH]

Sertraline Hydrochloride: Selective serotonin uptake inhibitor. [NIH] Serum: The clear liquid part of the blood that remains after blood cells and clotting proteins have been removed. [NIH] Sex Characteristics: Those characteristics that distinguish one sex from the other. The primary sex characteristics are the ovaries and testes and their related hormones. Secondary sex characteristics are those which are masculine or feminine but not directly related to reproduction. [NIH] Sex Education: Education which increases the knowledge of the functional, structural, and behavioral aspects of human reproduction. [NIH] Sexual Abstinence: Refraining from sexual intercourse. [NIH] Shock: The general bodily disturbance following a severe injury; an emotional or moral upset occasioned by some disturbing or unexpected experience; disruption of the circulation, which can upset all body functions: sometimes referred to as circulatory shock. [NIH]

Shunt: A surgically created diversion of fluid (e.g., blood or cerebrospinal fluid) from one area of the body to another area of the body. [NIH] Sialyltransferases: A group of enzymes with the general formula CMP-Nacetylneuraminate:acceptor N-acetylneuraminyl transferase. They catalyze the transfer of N-

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acetylneuraminic acid from CMP-N-acetylneuraminic acid to an acceptor, which is usually the terminal sugar residue of an oligosaccharide, a glycoprotein, or a glycolipid. EC 2.4.99.-. [NIH]

Sibling Relations: Interactions and relationships between sisters and/or brothers. The concept also applies to animal studies. [NIH] Side effect: A consequence other than the one(s) for which an agent or measure is used, as the adverse effects produced by a drug, especially on a tissue or organ system other than the one sought to be benefited by its administration. [EU] Signal Transduction: The intercellular or intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GABA-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptormediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway. [NIH] Signs and Symptoms: Clinical manifestations that can be either objective when observed by a physician, or subjective when perceived by the patient. [NIH] Skeletal: Having to do with the skeleton (boney part of the body). [NIH] Skeleton: The framework that supports the soft tissues of vertebrate animals and protects many of their internal organs. The skeletons of vertebrates are made of bone and/or cartilage. [NIH] Skin Abnormalities: Congenital structural abnormalities of the skin. [NIH] Skull: The skeleton of the head including the bones of the face and the bones enclosing the brain. [NIH] Sleep Stages: Periods of sleep manifested by changes in EEG activity and certain behavioral correlates; includes Stage 1: sleep onset, drowsy sleep; Stage 2: light sleep; Stages 3 and 4: delta sleep, light sleep, deep sleep, telencephalic sleep. [NIH] Small intestine: The part of the digestive tract that is located between the stomach and the large intestine. [NIH] Smooth muscle: Muscle that performs automatic tasks, such as constricting blood vessels. [NIH]

Social Behavior: Any behavior caused by or affecting another individual, usually of the same species. [NIH] Social Change: Social process whereby the values, attitudes, or institutions of society, such as education, family, religion, and industry become modified. It includes both the natural process and action programs initiated by members of the community. [NIH] Social Conditions: The state of society as it exists or in flux. While it usually refers to society as a whole in a specified geographical or political region, it is applicable also to restricted strata of a society. [NIH] Social Environment: The aggregate of social and cultural institutions, forms, patterns, and processes that influence the life of an individual or community. [NIH]

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Social Support: Support systems that provide assistance and encouragement to individuals with physical or emotional disabilities in order that they may better cope. Informal social support is usually provided by friends, relatives, or peers, while formal assistance is provided by churches, groups, etc. [NIH] Social Work: The use of community resources, individual case work, or group work to promote the adaptive capacities of individuals in relation to their social and economic environments. It includes social service agencies. [NIH] Sodium: An element that is a member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23. With a valence of 1, it has a strong affinity for oxygen and other nonmetallic elements. Sodium provides the chief cation of the extracellular body fluids. Its salts are the most widely used in medicine. (From Dorland, 27th ed) Physiologically the sodium ion plays a major role in blood pressure regulation, maintenance of fluid volume, and electrolyte balance. [NIH] Sodium Channels: Cell membrane glycoproteins selective for sodium ions. Fast sodium current is associated with the action potential in neural membranes. [NIH] Soft tissue: Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body. [NIH] Solid tumor: Cancer of body tissues other than blood, bone marrow, or the lymphatic system. [NIH] Soma: The body as distinct from the mind; all the body tissue except the germ cells; all the axial body. [NIH] Somatic: 1. Pertaining to or characteristic of the soma or body. 2. Pertaining to the body wall in contrast to the viscera. [EU] Somatic cells: All the body cells except the reproductive (germ) cells. [NIH] Sorbitol: A polyhydric alcohol with about half the sweetness of sucrose. Sorbitol occurs naturally and is also produced synthetically from glucose. It was formerly used as a diuretic and may still be used as a laxative and in irrigating solutions for some surgical procedures. It is also used in many manufacturing processes, as a pharmaceutical aid, and in several research applications. [NIH] Spastic: 1. Of the nature of or characterized by spasms. 2. Hypertonic, so that the muscles are stiff and the movements awkward. 3. A person exhibiting spasticity, such as occurs in spastic paralysis or in cerebral palsy. [EU] Spasticity: A state of hypertonicity, or increase over the normal tone of a muscle, with heightened deep tendon reflexes. [EU] Spatial disorientation: Loss of orientation in space where person does not know which way is up. [NIH] Specialist: In medicine, one who concentrates on 1 special branch of medical science. [NIH] Species: A taxonomic category subordinate to a genus (or subgenus) and superior to a subspecies or variety, composed of individuals possessing common characters distinguishing them from other categories of individuals of the same taxonomic level. In taxonomic nomenclature, species are designated by the genus name followed by a Latin or Latinized adjective or noun. [EU] Specificity: Degree of selectivity shown by an antibody with respect to the number and types of antigens with which the antibody combines, as well as with respect to the rates and the extents of these reactions. [NIH] Spectrin: A high molecular weight (220-250 kDa) water-soluble protein which can be

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extracted from erythrocyte ghosts in low ionic strength buffers. The protein contains no lipids or carbohydrates, is the predominant species of peripheral erythrocyte membrane proteins, and exists as a fibrous coating on the inner, cytoplasmic surface of the membrane. [NIH]

Spectrum: A charted band of wavelengths of electromagnetic vibrations obtained by refraction and diffraction. By extension, a measurable range of activity, such as the range of bacteria affected by an antibiotic (antibacterial s.) or the complete range of manifestations of a disease. [EU] Speech pathologist: A specialist who evaluates and treats people with communication and swallowing problems. Also called a speech therapist. [NIH] Sperm: The fecundating fluid of the male. [NIH] Spinal cord: The main trunk or bundle of nerves running down the spine through holes in the spinal bone (the vertebrae) from the brain to the level of the lower back. [NIH] Spinal Cord Diseases: Pathologic conditions which feature spinal cord damage or dysfunction, including disorders involving the meninges and perimeningeal spaces surrounding the spinal cord. Traumatic injuries, vascular diseases, infections, and inflammatory/autoimmune processes may affect the spinal cord. [NIH] Spinal Nerves: The 31 paired peripheral nerves formed by the union of the dorsal and ventral spinal roots from each spinal cord segment. The spinal nerve plexuses and the spinal roots are also included. [NIH] Spleen: An organ that is part of the lymphatic system. The spleen produces lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach. [NIH] Sporadic: Neither endemic nor epidemic; occurring occasionally in a random or isolated manner. [EU] Stabilization: The creation of a stable state. [EU] Staging: Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. [NIH]

Statistically significant: Describes a mathematical measure of difference between groups. The difference is said to be statistically significant if it is greater than what might be expected to happen by chance alone. [NIH] Status Epilepticus: Repeated and prolonged epileptic seizures without recovery of consciousness between attacks. [NIH] Steel: A tough, malleable, iron-based alloy containing up to, but no more than, two percent carbon and often other metals. It is used in medicine and dentistry in implants and instrumentation. [NIH] Stereotyped Behavior: Relatively invariant mode of behavior elicited or determined by a particular situation; may be verbal, postural, or expressive. [NIH] Stereotypy: Unvarying repetition or unvarying persistence. [NIH] Sterility: 1. The inability to produce offspring, i.e., the inability to conceive (female s.) or to induce conception (male s.). 2. The state of being aseptic, or free from microorganisms. [EU] Sterilization: The destroying of all forms of life, especially microorganisms, by heat, chemical, or other means. [NIH] Steroid: A group name for lipids that contain a hydrogenated cyclopentanoperhydrophenanthrene ring system. Some of the substances included in this

Dictionary 309

group are progesterone, adrenocortical hormones, the gonadal hormones, cardiac aglycones, bile acids, sterols (such as cholesterol), toad poisons, saponins, and some of the carcinogenic hydrocarbons. [EU] Stimulant: 1. Producing stimulation; especially producing stimulation by causing tension on muscle fibre through the nervous tissue. 2. An agent or remedy that produces stimulation. [EU]

Stimulus: That which can elicit or evoke action (response) in a muscle, nerve, gland or other excitable issue, or cause an augmenting action upon any function or metabolic process. [NIH] Stomach: An organ of digestion situated in the left upper quadrant of the abdomen between the termination of the esophagus and the beginning of the duodenum. [NIH] Stool: The waste matter discharged in a bowel movement; feces. [NIH] Streptococci: A genus of spherical Gram-positive bacteria occurring in chains or pairs. They are widely distributed in nature, being important pathogens but often found as normal commensals in the mouth, skin, and intestine of humans and other animals. [NIH] Stress: Forcibly exerted influence; pressure. Any condition or situation that causes strain or tension. Stress may be either physical or psychologic, or both. [NIH] Stria: 1. A streak, or line. 2. A narrow bandlike structure; a general term for such longitudinal collections of nerve fibres in the brain. [EU] Stroke: Sudden loss of function of part of the brain because of loss of blood flow. Stroke may be caused by a clot (thrombosis) or rupture (hemorrhage) of a blood vessel to the brain. [NIH] Stroma: The middle, thickest layer of tissue in the cornea. [NIH] Subacute: Somewhat acute; between acute and chronic. [EU] Subclinical: Without clinical manifestations; said of the early stage(s) of an infection or other disease or abnormality before symptoms and signs become apparent or detectable by clinical examination or laboratory tests, or of a very mild form of an infection or other disease or abnormality. [EU] Subcutaneous: Beneath the skin. [NIH] Subiculum: A region of the hippocampus that projects to other areas of the brain. [NIH] Submandibular: Four to six lymph glands, located between the lower jaw and the submandibular salivary gland. [NIH] Submaxillary: Four to six lymph glands, located between the lower jaw and the submandibular salivary gland. [NIH] Subspecies: A category intermediate in rank between species and variety, based on a smaller number of correlated characters than are used to differentiate species and generally conditioned by geographical and/or ecological occurrence. [NIH] Supplementation: Adding nutrients to the diet. [NIH] Support group: A group of people with similar disease who meet to discuss how better to cope with their cancer and treatment. [NIH] Suppositories: A small cone-shaped medicament having cocoa butter or gelatin at its basis and usually intended for the treatment of local conditions in the rectum. [NIH] Suspensions: Colloids with liquid continuous phase and solid dispersed phase; the term is used loosely also for solid-in-gas (aerosol) and other colloidal systems; water-insoluble drugs may be given as suspensions. [NIH] Sympathomimetic: 1. Mimicking the effects of impulses conveyed by adrenergic postganglionic fibres of the sympathetic nervous system. 2. An agent that produces effects

310 Mental Retardation

similar to those of impulses conveyed by adrenergic postganglionic fibres of the sympathetic nervous system. Called also adrenergic. [EU] Symphysis: A secondary cartilaginous joint. [NIH] Synapses: Specialized junctions at which a neuron communicates with a target cell. At classical synapses, a neuron's presynaptic terminal releases a chemical transmitter stored in synaptic vesicles which diffuses across a narrow synaptic cleft and activates receptors on the postsynaptic membrane of the target cell. The target may be a dendrite, cell body, or axon of another neuron, or a specialized region of a muscle or secretory cell. Neurons may also communicate through direct electrical connections which are sometimes called electrical synapses; these are not included here but rather in gap junctions. [NIH] Synapsis: The pairing between homologous chromosomes of maternal and paternal origin during the prophase of meiosis, leading to the formation of gametes. [NIH] Synaptic: Pertaining to or affecting a synapse (= site of functional apposition between neurons, at which an impulse is transmitted from one neuron to another by electrical or chemical means); pertaining to synapsis (= pairing off in point-for-point association of homologous chromosomes from the male and female pronuclei during the early prophase of meiosis). [EU] Synaptic Membranes: Cell membranes associated with synapses. Both presynaptic and postsynaptic membranes are included along with their integral or tightly associated specializations for the release or reception of transmitters. [NIH] Synaptic Vesicles: Membrane-bound compartments which contain transmitter molecules. Synaptic vesicles are concentrated at presynaptic terminals. They actively sequester transmitter molecules from the cytoplasm. In at least some synapses, transmitter release occurs by fusion of these vesicles with the presynaptic membrane, followed by exocytosis of their contents. [NIH] Synergistic: Acting together; enhancing the effect of another force or agent. [EU] Systemic: Affecting the entire body. [NIH] Systolic: Indicating the maximum arterial pressure during contraction of the left ventricle of the heart. [EU] Tardive: Marked by lateness, late; said of a disease in which the characteristic lesion is late in appearing. [EU] Telencephalon: Paired anteriolateral evaginations of the prosencephalon plus the lamina terminalis. The cerebral hemispheres are derived from it. Many authors consider cerebrum a synonymous term to telencephalon, though a minority include diencephalon as part of the cerebrum (Anthoney, 1994). [NIH] Telomere: A terminal section of a chromosome which has a specialized structure and which is involved in chromosomal replication and stability. Its length is believed to be a few hundred base pairs. [NIH] Temporal: One of the two irregular bones forming part of the lateral surfaces and base of the skull, and containing the organs of hearing. [NIH] Teratogens: An agent that causes the production of physical defects in the developing embryo. [NIH] Testicular: Pertaining to a testis. [EU] Testis: Either of the paired male reproductive glands that produce the male germ cells and the male hormones. [NIH] Thalamic: Cell that reaches the lateral nucleus of amygdala. [NIH]

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Thalamic Diseases: Disorders of the centrally located thalamus, which integrates a wide range of cortical and subcortical information. Manifestations include sensory loss, movement disorders; ataxia, pain syndromes, visual disorders, a variety of neuropsychological conditions, and coma. Relatively common etiologies include cerebrovascular disorders; craniocerebral trauma; brain neoplasms; brain hypoxia; intracranial hemorrhages; and infectious processes. [NIH] Thalassemia: A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia. [NIH] Therapeutics: The branch of medicine which is concerned with the treatment of diseases, palliative or curative. [NIH] Threonine: An essential amino acid occurring naturally in the L-form, which is the active form. It is found in eggs, milk, gelatin, and other proteins. [NIH] Threshold: For a specified sensory modality (e. g. light, sound, vibration), the lowest level (absolute threshold) or smallest difference (difference threshold, difference limen) or intensity of the stimulus discernible in prescribed conditions of stimulation. [NIH] Thrombin: An enzyme formed from prothrombin that converts fibrinogen to fibrin. (Dorland, 27th ed) EC 3.4.21.5. [NIH] Thrombomodulin: A cell surface glycoprotein of endothelial cells that binds thrombin and serves as a cofactor in the activation of protein C and its regulation of blood coagulation. [NIH]

Thrombosis: The formation or presence of a blood clot inside a blood vessel. [NIH] Thrombus: An aggregation of blood factors, primarily platelets and fibrin with entrapment of cellular elements, frequently causing vascular obstruction at the point of its formation. Some authorities thus differentiate thrombus formation from simple coagulation or clot formation. [EU] Thyroid: A gland located near the windpipe (trachea) that produces thyroid hormone, which helps regulate growth and metabolism. [NIH] Thyrotropin: A peptide hormone secreted by the anterior pituitary. It promotes the growth of the thyroid gland and stimulates the synthesis of thyroid hormones and the release of thyroxine by the thyroid gland. [NIH] Thyroxine: An amino acid of the thyroid gland which exerts a stimulating effect on thyroid metabolism. [NIH] Tic: An involuntary compulsive, repetitive, stereotyped movement, resembling a purposeful movement because it is coordinated and involves muscles in their normal synergistic relationships; tics usually involve the face and shoulders. [EU] Tin: A trace element that is required in bone formation. It has the atomic symbol Sn, atomic number 50, and atomic weight 118.71. [NIH] Tinnitus: Sounds that are perceived in the absence of any external noise source which may take the form of buzzing, ringing, clicking, pulsations, and other noises. Objective tinnitus refers to noises generated from within the ear or adjacent structures that can be heard by other individuals. The term subjective tinnitus is used when the sound is audible only to the affected individual. Tinnitus may occur as a manifestation of cochlear diseases; vestibulocochlear nerve diseases; intracranial hypertension; craniocerebral trauma; and other conditions. [NIH] Tissue: A group or layer of cells that are alike in type and work together to perform a

312 Mental Retardation

specific function. [NIH] Tissue Culture: Maintaining or growing of tissue, organ primordia, or the whole or part of an organ in vitro so as to preserve its architecture and/or function (Dorland, 28th ed). Tissue culture includes both organ culture and cell culture. [NIH] Toilet Training: Conditioning to defecate and urinate in culturally acceptable places. [NIH] Tomography: Imaging methods that result in sharp images of objects located on a chosen plane and blurred images located above or below the plane. [NIH] Tonicity: The normal state of muscular tension. [NIH] Tooth Loss: The failure to retain teeth as a result of disease or injury. [NIH] Tooth Preparation: Procedures carried out with regard to the teeth or tooth structures preparatory to specified dental therapeutic and surgical measures. [NIH] Topical: On the surface of the body. [NIH] Toxic: Having to do with poison or something harmful to the body. Toxic substances usually cause unwanted side effects. [NIH] Toxicity: The quality of being poisonous, especially the degree of virulence of a toxic microbe or of a poison. [EU] Toxicology: The science concerned with the detection, chemical composition, and pharmacologic action of toxic substances or poisons and the treatment and prevention of toxic manifestations. [NIH] Toxins: Specific, characterizable, poisonous chemicals, often proteins, with specific biological properties, including immunogenicity, produced by microbes, higher plants, or animals. [NIH] Traction: The act of pulling. [NIH] Transaminases: A subclass of enzymes of the transferase class that catalyze the transfer of an amino group from a donor (generally an amino acid) to an acceptor (generally a 2-keto acid). Most of these enzymes are pyridoxyl phosphate proteins. (Dorland, 28th ed) EC 2.6.1. [NIH]

Transcription Factors: Endogenous substances, usually proteins, which are effective in the initiation, stimulation, or termination of the genetic transcription process. [NIH] Transcutaneous: Transdermal. [EU] Transcutaneous Electric Nerve Stimulation: Electrical stimulation of nerves and/or muscles to relieve pain; it is used less frequently to produce anesthesia. The optimal placements of electrodes or "trigger points" may correspond with acupuncture analgesia points. TENS is sometimes referred to as acupuncture-like when using a low frequency stimulus. [NIH] Transduction: The transfer of genes from one cell to another by means of a viral (in the case of bacteria, a bacteriophage) vector or a vector which is similar to a virus particle (pseudovirion). [NIH] Transfection: The uptake of naked or purified DNA into cells, usually eukaryotic. It is analogous to bacterial transformation. [NIH] Transferases: Transferases are enzymes transferring a group, for example, the methyl group or a glycosyl group, from one compound (generally regarded as donor) to another compound (generally regarded as acceptor). The classification is based on the scheme "donor:acceptor group transferase". (Enzyme Nomenclature, 1992) EC 2. [NIH] Transgenes: Genes that are introduced into an organism using gene transfer techniques. [NIH]

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Translation: The process whereby the genetic information present in the linear sequence of ribonucleotides in mRNA is converted into a corresponding sequence of amino acids in a protein. It occurs on the ribosome and is unidirectional. [NIH] Translational: The cleavage of signal sequence that directs the passage of the protein through a cell or organelle membrane. [NIH] Translocation: The movement of material in solution inside the body of the plant. [NIH] Transmitter: A chemical substance which effects the passage of nerve impulses from one cell to the other at the synapse. [NIH] Transplantation: Transference of a tissue or organ, alive or dead, within an individual, between individuals of the same species, or between individuals of different species. [NIH] Trauma: Any injury, wound, or shock, must frequently physical or structural shock, producing a disturbance. [NIH] Tremor: Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of cerebellar diseases, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of Parkinson disease. [NIH] Trigger zone: Dolorogenic zone (= producing or causing pain). [EU] Trinucleotide Repeat Expansion: DNA region comprised of a variable number of repetitive, contiguous trinucleotide sequences. The presence of these regions is associated with diseases such as Fragile X Syndrome and myotonic dystrophy. Many chromosome fragile sites (chromosome fragility) contain expanded trinucleotide repeats. [NIH] Trinucleotide Repeats: Microsatellite repeats consisting of three nucleotides dispersed in the euchromatic arms of chromosomes. [NIH] Trisomy: The possession of a third chromosome of any one type in an otherwise diploid cell. [NIH]

Trophoblast: The outer layer of cells of the blastocyst which works its way into the endometrium during ovum implantation and grows rapidly, later combining with mesoderm. [NIH] Tryptophan: An essential amino acid that is necessary for normal growth in infants and for nitrogen balance in adults. It is a precursor serotonin and niacin. [NIH] Tubulin: A microtubule subunit protein found in large quantities in mammalian brain. It has also been isolated from sperm flagella, cilia, and other sources. Structurally, the protein is a dimer with a molecular weight of approximately 120,000 and a sedimentation coefficient of 5.8S. It binds to colchicine, vincristine, and vinblastine. [NIH] Type 2 diabetes: Usually characterized by a gradual onset with minimal or no symptoms of metabolic disturbance and no requirement for exogenous insulin. The peak age of onset is 50 to 60 years. Obesity and possibly a genetic factor are usually present. [NIH] Tyrosine: A non-essential amino acid. In animals it is synthesized from phenylalanine. It is also the precursor of epinephrine, thyroid hormones, and melanin. [NIH] Ubiquitin: A highly conserved 76 amino acid-protein found in all eukaryotic cells. [NIH] Ultrasonography: The visualization of deep structures of the body by recording the reflections of echoes of pulses of ultrasonic waves directed into the tissues. Use of ultrasound for imaging or diagnostic purposes employs frequencies ranging from 1.6 to 10 megahertz. [NIH] Unconscious: Experience which was once conscious, but was subsequently rejected, as the

314 Mental Retardation

"personal unconscious". [NIH] Urea: A compound (CO(NH2)2), formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. [NIH] Ureters: Tubes that carry urine from the kidneys to the bladder. [NIH] Urethra: The tube through which urine leaves the body. It empties urine from the bladder. [NIH]

Urinary: Having to do with urine or the organs of the body that produce and get rid of urine. [NIH] Urinary Retention: Inability to urinate. The etiology of this disorder includes obstructive, neurogenic, pharmacologic, and psychogenic causes. [NIH] Urinary tract: The organs of the body that produce and discharge urine. These include the kidneys, ureters, bladder, and urethra. [NIH] Urinary tract infection: An illness caused by harmful bacteria growing in the urinary tract. [NIH]

Urinate: To release urine from the bladder to the outside. [NIH] Urine: Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra. [NIH] Urogenital: Pertaining to the urinary and genital apparatus; genitourinary. [EU] Uterus: The small, hollow, pear-shaped organ in a woman's pelvis. This is the organ in which a fetus develops. Also called the womb. [NIH] Vaccine: A substance or group of substances meant to cause the immune system to respond to a tumor or to microorganisms, such as bacteria or viruses. [NIH] Vagina: The muscular canal extending from the uterus to the exterior of the body. Also called the birth canal. [NIH] Valproic Acid: A fatty acid with anticonvulsant properties used in the treatment of epilepsy. The mechanisms of its therapeutic actions are not well understood. It may act by increasing GABA levels in the brain or by altering the properties of voltage dependent sodium channels. [NIH] Vascular: Pertaining to blood vessels or indicative of a copious blood supply. [EU] Vasodilator: An agent that widens blood vessels. [NIH] Vector: Plasmid or other self-replicating DNA molecule that transfers DNA between cells in nature or in recombinant DNA technology. [NIH] Vein: Vessel-carrying blood from various parts of the body to the heart. [NIH] Venous: Of or pertaining to the veins. [EU] Venous blood: Blood that has given up its oxygen to the tissues and carries carbon dioxide back for gas exchange. [NIH] Ventricle: One of the two pumping chambers of the heart. The right ventricle receives oxygen-poor blood from the right atrium and pumps it to the lungs through the pulmonary artery. The left ventricle receives oxygen-rich blood from the left atrium and pumps it to the body through the aorta. [NIH] Ventricular: Pertaining to a ventricle. [EU] Venules: The minute vessels that collect blood from the capillary plexuses and join together to form veins. [NIH]

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Vertebrae: A bony unit of the segmented spinal column. [NIH] Vestibular: Pertaining to or toward a vestibule. In dental anatomy, used to refer to the tooth surface directed toward the vestibule of the mouth. [EU] Vestibule: A small, oval, bony chamber of the labyrinth. The vestibule contains the utricle and saccule, organs which are part of the balancing apparatus of the ear. [NIH] Vestibulocochlear Nerve: The 8th cranial nerve. The vestibulocochlear nerve has a cochlear part (cochlear nerve) which is concerned with hearing and a vestibular part (vestibular nerve) which mediates the sense of balance and head position. The fibers of the cochlear nerve originate from neurons of the spiral ganglion and project to the cochlear nuclei (cochlear nucleus). The fibers of the vestibular nerve arise from neurons of Scarpa's ganglion and project to the vestibular nuclei. [NIH] Vestibulocochlear Nerve Diseases: Diseases of the vestibular and/or cochlear (acoustic) nerves, which join to form the vestibulocochlear nerve. Vestibular neuritis, cochlear neuritis, and acoustic neuromas are relatively common conditions that affect these nerves. Clinical manifestations vary with which nerve is primarily affected, and include hearing loss, vertigo, and tinnitus. [NIH] Veterinary Medicine: The medical science concerned with the prevention, diagnosis, and treatment of diseases in animals. [NIH] Villi: The tiny, fingerlike projections on the surface of the small intestine. Villi help absorb nutrients. [NIH] Vinblastine: An anticancer drug that belongs to the family of plant drugs called vinca alkaloids. It is a mitotic inhibitor. [NIH] Vincristine: An anticancer drug that belongs to the family of plant drugs called vinca alkaloids. [NIH] Viral: Pertaining to, caused by, or of the nature of virus. [EU] Viral vector: A type of virus used in cancer therapy. The virus is changed in the laboratory and cannot cause disease. Viral vectors produce tumor antigens (proteins found on a tumor cell) and can stimulate an antitumor immune response in the body. Viral vectors may also be used to carry genes that can change cancer cells back to normal cells. [NIH] Virulence: The degree of pathogenicity within a group or species of microorganisms or viruses as indicated by case fatality rates and/or the ability of the organism to invade the tissues of the host. [NIH] Virus: Submicroscopic organism that causes infectious disease. In cancer therapy, some viruses may be made into vaccines that help the body build an immune response to, and kill, tumor cells. [NIH] Virus Replication: The process of intracellular viral multiplication, consisting of the synthesis of proteins, nucleic acids, and sometimes lipids, and their assembly into a new infectious particle. [NIH] Viscera: Any of the large interior organs in any one of the three great cavities of the body, especially in the abdomen. [NIH] Visual Cortex: Area of the occipital lobe concerned with vision. [NIH] Visual field: The entire area that can be seen when the eye is forward, including peripheral vision. [NIH] Vitreous Body: The transparent, semigelatinous substance that fills the cavity behind the crystalline lens of the eye and in front of the retina. It is contained in a thin hyoid membrane and forms about four fifths of the optic globe. [NIH]

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Vitro: Descriptive of an event or enzyme reaction under experimental investigation occurring outside a living organism. Parts of an organism or microorganism are used together with artificial substrates and/or conditions. [NIH] Vivo: Outside of or removed from the body of a living organism. [NIH] Voice Disorders: Disorders of voice pitch, loudness, or quality. Dysphonia refers to impaired utterance of sounds by the vocal folds. [NIH] Wakefulness: A state in which there is an enhanced potential for sensitivity and an efficient responsiveness to external stimuli. [NIH] White blood cell: A type of cell in the immune system that helps the body fight infection and disease. White blood cells include lymphocytes, granulocytes, macrophages, and others. [NIH]

Withdrawal: 1. A pathological retreat from interpersonal contact and social involvement, as may occur in schizophrenia, depression, or schizoid avoidant and schizotypal personality disorders. 2. (DSM III-R) A substance-specific organic brain syndrome that follows the cessation of use or reduction in intake of a psychoactive substance that had been regularly used to induce a state of intoxication. [EU] Wound Healing: Restoration of integrity to traumatized tissue. [NIH] Xenograft: The cells of one species transplanted to another species. [NIH] X-ray: High-energy radiation used in low doses to diagnose diseases and in high doses to treat cancer. [NIH] Yeasts: A general term for single-celled rounded fungi that reproduce by budding. Brewers' and bakers' yeasts are Saccharomyces cerevisiae; therapeutic dried yeast is dried yeast. [NIH] Zygote: The fertilized ovum. [NIH] Zymogen: Inactive form of an enzyme which can then be converted to the active form, usually by excision of a polypeptide, e. g. trypsinogen is the zymogen of trypsin. [NIH]

317

INDEX 1 1-phosphate, 186, 187, 245 A Abdomen, 245, 254, 280, 283, 308, 309, 315 Abdominal, 245, 283, 293 Aberrant, 34, 42, 50, 62, 175, 245 Acceptor, 245, 293, 305, 312 Accommodation, 129, 245, 288 ACE, 52, 245 Acetylcholine, 186, 188, 245, 258, 291 Acidemia, 20, 245, 295 Acidosis, 102, 245 Acoustic, 203, 245, 315 Actin, 245, 287, 288 Actinin, 245, 268 Activities of Daily Living, 190, 229, 245, 302 Acupuncture Analgesia, 245, 312 Adaptability, 245, 256, 257 Adaptation, 10, 15, 18, 144, 245, 280, 291, 297 Adenine, 246, 301 Adenosine, 186, 188, 246, 281, 296 Adenovirus, 57, 246 Adenylate Cyclase, 174, 175, 180, 246 Adjustment, 62, 131, 142, 146, 158, 245, 246 Adolescence, 15, 53, 246, 294 Adrenal Cortex, 246, 262 Adrenal Glands, 246, 248 Adrenergic, 171, 174, 180, 186, 187, 188, 246, 250, 267, 269, 309 Adrenergic Agents, 174, 180, 246 Adrenoleukodystrophy, 35, 246, 295 Adverse Effect, 246, 259, 306 Aerobic, 105, 157, 246, 286, 293 Aerosol, 246, 309 Afferent, 246, 270 Affinity, 184, 246, 247, 251, 259, 307 Agar, 246, 296 Age Groups, 8, 247 Age of Onset, 165, 247, 254, 313 Aged, 80 and Over, 247 Agenesis, 98, 247 Agonist, 16, 32, 163, 247, 254, 267, 281, 288 Akathisia, 165, 166, 247, 250 Albumin, 57, 247, 297 Alexia, 247, 267

Algorithms, 247, 253 Alkaline, 245, 247, 248, 255 Alkaloid, 247, 254, 259 Alleles, 32, 36, 247 Allergen, 247, 265 Alternative medicine, 209, 247 Ameliorating, 26, 187, 247 Amenorrhea, 247, 249, 254 Amine, 247, 276 Amino Acid Sequence, 177, 248, 249, 270, 273, 299 Amino Acids, 179, 186, 188, 248, 273, 290, 295, 297, 299, 303, 305, 313, 314 Ammonia, 247, 248, 274, 277, 314 Amnesia, 172, 248 Amniotic Fluid, 248, 273 Amyloid, 37, 248 Amyloidosis, 8, 248 Anaesthesia, 248, 279 Anal, 69, 98, 99, 100, 101, 103, 112, 117, 120, 143, 248, 271, 283, 287 Analogous, 248, 267, 312 Analysis of Variance, 62, 248 Anatomical, 248, 268, 279, 294, 304 Anemia, 182, 208, 248, 272, 311 Anesthesia, 5, 248, 269, 312 Angina, 170, 173, 174, 175, 180, 182, 185, 187, 188, 248, 291 Angina Pectoris, 170, 173, 174, 175, 180, 182, 185, 187, 188, 248 Angiogenesis, 187, 249 Angioplasty, 187, 249 Animal model, 19, 25, 30, 46, 68, 249 Anions, 247, 249, 281 Anomalies, 13, 14, 22, 29, 46, 49, 51, 69, 74, 75, 76, 83, 85, 177, 249 Anorexia, 170, 173, 174, 175, 180, 182, 185, 187, 249 Anorexia Nervosa, 180, 249 Anoxia, 172, 249 Antagonism, 249, 259 Anterior chamber, 249, 281 Antibacterial, 249, 308 Antibiotic, 186, 249, 308 Antibodies, 176, 185, 249, 275, 278, 284, 287, 297 Antibody, 12, 229, 246, 249, 260, 275, 276, 278, 279, 285, 287, 307

318 Mental Retardation

Anticoagulant, 249, 299 Anticonvulsant, 249, 314 Antiemetic, 249, 250 Antigen, 246, 249, 260, 276, 277, 278, 279, 284 Anti-infective, 250, 257 Anti-inflammatory, 250, 294 Antioxidant, 250, 251 Antipsychotic, 50, 171, 250, 259, 290, 303 Antipsychotic Agents, 50, 250 Anxiety, 9, 76, 84, 116, 154, 170, 171, 173, 174, 175, 178, 180, 182, 185, 187, 188, 247, 250 Apathy, 250, 290 Aphasia, 173, 250 Apoptosis, 20, 28, 38, 250, 256 Aqueous, 171, 250, 252, 263, 268, 282 Arachidonic Acid, 250, 282 Arginase, 38, 46, 251 Arginine, 251, 291 Arterial, 251, 257, 277, 291, 299, 310 Arteries, 251, 254, 262, 281, 286, 288 Arterioles, 251, 254, 288 Ascorbic Acid, 177, 251 Aseptic, 251, 293, 308 Aspartate, 19, 184, 251 Assay, 32, 37, 38, 48, 74, 172, 251 Astrocytes, 18, 251 Asymptomatic, 23, 251 Ataxia, 14, 35, 66, 83, 87, 251, 257, 277, 311 Atrophy, 14, 74, 83, 98, 108, 177, 251, 283, 289, 290, 292 Attenuated, 99, 251 Atypical, 23, 36, 50, 62, 99, 165, 166, 251, 259, 303 Audiologist, 228, 251 Auditory, 25, 68, 118, 119, 157, 251, 275 Authorship, 100, 251 Autoimmune disease, 251, 252, 287 Autoimmunity, 175, 182, 252 Autonomic, 148, 245, 250, 252, 291, 295 Autonomic Nervous System, 252, 295 Axonal, 28, 55, 252 Axons, 18, 34, 55, 252, 265, 292 B Bacteria, 35, 249, 250, 252, 253, 265, 270, 286, 308, 309, 312, 314 Bacterial Physiology, 246, 252 Bacteriophage, 252, 296, 312 Bacterium, 252, 260, 276 Basal Ganglia, 163, 250, 251, 252, 254, 258 Basal Ganglia Diseases, 251, 252, 258

Base, 39, 171, 179, 246, 252, 255, 264, 272, 273, 281, 297, 310 Base Sequence, 252, 272, 273 Basement Membrane, 252, 270 Behavioral Sciences, 21, 112, 252 Behavioral Symptoms, 166, 252 Benign, 45, 164, 170, 173, 174, 175, 180, 182, 185, 187, 188, 252, 253, 254, 275, 289 Benign tumor, 164, 253 Beta-pleated, 248, 253 Bewilderment, 253, 261 Bilateral, 253, 294, 298, 303 Bile, 29, 253, 272, 283, 309 Bile Acids, 29, 253, 309 Bile Acids and Salts, 253 Bilirubin, 247, 253 Biochemical, 18, 29, 34, 35, 40, 58, 102, 164, 247, 253, 273, 274, 282, 305 Biogenesis, 35, 253 Biological Sciences, 21, 253 Biological therapy, 253, 275 Biophysics, 34, 253 Biosynthesis, 59, 251, 253, 263, 305 Biotechnology, 64, 65, 198, 209, 217, 253 Bipolar Disorder, 30, 165, 253 Bladder, 253, 279, 287, 289, 299, 314 Blastocyst, 28, 253, 261, 313 Blood Platelets, 253, 305 Blood pressure, 179, 254, 255, 257, 277, 278, 287, 307 Blood vessel, 164, 245, 249, 254, 255, 256, 257, 258, 269, 276, 281, 283, 306, 307, 309, 311, 314 Blot, 39, 254 Body Fluids, 254, 267, 297, 307 Bone Marrow, 254, 284, 288, 307 Bone scan, 254, 304 Bowel, 175, 182, 248, 254, 262, 266, 280, 282, 309 Bowel Movement, 254, 262, 266, 309 Bradykinin, 254, 291, 297 Brain Diseases, 254, 294, 301 Brain Neoplasms, 254, 277, 311 Brain Stem, 254, 257, 301 Branch, 227, 241, 254, 263, 268, 290, 294, 301, 307, 311 Breakdown, 200, 254, 266, 272 Bromocriptine, 163, 254 Bronchial, 254, 276 Bruxism, 200, 254 Bulbar, 255, 301

Index 319

Bulimia, 170, 173, 174, 175, 180, 182, 185, 187, 255 Butyric Acid, 177, 255 C Cachexia, 175, 180, 182, 255 Calcification, 97, 255 Calcium, 255, 260, 277, 286, 300, 306 Canonical, 49, 255 Capsules, 171, 179, 255, 267, 273 Carbohydrate, 51, 255, 274, 298, 305 Carbon Dioxide, 255, 264, 271, 314 Carcinogenic, 255, 279, 299, 309 Carcinoma, 179, 255 Cardiomyopathy, 255 Cardiovascular, 76, 175, 182, 185, 255, 282, 305 Cardiovascular Abnormalities, 185, 255 Cardiovascular disease, 76, 175, 182, 255 Cardiovascular System, 255 Carotene, 256, 303 Case report, 4, 5, 78, 98, 105, 256, 259 Caspase, 38, 256 Cataracts, 66, 94, 104, 256 Catecholamine, 256, 267 Catheterization, 249, 256 Caveolae, 38, 256 Caveolins, 256 Cell Adhesion, 18, 31, 256 Cell Adhesion Molecules, 31, 256 Cell Cycle, 28, 256 Cell Death, 46, 250, 256, 289 Cell Differentiation, 175, 183, 256, 306 Cell Division, 30, 252, 256, 257, 263, 275, 285, 286, 296, 299, 304 Cell membrane, 17, 184, 256, 265, 272, 281, 296, 307, 310 Cell Membrane Structures, 256 Cell proliferation, 175, 183, 256, 306 Cell Respiration, 257, 286, 293 Cell Survival, 257, 275 Central Nervous System Infections, 257, 275, 277 Ceramide, 38, 257 Cerebellar, 19, 25, 66, 74, 87, 94, 96, 108, 251, 257, 302, 313 Cerebellar Diseases, 251, 257, 313 Cerebellum, 34, 49, 177, 254, 257, 302 Cerebral Infarction, 257, 277 Cerebral Palsy, 4, 119, 160, 172, 183, 201, 257, 307 Cerebrospinal, 257, 277, 305 Cerebrospinal fluid, 257, 277, 305

Cerebrovascular, 252, 255, 257, 311 Cerebrum, 257, 310 Character, 248, 257, 258, 264 Chemoreceptor, 250, 257 Child Care, 55, 206, 257 Chlorhexidine, 4, 257 Cholesterol, 29, 38, 164, 253, 256, 257, 262, 309 Cholinergic, 250, 258 Chondrodysplasia Punctata, 258, 295 Chondroitin sulfate, 17, 258 Chorea, 250, 258 Chorioretinitis, 258, 303 Choroid, 258, 303 Chromaffin System, 258, 269 Chromatin, 32, 250, 258, 291 Chromosomal, 13, 22, 41, 51, 60, 88, 93, 101, 104, 178, 185, 208, 232, 258, 273, 287, 310 Chromosome Aberrations, 24, 40, 258 Chromosome Abnormalities, 166, 258 Chromosome Fragility, 258, 313 Chronic, 4, 23, 42, 59, 68, 175, 182, 203, 228, 255, 258, 266, 279, 283, 292, 300, 309 Chronic Disease, 175, 182, 255, 258 Circulatory system, 258, 269, 280 CIS, 28, 258, 303 Citrus, 251, 259 Clamp, 19, 259 Clear cell carcinoma, 259, 265 Cleft Palate, 95, 203, 259 Clinical Medicine, 259, 298 Clinical study, 259, 262 Clinical trial, 13, 35, 57, 163, 167, 217, 259, 262, 300, 301 Clone, 24, 48, 180, 259 Cloning, 37, 42, 47, 51, 59, 110, 170, 187, 196, 253, 259, 283 Clozapine, 71, 259 Cochlea, 259, 280 Cochlear, 259, 311, 315 Cochlear Diseases, 259, 311 Coenzyme, 251, 259 Cofactor, 259, 299, 311 Cognition, 14, 20, 33, 43, 62, 85, 108, 259, 282, 289 Colchicine, 259, 313 Collagen, 252, 260, 271, 273, 297 Collapse, 254, 260 Colloidal, 247, 260, 268, 309 Coloboma, 81, 260 Combinatorial, 19, 41, 260

320 Mental Retardation

Communication Disorders, 6, 168, 216, 260 Competency, 100, 137, 260 Complement, 260, 273, 281, 297 Complementary and alternative medicine, 115, 122, 260 Complementary medicine, 115, 260 Complementation, 45, 261 Computational Biology, 217, 261 Computed tomography, 261, 304 Computer-Assisted Instruction, 55, 261 Computerized axial tomography, 261, 304 Computerized tomography, 164, 166, 261 Conception, 261, 271, 308 Cone, 126, 129, 261, 296, 309 Confusion, 30, 261, 266, 278, 290 Congestion, 250, 261 Congestive heart failure, 170, 187, 261 Conjugated, 253, 261, 263 Connective Tissue, 251, 254, 260, 261, 271, 272, 273, 303 Connexins, 46, 261, 272 Consciousness, 261, 264, 266, 301, 308 Constipation, 250, 261 Constitutional, 47, 261, 303 Consultation, 45, 61, 261 Consumption, 262, 265 Continence, 200, 262 Contraindications, ii, 262 Controlled clinical trial, 24, 44, 262 Controlled study, 42, 106, 112, 262 Cooperative group, 27, 262 Coordination, 5, 27, 49, 257, 262, 287 Coronary, 187, 249, 255, 262, 270, 277, 286, 288, 291 Coronary Circulation, 249, 262, 291 Coronary heart disease, 255, 262 Coronary Thrombosis, 262, 286, 288 Corpus, 19, 262 Corpus Callosum, 19, 262 Cortex, 34, 55, 75, 76, 251, 254, 262, 269, 270, 271, 289, 302 Cortical, 48, 49, 74, 262, 270, 304, 311 Cortisol, 16, 247, 262 Cranial, 49, 257, 262, 270, 275, 280, 289, 292, 295, 301, 315 Craniocerebral Trauma, 252, 262, 275, 277, 311 Craniofacial Abnormalities, 95, 262 Creatine, 109, 262 Creatinine, 262, 263 Crossing-over, 263, 302

Cryofixation, 263 Cryopreservation, 28, 263 Cues, 131, 173, 263 Curative, 196, 263, 311 Cyclic, 184, 185, 246, 263, 275, 291, 296, 304 Cystathionine beta-Synthase, 20, 263, 277 Cysteine, 188, 263 Cystine, 263 Cytochrome, 44, 263 Cytogenetics, 40, 44, 67, 101, 104, 105, 152, 263 Cytokine, 38, 263 Cytomegalovirus, 24, 186, 188, 263 Cytopenia, 175, 182, 263 Cytoplasm, 250, 256, 263, 264, 274, 288, 291, 303, 310 Cytoskeletal Proteins, 48, 263, 268 Cytoskeleton, 21, 263, 264 Cytotoxic, 264, 306 D Databases, Bibliographic, 217, 264 De novo, 38, 54, 60, 75, 264 Deamination, 264, 314 Decarboxylation, 264, 276 Degenerative, 19, 55, 61, 187, 264, 287, 303 Dehydration, 51, 264 Deinstitutionalization, 4, 5, 6, 75, 102, 124, 125, 126, 129, 132, 136, 138, 149, 200, 202, 264 Deletion, 13, 22, 60, 81, 91, 110, 250, 264, 273 Delirium, 170, 173, 174, 175, 180, 182, 185, 187, 188, 250, 264 Delusions, 264, 300 Demyelinating Diseases, 46, 264 Dendrites, 55, 264, 265, 290 Dendritic, 31, 55, 61, 75, 76, 264 Density, 70, 135, 264, 292 Dental Care, 5, 6, 7, 8, 10, 11, 72, 107, 108, 199, 200, 205, 264, 265 Dental Care for Children, 5, 7, 8, 265 Dental Caries, 201, 265 Dental Plaque, 10, 265 Dental Staff, 203, 265 Dentate Gyrus, 265, 276 Dentists, 5, 8, 10, 265 Depolarization, 265, 306 Depressive Disorder, 265, 283 Dermatitis, 175, 182, 265 DES, 80, 84, 96, 109, 265 Desensitization, 9, 154, 265

Index 321

Developed Countries, 201, 265 Dextroamphetamine, 265, 286 Diabetes Mellitus, 179, 265, 274, 276, 280 Diagnostic procedure, 169, 209, 265 Diastolic, 266, 277 Dichloroacetate, 24, 44, 266 Digestion, 253, 254, 266, 280, 283, 309 Digestive system, 168, 266 Dilatation, 249, 266, 298 Dilation, 254, 266, 277 Dilator, 266, 291 Dimerization, 103, 266 Diploid, 261, 266, 287, 296, 313 Discrimination, 5, 18, 80, 139, 266 Discrimination Learning, 18, 266 Disease Progression, 9, 266 Disease Susceptibility, 54, 170, 266 Disorientation, 261, 264, 266 Dissection, 58, 77, 266 Dissociation, 246, 266 Distal, 29, 60, 65, 87, 93, 109, 252, 266, 295, 300 Diuretic, 266, 307 Dizziness, 203, 266 Dominance, 267, 282 Dopamine, 32, 163, 174, 180, 186, 188, 250, 254, 259, 265, 267, 296, 303 Dorsal, 87, 267, 289, 298, 308 Dorsum, 267 Dosage Forms, 171, 179, 267 Down syndrome, 8, 12, 20, 25, 26, 36, 43, 58, 62, 70, 71, 76, 96, 102, 120, 172, 201, 228, 267 Drive, ii, vi, 5, 6, 27, 111, 200, 205, 267 Drug Design, 176, 267 Drug Interactions, 267 Duct, 256, 267, 304 Duodenum, 253, 267, 290, 309 Dyes, 166, 248, 267, 291 Dynein, 267, 286 Dynorphins, 267, 292 Dyskinesia, 50, 81, 250, 267 Dyslexia, 20, 55, 203, 267, 282 Dysplasia, 74, 83, 267 Dystonia, 203, 250, 268 Dystrophin, 70, 268 Dystrophy, 49, 73, 74, 94, 121, 201, 268 E Effector, 174, 180, 188, 245, 260, 268, 290, 296 Effector cell, 268, 290

Efficacy, 30, 41, 57, 123, 154, 163, 192, 267, 268 Elective, 268 Electrolyte, 264, 268, 307 Electrons, 250, 252, 268, 281, 293, 301 Electrophoresis, 22, 268 Electrophysiological, 34, 268 Embryo, 28, 46, 253, 256, 268, 279, 287, 310 Emesis, 250, 268 Empirical, 57, 62, 133, 268 Emulsion, 268, 272 Enamel, 265, 268 Encephalopathy, 23, 269 Endarterectomy, 249, 269 Endocrine Glands, 269 Endocrine System, 164, 172, 269 Endocytosis, 256, 269 Endorphins, 269, 292, 299 Endothelium, 269, 291 Endothelium-derived, 269, 291 Enkephalin, 16, 269, 299 Entorhinal Cortex, 269, 276 Enuresis, 182, 269 Environmental Health, 216, 218, 269 Enzymatic, 29, 255, 256, 260, 265, 269, 276, 295, 303 Enzyme, 38, 174, 175, 180, 245, 246, 251, 256, 259, 263, 267, 268, 269, 275, 283, 285, 296, 297, 300, 301, 306, 311, 312, 316 Epidemiological, 53, 230, 269 Epinephrine, 246, 267, 269, 291, 313 Epithelial, 51, 269 Epithelial Cells, 51, 269 Epithelium, 252, 269, 281 Ergot, 254, 269 Erythritol, 10, 270 Erythrocytes, 248, 254, 270, 302 Esophagus, 266, 270, 309 Ether, 270, 295 Eukaryotic Cells, 263, 270, 279, 293, 313 Evoke, 270, 309 Excipient, 177, 270 Excitation, 184, 257, 270 Excitatory, 184, 270, 274, 281 Excitatory Amino Acid Agonists, 270, 281 Excrete, 270, 302 Exogenous, 270, 295, 313 Exon, 47, 270 Extensor, 270, 300 Extracellular, 17, 50, 58, 188, 248, 251, 261, 269, 270, 271, 307 Extracellular Matrix, 17, 50, 261, 270, 271

322 Mental Retardation

Extracellular Space, 270 Extrapyramidal, 247, 250, 267, 270 Eye Infections, 246, 270 Eye Movements, 270, 301 F Facial, 21, 29, 37, 47, 49, 69, 73, 79, 85, 87, 88, 93, 97, 101, 110, 144, 150, 152, 164, 202, 232, 262, 270 Facial Nerve, 49, 270 Family Planning, 217, 271 Family Relations, 15, 271 Fasciculation, 19, 271, 290 Fat, 181, 250, 253, 254, 255, 256, 257, 262, 271, 283, 287, 303, 307 Fathers, 15, 144, 271 Fatigue, 271, 275 Fatty acids, 29, 182, 247, 271, 283 Fetal Alcohol Syndrome, 69, 225, 271 Fetal Death, 208, 271 Fetus, 32, 271, 298, 314 Fibrin, 271, 311 Fibrinogen, 271, 297, 311 Fibroblasts, 104, 164, 271 Fibrosis, 27, 187, 271, 304 Fissure, 259, 260, 262, 265, 271 Fixation, 181, 182, 263, 271 Fluorescence, 22, 32, 41, 60, 76, 272 Folate, 272 Fold, 32, 56, 271, 272 Folic Acid, 177, 272 Forearm, 254, 272 Fossa, 257, 272 Fovea, 271, 272 Frameshift, 39, 75, 272 Frameshift Mutation, 39, 75, 272 Fungi, 270, 272, 274, 286, 316 G Gallbladder, 245, 266, 272 Ganglia, 87, 245, 252, 272, 289, 295 Ganglioside, 59, 272 Gap Junctions, 17, 46, 261, 272, 310 Gas, 248, 255, 272, 277, 291, 309, 314 Gastric, 267, 272, 276 Gastrin, 272, 277 Gastrointestinal, 51, 164, 254, 269, 272, 282, 305 Gastrointestinal tract, 164, 272, 282, 305 Gelatin, 273, 274, 309, 311 Gene Deletion, 39, 273 Gene Expression, 28, 273 Genetic Code, 273, 291 Genetic Counseling, 22, 30, 273

Genetic Engineering, 253, 259, 273 Genetic Techniques, 165, 273 Genetic testing, 30, 164, 273 Genetic transcription, 273, 299, 312 Genital, 69, 95, 259, 273, 314 Genitourinary, 273, 314 Genomics, 15, 22, 86, 170, 185, 187, 273 Genotype, 23, 36, 37, 43, 54, 57, 177, 273, 295 Germ Cells, 273, 285, 293, 307, 310 Gestation, 20, 260, 273, 295 Gestational, 183, 273 Gestational Age, 183, 273 Gland, 246, 258, 273, 293, 299, 304, 309, 311 Glucose, 65, 107, 251, 265, 273, 274, 275, 278, 280, 307 Glucose Intolerance, 265, 274 Glutamate, 19, 38, 61, 183, 184, 274, 281, 285 Glutamic Acid, 272, 274 Glutamine, 57, 274 Glycerol, 255, 274, 296 Glycine, 253, 274, 305 Glycoprotein, 51, 271, 274, 306, 311 Glycosaminoglycan, 258, 274 Glycosuria, 182, 274 Glycosylation, 59, 73, 186, 274 Gonads, 274, 278 Governing Board, 274, 298 Government Agencies, 27, 274, 298 Grade, 125, 135, 274 Graft, 274, 277, 279 Granulocytes, 274, 282, 306, 316 Grasses, 272, 274 Group Homes, 3, 136, 139, 140, 155, 158, 200, 274 Growth factors, 47, 175, 183, 188, 275 Guanylate Cyclase, 275, 291 Gynaecomastia, 75, 275 H Hammer, 34, 83, 275, 293 Handicap, 39, 102, 138, 150, 275 Haptens, 246, 275 Headache, 275, 277, 278 Health Behavior, 201, 275 Health Promotion, 190, 218, 275 Health Services, iv, 11, 13, 199, 219, 220, 275 Health Status, 26, 37, 201, 219, 275 Hearing aid, 228, 275 Hearing Disorders, 260, 275

Index 323

Heart attack, 255, 275 Heart failure, 173, 174, 175, 180, 182, 185, 187, 275 Heme, 253, 263, 275 Hemoglobin, 248, 270, 275, 282, 311 Hemolytic, 276, 311 Hemorrhage, 262, 275, 276, 309 Hemostasis, 276, 305 Hepatic, 57, 247, 264, 276 Hepatocyte, 57, 276 Hereditary, 70, 73, 87, 276, 287, 289, 303, 311 Heredity, 166, 273, 276 Heterogeneity, 8, 35, 93, 246, 276 Hippocampus, 33, 265, 276, 309 Histamine, 186, 188, 250, 276 Histidine, 276 Histology, 44, 276, 294 Histone Deacetylase, 66, 276 Homeostasis, 35, 276 Homeotic, 47, 276 Homogeneous, 24, 44, 276, 295 Homologous, 48, 54, 185, 247, 261, 263, 276, 304, 310 Homozygotes, 45, 267, 276 Hormonal, 54, 251, 276 Hormone, 174, 175, 180, 186, 188, 262, 265, 269, 272, 276, 277, 280, 285, 299, 303, 304, 306, 311 Host, 57, 252, 277, 278, 282, 315 Housekeeping, 274, 277 Human Development, 14, 25, 62, 112, 163, 164, 165, 166, 190, 206, 216, 226, 227, 230, 277 Hybrid, 41, 45, 57, 259, 277 Hybridization, 22, 41, 54, 60, 277 Hydrocephalus, 19, 277, 280 Hydrogen, 245, 247, 252, 255, 277, 286, 291, 293 Hydrolases, 277, 284 Hydrolysis, 174, 175, 180, 251, 277, 281, 296, 297, 300 Hydrophilic, 186, 188, 277 Hydrophobic, 186, 188, 277, 281 Hyperammonemia, 35, 38, 277 Hypercalcemia, 37, 202, 277 Hyperhomocysteinemia, 263, 277 Hypersensitivity, 247, 265, 277, 282, 303 Hypertension, 170, 173, 174, 175, 179, 180, 182, 185, 187, 188, 210, 255, 277, 280 Hypertrophic cardiomyopathy, 87, 278

Hypertrophy, 73, 170, 173, 174, 175, 180, 182, 185, 187, 188, 278 Hypoglycaemia, 264, 278 Hypogonadism, 179, 185, 278 Hypoplasia, 19, 25, 74, 85, 96, 278 Hypotension, 170, 171, 173, 174, 175, 180, 182, 185, 187, 250, 278, 290 Hypothalamus, 245, 252, 254, 269, 278, 290, 299 Hypothyroidism, 89, 110, 278 Hypotonia, 102, 164, 177, 257, 258, 278 Hypoxia, 264, 278, 311 I Id, 113, 121, 231, 232, 240, 242, 278 Idiopathic, 54, 278 Ileum, 278, 290 Immune response, 46, 249, 252, 275, 278, 315 Immune system, 22, 252, 253, 268, 278, 282, 284, 287, 314, 316 Immunodeficiency, 175, 182, 229, 278 Immunodeficiency syndrome, 229, 278 Immunoglobulin, 249, 278, 287 Immunohistochemistry, 38, 278 Immunologic, 273, 278 Immunology, 246, 278 Immunotherapy, 253, 265, 278 Impairment, 14, 30, 34, 49, 132, 157, 172, 176, 200, 228, 251, 253, 264, 267, 270, 279, 285, 300 In situ, 22, 38, 41, 60, 76, 279, 298 In Situ Hybridization, 22, 38, 41, 60, 76, 279 In vitro, 19, 20, 28, 29, 34, 39, 40, 45, 279, 312 In vivo, 20, 29, 32, 34, 35, 39, 40, 45, 57, 103, 279 Incontinence, 200, 277, 279 Indicative, 191, 279, 294, 314 Induction, 25, 38, 250, 279, 299 Infancy, 20, 59, 179, 258, 279 Infant, Newborn, 247, 279 Infantile, 37, 77, 91, 279, 283, 295 Infarction, 257, 279 Infection, 12, 25, 40, 170, 229, 251, 253, 263, 264, 270, 278, 279, 284, 290, 303, 309, 316 Infertility, 254, 279 Inflammation, 187, 201, 247, 250, 258, 265, 270, 271, 279, 282, 290, 292, 303 Informed Consent, 9, 28, 86, 279 Ingestion, 279, 294, 297 Inhalation, 246, 279, 297

324 Mental Retardation

Initiation, 8, 279, 299, 312 Inner ear, 185, 259, 280 Innervation, 270, 280, 292, 301 Inositol, 280, 285, 304 Inotropic, 267, 280 Insight, 40, 50, 280 Insomnia, 71, 280 Institutionalization, 10, 59, 280 Insulator, 280, 287 Insulin, 280, 313 Intention tremor, 14, 280 Intercellular Junctions, 46, 280 Intermittent, 280, 284 Interstitial, 22, 60, 90, 270, 280, 302 Intestinal, 172, 256, 280 Intestine, 253, 254, 280, 282, 309 Intoxication, 264, 280, 316 Intracellular, 18, 41, 175, 183, 185, 273, 279, 280, 284, 285, 291, 302, 304, 306, 315 Intracellular Membranes, 280, 285 Intracranial Hemorrhages, 277, 280, 311 Intracranial Hypertension, 275, 277, 280, 311 Intracranial Pressure, 280, 292 Intramuscular, 171, 178, 179, 280 Intraocular, 260, 280 Intravenous, 57, 280 Intrinsic, 246, 252, 280 Invasive, 280, 284 Involuntary, 90, 252, 258, 269, 281, 288, 311 Ion Channels, 185, 251, 281, 290 Ions, 252, 266, 268, 277, 281, 286, 300, 307 Iris, 81, 249, 281 Ischemia, 251, 272, 281, 292 Ischemic stroke, 187, 281 Isoprenoid, 29, 281 K Kainate, 184, 281 Kainic Acid, 184, 281 Karyotype, 41, 281 Kb, 89, 216, 281 Keratolytic, 265, 281 Keto, 281, 312 Kinesin, 281, 286 L Labyrinth, 259, 280, 281, 305, 315 Lacrimal, 270, 281 Language Development, 26, 155, 281 Language Disorders, 8, 9, 11, 260, 282 Large Intestine, 266, 280, 282, 302, 306 Latent, 20, 282

Laterality, 72, 282 Laxative, 247, 282, 307 Learning Disorders, 31, 224, 282 Lectin, 282, 285 Length of Stay, 10, 282 Lens, 104, 256, 282, 302, 315 Lesion, 183, 282, 283, 301, 310 Lethal, 21, 282 Lethargy, 277, 278, 282 Leucine, 29, 282 Leucocyte, 282, 284 Leukemia, 29, 282 Leukotrienes, 29, 251, 282 Library Services, 193, 240, 282 Lichens, 270, 282 Life cycle, 246, 272, 282 Life Expectancy, 8, 36, 172, 194, 283 Ligament, 283, 299 Ligands, 175, 183, 186, 256, 283 Ligase, 80, 283 Linear Models, 15, 283 Linkage, 47, 51, 92, 93, 109, 283 Lip, 4, 77, 93, 283 Lipid, 44, 256, 274, 280, 281, 283, 287 Lipodystrophy, 93, 283 Lipoxygenase, 282, 283 Lithium, 177, 178, 250, 283 Liver, 57, 62, 92, 245, 247, 248, 251, 253, 263, 266, 268, 272, 276, 283, 304, 314 Liver scan, 283, 304 Lobe, 102, 257, 283, 299 Localization, 38, 59, 63, 92, 120, 278, 283 Localized, 48, 63, 70, 177, 185, 248, 263, 265, 268, 271, 279, 283, 296 Longitudinal study, 15, 53, 92, 283 Long-Term Care, 39, 283 Loop, 41, 43, 284 Lymph, 258, 269, 284, 309 Lymphatic, 269, 279, 284, 307, 308 Lymphoblasts, 42, 284 Lymphocyte, 250, 284, 285 Lymphocytic, 43, 284 Lymphoid, 249, 282, 284 Lysosomal Storage Diseases, 27, 284 Lytic, 25, 284 M Magnetic Resonance Imaging, 20, 164, 166, 284, 304 Malformation, 31, 51, 91, 284 Malignant, 22, 48, 254, 284, 289 Malnutrition, 247, 251, 255, 284 Mammary, 275, 284

Index 325

Mammogram, 255, 284, 286 Mania, 171, 178, 284 Manic, 170, 173, 174, 175, 180, 182, 185, 187, 188, 250, 253, 283, 284, 300 Manic-depressive psychosis, 284, 300 Manifest, 35, 40, 252, 284 Maternal Behavior, 22, 284 Medial, 102, 284, 292 Mediate, 20, 25, 49, 175, 183, 256, 267, 284 Mediator, 175, 183, 284, 305 Medicament, 171, 178, 285, 309 MEDLINE, 217, 285 Megalencephaly, 43, 285 Megaloblastic, 272, 285 Meiosis, 36, 46, 285, 310 Melanin, 281, 285, 296, 313 Melanoblasts, 285, 296 Memantine, 19, 285 Membrane Glycoproteins, 285 Membrane Proteins, 36, 256, 285, 308 Memory, 12, 25, 33, 34, 43, 45, 118, 129, 135, 143, 149, 152, 248, 249, 264, 285 Meninges, 25, 257, 262, 285, 308 Menopause, 36, 285, 298 Menstruation, 247, 285 Mental deficiency, 271, 285 Mental Disorders, 168, 176, 231, 285, 300 Mental Processes, 266, 285, 300 Mesolimbic, 285 Metabolic disorder, 35, 277, 285, 295 Metabotropic, 61, 285 Metastasis, 256, 286 Methylphenidate, 32, 286 MI, 244, 286 Microbiology, 246, 251, 286 Microcalcifications, 255, 286 Microorganism, 259, 286, 316 Micro-organism, 265, 286, 296 Microscopy, 18, 41, 82, 252, 286 Migration, 19, 25, 48, 49, 55, 286, 296 Mitochondria, 20, 286, 293 Mitochondrial Swelling, 286, 289 Mitosis, 250, 286 Mitotic, 48, 286, 287, 315 Mobility, 229, 286 Modeling, 154, 155, 267, 286 Modification, 53, 117, 140, 144, 273, 286, 301 Molecular Motors, 21, 286 Molecular Structure, 46, 286 Monitor, 90, 263, 287, 291 Monoclonal, 176, 287

Monoclonal antibodies, 176, 287 Monosomy, 47, 287 Morphogenesis, 28, 31, 271, 287 Morphological, 61, 268, 287 Morula, 253, 287 Mosaicism, 81, 104, 287 Motility, 49, 287, 305 Motor Activity, 287, 301 Motor nerve, 271, 287, 295 Motor Skills, 33, 140, 143, 153, 210, 287 Movement Disorders, 14, 81, 250, 287, 311 Mucins, 51, 265, 287, 304 Mucus, 287 Multiple sclerosis, 188, 287 Multivariate Analysis, 62, 287 Muscle Contraction, 268, 287 Muscle Fibers, 287, 288 Muscular Diseases, 288, 290, 294, 301 Muscular Dystrophies, 268, 288 Musculature, 49, 288 Mutagenesis, 40, 51, 288 Mutagens, 272, 288 Myelin, 46, 183, 264, 287, 288 Myelosuppression, 175, 182, 288 Myocardial infarction, 170, 173, 174, 175, 180, 182, 185, 187, 188, 250, 262, 286, 288 Myocardial Ischemia, 248, 288 Myocardium, 248, 286, 288 Myofibrils, 268, 288 Myopia, 87, 288, 289, 302 Myosin, 21, 184, 185, 286, 287, 288 Myotonic Dystrophy, 288, 313 N Naloxone, 288 Naltrexone, 16, 288 Narcolepsy, 265, 286, 288 Narcotic, 288, 289 Natural selection, 253, 289 Nausea, 249, 250, 267, 289 NCI, 1, 167, 215, 259, 289 Nearsightedness, 288, 289 Necrosis, 20, 183, 250, 257, 279, 286, 288, 289 Neocortex, 75, 289 Neonatal, 19, 23, 38, 57, 86, 183, 289, 295 Neonatal period, 183, 289 Neoplasia, 289 Neoplasm, 289 Neoplastic, 21, 289 Nervous System, 17, 18, 19, 20, 23, 24, 34, 49, 58, 164, 184, 245, 246, 252, 254, 257,

326 Mental Retardation

259, 265, 267, 272, 274, 282, 284, 286, 287, 289, 290, 292, 295, 305, 309 Networks, 21, 142, 289 Neural, 18, 33, 41, 43, 57, 246, 248, 285, 289, 296, 307 Neural Crest, 285, 289, 296 Neurodegenerative Diseases, 38, 187, 252, 289 Neurogenic, 289, 314 Neuroleptic, 99, 166, 186, 247, 250, 259, 289 Neurologic, 14, 58, 165, 277, 290, 295 Neurology, 19, 23, 40, 44, 68, 85, 87, 90, 91, 95, 96, 106, 107, 112, 290 Neuroma, 203, 290 Neuromuscular, 20, 73, 74, 89, 94, 245, 290, 292, 294, 301 Neuromuscular Diseases, 290, 294, 301 Neuromuscular Junction, 245, 290, 292 Neuronal, 18, 19, 20, 28, 29, 33, 34, 40, 43, 48, 52, 55, 82, 183, 290 Neurons, 18, 31, 33, 40, 48, 55, 87, 184, 264, 265, 270, 272, 289, 290, 310, 315 Neuropathy, 8, 87, 89, 290, 295 Neuropeptide, 186, 290 Neurophysiology, 16, 80, 265, 290 Neuropsychological Tests, 36, 290 Neuropsychology, 20, 40, 290 Neuroretinitis, 290, 303 Neurosecretory Systems, 269, 290 Neurotensin, 174, 290 Neurotoxicity, 281, 290 Neurotransmitters, 38, 178, 188, 290 Neutropenia, 175, 182, 291 Neutrophils, 274, 291 Night Blindness, 291, 303 Nitric Oxide, 19, 38, 291 Nitrogen, 57, 247, 271, 274, 291, 313 Nitroglycerin, 19, 291 Nonverbal Communication, 8, 144, 260, 291 Norepinephrine, 246, 267, 291 Nuclear, 21, 44, 49, 51, 120, 252, 268, 270, 271, 289, 291 Nuclear Family, 271, 291 Nuclei, 268, 273, 284, 286, 291, 292, 315 Nucleic acid, 40, 184, 252, 273, 277, 279, 288, 291, 301, 315 Nucleic Acid Hybridization, 277, 291 Nucleus, 48, 166, 250, 252, 258, 263, 270, 285, 291, 299, 310, 315

O Occipital Lobe, 291, 315 Occupational Therapy, 191, 202, 292 Ocular, 95, 219, 292 Ointments, 267, 292, 294 Opacity, 256, 264, 292 Operon, 292, 299 Ophthalmology, 81, 118, 271, 292 Ophthalmoplegia, 74, 108, 292 Opioid Peptides, 16, 267, 269, 292 Opsin, 292, 303 Optic Atrophy, 66, 73, 292 Optic Chiasm, 278, 292 Optic Disk, 292 Optic Nerve, 290, 292, 303 Oral Health, 3, 4, 5, 10, 199, 200, 201, 219, 230, 292 Oral Hygiene, 3, 5, 201, 230, 292 Organ Culture, 293, 312 Organelles, 263, 264, 281, 293 Orofacial, 4, 293 Orthostatic, 250, 293 Osmotic, 247, 286, 293 Ossicles, 275, 293 Osteodystrophy, 70, 293 Osteoporosis, 170, 173, 174, 175, 180, 182, 185, 187, 188, 293 Outpatient, 61, 293 Ovarian Cysts, 62, 293 Ovary, 274, 293 Overdosage, 171, 293 Ovum, 273, 282, 287, 293, 299, 313, 316 Oxidation, 29, 36, 245, 250, 263, 293 Oxidative metabolism, 282, 293 P Palate, 77, 259, 293 Palliative, 293, 311 Palsy, 183, 293 Pancreas, 245, 266, 280, 293 Pancreatic, 185, 186, 293, 294 Pancreatic Polypeptide, 185, 186, 294 Paraffin, 10, 294 Paralysis, 34, 255, 292, 294, 300, 301, 307 Paraplegia, 19, 73, 294 Parasite, 294 Parasitic, 24, 294 Parenchyma, 25, 294 Parkinsonism, 250, 294 Paroxysmal, 81, 106, 248, 294 Particle, 21, 294, 312, 315 Patch, 19, 294

Index 327

Pathogenesis, 9, 15, 18, 20, 23, 25, 46, 50, 63, 67, 294 Pathologic, 245, 250, 254, 262, 277, 294, 300, 303, 308 Pathologic Processes, 250, 294 Pathologies, 176, 294 Pathophysiology, 23, 38, 163, 294 Patient Advocacy, 232, 294 Patient Compliance, 4, 10, 294 Patient Education, 10, 228, 238, 240, 244, 294 Pediatrics, 17, 20, 23, 24, 26, 28, 29, 34, 37, 47, 53, 66, 79, 85, 89, 100, 116, 224, 294 Pedigree, 79, 108, 294 Pelvic, 294, 299 Peptide, 277, 292, 295, 297, 299, 300, 311 Perception, 26, 57, 147, 149, 261, 275, 295, 304 Perinatal, 24, 183, 295 Periodontal disease, 3, 201, 295 Peripheral blood, 32, 295 Peripheral Nervous System, 19, 87, 264, 289, 290, 293, 294, 295, 301 Peripheral Nervous System Diseases, 290, 294, 295, 301 Peripheral Neuropathy, 13, 73, 87, 295 Periventricular Leukomalacia, 183, 295 Peroxisomal Disorders, 35, 295 Petroleum, 294, 295 Phallic, 271, 295 Pharmaceutical Solutions, 267, 295 Pharmacokinetics, 267, 295 Pharmacologic, 248, 295, 312, 314 Phenotype, 13, 14, 19, 21, 23, 29, 33, 36, 37, 40, 43, 45, 51, 54, 58, 80, 170, 187, 261, 273, 295 Phenylalanine, 296, 313 Phosphodiesterase, 174, 180, 188, 296 Phospholipases, 296, 306 Phospholipids, 271, 280, 296 Phosphorus, 255, 296 Phosphorylates, 48, 64, 71, 296 Phosphorylation, 31, 59, 175, 183, 186, 296 Photoreceptor, 185, 296 Physical Examination, 164, 166, 273, 296 Physical Fitness, 98, 210, 296 Physical Therapy, 202, 296 Physiologic, 247, 253, 285, 296, 302, 303, 313 Physiology, 8, 32, 34, 58, 253, 268, 290, 296 Piebaldism, 75, 296 Pigments, 256, 296, 303

Pitch, 296, 316 Plants, 247, 253, 255, 259, 274, 282, 291, 296, 312 Plaque, 10, 103, 249, 257, 296 Plasma, 29, 38, 247, 249, 256, 268, 271, 273, 274, 276, 294, 297, 300, 305 Plasma cells, 249, 297 Plasma protein, 247, 297, 300 Plasticity, 16, 34, 42, 53, 55, 61, 64, 67, 82, 297 Plasticizers, 182, 297 Platelet Activation, 297, 306 Platelet Aggregation, 291, 297 Platelet Count, 62, 297 Platelets, 288, 291, 297, 311 Platinum, 284, 297 Point Mutation, 23, 44, 297 Poisoning, 244, 264, 269, 280, 289, 297 Policy Making, 274, 297 Polymerase, 297, 298, 299 Polymorphic, 99, 170, 265, 297 Polymorphism, 177, 297 Polypeptide, 175, 183, 186, 248, 260, 271, 277, 294, 297, 299, 311, 316 Polysaccharide, 250, 274, 298, 300 Posterior, 248, 251, 257, 258, 267, 281, 291, 293, 298 Postmenopausal, 293, 298 Postnatal, 271, 298 Postsynaptic, 31, 32, 298, 306, 310 Post-synaptic, 34, 298 Post-translational, 59, 186, 298 Post-traumatic, 287, 298 Postural, 298, 308 Potentiation, 298, 306 Practice Guidelines, 27, 220, 228, 231, 298 Preclinical, 57, 98, 298 Precursor, 29, 37, 186, 251, 267, 268, 269, 291, 296, 298, 299, 300, 313 Pregnancy Tests, 273, 298 Prenatal, 33, 102, 120, 172, 177, 268, 271, 298 Presbycusis, 228, 298 Presynaptic, 32, 298, 310 Prevalence, 4, 7, 11, 14, 56, 87, 124, 126, 147, 160, 201, 219, 230, 231, 298 Primed In Situ Labeling, 105, 298 Private Sector, 190, 298 Probe, 41, 177, 298 Programmed Instruction, 261, 298 Progression, 14, 46, 53, 249, 298 Projection, 18, 291, 292, 299, 302

328 Mental Retardation

Prolactin, 254, 299 Promoter, 32, 39, 57, 90, 299 Promotor, 59, 299 Prone, 22, 299 Pro-Opiomelanocortin, 269, 292, 299 Prophase, 299, 310 Prophylaxis, 4, 299 Prospective study, 283, 299 Prostate, 38, 299 Prosthesis, 173, 299 Protein C, 21, 103, 173, 174, 180, 186, 187, 188, 247, 248, 252, 299, 308, 314 Protein Conformation, 248, 299 Protein Kinases, 30, 175, 299 Protein S, 42, 53, 61, 82, 174, 175, 180, 183, 188, 198, 253, 273, 299, 303 Proteoglycans, 17, 252, 300 Proteolytic, 186, 260, 271, 300 Prothrombin, 300, 311 Protocol, 6, 39, 300 Proximal, 29, 77, 266, 298, 300 Pruritus, 250, 300 Psoriasis, 175, 176, 182, 300 Psychiatric, 6, 36, 50, 75, 81, 98, 108, 165, 166, 192, 198, 260, 285, 300 Psychic, 300, 304 Psychogenic, 300, 314 Psychology, 15, 16, 27, 42, 88, 100, 102, 121, 203, 219, 266, 290, 300 Psychometric testing, 54, 300 Psychomotor, 264, 290, 300 Psychopathology, 77, 78, 158, 300 Psychophysiology, 290, 300 Psychosis, 41, 165, 178, 250, 273, 300 Psychotropic, 28, 71, 300 Ptosis, 74, 300 Public Health, 7, 14, 36, 87, 108, 143, 198, 201, 218, 219, 220, 301 Public Policy, 126, 217, 301 Publishing, 64, 301 Pulmonary, 254, 262, 282, 301, 314 Pulmonary Artery, 254, 301, 314 Pulse, 22, 287, 301 Purines, 252, 301, 305 Pyridoxal, 263, 301 Pyridoxal Phosphate, 263, 301 Q Quadriplegia, 98, 301 Quality of Life, 59, 141, 143, 147, 148, 150, 158, 200, 201, 232, 301 R Race, 8, 49, 143, 281, 286, 301

Radiation, 248, 272, 301, 304, 316 Radioactive, 254, 277, 283, 287, 291, 301, 304 Radiography, 273, 301 Randomized, 24, 28, 30, 62, 268, 301 Reality Testing, 300, 301 Receptors, Serotonin, 302, 305 Recombinant, 28, 170, 173, 174, 175, 180, 182, 185, 186, 302, 314 Recombination, 48, 60, 302 Rectum, 254, 266, 272, 279, 282, 299, 302, 309 Recurrence, 253, 284, 302 Red blood cells, 270, 276, 288, 302 Red Nucleus, 251, 302 Refer, 1, 200, 260, 266, 269, 271, 272, 283, 289, 292, 300, 302, 315 Refraction, 288, 302, 308 Refractive Power, 288, 302 Regeneration, 34, 46, 48, 302 Regimen, 268, 294, 302 Reliability, 71, 78, 133, 302 Remission, 253, 284, 302 Renal failure, 264, 276, 302 Renal tubular, 97, 302 Renal tubular acidosis, 97, 302 Research Support, 8, 302 Resident physician, 7, 302 Residential Facilities, 6, 136, 158, 202, 302 Resolving, 35, 302 Resorption, 277, 303 Restless legs, 166, 303 Restoration, 100, 296, 303, 316 Retina, 47, 185, 258, 282, 288, 290, 292, 303, 315 Retinal, 47, 179, 261, 292, 303 Retinitis, 94, 185, 303 Retinitis Pigmentosa, 94, 185, 303 Retinol, 303 Retinopathy, 179, 303 Rheumatism, 303 Rheumatoid, 175, 176, 182, 303 Rheumatoid arthritis, 175, 176, 182, 303 Ribose, 246, 303 Ribosome, 303, 313 Risk factor, 7, 12, 37, 78, 231, 277, 299, 303 Risperidone, 50, 71, 99, 106, 107, 112, 171, 178, 303 Rod, 252, 259, 296, 303 Rural Population, 304 S Saline, 181, 182, 304

Index 329

Saliva, 10, 16, 103, 304 Salivary, 16, 200, 263, 265, 266, 270, 304, 309 Salivary glands, 263, 265, 266, 270, 304 Scans, 166, 304 Schizoid, 304, 316 Schizotypal Personality Disorder, 304, 316 Sclerosis, 69, 187, 287, 304 Screening, 16, 20, 41, 47, 53, 54, 62, 67, 89, 101, 117, 170, 179, 208, 219, 227, 259, 304 Second Messenger Systems, 290, 304 Secretion, 254, 276, 278, 287, 304, 305 Secretory, 304, 310 Segmental, 58, 96, 304 Segmentation, 304 Segregation, 130, 302, 304 Seizures, 7, 61, 106, 109, 264, 294, 304, 308 Self Care, 6, 245, 305 Self-Injurious Behavior, 16, 62, 71, 165, 305 Semen, 299, 305 Semicircular canal, 280, 305 Semisynthetic, 254, 305 Senile, 250, 293, 298, 305 Septic, 175, 176, 182, 251, 305 Sequence Analysis, 47, 305 Sequencing, 33, 54, 305 Serine, 77, 175, 182, 263, 305 Serotonin, 163, 186, 188, 250, 259, 302, 303, 305, 313 Sertraline, 163, 305 Sertraline Hydrochloride, 163, 305 Serum, 117, 247, 260, 305 Sex Characteristics, 246, 305 Sex Education, 124, 229, 305 Sexual Abstinence, 229, 305 Shock, 175, 176, 182, 305, 313 Shunt, 29, 305 Sialyltransferases, 59, 305 Sibling Relations, 71, 154, 306 Side effect, 50, 62, 69, 166, 171, 246, 247, 250, 253, 288, 306, 312 Signal Transduction, 41, 59, 173, 174, 175, 180, 183, 188, 256, 280, 306 Signs and Symptoms, 37, 302, 306 Skeletal, 41, 75, 95, 172, 259, 278, 288, 306 Skeleton, 245, 306 Skin Abnormalities, 66, 306 Skull, 262, 280, 306, 310 Sleep Stages, 56, 306 Small intestine, 267, 277, 278, 280, 306, 315 Smooth muscle, 276, 288, 291, 306 Social Behavior, 79, 117, 134, 306

Social Change, 9, 150, 306 Social Conditions, 10, 306 Social Environment, 301, 306 Social Support, 80, 86, 149, 155, 307 Social Work, 133, 142, 145, 150, 153, 203, 206, 307 Sodium, 20, 307, 314 Sodium Channels, 20, 307, 314 Soft tissue, 200, 254, 292, 306, 307 Solid tumor, 249, 307 Soma, 307 Somatic, 48, 246, 285, 286, 295, 307 Somatic cells, 285, 286, 307 Sorbitol, 10, 307 Spastic, 19, 73, 85, 87, 98, 136, 307 Spasticity, 307 Spatial disorientation, 266, 307 Specialist, 166, 234, 266, 307, 308 Specificity, 30, 246, 307 Spectrin, 268, 307 Spectrum, 23, 29, 43, 58, 78, 229, 308 Speech pathologist, 202, 308 Sperm, 258, 308, 313 Spinal cord, 34, 245, 251, 254, 257, 258, 285, 289, 290, 294, 295, 301, 308 Spinal Cord Diseases, 294, 301, 308 Spinal Nerves, 295, 308 Spleen, 248, 263, 284, 308 Sporadic, 289, 308 Stabilization, 31, 308 Staging, 24, 304, 308 Statistically significant, 10, 308 Status Epilepticus, 95, 308 Steel, 259, 308 Stereotyped Behavior, 50, 163, 308 Stereotypy, 50, 59, 117, 308 Sterility, 185, 279, 308 Sterilization, 90, 308 Steroid, 253, 262, 308 Stimulant, 265, 276, 286, 309 Stimulus, 18, 54, 159, 267, 268, 270, 280, 281, 309, 311, 312 Stomach, 245, 266, 270, 272, 277, 289, 306, 308, 309 Stool, 279, 282, 309 Streptococci, 11, 309 Stress, 4, 7, 32, 86, 127, 138, 139, 144, 145, 146, 155, 200, 252, 256, 262, 289, 303, 309 Stria, 32, 309 Stroke, 168, 172, 183, 187, 210, 216, 225, 226, 227, 255, 281, 309 Stroma, 281, 294, 309

330 Mental Retardation

Subacute, 279, 309 Subclinical, 279, 304, 309 Subcutaneous, 171, 178, 283, 309 Subiculum, 276, 309 Submandibular, 309 Submaxillary, 51, 309 Subspecies, 307, 309 Supplementation, 29, 117, 309 Support group, 203, 309 Suppositories, 171, 179, 273, 309 Suspensions, 171, 309 Sympathomimetic, 265, 267, 269, 291, 309 Symphysis, 299, 310 Synapses, 64, 291, 310 Synapsis, 310 Synaptic, 16, 31, 34, 42, 44, 53, 61, 64, 67, 306, 310 Synaptic Membranes, 31, 310 Synaptic Vesicles, 310 Synergistic, 27, 299, 310, 311 Systemic, 248, 254, 264, 269, 279, 280, 310 Systolic, 277, 310 T Tardive, 250, 310 Telencephalon, 252, 310 Telomere, 41, 60, 101, 310 Temporal, 45, 59, 74, 102, 275, 276, 310 Teratogens, 51, 310 Testicular, 152, 175, 182, 310 Testis, 274, 310 Thalamic, 251, 310, 311 Thalamic Diseases, 251, 311 Thalassemia, 67, 311 Therapeutics, 311 Threonine, 77, 175, 182, 305, 311 Threshold, 144, 277, 311 Thrombin, 186, 188, 271, 297, 299, 300, 311 Thrombomodulin, 299, 311 Thrombosis, 299, 309, 311 Thrombus, 262, 279, 281, 288, 297, 311 Thyroid, 278, 311, 313 Thyrotropin, 278, 311 Thyroxine, 247, 296, 311 Tic, 68, 311 Tin, 295, 297, 311 Tinnitus, 228, 311, 315 Tissue Culture, 15, 18, 20, 38, 44, 312 Toilet Training, 200, 312 Tomography, 44, 312 Tonicity, 268, 312 Tooth Loss, 201, 312 Tooth Preparation, 245, 312

Topical, 257, 294, 312 Toxic, iv, 197, 274, 290, 295, 312 Toxicity, 267, 312 Toxicology, 22, 184, 197, 218, 312 Toxins, 250, 279, 287, 292, 312 Traction, 259, 312 Transaminases, 62, 312 Transcription Factors, 59, 90, 312 Transcutaneous, 16, 312 Transcutaneous Electric Nerve Stimulation, 16, 312 Transduction, 174, 175, 180, 183, 188, 306, 312 Transfection, 253, 312 Transferases, 274, 312 Transgenes, 38, 312 Translation, 53, 65, 103, 132, 313 Translational, 21, 26, 27, 53, 103, 313 Translocation, 24, 42, 48, 60, 74, 75, 81, 95, 104, 232, 258, 313 Transmitter, 245, 251, 267, 281, 284, 291, 310, 313 Transplantation, 48, 91, 92, 313 Trauma, 264, 289, 313 Tremor, 14, 280, 294, 313 Trigger zone, 250, 313 Trinucleotide Repeat Expansion, 14, 313 Trinucleotide Repeats, 313 Trisomy, 36, 47, 96, 104, 172, 313 Trophoblast, 253, 313 Tryptophan, 260, 305, 313 Tubulin, 43, 313 Type 2 diabetes, 87, 313 Tyrosine, 31, 38, 122, 267, 313 U Ubiquitin, 53, 313 Ultrasonography, 273, 313 Unconscious, 278, 313 Urea, 57, 251, 314 Ureters, 314 Urethra, 299, 314 Urinary, 97, 104, 170, 173, 174, 175, 180, 182, 185, 187, 188, 208, 269, 273, 277, 279, 314 Urinary Retention, 170, 173, 174, 175, 180, 182, 185, 187, 188, 314 Urinary tract, 104, 208, 314 Urinary tract infection, 104, 208, 314 Urinate, 312, 314 Urine, 253, 262, 263, 266, 269, 274, 279, 314 Urogenital, 51, 273, 314 Uterus, 262, 285, 290, 314

Index 331

V Vaccine, 300, 314 Vagina, 265, 285, 314 Valproic Acid, 112, 314 Vascular, 187, 258, 269, 277, 279, 291, 308, 311, 314 Vasodilator, 254, 267, 270, 276, 314 Vector, 35, 57, 312, 314 Vein, 280, 291, 314 Venous, 257, 291, 297, 299, 314 Venous blood, 257, 297, 314 Ventricle, 276, 278, 301, 310, 314 Ventricular, 96, 106, 187, 277, 314 Venules, 254, 314 Vertebrae, 308, 315 Vestibular, 185, 315 Vestibule, 259, 280, 305, 315 Vestibulocochlear Nerve, 311, 315 Vestibulocochlear Nerve Diseases, 311, 315 Veterinary Medicine, 217, 315 Villi, 277, 315 Vinblastine, 313, 315 Vincristine, 313, 315 Viral, 20, 24, 34, 35, 57, 170, 173, 174, 175, 180, 182, 187, 312, 315 Viral vector, 34, 35, 57, 315

Virulence, 251, 312, 315 Virus, 25, 35, 229, 252, 257, 273, 296, 312, 315 Virus Replication, 25, 315 Viscera, 307, 315 Visual Cortex, 53, 315 Visual field, 292, 303, 315 Vitreous Body, 258, 303, 315 Vitro, 316 Vivo, 23, 32, 34, 35, 40, 44, 57, 127, 154, 316 Voice Disorders, 203, 316 W Wakefulness, 264, 316 White blood cell, 245, 249, 284, 287, 288, 291, 297, 316 Withdrawal, 50, 264, 316 Wound Healing, 187, 256, 316 X Xenograft, 249, 316 X-ray, 97, 164, 166, 261, 272, 284, 291, 304, 316 Y Yeasts, 272, 295, 316 Z Zygote, 261, 287, 316 Zymogen, 299, 316

332 Mental Retardation

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