The Official Parent's Sourcebook on Chiari Malformation: Updated Directory for the Internet Age

THE OFFICIAL PARENT’S SOURCEBOOK on CHIARI MALFORMATION J AMES N. P ARKER , M.D. AND P HILIP M. P ARKER , P H .D., E ...

Author: Icon Health Publications

6 downloads 275 Views 840KB Size Report

This content was uploaded by our users and we assume good faith they have the permission to share this book. If you own the copyright to this book and it is wrongfully on our website, we offer a simple DMCA procedure to remove your content from our site. Start by pressing the button below!

Report copyright / DMCA form

DOWNLOAD PDF

THE OFFICIAL PARENT’S SOURCEBOOK

on

CHIARI

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

ii

ICON Health Publications ICON Group International, Inc. 4370 La Jolla Village Drive, 4th Floor San Diego, CA 92122 USA Copyright Ó2003 by ICON Group International, Inc. Copyright Ó2003 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: Tiffany LaRochelle 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 as a substitute for consultation with your child’s physician. All matters regarding your child’s health require medical supervision. 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, in close consultation with a qualified physician. The reader is advised to always check product information (package inserts) for changes and new information regarding dose and contraindications before administering 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., 1960The Official Parent’s Sourcebook on Chiari Malformation: A Revised and Updated Directory for the Internet Age/James N. Parker and Philip M. Parker, editors p. cm. Includes bibliographical references, glossary and index. ISBN: 0-597-83674-4 1. Chiari Malformation-Popular works. I. Title.

iii

Disclaimer This publication is not intended to be used for the diagnosis or treatment of a health problem or as a substitute for consultation with licensed medical professionals. 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, or the authors are not responsible for the content of any Web pages nor publications referenced in this publication.

Copyright Notice If a physician wishes to copy limited passages from this sourcebook for parent use, this right is automatically granted without written permission from ICON Group International, Inc. (ICON Group). However, all of ICON Group publications are copyrighted. 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 sourcebook.

iv

Dedication To the healthcare professionals dedicating their time and efforts to the study of Chiari malformation.

Acknowledgements The collective knowledge generated from academic and applied research summarized in various references has been critical in the creation of this sourcebook which is best viewed as a comprehensive compilation and collection of information prepared by various official agencies which directly or indirectly are dedicated to Chiari malformation. All of the Official Parent’s Sourcebooks 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 sourcebook. 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 LaRochelle for her excellent editorial support.

v

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 the Official Parent’s Sourcebook series published 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 the Official Parent’s Sourcebook series published by ICON Health Publications.

vi

About ICON Health Publications In addition to Chiari malformation, Official Parent’s Sourcebooks are available for the following related topics: ·

The Official Patient's Sourcebook on Aicardi Syndrome

·

The Official Patient's Sourcebook on Craniosynostosis

·

The Official Patient's Sourcebook on Dandy Walker Syndrome

·

The Official Patient's Sourcebook on Encephaloceles

·

The Official Patient's Sourcebook on Shaken Baby Syndrome

·

The Official Patient's Sourcebook on Spina Bifida

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

vii

Table of Contents INTRODUCTION ...................................................................................... 1

Overview ...............................................................................................................1 Organization .........................................................................................................3 Scope ......................................................................................................................3 Moving Forward ...................................................................................................4

PART I: THE ESSENTIALS ................................................. 7 CHAPTER 1. THE ESSENTIALS ON CHIARI MALFORMATION: GUIDELINES ........................................................................................... 9

Overview ...............................................................................................................9 What Is Chiari Malformation?............................................................................10 Is There Any Treatment? ....................................................................................11 What Is the Prognosis? .......................................................................................11 What Research Is Being Done? ...........................................................................11 For More Information..........................................................................................12 More Guideline Sources ......................................................................................12 Vocabulary Builder..............................................................................................15

CHAPTER 2. SEEKING GUIDANCE ....................................................... 17

Overview .............................................................................................................17 Associations and Chiari Malformation ...............................................................17 Finding Associations ...........................................................................................20 Finding Doctors...................................................................................................21 Finding a Neurologist .........................................................................................23 Selecting Your Doctor .........................................................................................23 Working with Your Child’s Doctor.....................................................................23 Broader Health-Related Resources ......................................................................24

CHAPTER 3. CLINICAL TRIALS AND CHIARI MALFORMATION .......... 27

Overview .............................................................................................................27 Recent Trials on Chiari Malformation ................................................................30 Benefits and Risks................................................................................................31 Keeping Current on Clinical Trials.....................................................................34 General References...............................................................................................35 Vocabulary Builder..............................................................................................36

PART II: ADDITIONAL RESOURCES AND ADVANCED MATERIAL.................................................. 37 CHAPTER 4. STUDIES ON CHIARI MALFORMATION............................ 39

Overview .............................................................................................................39 Federally Funded Research on Chiari Malformation ..........................................39 The National Library of Medicine: PubMed .......................................................54 Vocabulary Builder..............................................................................................71

viii Contents

CHAPTER 5. BOOKS ON CHIARI MALFORMATION .............................. 79

Overview .............................................................................................................79 The National Library of Medicine Book Index ....................................................79 Chapters on Chiari Malformation .......................................................................84 General Home References ....................................................................................86 Vocabulary Builder..............................................................................................87

CHAPTER 6. MULTIMEDIA ON CHIARI MALFORMATION ................... 89

Overview .............................................................................................................89 Bibliography: Multimedia on Chiari Malformation............................................89 Vocabulary Builder..............................................................................................92

CHAPTER 7. PERIODICALS AND NEWS ON CHIARI MALFORMATION 95

Overview .............................................................................................................95 News Services and Press Releases.......................................................................95 Newsletter Articles..............................................................................................97 Academic Periodicals covering Chiari Malformation .........................................99 Vocabulary Builder............................................................................................101

CHAPTER 8. PHYSICIAN GUIDELINES AND DATABASES ................... 103

Overview ...........................................................................................................103 NIH Guidelines .................................................................................................103 NIH Databases ..................................................................................................104 Other Commercial Databases ............................................................................107 The Genome Project and Chiari Malformation .................................................108 Specialized References .......................................................................................112 Vocabulary Builder............................................................................................114

PART III. APPENDICES .................................................. 115 APPENDIX A. RESEARCHING ALTERNATIVE MEDICINE ................... 117

Overview ...........................................................................................................117 What Is CAM? ..................................................................................................118 What Are the Domains of Alternative Medicine? ............................................118 Can Alternatives Affect My Child’s Treatment?..............................................122 Finding CAM References on Chiari Malformation ..........................................122 Additional Web Resources.................................................................................123 General References.............................................................................................124

APPENDIX B. RESEARCHING NUTRITION.......................................... 127

Overview ...........................................................................................................127 Food and Nutrition: General Principles............................................................127 Finding Studies on Chiari Malformation..........................................................132 Federal Resources on Nutrition.........................................................................133 Additional Web Resources.................................................................................134 Vocabulary Builder............................................................................................134

APPENDIX C. FINDING MEDICAL LIBRARIES .................................... 137

Overview ...........................................................................................................137

Contents

ix

Preparation ........................................................................................................137 Finding a Local Medical Library.......................................................................138 Medical Libraries in the U.S. and Canada ........................................................138

APPENDIX D. YOUR CHILD’S RIGHTS AND INSURANCE................... 145

Overview ...........................................................................................................145 Your Child’s Rights as a Patient.......................................................................145 Parent Responsibilities ......................................................................................149 Choosing an Insurance Plan .............................................................................150 Medicaid ............................................................................................................152 NORD’s Medication Assistance Programs ......................................................152 Additional Resources.........................................................................................153

ONLINE GLOSSARIES.................................................... 155 Online Dictionary Directories ..........................................................................156

CHIARI MALFORMATION GLOSSARY ................... 157 General Dictionaries and Glossaries .................................................................169

INDEX................................................................................... 171

1

INTRODUCTION Overview Dr. C. Everett Koop, former U.S. Surgeon General, once said, “The best prescription is knowledge.”1 The Agency for Healthcare Research and Quality (AHRQ) of the National Institutes of Health (NIH) echoes this view and recommends that all parents incorporate education into the treatment process. According to the AHRQ: Finding out more about your [child’s] condition is a good place to start. By contacting groups that support your [child’s] condition, visiting your local library, and searching on the Internet, you can find good information to help guide your decisions for your [child’s] treatment. Some information may be hard to find—especially if you don’t know where to look.2 As the AHRQ mentions, finding the right information is not an obvious task. Though many physicians and public officials had thought that the emergence of the Internet would do much to assist parents in obtaining reliable information, 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.3 Quotation from http://www.drkoop.com. The Agency for Healthcare Research and Quality (AHRQ): http://www.ahcpr.gov/consumer/diaginfo.htm. 3 From the NIH, National Cancer Institute (NCI): http://cancertrials.nci.nih.gov/beyond/evaluating.html. 1 2

2

Chiari Malformation

Since the late 1990s, physicians have seen a general increase in parent Internet usage rates. Parents frequently enter their children’s doctor’s offices with printed Web pages of home remedies in the guise of latest medical research. This scenario is so common that doctors often spend more time dispelling misleading information than guiding children through sound therapies. The Official Parent’s Sourcebook on Chiari Malformation has been created for parents who have decided to make education and research an integral part of the treatment process. The pages that follow will tell you where and how to look for information covering virtually all topics related to Chiari malformation, from the essentials to the most advanced areas of research. The title of this book includes the word “official.” This reflects the fact that the sourcebook draws from public, academic, government, and peerreviewed research. Selected readings from various agencies are reproduced to give you some of the latest official information available to date on Chiari malformation. Given parents’ increasing sophistication in using the Internet, abundant references to reliable Internet-based resources are provided throughout this sourcebook. Where possible, guidance is provided on how to obtain free-ofcharge, primary research results as well as more detailed information via the Internet. E-book and electronic versions of this sourcebook are fully interactive with each of the Internet sites mentioned (clicking on a hyperlink automatically opens your browser to the site indicated). Hard copy users of this sourcebook can type cited Web addresses directly into their browsers to obtain access to the corresponding sites. Since we are working with ICON Health Publications, hard copy Sourcebooks are frequently updated and printed on demand to ensure that the information provided is current. In addition to extensive references accessible via the Internet, every chapter presents a “Vocabulary Builder.” Many health guides offer glossaries of technical or uncommon terms in an appendix. In editing this sourcebook, we have decided to place a smaller glossary within each chapter that covers terms used in that chapter. Given the technical nature of some chapters, you may need to revisit many sections. Building one’s vocabulary of medical terms in such a gradual manner has been shown to improve the learning process. We must emphasize that no sourcebook on Chiari malformation should affirm that a specific diagnostic procedure or treatment discussed in a research study, patent, or doctoral dissertation is “correct” or your child’s

Introduction

3

best option. This sourcebook is no exception. Each child is unique. Deciding on appropriate options is always up to parents in consultation with their children’s physicians and healthcare providers.

Organization This sourcebook is organized into three parts. Part I explores basic techniques to researching Chiari malformation (e.g. finding guidelines on diagnosis, treatments, and prognosis), followed by a number of topics, including information on how to get in touch with organizations, associations, or other parent networks dedicated to Chiari malformation. It also gives you sources of information that can help you find a doctor in your local area specializing in treating Chiari malformation. Collectively, the material presented in Part I is a complete primer on basic research topics for Chiari malformation. Part II moves on to advanced research dedicated to Chiari malformation. Part II is intended for those willing to invest many hours of hard work and study. It is here that we direct you to the latest scientific and applied research on Chiari malformation. When possible, contact names, links via the Internet, and summaries are provided. It is in Part II where the vocabulary process becomes important as authors publishing advanced research frequently use highly specialized language. In general, every attempt is made to recommend “free-to-use” options. Part III provides appendices of useful background reading covering Chiari malformation or related disorders. The appendices are dedicated to more pragmatic issues facing parents. Accessing materials via medical libraries may be the only option for some parents, so a guide is provided for finding local medical libraries which are open to the public. Part III, therefore, focuses on advice that goes beyond the biological and scientific issues facing children with Chiari malformation and their families.

Scope While this sourcebook covers Chiari malformation, doctors, research publications, and specialists may refer to your child’s condition using a variety of terms. Therefore, you should understand that Chiari malformation is often considered a synonym or a condition closely related to the following: ·

Arnold-chiari Malformation

4

Chiari Malformation

·

Cerebellomedullary Malformation Syndrome

In addition to synonyms and related conditions, physicians may refer to Chiari malformation using certain coding systems. The International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) is the most commonly used system of classification for the world’s illnesses. Your physician may use this coding system as an administrative or tracking tool. The following classification is commonly used for Chiari malformation:4 ·

741.0 with hydrocephalus

For the purposes of this sourcebook, we have attempted to be as inclusive as possible, looking for official information for all of the synonyms relevant to Chiari malformation. You may find it useful to refer to synonyms when accessing databases or interacting with healthcare professionals and medical librarians.

Moving Forward Since the 1980s, the world has seen a proliferation of healthcare guides covering most illnesses. Some are written by parents, patients, or their family members. These generally take a layperson’s approach to understanding and coping with an illness or disorder. They can be uplifting, encouraging, and highly supportive. Other guides are authored by physicians or other healthcare providers who have a more clinical outlook. Each of these two styles of guide has its purpose and can be quite useful. As editors, we have chosen a third route. We have chosen to expose you to as many sources of official and peer-reviewed information as practical, for the purpose of educating you about basic and advanced knowledge as recognized by medical science today. You can think of this sourcebook as your personal Internet age reference librarian. Why “Internet age”? When their child has been diagnosed with Chiari malformation, parents will often log on to the Internet, type words into a search engine, and receive several Web site listings which are mostly irrelevant or redundant. Parents are left to wonder where the relevant This list is based on the official version of the World Health Organization’s 9th Revision, International Classification of Diseases (ICD-9). According to the National Technical Information Service, “ICD-9CM extensions, interpretations, modifications, addenda, or errata other than those approved by the U.S. Public Health Service and the Health Care Financing Administration are not to be considered official and should not be utilized. Continuous maintenance of the ICD-9-CM is the responsibility of the federal government.” 4

Introduction

5

information is, and how to obtain it. Since only the smallest fraction of information dealing with Chiari malformation is even indexed in search engines, a non-systematic approach often leads to frustration and disappointment. With this sourcebook, we hope to direct you to the information you need that you would not likely find using popular Web directories. Beyond Web listings, in many cases we will reproduce brief summaries or abstracts of available reference materials. These abstracts often contain distilled information on topics of discussion. While we focus on the more scientific aspects of Chiari malformation, there is, of course, the emotional side to consider. Later in the sourcebook, we provide a chapter dedicated to helping you find parent groups and associations that can provide additional support beyond research produced by medical science. We hope that the choices we have made give you and your child the most options in moving forward. In this way, we wish you the best in your efforts to incorporate this educational approach into your child’s treatment plan. The Editors

7

PART I: THE ESSENTIALS

ABOUT PART I Part I has been edited to give you access to what we feel are “the essentials” on Chiari malformation. The essentials typically include a definition or description of the condition, a discussion of who it affects, the signs or symptoms, tests or diagnostic procedures, and treatments for disease. Your child’s doctor or healthcare provider may have already explained the essentials of Chiari malformation to you or even given you a pamphlet or brochure describing the condition. Now you are searching for more in-depth information. As editors, we have decided, nevertheless, to include a discussion on where to find essential information that can complement what the doctor has already told you. In this section we recommend a process, not a particular Web site or reference book. The process ensures that, as you search the Web, you gain background information in such a way as to maximize your understanding.

9

CHAPTER 1. THE ESSENTIALS ON CHIARI MALFORMATION: GUIDELINES Overview Official agencies, as well as federally funded institutions supported by national grants, frequently publish a variety of guidelines on Chiari malformation. 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. The great advantage of guidelines over other sources is that they are often written with the parent in mind. Since new guidelines on Chiari malformation can appear at any moment and be published by a number of sources, the best approach to finding guidelines is to systematically scan the Internet-based services that post them.

The National Institutes of Health (NIH)5 The National Institutes of Health (NIH) is the first place to search for relatively current guidelines and fact sheets on Chiari malformation. Originally founded in 1887, the NIH is one of the world’s foremost medical research centers and the federal focal point for medical research in the United States. At any given time, the NIH supports some 35,000 research grants at universities, medical schools, and other research and training institutions, both nationally and internationally. The rosters of those who have conducted research or who have received NIH support over the years include the world’s most illustrious scientists and physicians. Among them are 97 scientists who have won the Nobel Prize for achievement in medicine.

5

Adapted from the NIH: http://www.nih.gov/about/NIHoverview.html.

10 Chiari Malformation

There is no guarantee that any one Institute will have a guideline on a specific medical condition, though the National Institutes of Health collectively publish over 600 guidelines for both common and rare disorders. The best way to access NIH guidelines is via the Internet. Although the NIH is organized into many different Institutes and Offices, the following is a list of key Web sites where you are most likely to find NIH clinical guidelines and publications dealing with Chiari malformation and associated conditions: ·

Office of the Director (OD); guidelines consolidated across agencies available at http://www.nih.gov/health/consumer/conkey.htm

·

National Library of Medicine (NLM); extensive encyclopedia (A.D.A.M., Inc.) with guidelines available at http://www.nlm.nih.gov/medlineplus/healthtopics.html

·

National Institute of Neurological Disorders and Stroke (NINDS); http://www.ninds.nih.gov/health_and_medical/disorder_index.htm

Among the above, the National Institute of Neurological Disorders and Stroke (NINDS) is particularly noteworthy. The mission of the NINDS is to reduce the burden of neurological disease—a burden borne by every age group, by every segment of society, by people all over the world.6 To support this mission, the NINDS conducts, fosters, coordinates, and guides research on the causes, prevention, diagnosis, and treatment of neurological disorders and stroke, and supports basic research in related scientific areas. The following patient guideline was recently published by the NINDS on Chiari malformation.

What Is Chiari Malformation?7 Arnold-Chiari Malformation is a condition in which the cerebellum portion of the brain protrudes into the spinal canal. It may or may not be apparent at birth. Arnold-Chiari I type malformation usually causes symptoms in young adults and is often associated with syringomyelia, in which a tubular cavity develops within the spinal cord. Arnold-Chiari II type malformation is associated with myelomeningocele (a defect of the spine) and hydrocephalus (increased cerebrospinal fluid and pressure within the brain), which usually This paragraph has been adapted from the NINDS: http://www.ninds.nih.gov/about_ninds/mission.htm. “Adapted” signifies that a passage has been reproduced exactly or slightly edited for this book. 7 Adapted from The National Institute of Neurological Disorders and Stroke (NINDS): http://www.ninds.nih.gov/health_and_medical/disorders/chiari_doc.htm. 6

Guidelines 11

are apparent at birth. Myelomeningocele usually causes paralysis of the legs and, less commonly, the arms. If left untreated, hydrocephalus can cause mental impairment. Either type of Arnold-Chiari Malformation can cause symptoms of headache, vomiting, difficulty swallowing, and hoarseness. Adults and adolescents who are unaware they have Arnold-Chiari I type malformation may develop headache that is predominantly located in the back of the head and is increased by coughing or straining. Symptoms of progressive brain impairment may include dizziness, an impaired ability to coordinate movement, double vision, and involuntary, rapid, downward eye movements.

Is There Any Treatment? Infants and children with myelomeningocele may require surgery to repair protrusion of the meningeal sac into the spinal cord. Hydrocephalus may be treated with surgical implantation of a shunt to relieve increased pressure on the brain. Some adults with Arnold-Chiari Malformation may benefit from surgery in which the existing opening in the back of the skull is enlarged.

What Is the Prognosis? Infants with very severe malformations may have life-threatening complications. Most patients who have surgery experience a reduction in their symptoms. Some patients may experience prolonged periods of relative stability.

What Research Is Being Done? Research supported by the NINDS includes studies to understand how the brain and nervous system normally develop and function and how they are affected by disease and trauma. These studies contribute to a greater understanding of congenital birth defects, such as Arnold-Chiari Malformation, and open promising new doors to potential treatments.

12 Chiari Malformation

For More Information For more information, contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 [email protected] http://www.modimes.org Tel: 914-428-7100 / 888-MODIMES (663-4637) Fax: 914-428-8203 National Organization for Rare Disorders (NORD) P.O. Box 8923 (100 Route 37) New Fairfield, CT 06812-8923 [email protected] http://www.rarediseases.org Tel: 203-746-6518 / 800-999-NORD (6673) Fax: 203-746-6481 Spina Bifida Association of America 4590 MacArthur Blvd. NW Suite 250 Washington, DC 20007-4266 [email protected] http://www.sbaa.org Tel: 202-944-3285 / 800-621-3141 Fax: 202-944-3295

More Guideline Sources The guideline above on Chiari malformation is only one example of the kind of material that you can find online and free of charge. The remainder of this chapter will direct you to other sources which either publish or can help you find additional guidelines on topics related to Chiari malformation. Many of the guidelines listed below address topics that may be of particular relevance to your child’s specific situation, while certain guidelines will apply to only some children with Chiari malformation. 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

Guidelines 13

cases where the contact information is provided, contacting the publisher or author directly.

Topic Pages: MEDLINEplus For parents wishing to go beyond guidelines published by specific Institutes of the NIH, the National Library of Medicine has created a vast and parentoriented healthcare information portal called MEDLINEplus. Within this Internet-based system are “health topic pages.” You can think of a health topic page as a guide to patient guides. 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. 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.

The Combined Health Information Database (CHID) CHID Online is a reference tool that maintains a database directory of thousands of journal articles and educational guidelines on Chiari malformation and related conditions. One of the advantages of CHID over other sources is that it 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: ·

Medical Facts about Spina Bifida Source: Baltimore, MD: Kennedy Krieger Institute. 1995. [12 p.]. Contact: Available from Kennedy Krieger Institute. 707 North Broadway, Baltimore, MD 21205. (800) 873-3377 or (410) 550-9000; FAX (410) 5509498; TTY (410) 550-9806. PRICE: Free.

14 Chiari Malformation

Summary: This booklet, written for patients and their families, provides general information about spina bifida. The types of spina bifida and how they affect the spinal nerves are described. Complications such as Chiari malformation, hydrocephalus, learning disabilities, seizures, and allergies are also discussed. A glossary of medical terms is included.

Healthfinder™ Healthfinder™ is an additional source sponsored by the U.S. Department of Health and Human Services which 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: ·

Klippel Trenaunay Syndrome Patient Education Summary: The information on this web page is designed to assist parents of newly-diagnosed patients with Klippel-Trenaunay (K-P) syndrome, a rare congenital malformation that may include a port-wine stain or Source: National Organization for Rare Disorders, Inc. http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&R ecordID=4653

·

Klippel-Trenaunay Syndrome Fact Sheet Summary: An overview of the Klippel-Trenaunay (K-T) syndrome, a rare congenital malformation that may include a port-wine stain or Source: Klippel-Trenaunay Syndrome Support Group http://www.healthfinder.gov/scripts/recordpass.asp?RecordType=0&R ecordID=4652

The NIH Search Utility After browsing the references listed at the beginning of this chapter, you may want to explore the NIH search utility. This allows you to search for documents on over 100 selected Web sites that comprise the NIH-WEBSPACE. 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 Chiari malformation. The drawbacks of this approach are that the information is not organized by theme and that the references are often a mix of information for professionals and parents. Nevertheless, a

Guidelines 15

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 that often link to government sites are available to the public. 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/

·

WebMDÒHealth: http://my.webmd.com/health_topics

Vocabulary Builder The material in this chapter may have contained a number of unfamiliar words. The following Vocabulary Builder introduces you to terms used in this chapter that have not been covered in the previous chapter: 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]

Asymptomatic: Showing or causing no symptoms. [EU] 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] Dizziness: An imprecise term which may refer to a sense of spatial disorientation, motion of the environment, or lightheadedness. [NIH] Hydrocephalus: A condition marked by dilatation of the cerebral ventricles, most often occurring secondarily to obstruction of the cerebrospinal fluid

16 Chiari Malformation

pathways, and accompanied by an accumulation of cerebrospinal fluid within the skull; the fluid is usually under increased pressure, but occasionally may be normal or nearly so. It is typically characterized by enlargement of the head, prominence of the forehead, brain atrophy, mental deterioration, and convulsions; may be congenital or acquired; and may be of sudden onset (acute h.) or be slowly progressive (chronic or primary b.). [EU]

Implantation: The insertion or grafting into the body of biological, living, inert, or radioactive material. [EU] Malformation: A morphologic defect resulting from an intrinsically abnormal developmental process. [EU] Mental: Pertaining to the mind; psychic. 2. (L. mentum chin) pertaining to the chin. [EU] Neurology: A medical specialty concerned with the study of the structures, functions, and diseases of the nervous system. [NIH] Neurosurgery: A surgical specialty concerned with the treatment of diseases and disorders of the brain, spinal cord, and peripheral and sympathetic nervous system. [NIH] Paralysis: Loss or impairment of motor function in a part due to lesion of the neural or muscular mechanism; also by analogy, impairment of sensory function (sensory paralysis). In addition to the types named below, paralysis is further distinguished as traumatic, syphilitic, toxic, etc., according to its cause; or as obturator, ulnar, etc., according to the nerve part, or muscle specially affected. [EU] Progressive: Advancing; going forward; going from bad to worse; increasing in scope or severity. [EU] 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] Shunt: 1. to turn to one side; to divert; to bypass. 2. a passage or anastomosis between two natural channels, especially between blood vessels. Such structures may be formed physiologically (e.g. to bypass a thrombosis) or they may be structural anomalies. 3. a surgically created anastomosis; also, the operation of forming a shunt. [EU] Skull: The skeleton of the head including the bones of the face and the bones enclosing the brain. [NIH]

17

CHAPTER 2. SEEKING GUIDANCE Overview Some parents are comforted by the knowledge that a number of organizations dedicate their resources to helping people with Chiari malformation. These associations can become invaluable sources of information and advice. Many associations offer parent support, financial assistance, and other important services. Furthermore, healthcare research has shown that support groups often help people to better cope with their conditions.8 In addition to support groups, your child’s physician can be a valuable source of guidance and support. In this chapter, we direct you to resources that can help you find parent organizations and medical specialists. We begin by describing how to find associations and parent groups that can help you better understand and cope with your child’s condition. The chapter ends with a discussion on how to find a doctor that is right for your child.

Associations and Chiari Malformation In addition to associations or groups that your child’s doctor might recommend, we suggest that you consider the following list (if there is a fee for an association, you may want to check with your child’s insurance provider to find out if the cost will be covered):

Churches, synagogues, and other houses of worship might also have groups that can offer you the social support you need.

8

18 Chiari Malformation

·

American Syringomyelia Alliance Project, Inc Address: American Syringomyelia Alliance Project, Inc. 300 Green Street, Suite 206, P.O. Box 1586, Longview, TX 75606-1586 Telephone: (903) 236-7079 Toll-free: (800) 272-7282 Fax: (903) 757-7456 Email: [email protected] Web Site: http://www.asap4sm.com Background: The American Syringomyelia Alliance Project (ASAP), which was established in 1988, is a national nonprofit organization that functions as a clearinghouse for information on syringomyelia, a rare progressive condition characterized by the presence of abnormal, fluidfilled cavities within the spinal cord and Chiari malformation. The organization provides information to people with syringomyelia and Chiari malformation and seeks to increase public awareness of the disorder. The Project also encourages medical research and seeks to raise funds for the investigation into the possible causes of and the development of new treatments for the disorder. The American Syringomyelia Alliance Project publishes a newsletter and produces informational materials and videotapes of lectures on syringomyelia and Chiari malformation by prominent neurosurgeons. The Project also holds an annual conference to bring people with syringomyelia and/or Chiari malformation together and has a self-help network project.

·

Back Pain Association of America, Inc Address: Back Pain Association of America, Inc. P.O. Box 135, Pasadena, MD 21123-0135 Telephone: (410) 255-3633 Toll-free: (888) 663-4637 Fax: (410) 255- 7338 Email: [email protected] Background: The Back Pain Association of America, Inc. (BPAA) is a national nonprofit organization dedicated to providing information and support to people who are affected by back and neck pain, their family members, friends, and health care professionals. Established in 1991 and consisting of nearly 4,000 members, BPAA offers programs and information to help affected individuals learn more about their spinal disorders and ways to cope with them. The organization also has a program to help individuals prevent back injuries. BPAA publishes a self-titled quarterly newsletter that helps readers stay informed of updated information and new forms of treatment. The organization's

Seeking Guidance 19

'Friends Across America' networking program enables affected individuals to exchange information and support via telephone. BPAA also has a physician referral service as well as an information service for physicians who treat back and neck pain. In addition, the Association also promotes research and offers a variety of fact sheets including 'The Relationship Between Nerve Damage and Leg Pain,' 'Urinary Problems and Diseases of the Spine,' 'Arachnoiditis, Questions and Answers,' and 'A Guide to Abdominal and Stretching Exercises.'. Relevant area(s) of interest: Spina Bifida ·

Canadian Syringomyelia Network Address: Canadian Syringomyelia Markham, Ontario, L3P 5X7, Canada

Network

69

Penny

Crescent,

Telephone: (905) 471-8278 Toll-free: (888) 663-4637 Fax: (905) 882-8367 Email: [email protected] Web Site: http://www.passport.ca/~csn Background: The Canadian Syringomyelia Network (CSN) is a nonprofit organization dedicated to serving as a support network for people with syringomyelia, a rare progressive condition characterized by the presence of abnormal, fluid-filled cavities within the spinal cord. Established in 1992, CSN acts as an information and referral network; builds awareness among the community; assists those who wish to establish support groups; compiles a database of people in Canada who have shown an interest in CSN and its mission; and produces a newsletter for its members and those interested in CSN's activities. The Canadian Syringomyelia Network also organizes periodic meetings and conferences, offers patient networking services, provides referrals, and produces educational materials including brochures, reports, and a regular newsletter. The Network maintains a web site at http://www.passport.ca/~csn. ·

March of Dimes Birth Defects Foundation Address: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue, White Plains, NY 10605 Telephone: (914) 428-7100 Toll-free: (888) 663-4637 Fax: (914) 997-4763 Email: [email protected] Web Site: http://www.modimes.org

20 Chiari Malformation

Background: The March of Dimes Birth Defects Foundation is a national not-for- profit organization that was established in 1938. The mission of the Foundation is to improve the health of babies by preventing birth defects and infant mortality. Through the Campaign for Healthier Babies, the March of Dimes funds programs of research, community services, education, and advocacy. Educational programs that seek to prevent birth defects are important to the Foundation and to that end it produces a wide variety of printed informational materials and videos. The March of Dimes public health educational materials provide information encouraging health- enhancing behaviors that lead to a healthy pregnancy and a healthy baby. Relevant area(s) of interest: Aicardi Syndrome, Spina Bifida

Finding Associations There are several Internet directories that provide lists of medical associations with information on or resources relating to diseasex. By consulting all of associations listed in this chapter, you will have nearly exhausted all sources for patient associations concerned with diseasex. 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 Chiari malformation. 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.

DIRLINE A comprehensive source of information on associations is the DIRLINE database maintained by the National Library of Medicine. The database comprises some 10,000 records of organizations, research centers, and government institutes and associations which primarily focus on health and biomedicine. DIRLINE is available via the Internet at the following Web site: http://dirline.nlm.nih.gov. Simply type in “Chiari malformation” (or a synonym) or the name of a topic, and the site will list information contained in the database on all relevant organizations.

Seeking Guidance 21

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 “Chiari malformation”. 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.” By making these selections and typing in “Chiari malformation” (or synonyms) into the “For these words:” box, you will only receive results on organizations dealing with Chiari malformation. You should check back periodically with this database since it is updated every 3 months. 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 specific medical conditions. You can access this database at the following Web site: http://www.rarediseases.org/search/orgsearch.html. Type “diseasex” (or a synonym) in the search box, and click “Submit Query.”

Online Support Groups In addition to support groups, commercial Internet service providers offer forums and chat rooms to discuss different illnesses and conditions. WebMDÒ, for example, offers such a service at its Web site: http://boards.webmd.com/roundtable. These online communities can help you connect with a network of people whose concerns are similar to yours. Online support groups are places where people can talk informally. If you read about a novel approach, consult with your child’s doctor or other healthcare providers, as the treatments or discoveries you hear about may not be scientifically proven to be safe and effective.

Finding Doctors All parents must go through the process of selecting a physician for their children with Chiari malformation. While this process will vary, the Agency

22 Chiari Malformation

for Healthcare Research and Quality makes a number of suggestions, including the following:9 ·

If your child is in a managed care plan, check the plan’s list of doctors first.

·

Ask doctors or other health professionals who work with doctors, such as hospital nurses, for referrals.

·

Call a hospital’s doctor referral service, but keep in mind that these services usually refer you to doctors on staff at that particular hospital. The services do not have information on the quality of care that these doctors provide.

·

Some local medical societies offer lists of member doctors. Again, these lists do not have information on the quality of care that these doctors provide.

Additional steps you can take to locate doctors include the following: ·

Check with the associations listed earlier in this chapter.

·

Information on doctors in some states is available on the Internet at http://www.docboard.org. This Web site is run by “Administrators in Medicine,” a group of state medical board directors.

·

The American Board of Medical Specialties can tell you if your child’s doctor is board certified. “Certified” means that the doctor has completed a training program in a specialty and has passed an exam, or “board,” to assess his or her knowledge, skills, and experience to provide quality patient care in that specialty. Primary care doctors may also be certified as specialists. The AMBS Web site is located at http://www.abms.org/newsearch.asp.10 You can also contact the ABMS by phone at 1-866-ASK-ABMS.

·

You can call the American Medical Association (AMA) at 800-665-2882 for information on training, specialties, and board certification for many licensed doctors in the United States. This information also can be found in “Physician Select” at the AMA’s Web site: http://www.amaassn.org/aps/amahg.htm.

If the previous sources did not meet your needs, you may want to log on to the Web site of the National Organization for Rare Disorders (NORD) at http://www.rarediseases.org/. NORD maintains a database of doctors with expertise in various rare medical conditions. The Metabolic Information This section is adapted from the AHRQ: www.ahrq.gov/consumer/qntascii/qntdr.htm. While board certification is a good measure of a doctor’s knowledge, it is possible to receive quality care from doctors who are not board certified.

9

10

Seeking Guidance 23

Network (MIN), 800-945-2188, also maintains a database of physicians with expertise in various metabolic diseases.

Finding a Neurologist The American Academy of Neurology allows you to search for member neurologists by name or location. To use this service, go to http://www.aan.com/, select “Find a Neurologist” from the toolbar. Enter your search criteria, and click “Search.” To find out more information on a particular neurologist, click on the physician’s name. If the previous sources did not meet your needs, you may want to log on to the Web site of the National Organization for Rare Disorders (NORD) at http://www.rarediseases.org/. NORD maintains a database of doctors with expertise in various rare diseases. The Metabolic Information Network (MIN), 800-945-2188, also maintains a database of physicians with expertise in various metabolic diseases.

Selecting Your Doctor11 When you have compiled a list of prospective doctors, call each of their offices. First, ask if the doctor accepts your child’s health insurance plan and if he or she is taking new patients. If the doctor is not covered by your child’s plan, ask yourself if you are prepared to pay the extra costs. The next step is to schedule a visit with your first choice. During the first visit you will have the opportunity to evaluate your child’s doctor and to find out if your child feels comfortable with him or her.

Working with Your Child’s Doctor12 Research has shown that parents who have good relationships with their children’s doctors tend to be more satisfied with their children’s care. Here are some tips to help you and your child’s doctor become partners:

11 This

section has been adapted from the AHRQ: www.ahrq.gov/consumer/qntascii/qntdr.htm. 12 This section has been adapted from the AHRQ: www.ahrq.gov/consumer/qntascii/qntdr.htm.

24 Chiari Malformation

·

You know important things about your child’s symptoms and health history. Tell the doctor what you think he or she needs to know.

·

Always bring any medications your child is currently taking with you to the appointment, or you can bring a list of your child’s medications including dosage and frequency information. Talk about any allergies or reactions your child has had to medications.

·

Tell your doctor about any natural or alternative medicines your child is taking.

·

Bring other medical information, such as x-ray films, test results, and medical records.

·

Ask questions. If you don’t, the doctor will assume that you understood everything that was said.

·

Write down your questions before the doctor’s visit. List the most important ones first to make sure that they are addressed.

·

Ask the doctor to draw pictures if you think that this will help you and your child understand.

·

Take notes. Some doctors do not mind if you bring a tape recorder to help you remember things, but always ask first.

·

Take information home. Ask for written instructions. Your child’s doctor may also have brochures and audio and videotapes on Chiari malformation.

By following these steps, you will enhance the relationship you and your child have with the physician.

Broader Health-Related Resources In addition to the references above, the NIH has set up guidance Web sites that can help parents find healthcare professionals. These include:13 ·

Caregivers: http://www.nlm.nih.gov/medlineplus/caregivers.html

·

Choosing a Doctor or Healthcare Service: http://www.nlm.nih.gov/medlineplus/choosingadoctororhealthcareserv ice.html

·

Hospitals and Health Facilities: http://www.nlm.nih.gov/medlineplus/healthfacilities.html

You can access this information at: http://www.nlm.nih.gov/medlineplus/healthsystem.html.

13

Seeking Guidance 25

27

CHAPTER 3. CLINICAL MALFORMATION

TRIALS

AND

CHIARI

Overview Very few medical conditions have a single treatment. The basic treatment guidelines that your child’s physician has discussed with you, or those that you have found using the techniques discussed in Chapter 1, may provide you with all that you will require. For some patients, current treatments can be enhanced with new or innovative techniques currently under investigation. In this chapter, we will describe how clinical trials work and show you how to keep informed of trials concerning Chiari malformation.

What Is a Clinical Trial?14 Clinical trials involve the participation of people in medical research. Most medical research begins with studies in test tubes and on animals. Treatments that show promise in these early studies may then be tried with people. The only sure way to find out whether a new treatment is safe, effective, and better than other treatments for Chiari malformation is to try it on patients in a clinical trial.

The discussion in this chapter has been adapted from the NIH and the NEI: http://www.nei.nih.gov/health/clinicaltrials%5Ffacts/index.htm.

14

28 Chiari Malformation

What Kinds of Clinical Trials Are There? Clinical trials are carried out in three phases: ·

Phase I. Researchers first conduct Phase I trials with small numbers of patients and healthy volunteers. If the new treatment is a medication, researchers also try to determine how much of it can be given safely.

·

Phase II. Researchers conduct Phase II trials in small numbers of patients to find out the effect of a new treatment on Chiari malformation.

·

Phase III. Finally, researchers conduct Phase III trials to find out how new treatments for Chiari malformation compare with standard treatments already being used. Phase III trials also help to determine if new treatments have any side effects. These trials--which may involve hundreds, perhaps thousands, of people--can also compare new treatments with no treatment. How Is a Clinical Trial Conducted?

Various organizations support clinical trials at medical centers, hospitals, universities, and doctors’ offices across the United States. The “principal investigator” is the researcher in charge of the study at each facility participating in the clinical trial. Most clinical trial researchers are medical doctors, academic researchers, and specialists. The “clinic coordinator” knows all about how the study works and makes all the arrangements for your child’s visits. All doctors and researchers who take part in the study on Chiari malformation carefully follow a detailed treatment plan called a protocol. This plan fully explains how the doctors will treat your child in the study. The “protocol” ensures that all patients are treated in the same way, no matter where they receive care. Clinical trials are controlled. This means that researchers compare the effects of the new treatment with those of the standard treatment. In some cases, when no standard treatment exists, the new treatment is compared with no treatment. Patients who receive the new treatment are in the treatment group. Patients who receive a standard treatment or no treatment are in the “control” group. In some clinical trials, patients in the treatment group get a new medication while those in the control group get a placebo. A placebo is a harmless substance, a “dummy” pill, that has no effect on Chiari malformation. In other clinical trials, where a new surgery or device (not a medicine) is being tested, patients in the control group may receive a “sham treatment.” This treatment, like a placebo, has no effect on Chiari malformation and will not harm your child.

Clinical Trials 29

Researchers assign patients “randomly” to the treatment or control group. This is like flipping a coin to decide which patients are in each group. If you choose to have your child participate in a clinical trial, you will not know which group he or she will be appointed to. The chance of any patient getting the new treatment is about 50 percent. You cannot request that your child receive the new treatment instead of the placebo or “sham” treatment. Often, you will not know until the study is over whether your child has been in the treatment group or the control group. This is called a “masked” study. In some trials, neither doctors nor patients know who is getting which treatment. This is called a “double masked” study. These types of trials help to ensure that the perceptions of the participants or doctors will not affect the study results. Natural History Studies Unlike clinical trials in which patient volunteers may receive new treatments, natural history studies provide important information to researchers on how Chiari malformation develops over time. A natural history study follows patient volunteers to see how factors such as age, sex, race, or family history might make some people more or less at risk for Chiari malformation. A natural history study may also tell researchers if diet, lifestyle, or occupation affects how a medical condition develops and progresses. Results from these studies provide information that helps answer questions such as: How fast will a medical condition usually progress? How bad will the condition become? Will treatment be needed? What Is Expected of Your Child in a Clinical Trial? Not everyone can take part in a clinical trial for a specific medical condition. Each study enrolls patients with certain features or eligibility criteria. These criteria may include the type and stage of the condition, as well as, the age and previous treatment history of the patient. You or your child’s doctor can contact the sponsoring organization to find out more about specific clinical trials and their eligibility criteria. If you would like your child to participate in a clinical trial, your child’s doctor must contact one of the trial’s investigators and provide details about his or her diagnosis and medical history. When participating in a clinical trial, your child may be required to have a number of medical tests. Your child may also need to take medications

30 Chiari Malformation

and/or undergo surgery. Depending upon the treatment and the examination procedure, your child may be required to receive inpatient hospital care. He or she may have to return to the medical facility for followup examinations. These exams help find out how well the treatment is working. Follow-up studies can take months or years. However, the success of the clinical trial often depends on learning what happens to patients over a long period of time. Only patients who continue to return for follow-up examinations can provide this important long-term information.

Recent Trials on Chiari Malformation The National Institutes of Health and other organizations sponsor trials on various medical conditions. Because funding for research goes to the medical areas that show promising research opportunities, it is not possible for the NIH or others to sponsor clinical trials for every medical condition at all times. The following lists recent trials dedicated to Chiari malformation.15 If the trial listed by the NIH is still recruiting, your child may be eligible. If it is no longer recruiting or has been completed, then you can contact the sponsors to learn more about the study and, if published, the results. Further information on the trial is available at the Web site indicated. Please note that some trials may no longer be recruiting patients or are otherwise closed. Before contacting sponsors of a clinical trial, consult with your child’s physician who can help you determine if your child might benefit from participation. ·

Genetic Analysis of the Chiari I Malformation Condition(s): Syringomyelia; Type I Arnold Chiari Malformation Study Status: This study is currently recruiting patients. Sponsor(s): National Institute of Neurological Disorders and Stroke (NINDS) Purpose - Excerpt: The purpose of this study is to better understand the genetic factors related to the Chiari I malformation. In people with this abnormality, the lower part of the skull is smaller than normal. As a result, the lowest part of the brain, called the cerebellar tonsils, protrudes out of the hole at the bottom of the skull into the spinal canal. This study will try to discover the location of the genes responsible for the malformation. Candidates for this study are: 1) Patients with Chiari I malformation who also have a family member with the abnormality or a family member with syringomyelia (a cyst in the spinal cord that is often associated with the Chiari I malformation). 2) Family members of patients

15

These are listed at www.ClinicalTrials.gov.

Clinical Trials 31

with the Chiari I malformation. Participants will have a medical history and physical and neurologic examinations. They will undergo magnetic resonance imaging (MRI) of the brain and cervical (neck) spinal cord to measure the size of the head and determine the presence of the Chiari I malformation and syringomyelia. A small blood sample (about 2 tablespoons) will be drawn for DNA studies relating to the Chiari I malformation. Study Type: Observational Contact(s): Maryland; National Institute of Neurological Disorders and Stroke (NINDS), 9000 Rockville Pike Bethesda, Maryland, 20892, United States; Recruiting; Patient Recruitment and Public Liaison Office 1-800411-1222 [email protected]; TTY 1-866-411-1010 Web Site: http://clinicaltrials.gov/ct/gui/show/NCT00004738;jsessionid=C2A3E1 35F7DEA40C8D1B46F9E5A1C739

Benefits and Risks16 What Are the Benefits of Participating in a Clinical Trial? If you are considering a clinical trial, it is important to realize that your child’s participation can bring many benefits: ·

A new treatment could be more effective than the current treatment for Chiari malformation. Although only half of the participants in a clinical trial receive the experimental treatment, if the new treatment is proved to be more effective and safer than the current treatment, then those patients who did not receive the new treatment during the clinical trial may be among the first to benefit from it when the study is over.

·

If the treatment is effective, then it may improve your child’s health.

·

Clinical trial patients receive the highest quality of medical care. Experts watch them closely during the study and may continue to follow them after the study is over.

·

People who take part in trials contribute to scientific discoveries that may help others with Chiari malformation. In cases where certain medical

This section has been adapted from ClinicalTrials.gov, a service of the National Institutes of Health: http://www.clinicaltrials.gov/ct/gui/c/a1r/info/whatis?JServSessionIdzone_ct=9jmun6f291.

16

32 Chiari Malformation

conditions run in families, your child’s participation may lead to better care or prevention for you and other family members. The Informed Consent Once you agree to have your child take part in a clinical trial, you will be asked to sign an “informed consent.” This document explains a clinical trial’s risks and benefits, the researcher’s expectations of you and your child, and your child’s rights as a patient. What Are the Risks? Clinical trials may involve risks as well as benefits. Whether or not a new treatment will work cannot be known ahead of time. There is always a chance that a new treatment may not work better than a standard treatment. There is also the possibility that it may be harmful. The treatment your child receives may cause side effects that are serious enough to require medical attention. How Is Your Child’s Safety Protected? Clinical trials can raise fears of the unknown. Understanding the safeguards that protect your child can ease some of these fears. Before a clinical trial begins, researchers must get approval from their hospital’s Institutional Review Board (IRB), an advisory group that makes sure a clinical trial is designed to protect your child’s safety. During a clinical trial, doctors will closely watch your child to see if the treatment is working and if he or she is experiencing any side effects. All the results are carefully recorded and reviewed. In many cases, experts from the Data and Safety Monitoring Committee carefully monitor each clinical trial and can recommend that a study be stopped at any time. Your child will only be asked to participate in a clinical trial as a volunteer with your informed consent.

Clinical Trials 33

What Are Your Child’s Rights in a Clinical Trial? If your child is eligible for a clinical trial, you will be given information to help you decide whether or not you want him or her to participate. You and your child have the right to: ·

Information on all known risks and benefits of the treatments in the study.

·

Know how the researchers plan to carry out the study, for how long, and where.

·

Know what is expected of your child.

·

Know any costs involved for you or your child’s insurance provider.

·

Know before any of your child’s medical or personal information is shared with other researchers involved in the clinical trial.

·

Talk openly with doctors and ask any questions.

After your child joins a clinical trial, you and your child have the right to: ·

Leave the study at any time. Participation is strictly voluntary.

·

Receive any new information about the new treatment.

·

Continue to ask questions and get answers.

·

Maintain your child’s privacy. Your child’s name will not appear in any reports based on the study.

·

Know whether your child participated in the treatment group or the control group (once the study has been completed).

What about Costs? In some clinical trials, the research facility pays for treatment costs and other associated expenses. You or your child’s insurance provider may have to pay for costs that are considered standard care. These things may include inpatient hospital care, laboratory and other tests, and medical procedures. You also may need to pay for travel between your home and the clinic. You should find out about costs before committing your child to participation in the trial. If your child has health insurance, find out exactly what it will cover. If your child does not have health insurance, or if your child’s insurance policy will not cover care, talk to the clinic staff about other options for covering the costs.

34 Chiari Malformation

What Questions Should You Ask before Your Child Participates in a Clinical Trial? Questions you should ask when deciding whether or not to enroll your child in a clinical trial include the following: ·

What is the purpose of the clinical trial?

·

What are the standard treatments for Chiari malformation? Why do researchers think the new treatment may be better? What is likely to happen to my child with or without the new treatment?

·

What tests and treatments will my child need? Will my child need surgery? Medication? Hospitalization?

·

How long will the treatment last? How often will my child have to come back for follow-up exams?

·

What are the treatment’s possible benefits to my child’s condition? What are the short- and long-term risks? What are the possible side effects?

·

Will the treatment be uncomfortable? Will it make my child sick? If so, for how long?

·

How will my child’s health be monitored?

·

Where will my child need to go for the clinical trial?

·

How much will it cost to participate in the study? What costs are covered by the study? How much will my child’s health insurance cover?

·

Who will be in charge of my child’s care?

·

Will taking part in the study affect my child’s daily life?

·

How does my child feel about taking part in a clinical trial? Will other family members benefit from my child’s contributions to new medical knowledge?

Keeping Current on Clinical Trials Various government agencies maintain databases on trials. The U.S. National Institutes of Health, through the National Library of Medicine, has developed ClinicalTrials.gov to provide the public and physicians with current information about clinical research across the broadest number of medical 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

Clinical Trials 35

receives about 2 million hits per month and hosts approximately 5,400 visitors daily. To access this database, simply go to their Web site (www.clinicaltrials.gov) and search by “Chiari malformation” (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 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#Clinica l_Trials

General References The following references describe clinical trials and experimental medical research. They have been selected to ensure that they are likely to be available from your local or online bookseller or university medical library. These references are usually written for healthcare professionals, so you may consider consulting with a librarian or bookseller who might recommend a particular reference. The following includes some of the most readily available references (sorted alphabetically by title; hyperlinks provide rankings, information and reviews at Amazon.com): ·

A Guide to Patient Recruitment : Today’s Best Practices & Proven Strategies by Diana L. Anderson; Paperback - 350 pages (2001), CenterWatch, Inc.; ISBN: 1930624115; http://www.amazon.com/exec/obidos/ASIN/1930624115/icongroupinter na

·

A Step-By-Step Guide to Clinical Trials by Marilyn Mulay, R.N., M.S., OCN; Spiral-bound - 143 pages Spiral edition (2001), Jones & Bartlett Pub; ISBN: 0763715697;

36 Chiari Malformation

http://www.amazon.com/exec/obidos/ASIN/0763715697/icongroupinter na ·

The CenterWatch Directory of Drugs in Clinical Trials by CenterWatch; Paperback - 656 pages (2000), CenterWatch, Inc.; ISBN: 0967302935; http://www.amazon.com/exec/obidos/ASIN/0967302935/icongroupinter na

·

Extending Medicare Reimbursement in Clinical Trials by Institute of Medicine Staff (Editor), et al; Paperback 1st edition (2000), National Academy Press; ISBN: 0309068886; http://www.amazon.com/exec/obidos/ASIN/0309068886/icongroupinter na

·

Handbook of Clinical Trials by Marcus Flather (Editor); Paperback (2001), Remedica Pub Ltd; ISBN: 1901346293; http://www.amazon.com/exec/obidos/ASIN/1901346293/icongroupinter na

Vocabulary Builder The following vocabulary builder gives definitions of words used in this chapter that have not been defined in previous chapters: Cerebellar: Pertaining to the cerebellum. [EU] Cyst: Any closed cavity or sac; normal or abnormal, lined by epithelium, and especially one that contains a liquid or semisolid material. [EU]

37

PART II: ADDITIONAL RESOURCES AND ADVANCED MATERIAL

ABOUT PART II In Part II, we introduce you to additional resources and advanced research on Chiari malformation. All too often, parents who conduct their own research are overwhelmed by the difficulty in finding and organizing information. The purpose of the following chapters is to provide you an organized and structured format to help you find additional information resources on Chiari malformation. In Part II, as in Part I, our objective is not to interpret the latest advances on Chiari malformation or render an opinion. Rather, our goal is to give you access to original research and to increase your awareness of sources you may not have already considered. In this way, you will come across the advanced materials often referred to in pamphlets, books, or other general works. Once again, some of this material is technical in nature, so consultation with a professional familiar with Chiari malformation is suggested.

39

CHAPTER 4. STUDIES ON CHIARI MALFORMATION Overview Every year, academic studies are published on Chiari malformation or related conditions. Broadly speaking, there are two types of studies. The first are peer reviewed. Generally, the content of these studies has been reviewed by scientists or physicians. Peer-reviewed studies are typically published in scientific journals and are usually available at medical libraries. The second type of studies is non-peer reviewed. These works include summary articles that do not use or report scientific results. These often appear in the popular press, newsletters, or similar periodicals. In this chapter, we will show you how to locate peer-reviewed references and studies on Chiari malformation. We will begin by discussing research that has been summarized and is free to view by the public via the Internet. We then show you how to generate a bibliography on Chiari malformation and teach you how to keep current on new studies as they are published or undertaken by the scientific community.

Federally Funded Research on Chiari Malformation The U.S. Government supports a variety of research studies relating to Chiari malformation and associated conditions. These studies are tracked by the Office of Extramural Research at the National Institutes of Health.17 CRISP 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).

17

40 Chiari Malformation

(Computerized Retrieval of Information on Scientific Projects) is a searchable database of federally funded biomedical research projects conducted at universities, hospitals, and other institutions. Visit CRISP at http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen. You can perform targeted searches by various criteria including geography, date, as well as topics related to Chiari malformation and related conditions. 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 Chiari malformation and related conditions. In some cases, therefore, it may be difficult to understand how some basic or fundamental research could eventually translate into medical practice. The following sample is typical of the type of information found when searching the CRISP database for Chiari malformation: ·

Project Title: ADENOSINE AND THE NEURONAL RESPONSE TO HYPOXIA AND ISCHE Principal Investigator & Institution: Fowler, John C.; Associate Professor; Physiology; Texas Tech University Health Scis Center Health Sciences Center Lubbock, Tx 79430 Timing: Fiscal Year 2000; Project Start 1-JUL-1999; Project End 0-JUN2002 Summary: (Verbatim from the Applicant's Abstract) The long-term goal of this research is to understand the (patho) physiologic role of the inhibitory neuromodulator adenosine in the brain's early resp0onse to stroke. Understanding adenosine's contribution is important because adenosine-based pharmacological interventions show promise in reducing neuronal hypoxic/ischemic injury in a number of animal models [31,34,83,87,117]. The specific focus if this proposal is to extend to an in vivo model of the PI's previous series of studies performed in the in vitro hippocampal slice. These in vitro studies detailed adenosine's substantial mediation of the early reversible depression of synaptic transmission and adenosine's interaction with the anoxic depolarization in response to ischemic-like conditions [39-42,44]. Surprisingly, in spite of an extensive literature describing adenosine's role I in vitro slice preparations [9,24,52,69,89,90,142], there has yet to be, to the best of our knowledge, a demonstration of adenosine's role in vivo. The following specific aims, therefore, focus on examining the contribution of salient observations made in vitro to an in vivo hypoxic/ischemic rat hippocampal model. The following specific aims will be addressed: Specific Aim 1. Determine the role of adenosine receptors in the in the

Studies 41

early hypoxic/ischemic depression of synaptic transmission in vivo. Specific Aim 2. Examine the relationship between adensone receptor activation, inhibition of evoked synaptic potentials and tissue pO2 in vivo. Specific Aim 3. Determine the influence of adenosine receptor activation on development of the anoxic depolarization during extended hypoxia/ischemia in vivo. And, examine adenosine receptor function in the post-ischemic period after the anoxic depolarization. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: DEHYDROASCORBIC ACID AS THERAPY FOR ISCHEMIC STROKE Principal Investigator & Institution: Boyd, Thomas A.; ; Progenics Pharmaceuticals, Inc. 777 Old Saw Mill River Rd Tarrytown, Ny 10591 Timing: Fiscal Year 2001; Project Start 0-SEP-2001; Project End 1-JUL-2003 Summary: adapted from applicant's abstract): A major goal of stroke research is the development of new treatment modalities to limit the extent of brain injury following acute ischemic injury. Ischemic stroke is associated with a rapid increase in free radical content in the brain and a depletion of natural antioxidants such as ascorbic acid. Delivery of ascorbic acid to the site of ischemic injury is limited by its inability to cross the blood-brain barrier. Dehydroascorbic acid, the oxidized form of ascorbic acid, readily crosses the blood-brain barrier via a facilitative transport mechanism and is converted into ascorbic acid after entering the brain. Dehydroascorbic acid has shown preliminary evidence of efficacy in a murine stroke model. This compound thus has significant potential as a therapeutic agent for stroke. The first aim of this Phase I project is to prepare an adequate supply of pure dehydroascorbic acid for testing. As large quantities of pure compound are not presently available, a new preparative, large-scale route will be developed. New analytical methods will be developed for determining the compound's identity and purity and for quantitating levels of unwanted impurities. The second aim is to formulate a convenient and stable solution for injection. This is feasible provided the buffer components are tightly optimized and the stability is confirmed by analytical testing. The final aim is to demonstrate in a small animal model that ehydroascorbic acid decreases neuronal injury after an occlusive infarct. This will be done in the mouse middle cerebral artery occlusion model. Dehydroascorbic acid will be administered intravenously at various doses and intervals following the onset of ischemia, and its effects on infarct volume, neurological function, cerebral blood flow, and mortality will be determined. A significant beneficial effect in this model in comparison with either vehicle or ascorbic acid controls will provide in vivo proof-of concept of the

42 Chiari Malformation

approach and will confine the value of additional studies of dehydroascorbic acid as a therapeutic agent in ischemic stroke. PROPOSED COMMERCIAL APPLICATIONS: Dehydroascorbic acid is a novel treatment for patients who have suffered an ischemic stroke. Dehydroascorbic acid has a unique ability to enter the brain and to elevate tissue levels of ascorbic acid, a natural antioxidant. This project seeks to demonstrate that dehydroascorbic acid is efficacious in a murine stroke model in the interval of time immediately following an ischemic event, a critical period for patients with ischemic stroke. These studies will support eventual clinical testing of the compound. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: ENHANCED CAROTID BIFURCATION PHANTOM FOR MRI/A Principal Investigator & Institution: Burke, Thomas M.; ; Phantoms by Design 1507 175Th Pl Se Bothell, Wa 98012 Timing: Fiscal Year 2002; Project Start 1-SEP-2000; Project End 1-JAN2003 Summary: (provided by applicant): Non invasive MRI evaluation of the carotid artery wall lumen and blood flow patterns is hampered by the lack of a comprehensive and anatomically correct commercially available flow phantom. The objective of this project is to develop an advanced artificial carotid artery flow phantom for use in developing MRI applications to identify patients at risk for transient ischemic attack or strokes. In Phase I, proof-of-concept was established. Phase II technical aims are selected to systematically expand the knowledge and experience base of key technologies: 1) lumen design, based on image data, 2) wall mechanics, with regional variation, 3) image morphology, including two distinct layers with fibrous cap lesion and 4) fluid motion with custom pulsatile flow pump and experimental results compared to hemodynamic simulations. Prototypes are constructed and evaluated in: academic, clinical and industrial environments to gauge commercial potential and identify key functional requirements for commercial development. Materials and preparations are optimized to obtain precision and stability of parameters on the order of 20 percent over 18 months. A comprehensive flow phantom will be critical for the development, validation and training of medical personnel on using state of the art MRI screening protocols for atherosclerotic carotid artery disease. PROPOSED COMMERCIAL APPLICATION: The magnitude of the clinical need for a non invasive means to identify patients at risk and stage carotid artery surgery is so large and the commercial availability of adequate MRI tools so limited as to create a lucrative market niche for highly advanced

Studies 43

anthropomorphic flow phantoms targeted at cerebrovascular disease. Products developed from this project will address current and future needs in clinical research, QA, and personnel training applications. (See Product Development Plan, pages 45-50). Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: HEMORRHAGIC AND ISCHEMIC STROKE AMONG BLACKS AND WHITES Principal Investigator & Institution: Broderick, Joseph P.; Professor and Chair; Neurology; University of Cincinnati 2624 Clifton Ave Cincinnati, Oh 45221 Timing: Fiscal Year 2000; Project Start 2-SEP-1992; Project End 1-MAY2003 Summary: (Adapted from Investigator's Abstract) The primary goal of the proposed studies is to identify temporal trends in the incidence rate, causes, treatment and outcome of stroke among a large biracial metropolitan population in Cincinnati. There are 1,299,901 people in the greater metropolitan area, 187,806 of whom are black, a percentage that compares favorably with the U.S. population as a whole. The investigators propose to identify every hospitalized or autopsied and almost all non-hospitalized strokes during 1/1/99 through 12/31/99 at all 19 regional hospitals and 147 outpatient screening sites as per the previous five-year funding period. They will identify and abstract detailed information from the medical record for every potential stroke case (methods identical to their ongoing five-year study). In addition, they plan on contacting 500 new ischemic stroke patients (250 blacksexcluding TIAs) and/or their families, to obtain detailed information concerning prior risk factors, knowledge about stroke, outcome following stroke, and buccal cells and/or blood for genetic material. They will replicate a random digit-dialing telephone surgery of 2000 persons from the general population during the year 2000. The estimated 3000 strokes and 700 TIAs as well as the 2000 surveyed persons from the general population will be used to test the following primary hypotheses. 1) The age, sex, and race-adjusted incidence rate of all first-ever stroke, cerebral infarction, and subarachnoid hemorrhage will decrease and the rate of intracerebral hemorrhage will increase during 1999 as compared to incidence rates during 1988 and 1993/4. 2) The 3-month mortality after stroke will be similar for blacks and whites and unchanged from 1993/94, but functional outcome at 3-months as measured by the Barthel Index, Pfeffer Instrumental Activities of Daily Living Scale, and Medical Outcome Study will be poorer among blacks as compared to whites. The investigators state that the long-term goal of this research is to study

44 Chiari Malformation

temporal trends in stroke incidence rates, treatment, and outcome and their relationships to the prevalence of environmental and genetic risk factors for stroke as well as to changes in public knowledge of stroke risk factors and warning signs. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: NEURONAL INJURY AND BLOOD GENOMICS Principal Investigator & Institution: Sharp, Frank R.; Professor and ViceChairman; Neurology; University of Cincinnati 2624 Clifton Ave Cincinnati, Oh 45221 Timing: Fiscal Year 2002; Project Start 1-JUN-2002; Project End 1-MAY2006 Summary: (Adapted from applicant?s abstract): It is hypothesized that the white blood cell genomic response can be used to deduce the presence of neuronal injury due to acute neurological diseases, and that the blood genomic response patterns can be used to differentiate between the diseases causing the neuronal injury. Our preliminary data using microarray technology show unique patterns of gene expression by lymphocytes of adult rats subjected to ischemic strokes, hemorrhagic strokes, status epilepticus, hypoxia, hypoglycemia and sham-surgeries as compared to untouched controls. The first Aim of this proposal will determine whether short durations of global cerebral ischemia, focal cerebral ischemia (transient ischemic attack), hypoglycemia and seizures produce different white blood cell genomic responses in rats that can be used to differentiate between these conditions hours to days later. The second Aim will determine whether long durations of global ischemia, hypoglycemia and status epilepticus regulate specific genes in white blood cells in response to the diffuse neuronal injury caused by all of these conditions, and whether these genes can serve as indicators of the diffuse neuronal injury. The genomic expression of neutrophils, lymphocytes and whole blood will be examined at various times after cerebral ischemia, insulin-induced hypoglycemia, seizures and status epilepticus. Genes regulated in the different white blood cells by these conditions will be correlated with the presence of diffuse neuronal cell death in brain using TUNEL staining. The third set of Aims will determine whether the same genes regulated in white blood cells of rodents following single seizures and status epilepticus are also regulated in the white blood cells of men and women patients following seizures and status epilepticus. These studies will also determine whether blood genomic responses can be used to distinguish whether patients have had seizures, pseudoseizures or syncope, and whether some of the neuronal injury-related genes regulated in the blood of rodents with status

Studies 45

epilepticus are regulated in patients with status epilepticus. Genes regulated more than two fold on microarrays will be confirmed by quantitative RT-PCR for all of the aims. The goal is to objectively differentiate seizures, syncope, global cerebral ischemia, hypoglycemia, and transient ischemic attacks hours to days after they occur; and to begin to identify blood genomic markers of neuronal death associated with acute neurological diseases that might also be useful in chronic neurological diseases. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: PILOT STUDY OF TNK-TPA IN ACUTE ISCHEMIC STROKE Principal Investigator & Institution: Haley, Elliot C.; Professor; Neurology; University of Virginia Charlottesville Box 400195 Charlottesville, Va 22904 Timing: Fiscal Year 2002; Project Start 1-DEC-1999; Project End 0-NOV2003 Summary: Ischemic stroke continues to be a major public health problem exacting billions of dollars from society in medical care costs and lost wages, as well as incalculable suffering to victims and their families. Recently, thrombolytic therapy with intravenous recombinant tissue plasminogen activator (rt-PA) was shown to improve neurological and functional outcome in patients with acute ischemic stroke who could be treated with 3 hours of onset. However, the risk of symptomatic intracranial hemorrhage complicating rt-PA treatment was 6.4%, and 39% of patients either died or were severely disabled despite treatment. A novel, second generation thrombolytic drug, TNK-TPA, has been specificity engineered to be more fibrin specific than rt-PA, and has been shown in experimental models of thrombosis to be more potent and active more quickly than rt- PA. In experimental stroke, cerebrovascular clots were lysed more quickly, and the incidence of intracranial hemorrhage was reduced. In human studies of myocardial infarction, the drug is well tolerated, and the incidence of intracranial hemorrhage complicating treatment is not greater, and may be less than that with rtPA. This proposal describes a multi-center, open-label, pilot doseescalation safety trial of TNK-TPA in patients with acute ischemic stroke who can be treated within 3 hours. The primary hypothesis to be tested is that TNK-TPA may be safely administered to patients with acute ischemic stroke at doses which may be associated with improvement in neurological outcome. The primary endpoint of the study will be the incidence of symptomatic intracranial hemorrhage within 36 hours of treatment. Safety monitoring and sample size calculations are designed

46 Chiari Malformation

so that enrollment will cease if the observed intracranial hemorrhage rate exceeds that reported with rt-PA treatment of stroke. Drug activity will be gauged by the proportion of patients with major early neurological improvement as determined by an 8 or more point improvement on the National Institutes of Health Stroke Scale or a perfect score of 0 at 24 hours after stroke onset. Outcome at 3 months will be assessed with the Barthel Index, the modified Rankin Sale, and a global outcome statistic to facilitate sample size calculations for subsequent studies. Sixty to 100 patients will be enrolled in dosage tiers of up to 25 patients each. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: PREDICTORS OF RECURRENT STROKE IN THE PROGRESS STUDY Principal Investigator & Institution: Campbell, Duncan J.; ; St. Vincent's Institute of Med Res 9 Princes St, Fitzroy Victoria, Timing: Fiscal Year 2002; Project Start 0-SEP-2002; Project End 1-AUG2004 Summary: (provided by applicant): The objective is to investigate mechanisms of recurrent stroke. This study is based on plasma samples taken on enrolment from a subset of the 6105 subjects in the perindopril protection against recurrent stroke (PROGRESS) study. The PROGRESS study was a randomized double-blind controlled study of a perindoprilbased blood pressure lowering regimen among individuals with stroke or transient ischemic attack within the previous 5 years. This project has two main aims: 1. To identify whether NMR-determined lipoprotein profiles, and plasma levels of homocysteine, protein carbonyls, chlorotyrosine, soluble vascular cell adhesion molecule (sVCAM-1), C-reactive peptide (CRP), active renin, and the amino-terminal pro-B-type natriuretic peptide (NT-proBNP) predict recurrent stroke in PROGRESS study participants. 2. To identify whether nuclear magnetic resonance (NMR)determined lipoprotein profiles, and plasma levels of homocysteine, protein carbonyls, chlorotyrosine, sVCAM-1, CRP, active renin, and NTproBNP predict benefit from perindopril-based antihypertensive therapy. This will be a nested case-control study. From the plasma samples obtained from 6105 subjects on enrolment in the PROGRESS study, 727 are from subjects who experienced recurrent stroke during the study. Our cases will be the 325 from this 727 from whom plasma samples were collected more than one month after the qualifying event and frozen at 80xC within 24 hours. Each case will be matched to two control subjects who had no cardiovascular event. The data will be related to clinical markers of cardiovascular risk on enrolment. We will determine which of these plasma markers provides an independent measure of risk of

Studies 47

recurrent stroke. In addition, we will determine those plasma markers that provide an independent measure of reduction in risk of recurrent stroke by perindopril-based antihypertensive therapy, thereby providing a means to identify those subjects most likely to benefit from such therapy, and conversely, those subjects in whom alternative preventative strategies are required. In addition, plasma markers that predict risk of recurrent stroke may indicate novel therapies to prevent this condition. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: SOCIOBIOLOGICAL FACTORS IN EXPERIMENTAL STROKE OUTCOME Principal Investigator & Institution: Devries, a C.; Anesthesiology/Crit Care Med; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2000; Project Start 1-JUL-2000; Project End 1-DEC2000 Summary: Both acute and chronic stress can have long-term physiological and psychological consequences, some of which can be ameliorated through positive social interaction. The current proposal will examine the effects of prior exposure to positive social interaction versus social stress on the histological, physiological and behavioral consequences of experimental stroke. In addition, alterations in the corticosteroid, glutamate and antioxidant responses to experimental stroke will be examined as possible mechanisms through which social cues can influence histological and functional outcome in this model. The data collected as part Of this proposal will increase our understanding of the basic pathophysiological mechanism of cerebral ischemia and the roles that social interaction and the hypothalamic-pituitary-adrenal axis play in modulating excitotoxic neuronal death. Ultimately, understanding the factors that modulate neurotoxicity and behavioral outcomes may facilitate the development of therapeutic approaches for the prevention and treatment of cerebral ischemia. Taken together, the proposed studies will provide a neurobiological foundation on which to study the effects of social cues on experimental stroke outcome, and may provide insights into the mechanisms underlying individual differences in outcome following an ischemic event. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen

·

Project Title: SODIUM IMAGING IS ISCHEMIC HEART DISEASE Principal Investigator & Institution: Bottomley, Paul A.; Russell H. Morgan Professor; Radiology; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218

48 Chiari Malformation

Timing: Fiscal Year 2000; Project Start 1-JUL-1999; Project End 0-JUN2003 Summary: The exchange of intra- and extra-cellular sodium and potassium ions is essential to cell function and integrity. In the heart, evidence from animal and human radionuclide imaging studies of potassium and potassium analogs, and from biochemical and magnetic resonance spectroscopy (MRS) studies of animal models, indicates that sodium- potassium pump function is compromised during periods of myocardial ischemia and that it is lost in non-viable, infarcted tissue as intra- and extra-cellular pools equilibrate. Sodium (23Na) magnetic resonance imaging (MRI) is uniquely able to image and measure noninvasively naturally abundant, endogenous sodium in the body. 23Na MRI at magnetic fields of > 2.7 Tesla (T) in animal models demonstrate a 2-fold increase in 23Na signal levels in nonviable, histologically-confirmed, acute reperfused myocardial infarction (MI). Owing to its higher tissue concentration and sensitivity and its short relaxation time, 23Na MRI has an enormous sensitivity advantage compared, for example, with the detection of high-energy phosphate metabolites by phosphorus (31P) MRI (approximately 80- fold). Thus 23Na MRI is a potentially unique and important tool for assessing cellular metabolic and ionic function through altered sodium levels in patients with ischemic heart disease and/or MI. Yet 23Na is not now routinely possible on clinical 1.5T MRI scanners. Moreover, human 23Na MRI has never benefitted from new MRI hardware and software technology. In preliminary studies we implemented 23Na MRI on a clinical MRI scanner, and demonstrate altered 23Na MRI levels in MI. We show preliminary stress-23Na MRI data from patients with stressinduced ischemia detected metabolically by 31P MRS. Here we propose to develop and optimize human cardiac 23Na MRI on a clinical 1.5 T MRI/MRS system, by implementing high-speed MRI, 23Na phased-array detection, resolution-enhancement using a priori anatomic information, and methods of suppressing 23Na signals from ventricular blood. We will use optimized 23Na MRI to characterize normal and infarcted human myocardium, and to test the hypotheses that 23Na MRI can differentiate normal from non-viable reperfused MI in patients as detected by radionuclide imaging, and compared with contrastenhanced MRI. Further, the hypothesis that optimized 23Na MRI can detect stress-induced changes in sodium in energetically- compromised myocardium will be tested in combined stress-23Na/3 1P metabolic studies. The availability of thousands of clinical MRI scanners offers a great opportunity for advancing 23Na MRI as a tool for assessing sodium pump function in human heart disease.

Studies 49

Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: STRESS AND MYOCARDIAL MECHANISMS AND TREATMENT

ISCHEMIA--

Principal Investigator & Institution: Blumenthal, James A.; Professor of Medical Psychology; Psychiatry and Behavioral Scis; Duke University Durham, Nc 27706 Timing: Fiscal Year 2000; Project Start 5-SEP-1998; Project End 1-AUG2003 Summary: (adapted from the applicant's abstract): The presence of transient myocardial ischemia is an important functional expression of coronary heart disease (CHD). Recent research has shown that mental stress can trigger ischemia in the laboratory and during daily life, and that mental stress-induced ischemia is associated with increased risk for coronary events over and above exercise-induced ischemia. The present study is designed to examine the extent to which a program of exercise or stress management training reduces ischemic activity measured in the laboratory using radionuclide ventriculography and during daily life using ambulatory monitoring. In addition, we propose to examine the bio-behavioral mechanisms by which mental stress triggers ischemia, and the mechanisms by which the inter-ventions reduce ischemic activity. Two hundred and ten patients with documented CHD and evidence of exercise-induced myocardial ischemia will serve as subjects for this study. Patients will be withdrawn from anti-ischemic medications and undergo comprehensive evaluations both in-hospital and out-of-hospital. In-hospital assessments will include provocative mental stress testing to elicit myocardial ischemia, ultrasound testing to assess endothelial function, and psychometric testing to assess underlying personality traits and behavioral dispositions. The out-of-hospital assessment will consist of 48-hour ischemia monitoring with concurrent blood pressure monitoring and behavioral diary recording. Following completion of the assessments, patients will resume their usual medications and will be randomly assigned to either Aerobic Exercise, Stress Management or Education/Routine Care. Patients will be re-evaluated after the fourmonth treatment program and thereafter followed for at least six months. It is hypothesized that exercise and stress management training will be associated with a reduction in ischemic activity, associated with reductions in blood pressure and systemic vascular resistance, and improved endothelial function. Data from this study will provide new scientific insights into the mechanisms of stress-induced myocardial ischemia, as well as important knowledge regarding the clinical benefits

50 Chiari Malformation

of exercise and stress management training in the treatment of CHD patients with myocardial ischemia. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: STROKE OUTCOME AND NEUROIMAGING OF INTRACRANIAL STENOSIS Principal Investigator & Institution: Feldmann, Edward; Associat Professor; Rhode Island Hospital (Providence, Ri) 593 Eddy St Providence, Ri 02902 Timing: Fiscal Year 2000; Project Start 5-SEP-1999; Project End 0-JUN2004 Summary: Intracranial atherosclerosis is responsible for 50,000 ischemic strokes each year in the USA. Noninvasive testing such as transcranial Doppler ultrasound (TCD) and magnetic resonance angiography (MRA) to identify intracranial atherosclerosis is in widespread use but has not been rigorously validated against the gold standard, catheter angiography. The recently NIH-funded WASID trial (Warfarin Aspirin Symptomatic Intracranial Disease) will compare warfarin with aspirin for stroke prevention in patients with intracranial atherosclerosis. WASID requires performance of angiography along with TCD and MRA, providing an opportunity to critically evaluate these nonivasive tests. Main Objective. The purpose of SONIA is to validate the noninvasive diagnosis of intracranial atherosclerosis. The primary aim of SONIA is to define velocity values on TCD and anatomic abnormalities on MRA that identify severe (50-99 percent) intracranial stenosis or large, proximal arteries seen on catheter angiography. SONIA will define the criteria, or cutpoints, for an abnormal TCD or MRA and show that they perform with a reliable positive predictive value (PPV). Study Design. SONIA will be conducted in collaboration with WASID. Study-wide cutpoints defining positive TCD and MRA have been developed and reviewed by the site investigators of WASID. Hard copy angiography, TCD and MRA generated in WASID will be centrally read in SONIA. The performance of TCD and MRA will be monitored and analyzed in SONIA to demonstrate cutpoints that achieve a PPV of 80 percent for the identification of severe intracranial stenosis on angiography. Conclusions. Central readings will be used to validate the cutpoints that function with a PPV of 80 percent and to develop measures of negative predictive value (NPV), inter- and intra-observer variability. Sensitivity and specificity will be determined after adjustment for verification bias and employed in receiver-operator characteristic analyses. SONIA will use these techniques to develop TCD or MRA cutpoints that minimize the clinical consequences of test errors

Studies 51

that occur in the noninvasive evaluation of patients with suspected intracranial atherosclerosis. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: STROKE REGISTRY Principal Investigator & Institution: Bruno, Askiel; ; University of New Mexico Albuquerque Albuquerque, Nm 87131 Timing: Fiscal Year 2000 Summary: Patients presenting with stroke or transient ischemic attack will be entered into a stroke registry. Recorded will be age, race, gender, vascular risk factors, stroke type and location, and in-hospital mortality. This information will later be used to study important issues regarding stroke epidemiology, mechanism, and prognosis. Biostatistician ONLY. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen

·

Project Title: TIA-INDUCED ISCHEMIC TOLERANCE VIA P450 EPOXYGENASE Principal Investigator & Institution: Alkayed, Nabil; Anesthesiology/Crit Care Med; Johns Hopkins University 3400 N Charles St Baltimore, Md 21218 Timing: Fiscal Year 2002; Project Start 1-JUN-2002; Project End 0-APR2006 Summary: (provided by applicant): The project seeks to determine if prior exposure to mild ischemia, such as occurs during Transient Ischemic Attack (TIA), alters the brain's response to subsequent stroke by serving as a preconditioning stimulus and triggering neuroprotective mechanisms. Specifically, we will test the hypothesis that experimental TIA reduces damage from subsequent stroke by a mechanism that involves upregulation of cytochrome P450 2C11, a P450 AA epoxygenase expressed in astrocytes, which catalyzes the conversion of arachidonic acid (AA) to biologically active epoxyeicosatrienoic acids (EETs). The Specific Aims of the project are: 1) To determine if experimental TIA protects brain from subsequent stroke by EETs-mediated mechanism, 2) To determine if TIA-enhanced EETs production is due to upregulation in astrocytes of the biosynthetic enzyme P450 2C11, 3) To determine if genetic and pharmacological manipulations of EETs synthetic and breakdown pathways mimic the protection acquired by TIA, and 4) To determine if ischemic tolerance in TIA-preconditioned brain is due to EETs-mediated activation of phosphoinositide 3-kinase (P13-K) /Akt cell survival signaling cascade in neurons. To simulate TIA, three 10-minute periods of middle cerebral artery occlusion (MCAO) will be induced in

52 Chiari Malformation

rats and mice; three days later, a more severe 2-hour ischemia is produced and infarct size is measured at 1 and 7 days to assess short- and long term tissue outcome after stroke. The level of endogenous EETs in brain will be manipulated using pharmacological and genetic tools and measured using in-vivo microdialysis. Expression of P450 2C11 will be examined using RNase protection assay, Western blotting, immunohistochemistry and in-situ hybridization. Western blotting with anti-phospho-Akt antibody will assess Akt activation and the importance of this pathway to ischemic tolerance will be evaluated using specific P13-K/Akt inhibitors. The studies explore a potential endogenous mechanism of protection from cerebral ischemia, which can be targeted as a novel strategy for stroke injury prevention and treatment. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: WARFARIN/ASPRIN INTRACRANIAL DISEASE STUDY

SYMPTOMATIC

Principal Investigator & Institution: Chimowitz, Marc I.; ; Emory University 1380 S Oxford Rd Atlanta, Ga 30322 Timing: Fiscal Year 2001; Project Start 1-OCT-1974; Project End 0-NOV2004 Summary: The purpose of this study is to compare the efficacy and safety of warfarin (INR 2-3) with high dose aspirin (1300 mg per day) for preventing stroke and vascular death in patients with transient ischemic attack or non-server stroke caused by symptomatic stenosis of a major intracranial artery. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen ·

Project Title: WOMENS ESTROGEN FOR STROKE TRIAL Principal Investigator & Institution: Horwitz, Ralph I.; Internal Medicine; Yale University New Haven, Ct 06520 Timing: Fiscal Year 2000; Project Start 1-JUN-1993; Project End 1-JUL2002 Summary: The Women's Estrogen for Stroke Trial (WEST) is a randomized, double- blind, placebo-controlled trial testing whether estrogen treatment, compared to placebo, reduces the risk of death or recurrent stroke in postmenopausal women with a transient ischemic attack or first non- disabling stroke. Patient accrual, which began in December 1993, was based on a sample size target of 652 women to detect a difference in endpoints of 10 percent (25 percent in placebo group and 15 percent in estrogen group). To date, the WEST has enrolled 561 patients and has been remarkably successful in maintaining high protocol

Studies 53

adherence, patient participation, and clinical safety. Enrollment in WEST will be completed by May 31, 1998, before the start of the continuation period. Therefore, the principal aim for the competing renewal is to complete the WEST as originally proposed to ensure desired average length of patient follow-up of 3.5 years and to permit the necessary data management and analysis. The additional funded time is also needed to enable the trial investigators to arrange for appropriate termination of subject participation. A second aim for the competing renewal is to acquire new data about the clinical course and outcomes of TIA and nondisabling stroke in elderly women, and on the impact of estrogens generally in this special population. Indeed, few studies have had the opportunity to acquire detailed clinical, behavioral and cognitive data on the clinical course of stroke in a group of women this age who began their participation with high levels of functional independence. The goals of WEST are even more salient to the health of American women today than when they were proposed in 1992. Even now in 1997 no clinical trial data are available demonstrating estrogen's effect on vascular endpoints, including cerebrovascular, that would help postmenopausal patients and their physicians decide whether and when to use estrogens. Thus, the results of WEST promise to provide American women with urgently needed information that may help to reduce the risk of stroke and strokerelated mortality. Website: http://crisp.cit.nih.gov/crisp/crisp_query.generate_screen

54 Chiari Malformation

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. 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 the public.18 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 Chiari malformation, simply go to the PubMed Web site at www.ncbi.nlm.nih.gov/pubmed. Type “Chiari malformation” (or synonyms) into the search box, and click “Go.” The following is the type of output you can expect from PubMed for “Chiari malformation” (hyperlinks lead to article summaries): ·

A case of syringomyelia with type I Arnold-Chiari malformation (ACM): growth hormone (GH) therapy and the size of syrinx on serial MR images. Author(s): Takakuwa S, Asai A, Igarashi N. Source: Endocr J. 1996 October; 43 Suppl: S129-30. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9076361&dopt=Abstract

·

A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation. Author(s): Ito S, Matsui K, Ohsaki E, Goto A, Takagi K, Koresawa M, Ito S, Sekido K, Suzuki M, Torikai K, Aida N. Source: Brain & Development. 1996 July-August; 18(4): 307-11. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8879651&dopt=Abstract

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.

18

Studies 55

·

Abductor vocal fold palsy as a manifestation of type one Arnold Chiari malformation. Author(s): Allsopp GM, Karkanevatos A, Bickerton RC. Source: The Journal of Laryngology and Otology. 2000 March; 114(3): 2213. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10829116&dopt=Abstract

·

Acute foramen magnum syndrome caused by an acquired Chiari malformation after lumbar drainage of cerebrospinal fluid: report of three cases. Author(s): Dagnew E, van Loveren HR, Tew JM Jr. Source: Neurosurgery. 2002 September; 51(3): 823-8; Discussion 828-9. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12188966&dopt=Abstract

·

Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia. Author(s): Alvarez D, Requena I, Arias M, Valdes L, Pereiro I, De la Torre R. Source: The European Respiratory Journal : Official Journal of the European Society for Clinical Respiratory Physiology. 1995 April; 8(4): 661-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7664871&dopt=Abstract

·

Adult Arnold-Chiari malformation: a postpartum case presentation. Author(s): O'Reilly SA, Toffol GJ. Source: J Am Osteopath Assoc. 1995 October; 95(10): 607-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8557551&dopt=Abstract

·

Adult Chiari malformation with headache and trigeminal dysesthesia. Author(s): Storrs TJ, Roberts CI. Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics. 1996 September; 82(3): 284-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8884826&dopt=Abstract

·

Airway abnormalities in patients with Arnold-Chiari malformation. Author(s): Choi SS, Tran LP, Zalzal GH.

56 Chiari Malformation

Source: Otolaryngology and Head and Neck Surgery. 1999 December; 121(6): 720-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10580226&dopt=Abstract ·

Antley-Bixler syndrome associated with Arnold-Chiari malformation. Author(s): Chang YT, Tsai FJ, Shen WC, Lin HC, Peng CT, Tsai CH. Source: Acta Paediatrica (Oslo, Norway : 1992). 2000 June; 89(6): 737-9. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10914975&dopt=Abstract

·

Arachnoid cyst with type I Arnold Chiari malformation presenting as syringomyelic syndrome. Author(s): Shukla R, Husain M, Sharma VP. Source: J Assoc Physicians India. 1996 September; 44(9): 658-9. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9251382&dopt=Abstract

·

Arnold Chiari malformation and nystagmus of skew. Author(s): Lavin P. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2001 March; 70(3): 416-7. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11248904&dopt=Abstract

·

Arnold-Chiari malformation and nystagmus of skew. Author(s): Pieh C, Gottlob I. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2000 July; 69(1): 124-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10864619&dopt=Abstract

·

Arnold-Chiari malformation in a captive African lion cub. Author(s): Shamir MH, Horowitz IH, Yakobson B, Ofri R. Source: J Wildl Dis. 1998 July; 34(3): 661-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9706583&dopt=Abstract

·

Arnold-chiari malformation in pregnancy. Author(s): Semple DA, McClure JH.

Studies 57

Source: Anaesthesia. 1996 June; 51(6): 580-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8694215&dopt=Abstract ·

Arnold-Chiari malformation with syringomyelia in an elderly woman. Author(s): Geroldi C, Frisoni GB, Bianchetti A, Trabucchi M, Bricolo A. Source: Age and Ageing. 1999 July; 28(4): 399-400. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10459795&dopt=Abstract

·

Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: a case report. Author(s): Ahmmed AU, Mackenzie I, Das VK, Chatterjee S, Lye RH. Source: The Journal of Laryngology and Otology. 1996 November; 110(11): 1060-4. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8944883&dopt=Abstract

·

Basilar invagination and Chiari malformation associated with cerebellar atrophy: report of two treated cases. Author(s): Goel A, Desai K, Bhatjiwale M, Muzumdar DP. Source: Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia. 2002 March; 9(2): 194-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11922714&dopt=Abstract

·

Beare-Stevenson cutis gyrata syndrome with Chiari malformation. Author(s): Wang TJ, Hung KS, Chen PK, Chuang WL, Shih TY, Lai BJ, Hsiao M. Source: Acta Neurochirurgica. 2002 July; 144(7): 743-5. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12181710&dopt=Abstract

·

Blepharoclonus and Arnold-Chiari malformation. Author(s): Jacome DE. Source: Acta Neurologica Scandinavica. 2001 August; 104(2): 113-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11493230&dopt=Abstract

·

Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and

58 Chiari Malformation

conjecture as to mechanism. Author(s): Rekate HL, Nadkarni TD, Teaford PA, Wallace D. Source: Neurosurgery. 1999 August; 45(2): 386-91. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10449086&dopt=Abstract ·

Central sleep apnoea in Arnold-Chiari malformation: evidence of pathophysiological heterogeneity. Author(s): Rabec C, Laurent G, Baudouin N, Merati M, Massin F, Foucher P, Brondel L, Reybet-Degat O. Source: The European Respiratory Journal : Official Journal of the European Society for Clinical Respiratory Physiology. 1998 December; 12(6): 1482-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9877513&dopt=Abstract

·

Cerebellar contusion associated with type I Chiari malformation following supratentorial head trauma: case report. Author(s): Couldwell WT, Zhang W, Allen R, Arce D, Stillerman CB. Source: Neurological Research. 1998 January; 20(1): 93-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9471110&dopt=Abstract

·

Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues. Author(s): Zolty P, Sanders MH, Pollack IF. Source: Sleep. 2000 August 1; 23(5): 637-43. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10947031&dopt=Abstract

·

Chiari malformation and syringomyelia in monozygotic twins: birth injury as a possible cause of syringomyelia--case report. Author(s): Iwasaki Y, Hida K, Onishi K, Nanba R. Source: Neurol Med Chir (Tokyo). 2000 March; 40(3): 176-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10842490&dopt=Abstract

·

Chiari malformation and tonsillar ectopia in twin brothers and father with autosomal dominant spondylo-epiphyseal dysplasia tarda. Author(s): Gripp KW, Scott CI Jr, Nicholson L, Magram G, Grissom LE.

Studies 59

Source: Skeletal Radiology. 1997 February; 26(2): 131-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9060107&dopt=Abstract ·

Chiari malformation associated with vitamin D-resistant rickets: case report. Author(s): Kuether TA, Piatt JH. Source: Neurosurgery. 1998 May; 42(5): 1168-71. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9588565&dopt=Abstract

·

Chiari malformation type 1 and osteoporosis. Author(s): Koch CA, Heiss JD, Pacak K, Krakoff J, Winer KK, Wassermann EM. Source: Neurosurgical Review. 2000 September; 23(3): 171-2. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11086745&dopt=Abstract

·

Chiari malformation type I: a new MRI classification. Author(s): Amer TA, el-Shmam OM. Source: Magnetic Resonance Imaging. 1997; 15(4): 397-403. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9223040&dopt=Abstract

·

Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome. Author(s): Hultman CS, Riski JE, Cohen SR, Burstein FD, Boydston WR, Hudgins RJ, Grattan-Smith D, Uhas K, Simms C. Source: Plastic and Reconstructive Surgery. 2000 July; 106(1): 16-24. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10883607&dopt=Abstract

·

Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia. Author(s): Fanfulla F, Eleftheriou D, Patruno V, Bruschi C, Rampulla C. Source: Monaldi Arch Chest Dis. 1998 April; 53(2): 138-41. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9689798&dopt=Abstract

60 Chiari Malformation

·

Combined anterior and posterior approach for managing basilar invagination associated with type I Chiari malformation. Author(s): Zileli M, Cagli S. Source: Journal of Spinal Disorders & Techniques. 2002 August; 15(4): 284-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12177543&dopt=Abstract

·

Corticospinal pathway and exercise hyperpnea: lessons from a patient with Arnold Chiari malformation. Author(s): Haouzi P, Marchal J, Allioui EM, Hannhart B, Chalon B, Braun M. Source: Respiration Physiology. 2000 October; 123(1-2): 13-22. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10996184&dopt=Abstract

·

Current opinions for the treatment of syringomyelia and chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Author(s): Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson BS. Source: Pediatric Neurosurgery. 2000 December; 33(6): 311-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11182642&dopt=Abstract

·

Decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation. Author(s): Lee JH, Chung CK, Kim HJ. Source: Spinal Cord : the Official Journal of the International Medical Society of Paraplegia. 2002 October; 40(10): 501-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12235531&dopt=Abstract

·

Effects of posterior fossa decompression with and without duraplasty on Chiari malformation-associated hydromyelia. Author(s): Munshi I, Frim D, Stine-Reyes R, Weir BK, Hekmatpanah J, Brown F. Source: Neurosurgery. 2000 June; 46(6): 1384-9; Discussion 1389-90. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10834643&dopt=Abstract

Studies 61

·

Esophageal dysphagia as the sole symptom in type I Chiari malformation. Author(s): Elta GH, Caldwell CA, Nostrant TT. Source: Digestive Diseases and Sciences. 1996 March; 41(3): 512-5. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8617124&dopt=Abstract

·

Expansive suboccipital cranioplasty for the treatment of syringomyelia associated with Chiari malformation. Author(s): Sakamoto H, Nishikawa M, Hakuba A, Yasui T, Kitano S, Nakanishi N, Inoue Y. Source: Acta Neurochirurgica. 1999; 141(9): 949-60; Discussion 960-1. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10526076&dopt=Abstract

·

Fetal alcohol syndrome with Arnold-Chiari malformation: report of one case. Author(s): Kan DC, Tsai FJ, Peng CT, Tsai CH. Source: Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1998 MarchApril; 39(2): 116-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9599902&dopt=Abstract

·

Formative evaluation of a World Wide Web-based Chiari malformation hypermedia program. Author(s): Hough B. Source: J Biocommun. 1997; 24(4): 22-32. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9494876&dopt=Abstract

·

Frontometaphyseal dysplasia: a case with Arnold-Chiari malformation and bracket epiphysis of the first metacarpal bone. Author(s): Boduroglu K, Tuncbilek E. Source: Pediatrics International : Official Journal of the Japan Pediatric Society. 1999 April; 41(2): 181-3. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10221024&dopt=Abstract

·

Headache in Chiari malformation: a distinct clinical entity? Author(s): Kesler R, Mendizabal JE.

62 Chiari Malformation

Source: J Am Osteopath Assoc. 1999 March; 99(3): 153-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10217909&dopt=Abstract ·

Hindbrain herniation syndromes: the Chiari malformations (I and II). Author(s): Cai C, Oakes WJ. Source: Semin Pediatr Neurol. 1997 September; 4(3): 179-91. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9323788&dopt=Abstract

·

Images in Pediatric Neurosurgery. Chiari malformations. Author(s): McLone DG. Source: Pediatric Neurosurgery. 2000 March; 32(3): 164. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10867566&dopt=Abstract

·

Infratentorial dysembryoplastic neuroepithelial tumor (DNT) associated with Arnold-Chiari malformation. Author(s): Yasha TC, Mohanty A, Radhesh S, Santosh V, Das S, Shankar SK. Source: Clin Neuropathol. 1998 November-December; 17(6): 305-10. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9832257&dopt=Abstract

·

Intraoperative cardiovascular collapse in an infant with Arnold-Chiari malformation. Author(s): Tanaka M, Harukuni I, Naito H. Source: Paediatric Anaesthesia. 1997; 7(2): 163-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9188119&dopt=Abstract

·

Intraoperative problem during surgery for Chiari malformation. Author(s): Sellery GR. Source: Canadian Journal of Anaesthesia = Journal Canadien D'anesthesie. 2001 July-August; 48(7): 718. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11495891&dopt=Abstract

·

Is Chiari malformation associated with increased levels of substance P and clinical symptoms in persons with fibromyalgia? Author(s): Bradley LA, Alarcon GS.

Studies 63

Source: Arthritis and Rheumatism. 1999 December; 42(12): 2731-2. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10616028&dopt=Abstract ·

Juvenile distal spinal muscular atrophy: a case with Arnold-Chiari malformation. Author(s): Celebisoy N, Uludag B, Yunten N. Source: Journal of Neurology. 1998 August; 245(8): 561-2. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9747924&dopt=Abstract

·

Letter to the editor concerning Arnold-Chiari malformation in a captive African lion cub. Author(s): Sundeep Chandra. Source: J Wildl Dis. 2000 January; 36(1): 190-1. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10682766&dopt=Abstract

·

Mechanisms of sudden death and autopsy findings in patients with Arnold-Chiari malformation and ventriculoatrial catheters. Author(s): Byard RW. Source: The American Journal of Forensic Medicine and Pathology : Official Publication of the National Association of Medical Examiners. 1996 September; 17(3): 260-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8870879&dopt=Abstract

·

Meningococcal meningitis with Arnold-Chiari malformation. Author(s): Jackson RM, Penrose-Stevens A. Source: The Journal of Infection. 1997 July; 35(1): 90-2. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9279736&dopt=Abstract

·

Neurofibromatosis type 1 and type I Chiari malformation: an unusual association. Author(s): Battistella PA, Perilongo G, Carollo C.

64 Chiari Malformation

Source: Child's Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery. 1996 June; 12(6): 336-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8816299&dopt=Abstract ·

Neurofibromatosis type I and Arnold-Chiari malformation. Author(s): Giustini S, Richetta A, Divona L, Faiola R, Trasimeni G, Pezza M, Amoruso G, Calvieri S. Source: Journal of the European Academy of Dermatology and Venereology : Jeadv. 2002 March; 16(2): 180-1. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12046833&dopt=Abstract

·

Neuropathic shoulder arthropathy associated with syringomyelia and Arnold-Chiari malformation (type I). Author(s): Riente L, Frigelli S, Delle SA. Source: The Journal of Rheumatology. 2002 March; 29(3): 638-9. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11908585&dopt=Abstract

·

Obstructive sleep apnoea with Arnold-Chiari malformation. Author(s): Doherty MJ, Spence DP, Young C, Calverley PM. Source: Thorax. 1995 June; 50(6): 690-1; Discussion 696-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7638817&dopt=Abstract

·

Orofacial pain as the sole manifestation of syringobulbiasyringomyelia associated with Arnold-Chiari malformation. Author(s): Penarrocha M, Okeson JP, Penarrocha MS, Angeles Cervello M. Source: J Orofac Pain. 2001 Spring; 15(2): 170-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11443828&dopt=Abstract

·

Otorhinolaryngologic manifestations in Chiari malformation. Author(s): Naya Galvez MJ, Fraile Rodrigo JJ, Liesa RF, Vicente Gonzalez EA, Garrido CM, Samperiz LC, Tajada JD.

Studies 65

Source: American Journal of Otolaryngology. 2002 March-April; 23(2): 99104. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11893978&dopt=Abstract ·

Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. Author(s): Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Source: Journal of Neurosurgery. 1997 January; 86(1): 40-7. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8988080&dopt=Abstract

·

Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Author(s): Hida K, Iwasaki Y, Koyanagi I, Abe H. Source: Surgical Neurology. 1999 April; 51(4): 383-90; Discussion 390-1. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10199291&dopt=Abstract

·

Periodic alternating nystagmus associated with Arnold-Chiari malformation. Author(s): Korres S, Balatsouras DG, Zournas C, Economou C, Gatsonis SD, Adamopoulos G. Source: The Journal of Laryngology and Otology. 2001 December; 115(12): 1001-4. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11779333&dopt=Abstract

·

Peripheral chemoreceptor function in children with myelomeningocele and Arnold-Chiari malformation type 2. Author(s): Gozal D, Arens R, Omlin KJ, Jacobs RA, Keens TG. Source: Chest. 1995 August; 108(2): 425-31. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7634879&dopt=Abstract

·

Posterior fossa decompression in syringomyelia associated with a Chiari malformation: a retrospective analysis of 22 patients. Author(s): Depreitere B, Van Calenbergh F, van Loon J, Goffin J, Plets C. Source: Clinical Neurology and Neurosurgery. 2000 June; 102(2): 91-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10817895&dopt=Abstract

66 Chiari Malformation

·

Posterior fossa decompression without duraplasty in infants and young children for treatment of Chiari malformation and achondroplasia. Author(s): Yundt KD, Park TS, Tantuwaya VS, Kaufman BA. Source: Pediatric Neurosurgery. 1996 November; 25(5): 221-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9309784&dopt=Abstract

·

Posttraumatic tremor and Arnold Chiari malformation: no sign of compression, but cure after surgical decompression. Author(s): Vanhatalo S, Paetau R, Mustonen K, Hernesniemi J, Riikonen R. Source: Movement Disorders : Official Journal of the Movement Disorder Society. 2000 May; 15(3): 581-3. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10830428&dopt=Abstract

·

Posturally evoked vomiting without nystagmus in a patient with Arnold-Chiari malformation. Author(s): Pollak L, Klein C, Rabey JM, Reichenthal E. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2001 September; 71(3): 414-5. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11534519&dopt=Abstract

·

Pseudotumour cerebri occurring in association with the Chiari malformation. Author(s): Sinclair N, Assaad N, Johnston I. Source: Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia. 2002 January; 9(1): 99-101. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11749033&dopt=Abstract

·

Radiology quiz case 2. Chiari malformation (type I). Author(s): Aferzon M, Reams CL. Source: Archives of Otolaryngology--Head & Neck Surgery. 2002 September; 128(9): 1104, 1106-7. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12220223&dopt=Abstract

·

Recurrent compression of Chiari malformation. Author(s): Cochrane DD.

Studies 67

Source: Pediatric Neurosurgery. 1996; 24(5): 277. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8933574&dopt=Abstract ·

Reevaluation of syringosubarachnoid shunt for syringomyelia with Chiari malformation. Author(s): Iwasaki Y, Hida K, Koyanagi I, Abe H. Source: Neurosurgery. 2000 February; 46(2): 407-12; Discussion 412-3. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10690730&dopt=Abstract

·

Resolution of Chiari malformation after repair of a congenital thoracic meningocele: case report and literature review. Author(s): Madhok R, Mazzola CA, Pollack IF. Source: Neurosurgery. 2002 December; 51(6): 1489-92. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12445356&dopt=Abstract

·

Respiratory arrest: a complication of Arnold-Chiari malformation in adults. Author(s): Omer S, al-Kawi MZ, Bohlega S, Bouchama A, Mclean D. Source: European Neurology. 1996; 36(1): 36-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8719648&dopt=Abstract

·

Respiratory failure in Arnold-Chiari malformation. Author(s): Hattingh TL, Butler JT, Biebuyck AL, Bolliger CT. Source: Respiration; International Review of Thoracic Diseases. 2002; 69(1): 86. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11844969&dopt=Abstract

·

Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations. Author(s): Portier F, Marianowski R, Morisseau-Durand MP, Zerah M, Manac'h Y. Source: International Journal of Pediatric Otorhinolaryngology. 2001 March; 57(3): 195-202. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11223451&dopt=Abstract

68 Chiari Malformation

·

Sagittal profiles of the spine in scoliosis associated with an ArnoldChiari malformation with or without syringomyelia. Author(s): Loder RT, Stasikelis P, Farley FA. Source: Journal of Pediatric Orthopedics. 2002 July-August; 22(4): 483-91. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12131445&dopt=Abstract

·

Simultaneous cerebral and spinal fluid pressure recordings in surgical indications of the Chiari malformation without myelodysplasia. Author(s): Hackel M, Benes V, Mohapl M. Source: Acta Neurochirurgica. 2001 September; 143(9): 909-17; Discussion 918. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11685623&dopt=Abstract

·

Sleep apnea syndrome associated with a type I Chiari malformation. Author(s): Yoshimi A, Nomura K, Furune S. Source: Brain & Development. 2002 January; 24(1): 49-51. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11751025&dopt=Abstract

·

Spondylolysis associated with Arnold-Chiari malformation and syringomyelia. Author(s): Rothman SL. Source: Spine. 1998 July 1; 23(13): 1506. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9670407&dopt=Abstract

·

Spondylolysis associated with Arnold-Chiari malformation and syringomyelia. A report of two cases. Author(s): Bowen JD, Malanga GA. Source: Spine. 1997 October 15; 22(20): 2458-63. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9355230&dopt=Abstract

·

Spontaneous intracranial hypotension: headache with a reversible Arnold-Chiari malformation. Author(s): Kasner SE, Rosenfeld J, Farber RE. Source: Headache. 1995 October; 35(9): 557-9. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8530283&dopt=Abstract

Studies 69

·

Spontaneous regeneration of the foramen magnum after decompressive suboccipital craniectomy in Chiari malformation: case report. Author(s): Aoki N, Oikawa A, Sakai T. Source: Neurosurgery. 1995 August; 37(2): 340-2. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7477791&dopt=Abstract

·

Sudden death and Chiari malformations. Author(s): Rocker GM, MacAulay MA, Sangalang V. Source: Intensive Care Medicine. 1995 July; 21(7): 621. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7593909&dopt=Abstract

·

Syringobulbia caused by delayed postoperative tethering of the cervical spinal cord - delayed complication of foramen magnum decompression for Chiari malformation. Author(s): Takahashi Y, Tajima Y, Ueno S, Tokutomi T, Shigemori M. Source: Acta Neurochirurgica. 1999; 141(9): 969-72; Discussion 972-3. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=10526078&dopt=Abstract

·

Syringomyelia and chiari malformation presenting as scoliosis in cystic fibrosis. Author(s): Rusakow LS, Guarin M, Lyon RM, Splaingard ML. Source: Pediatric Pulmonology. 1995 May; 19(5): 317-8. Review. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=7567208&dopt=Abstract

·

Syringomyelia associated with type I Chiari malformation. A 21-year retrospective study on 75 cases treated by foramen magnum decompression with a special emphasis on the value of tonsils resection. Author(s): Guyotat J, Bret P, Jouanneau E, Ricci AC, Lapras C. Source: Acta Neurochirurgica. 1998; 140(8): 745-54. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9810440&dopt=Abstract

70 Chiari Malformation

·

The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management. Author(s): Johnston I, Jacobson E, Besser M. Source: Acta Neurochirurgica. 1998; 140(5): 417-27; Discussion 427-8. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9728240&dopt=Abstract

·

The Chiari malformations: a constellation of anomalies. Author(s): Shuman RM. Source: Semin Pediatr Neurol. 1995 September; 2(3): 220-6. Review. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9422249&dopt=Abstract

·

The semantics of terminology: distinguishing Arnold-Chiari malformations from Chiari malformations. Author(s): Ehara S, Shimamura T. Source: The Journal of Bone and Joint Surgery. American Volume. 2002 February; 84-A(2): 321. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11861743&dopt=Abstract

·

Three-dimensional computerized tomography in the presurgical evaluation of Chiari malformations. Author(s): Duffau H, Sahel M, Sichez JP, Marro B. Source: Acta Neurochirurgica. 1998; 140(5): 429-35; Discussion 435-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=9728241&dopt=Abstract

·

Treatment of the Chiari malformation by Drs. H.E. James and A. Brant. Author(s): Hori A. Source: Child's Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery. 2002 October; 18(9-10): 461-2. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12382165&dopt=Abstract

·

Treatment of the Chiari malformation with bone decompression without durotomy in children and young adults. Author(s): James HE, Brant A.

Studies 71

Source: Child's Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery. 2002 May; 18(5): 202-6. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=12042917&dopt=Abstract ·

Unusual late responses in a patient with an Arnold-Chiari malformation. Author(s): Kutukcu Y, Odabasi Z, Vural O, Yardim M. Source: Electromyogr Clin Neurophysiol. 1999 June; 39(4): 213-6.

Vocabulary Builder 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] Aerobic: 1. having molecular oxygen present. 2. growing, living, or occurring in the presence of molecular oxygen. 3. requiring oxygen for respiration. [EU] 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] Anesthesiology: A specialty concerned with the study of anesthetics and anesthesia. [NIH] Angiography: Radiography of blood vessels after injection of a contrast medium. [NIH] Antibody: An immunoglobulin molecule that has a specific amino acid sequence by virtue of which it interacts only with the antigen that induced its synthesis in cells of the lymphoid series (especially plasma cells), or with antigen closely related to it. Antibodies are classified according to their ode of action as agglutinins, bacteriolysins, haemolysins, opsonins, precipitins, etc. [EU] Antihypertensive: An agent that reduces high blood pressure. [EU] Antioxidant: One of many widely used synthetic or natural substances added to a product to prevent or delay its deterioration by action of oxygen in the air. Rubber, paints, vegetable oils, and prepared foods commonly contain antioxidants. [EU] Apnea: A transient absence of spontaneous respiration. [NIH]

72 Chiari Malformation

Apnoea: Cessation of breathing. [EU] Arteries: The vessels carrying blood away from the heart. [NIH] Arthropathy: Any joint disease. [EU] Assay: Determination of the amount of a particular constituent of a mixture, or of the biological or pharmacological potency of a drug. [EU] Atrophy: A wasting away; a diminution in the size of a cell, tissue, organ, or part. [EU] Autopsy: Postmortem examination of the body. [NIH] Biochemical: Relating to biochemistry; characterized by, produced by, or involving chemical reactions in living organisms. [EU] Buccal: Pertaining to or directed toward the cheek. In dental anatomy, used to refer to the buccal surface of a tooth. [EU] Cardiac: Pertaining to the heart. [EU] Cardiovascular: Pertaining to the heart and blood vessels. [EU] Catheter: A tubular, flexible, surgical instrument for withdrawing fluids from (or introducing fluids into) a cavity of the body, especially one for introduction into the bladder through the urethra for the withdraw of urine. [EU]

Cerebral: Of or pertaining of the cerebrum or the brain. [EU] Cerebrospinal: Pertaining to the brain and spinal cord. [EU] Cerebrovascular: Pertaining to the blood vessels of the cerebrum, or brain. [EU]

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] Chronic: Persisting over a long period of time. [EU] 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] Contusion: A bruise; an injury of a part without a break in the skin. [EU] 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] 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] Depolarization: The process or act of neutralizing polarity. In neurophysiology, the reversal of the resting potential in excitable cell

Studies 73

membranes when stimulated, i.e., the tendency of the cell membrane potential to become positive with respect to the potential outside the cell. [EU] Dermatology: A medical specialty concerned with the skin, its structure, functions, diseases, and treatment. [NIH] Disposition: A tendency either physical or mental toward certain diseases. [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] Dysphagia: Difficulty in swallowing. [EU] Dysplasia: Abnormality of development; in pathology, alteration in size, shape, and organization of adult cells. [EU] Endogenous: Developing or originating within the organisms or arising from causes within the organism. [EU] Enzyme: A protein molecule that catalyses chemical reactions of other substances without itself being destroyed or altered upon completion of the reactions. Enzymes are classified according to the recommendations of the Nomenclature Committee of the International Union of Biochemistry. Each enzyme is assigned a recommended name and an Enzyme Commission (EC) number. They are divided into six main groups; oxidoreductases, transferases, hydrolases, lyases, isomerases, and ligases. [EU] Epiphyseal: Pertaining to or of the nature of an epiphysis. [EU] Estrogens: A class of sex hormones associated with the development and maintenance of secondary female sex characteristics and control of the cyclical changes in the reproductive cycle. They are also required for pregnancy maintenance and have an anabolic effect on protein metabolism and water retention. [NIH] Fibrin: The insoluble protein formed from fibrinogen by the proteolytic action of thrombin during normal clotting of blood. Fibrin forms the essential portion of the blood clot. [EU] Fibrosis: The formation of fibrous tissue; fibroid or fibrous degeneration [EU] Hemorrhage: Bleeding or escape of blood from a vessel. [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] Hypotension: Abnormally low blood pressure; seen in shock but not necessarily indicative of it. [EU] Hypothalamic: Of or involving the hypothalamus. [EU]

74 Chiari Malformation

Hypoxia: Reduction of oxygen supply to tissue below physiological levels despite adequate perfusion of the tissue by blood. [EU] Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents. [NIH] Infarction: 1. the formation of an infarct. 2. an infarct. [EU] 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 insulindependent diabetes mellitus. [NIH] Invasive: 1. having the quality of invasiveness. 2. involving puncture or incision of the skin or insertion of an instrument or foreign material into the body; said of diagnostic techniques. [EU] Ischemia: Deficiency of blood in a part, due to functional constriction or actual obstruction of a blood vessel. [EU] Lesion: Any pathological or traumatic discontinuity of tissue or loss of function of a part. [EU] Lipoprotein: Any of the lipid-protein complexes in which lipids are transported in the blood; lipoprotein particles consist of a spherical hydrophobic core of triglycerides or cholesterol esters surrounded by an amphipathic monolayer of phospholipids, cholesterol, and apolipoproteins; the four principal classes are high-density, low-density, and very-lowdensity lipoproteins and chylomicrons. [EU] Lumbar: Pertaining to the loins, the part of the back between the thorax and the pelvis. [EU] Lumen: The cavity or channel within a tube or tubular organ. [EU] Meningitis: Inflammation of the meninges. When it affects the dura mater, the disease is termed pachymeningitis; when the arachnoid and pia mater are involved, it is called leptomeningitis, or meningitis proper. [EU] Metabolite: process. [EU]

Any substance produced by metabolism or by a metabolic

Microdialysis: A technique for measuring extracellular concentrations of substances in tissues, usually in vivo, by means of a small probe equipped with a semipermeable membrane. Substances may also be introduced into the extracellular space through the membrane. [NIH] Myocardium: The muscle tissue of the HEART composed of striated, involuntary muscle known as cardiac muscle. [NIH] Neuronal: Pertaining to a neuron or neurons (= conducting cells of the nervous system). [EU]

Studies 75

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] Neutrophil: Having an affinity for neutral dyes. [EU] Nystagmus: An involuntary, rapid, rhythmic movement of the eyeball, which may be horizontal, vertical, rotatory, or mixed, i.e., of two varieties. [EU]

Orthopedics: A surgical specialty which utilizes medical, surgical, and physical methods to treat and correct deformities, diseases, and injuries to the skeletal system, its articulations, and associated structures. [NIH] Osteoporosis: Reduction in the amount of bone mass, leading to fractures after minimal trauma. [EU] Otolaryngology: A surgical specialty concerned with the study and treatment of disorders of the ear, nose, and throat. [NIH] Otorhinolaryngology: That branch of medicine concerned with medical and surgical treatment of the head and neck, including the ears, nose and throat. [EU]

Paraplegia: Paralysis of the legs and lower part of the body. [EU] Pediatrics: A medical specialty concerned with maintaining health and providing medical care to children from birth to adolescence. [NIH] Perindopril: An angiotensin-converting enzyme inhibitor. It is used in patients with hypertension and heart failure. [NIH] Physiologic: Normal; not pathologic; characteristic of or conforming to the normal functioning or state of the body or a tissue or organ; physiological. [EU]

Plasminogen: The inactive precursor of plasmin (=enzyme that catalyses the hydrolysis of peptide bonds at the carbonyl end of lysine or arginine residues). [EU] 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: Occurring after the menopause. [EU] Postoperative: Occurring after a surgical operation. [EU] Potassium: An element that is in the alkali group of metals. It has an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte and it plays a significant role in the regulation of fluid volume and maintenance of the water-electrolyte balance. [NIH] Prevalence: The total number of cases of a given disease in a specified population at a designated time. It is differentiated from INCIDENCE, which

76 Chiari Malformation

refers to the number of new cases in the population at a given time. [NIH] Proximal: Nearest; closer to any point of reference; opposed to distal. [EU] Psychiatry: The medical science that deals with the origin, diagnosis, prevention, and treatment of mental disorders. [NIH] Psychology: The science dealing with the study of mental processes and behavior in man and animals. [NIH] Radiology: A specialty concerned with the use of x-ray and other forms of radiant energy in the diagnosis and treatment of disease. [NIH] Receptor: 1. a molecular structure within a cell or on the surface characterized by (1) selective binding of a specific substance and (2) a specific physiologic effect that accompanies the binding, e.g., cell-surface receptors for peptide hormones, neurotransmitters, antigens, complement fragments, and immunoglobulins and cytoplasmic receptors for steroid hormones. 2. a sensory nerve terminal that responds to stimuli of various kinds. [EU] Recombinant: 1. a cell or an individual with a new combination of genes not found together in either parent; usually applied to linked genes. [EU] Regeneration: The natural renewal of a structure, as of a lost tissue or part. [EU]

Renin: An enzyme of the hydrolase class that catalyses cleavage of the leucine-leucine bond in angiotensin to generate angiotensin. 1. The enzyme is synthesized as inactive prorenin in the kidney and released into the blood in the active form in response to various metabolic stimuli. Not to be confused with rennin (chymosin). [EU] Resection: Excision of a portion or all of an organ or other structure. [EU] Rheumatology: A subspecialty of internal medicine concerned with the study of inflammatory or degenerative processes and metabolic derangement of connective tissue structures which pertain to a variety of musculoskeletal disorders, such as arthritis. [NIH] Rickets: A condition caused by deficiency of vitamin D, especially in infancy and childhood, with disturbance of normal ossification. The disease is marked by bending and distortion of the bones under muscular action, by the formation of nodular enlargements on the ends and sides of the bones, by delayed closure of the fontanelles, pain in the muscles, and sweating of the head. Vitamin D and sunlight together with an adequate diet are curative, provided that the parathyroid glands are functioning properly. [EU] Semantics: The relationships between symbols and their meanings. [NIH] Skeletal: Pertaining to the skeleton. [EU] Stenosis: Narrowing or stricture of a duct or canal. [EU] Subarachnoid: Situated or occurring between the arachnoid and the pia

Studies 77

mater. [EU] 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] Syncope: A temporary suspension of consciousness due to generalized cerebral schemia, a faint or swoon. [EU] Systemic: Pertaining to or affecting the body as a whole. [EU] Thoracic: Pertaining to or affecting the chest. [EU] Thrombolytic: 1. dissolving or splitting up a thrombus. 2. a thrombolytic agent. [EU] Thrombosis: The formation, development, or presence of a thrombus. [EU] Tolerance: 1. the ability to endure unusually large doses of a drug or toxin. 2. acquired drug tolerance; a decreasing response to repeated constant doses of a drug or the need for increasing doses to maintain a constant response. [EU]

Tomography: The recording of internal body images at a predetermined plane by means of the tomograph; called also body section roentgenography. [EU]

Tremor: An involuntary trembling or quivering. [EU] Venereology: A branch of medicine which deals with sexually transmitted disease. [NIH] Ventricular: Pertaining to a ventricle. [EU] 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] Warfarin: An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide. [NIH]

79

CHAPTER 5. BOOKS ON CHIARI MALFORMATION Overview This chapter provides bibliographic book references relating to Chiari malformation. You have many options to locate books on Chiari malformation. The simplest method is to go to your local bookseller and inquire about titles that they have in stock or can special order for you. Some parents, however, prefer online sources (e.g. www.amazon.com and www.bn.com). In addition to online booksellers, excellent sources for book titles on Chiari malformation include the Combined Health Information Database and the National Library of Medicine. Once you have found a title that interests you, visit your local public or medical library to see if it is available for loan.

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 “Chiari malformation” (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:19 In addition to LOCATORPlus, in collaboration with authors and publishers, the National Center for Biotechnology Information (NCBI) is 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

19

80 Chiari Malformation

·

Color atlas of congenital malformation syndromes. Author: Michael Baraitser, Robin M. Winter; Year: 1996; London; Baltimore: Mosby-Wolfe, 1996. ISBN: 0723420734 http://www.amazon.com/exec/obidos/ASIN/0723420734/icongroupin terna

·

Congenital malformation statistics, notifications: a statistical review of notifications of congenital malformations received as part of the England and Wales monitoring system. Author: Jones, Kenneth Lyons; Year: 1988; London: H.M.S.O., 1988. ISBN: 0116912251

·

Congenital malformation syndromes. Author: edited by D. Donnai and R.M. Winter; Year: 1995; London; New York: Chapman & Hall Medical, 1995. ISBN: 0412565900 http://www.amazon.com/exec/obidos/ASIN/0412565900/icongroupin terna

·

Craniofacial mesenchyme in morphogenesis and malformation: proceedings of the Sixth Annual Symposium of the Society of Craniofacial Genetics, held in Seattle, Washington, June 19, 1983. Author: editors, Kenneth S. Brown, Carlos F. Salinas, Natalie W. Paul; ass; Year: 1984; New York: Liss, c1984. ISBN: 0845110586 http://www.amazon.com/exec/obidos/ASIN/0845110586/icongroupin terna

·

Cytogenetics, environment, and malformation syndromes: the 1975 Birth Defects Conference held at Kansas City, Missouri. Author: sponsored by the University of Kansas Medical Center, College of Health Sciences and Hospital and the National Foundation-March of Di; Year: 1976; New York: Liss, c1976. ISBN: 0845110047 http://www.amazon.com/exec/obidos/ASIN/0845110047/icongroupin terna

·

Ductal plate malformation. Author: by Mogens Jørgen Jørgensen; Year: 1977; Copenhagen: Munksgaard, 1977. ISBN: 871620839

·

First Conference on the Clinical Delineation of Birth Defects. Editor: Daniel Bergsma; associate editors: Victor A. McKusick [et al.] assistant editor: Camille Jackson [et al. Author: Conference on the Clinical Delineation of Birth Defects (1st: 1968: Johns Hopkins Hospital, Baltimore); Year: 1969; New York, National Foundation] 1969.

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.

Books 81

·

Genetic & malformation syndromes in clinical medicine. Author: William L. Nyhan and Nadia O. Sakati; Year: 1976; Chicago: Year Book Medical Publishers, c1976. ISBN: 0815164866 http://www.amazon.com/exec/obidos/ASIN/0815164866/icongroupin terna

·

Malformation syndromes. Editor: Daniel Bergsma [et al.]. Author: Boston Conference on the Diagnosis, Genetics and Management of Birth Defects (1973); Year: 1974; New York, Intercontinental Medical Book Corp. [c1974]. ISBN: 0883720531 http://www.amazon.com/exec/obidos/ASIN/0883720531/icongroupin terna

·

Malformation syndromes; papers presented at the Johns Hopkins Hospital, sponsored by the Johns Hopkins Medical Institutions and the National Foundation-March of Dimes. Editor: Daniel Bergsma. Associate editors: Victor A. McKusick, Thaddeus E. Kelly, Edwar. Author: Birth Defects Conference (1974: Newport Beach, Calif.); Year: 1975; New York National Foundation-March of Dimes; [distributed by American Elsevier Pub. Co., c1975]. ISBN: 0444151559 http://www.amazon.com/exec/obidos/ASIN/0444151559/icongroupin terna

·

Mechanisms of congenital malformation. Proceedings of the second scientific conference of the Association for the Aid of Crippled Children, June 15 and 16, 1954. Harold Wolff, editor. Author: Association for the Aid of Crippled Children, New York. Scientific Conference (2d: 1954: New York); Year: 1954; New York, 1954.

·

Morphogenesis and malformation of face and brain: the first International Conference on Morphogenesis and Malformation held at Airlie House, Virginia, June 1974. Author: sponsored by the National Foundation-March of Dimes; editor, Daniel Bergsma, associate edi; Year: 1975; New York: Liss, c1975. ISBN: 0845110012 http://www.amazon.com/exec/obidos/ASIN/0845110012/icongroupin terna

·

Morphogenesis and malformation of the cardiovascular system: the Fourth International Workshop on Morphogenesis and Malformation held at Grand Canyon, Arizona, November 9-12, 1977. Author: sponsored by the National Foundation-March of Dimes; editors, Glenn C; Year: 1978; New York: Liss, c1978. ISBN: 0845110233 http://www.amazon.com/exec/obidos/ASIN/0845110233/icongroupin terna

·

Morphogenesis and malformation of the ear: Fifth International Workshop on Morphogenesis and Malformation held at Gulf State Park Resort, Gulf Shores, Alabama. Author: sponsored by March of

82 Chiari Malformation

Dimes Birth Defects Foundation; editor, Robert J. Gorlin; associate e; Year: 1980; New York: Liss, 1980. ISBN: 0845110381 http://www.amazon.com/exec/obidos/ASIN/0845110381/icongroupin terna ·

Morphogenesis and malformation of the genital system: the third International Conference on Morphogenesis and Malformation held at Lake Wilderness, Washington, July 1976. Author: sponsored by the National Foundation-March of Dimes; editors, Richard J. Blandau,; Year: 1977; New York: Liss, 1977. ISBN: 0845110098 http://www.amazon.com/exec/obidos/ASIN/0845110098/icongroupin terna

·

Morphogenesis and malformation of the limb: the second International Conference ... Author: editors, Daniel Bergsma, Widukind Lenz, assistant editor, Sue Conde Greene; Year: 1977; New York: Liss, c1977. ISBN: 084511008X http://www.amazon.com/exec/obidos/ASIN/084511008X/icongroupi nterna

·

Morphogenesis and malformation of the skin: sixth International Workshop on Morphogenesis and Malformation held at Lake Wilderness, Washington, September 18-20, 1980, sponsored by March of Dimes Birth Defects Foundation. Author: editor, RichardJ. Blandau, assoc; Year: 1981; New York: Liss, c1981. ISBN: 084511042X http://www.amazon.com/exec/obidos/ASIN/084511042X/icongroupi nterna

·

Mutation, cancer, and malformation. Author: edited by Ernest H.Y. Chu and Walderico M. Generoso; Year: 1984; New York: Plenum Press, c1984. ISBN: 0306418207 http://www.amazon.com/exec/obidos/ASIN/0306418207/icongroupin terna

·

New chromosomal and malformation syndromes: the 1974 Birth Defects Conference held at Newport Beach, California. Author: sponsored by the Harbor General Hospital Campus of the UCLA School of Medicine and the National Foundation-March of Dimes;editor, Daniel Be; Year: 1975; New York: Stratton Intercontinental Medical Book Corp., c1975. ISBN: 0883720329 http://www.amazon.com/exec/obidos/ASIN/0883720329/icongroupin terna

·

On malformation of the aortic valves as a cause of disease. Author: Peacock, Thomas Bevill, 1812-1882; Year: 1853; [Edinburgh, Murray and Gibb, 1853].

Books 83

·

Operative techniques in pediatric neurosurgery. Author: edited by A. Leland Albright, Ian F. Pollack, P. David Adelson; Year: 2001; New York: Thieme, 2001. ISBN: 0865778469 http://www.amazon.com/exec/obidos/ASIN/0865778469/icongroupin terna

·

Penetrance and variability in malformation syndromes. Author: editors, James J. O'Donnell, Bryan D. Hall; associate editor, Natalie W. Paul; assistant editor, Marilyn P. Orlando; Year: 1979; New York: Liss, 1979. ISBN: 0845110292 http://www.amazon.com/exec/obidos/ASIN/0845110292/icongroupin terna

·

Prematurity, congential malformation and birth injury. Proceedings of a conference sponsored by Association for the Aid of Crippled Children at the New York Academy of Medicine, June 5 and 6, 1952. Author: Association for the Aid of Crippled Children, New York. Scientific Conference (1st: 1952: New York); Year: 1953; New York, 1953.

·

Recognizable patterns of human malformation: genetic, embryologic and clinical aspects. Author: by David W. Smith; Year: 1976; Philadelphia: Saunders, 1976. ISBN: 0721683762 http://www.amazon.com/exec/obidos/ASIN/0721683762/icongroupin terna

·

Recognizable patterns of human malformation: genetic, embryologic and clinical aspects. Author: by David W. Smith with the assistance of Kenneth Lyons Jones; Year: 1982; Philadelphia: Saunders, 1982. ISBN: 0721683819

·

Recognizable patterns of human malformation; genetic, embryologic, and clinical aspects. Author: Smith, David W., 1926-1981; Year: 1970; Philadelphia, Saunders, 1970.

·

Smith's recognizable patterns of human malformation. Author: Kenneth Lyons Jones; Year: 1997; Philadelphia: Saunders, c1997. ISBN: 0721661157 http://www.amazon.com/exec/obidos/ASIN/0721661157/icongroupin terna

·

Surgical correction of intersexual genitalia and female genital malformation. Author: Waldemar Ch. Hecker; with a section on pediatric endocrinology by Dieter Knorr; translation by Terry C. Telger; Year: 1985; Berlin; New York: Springer-Verlag, c1985. ISBN: 0387153152 http://www.amazon.com/exec/obidos/ASIN/0387153152/icongroupin terna

·

Syringomyelia and the Chiari malformations. Author: AANS Publications Committee; John A. Anson, Edward C. Benzel, Issam A.

84 Chiari Malformation

Awad, editors; Year: 1997; Park Ridge, Ill.: American Association of Neurological Surgeons, c1997. ISBN: 1879284421 http://www.amazon.com/exec/obidos/ASIN/1879284421/icongroupin terna ·

Towards the prevention of the fetal malformation. Author: edited by J. B. Scrimgeour for the Edinburgh University Press; Year: 1978; Edinburgh: Edinburgh Univ. Press, c1978. ISBN: 0852243243 http://www.amazon.com/exec/obidos/ASIN/0852243243/icongroupin terna

Chapters on Chiari Malformation Frequently, Chiari malformation will be discussed within a book, perhaps within a specific chapter. In order to find chapters that are specifically dealing with Chiari malformation, an excellent source of abstracts is the Combined Health Information Database. You will need to limit your search to book chapters and Chiari malformation using the “Detailed Search” option. Go directly 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.” By making these selections and typing in “Chiari malformation” (or synonyms) into the “For these words:” box, you will only receive results on chapters in books. The following is a typical result when searching for book chapters on Chiari malformation: ·

Vertigo of Central Origin Source: in Canalis, R.F. and Lambert, P.R., eds. Ear: Comprehensive Otology. Philadelphia, PA: Lippincott Williams and Wilkins. 2000. p. 665679. Contact: Available from Lippincott Williams and Wilkins. P.O. Box 1600, Hagerstown, MD 21741. (800) 638-3030. Fax (301) 223-2300. Website: www.lww.com. PRICE: $179.00 plus shipping and handling. ISBN: 078171558X. Summary: This chapter on vertigo of central origin is from a textbook that offers complete coverage of the field of clinical otology (study of the ear). The book is oriented to serve both the otolaryngology resident as a practical learning tool and the practicing otolaryngologist as an updated reference source of clinical and basic information. Topics include differentiating central and peripheral causes of vertigo; the neurologic complications of ear infections, including epidural abscess and brain

Books 85

abscess; vascular disease, including vertebrobasilar insufficiency, infarction of the brainstem and cerebellum, hemorrhage into the brainstem and cerebellum, and the diagnosis of vascular causes of vertigo; migraine, including the clinical profile, migraine equivalents, and the mechanism of vertigo with migraine; tumors, including tumors of the cerebellopontine angle, and brain tumors; brain trauma, including the mechanism of brain injury, brainstem contusion, postconcussion syndrome, and the diagnosis of persistent dizziness after head trauma; cerebellar degeneration syndromes, including alcohol cerebellar degeneration and familial ataxia syndromes; disorders of the cranial vertebral junction, including the mechanism of brain damage, basilar impression, atlantoaxial dislocation, and Chiari malformation; multiple sclerosis; and vertigo and focal seizure disorders. For each disorder, the author discusses diagnosis, diagnostic tests, and treatment options. 3 figures. 2 tables. 74 references. ·

Common Neurotological Disorders Source: in Baloh, R.W. Dizziness, Hearing Loss, and Tinnitus. Philadelphia, PA: F.A. Davis Company. 1998. p. 139-198. Contact: Available from Oxford University Press, Inc. Business Office, 2001 Evans Road, Cary, NC 27513. (800) 451-7556 or (919) 677-0977. Fax (919) 677-1303. PRICE: $65.00 plus shipping and handling. Summary: This chapter on common neurotological disorders is from a textbook that presents a concise approach to evaluating patients with dizziness, hearing loss, and tinnitus. The first section outlines infections of the ear and temporal bone, including acute otitis media, chronic otomastoiditis, malignant external otitis, intracranial complications of otitic infection, toxic (serous) labyrinthitis, viral neurolabyrinthitis, herpes zoster oticus (Ramsay Hunt syndrome), and syphilitis labyrinthitis. The next section considers vascular disorders, including labyrinthine ischemia and infarction, labyrinthine hemorrhage, vertebrobasilar transient ischemic attacks, lateral medullary infarction (Wallenberg's syndrome), lateral pontomedullary infarction, cerebellar infarction, and cerebellar hemorrhage. Additional topics covered include benign positional vertigo (canalithiasis), Meniere's disease (endolymphatic hydrops), autoimmune inner ear disease, migraine (benign recurrent vertigo), tumors, trauma (otitic barotrauma, perilymph fistula, labyrinthine concussion, and noise-induced hearing loss), ototoxicity, otosclerosis, aging (presbycusis and disequilibrium of aging), developmental disorders (maldevelopment of the inner ear, and Chiari malformation), and multiple sclerosis. For each condition, the author summarizes the symptoms, signs, laboratory tests, and management

86 Chiari Malformation

strategies. Numerous tables and flowcharts guide the reader through the diagnostic workup. Important points are highlighted and presented in the margins of the text. 29 figures. 2 tables. 133 references.

General Home References In addition to references for Chiari malformation, you may want a general home medical guide that spans all aspects of home healthcare. The following list is a recent sample of such guides (sorted alphabetically by title; hyperlinks provide rankings, information, and reviews at Amazon.com): ·

Adams & Victor’s Principles Of Neurology by Maurice Victor, et al; Hardcover - 1692 pages; 7th edition (December 19, 2000), McGraw-Hill Professional Publishing; ISBN: 0070674973; http://www.amazon.com/exec/obidos/ASIN/0070674973/icongroupinter na

·

American Academy of Pediatrics Guide to Your Child’s Symptoms : The Official, Complete Home Reference, Birth Through Adolescence by Donald Schiff (Editor), et al; Paperback - 256 pages (January 1997), Villard Books; ISBN: 0375752579; http://www.amazon.com/exec/obidos/ASIN/0375752579/icongroupinter na

·

The Children’s Hospital Guide to Your Child’s Health and Development by Alan D. Woolf (Editor), et al; Hardcover - 796 pages, 1st edition (January 15, 2001), Perseus Books; ISBN: 073820241X; http://www.amazon.com/exec/obidos/ASIN/073820241X/icongroupinter na

·

Clinical Neuroanatomy Made Ridiculously Simple (MedMaster Series, 2000 Edition) by Stephen Goldberg; Paperback: 97 pages; 2nd edition (February 15, 2000), Medmaster; ISBN: 0940780461; http://www.amazon.com/exec/obidos/ASIN/0940780461/icongroupinter na

·

Helping Your Child in the Hospital: A Practical Guide for Parents by Nancy Keene, Rachel Prentice; Paperback - 176 pages, 3rd edition (April 15, 2002), O’Reilly & Associates; ISBN: 0596500114; http://www.amazon.com/exec/obidos/ASIN/0596500114/icongroupinter na

·

It’s Not a Tumor!: The Patient’s Guide to Common Neurological Problems by Robert Wiedemeyer; Paperback: (January 1996), Boxweed Pub; ISBN: 0964740796;

Books 87

http://www.amazon.com/exec/obidos/ASIN/0964740796/icongroupinter na ·

Medical Emergencies & Childhood Illnesses: Includes Your Child’s Personal Health Journal (Parent Smart) by Penny A. Shore, William Sears (Contributor); Paperback - 115 pages (February 2002), Parent Kit Corporation; ISBN: 1896833187; http://www.amazon.com/exec/obidos/ASIN/1896833187/icongroupinter na

·

Neurology for the Non-Neurologist by William J. Weiner (Editor), Christopher G. Goetz (Editor); Paperback (May 1999), Lippincott, Williams & Wilkins Publishers; ISBN: 0781717078; http://www.amazon.com/exec/obidos/ASIN/0781717078/icongroupinter na

·

Taking Care of Your Child: A Parent’s Guide to Complete Medical Care by Robert H. Pantell, M.D., et al; Paperback - 524 pages, 6th edition (March 5, 2002), Perseus Press; ISBN: 0738206016; http://www.amazon.com/exec/obidos/ASIN/0738206016/icongroupinter na

Vocabulary Builder Abscess: A localized collection of pus caused by suppuration buried in tissues, organs, or confined spaces. [EU] Ataxia: Failure of muscular coordination; irregularity of muscular action. [EU] Barotrauma: Injury following pressure changes; includes injury to the eustachian tube, ear drum, lung and stomach. [NIH] Benign: Not malignant; not recurrent; favourable for recovery. [EU] Chromosomal: Pertaining to chromosomes. [EU] Dislocation: The displacement of any part, more especially of a bone. Called also luxation. [EU] Endocrinology: A subspecialty of internal medicine concerned with the metabolism, physiology, and disorders of the endocrine system. [NIH] Epidural: Situated upon or outside the dura mater. [EU] Fistula: An abnormal passage or communication, usually between two internal organs, or leading from an internal organ to the surface of the body; frequently designated according to the organs or parts with which it communicates, as anovaginal, brochocutaneous, hepatopleural, pulmonoperitoneal, rectovaginal, urethrovaginal, and the like. Such passages

88 Chiari Malformation

are frequently created experimentally for the purpose of obtaining body secretions for physiologic study. [EU] Herpes: Any inflammatory skin disease caused by a herpesvirus and characterized by the formation of clusters of small vesicles. When used alone, the term may refer to herpes simplex or to herpes zoster. [EU] Labyrinthitis: Inflammation of the inner ear. [NIH] Malignant: Tending to become progressively worse and to result in death. Having the properties of anaplasia, invasion, and metastasis; said of tumours. [EU] Medullary: Pertaining to the marrow or to any medulla; resembling marrow. [EU] Morphogenesis: The development of the form of an organ, part of the body, or organism. [NIH] Neuroanatomy: Study of the anatomy of the nervous system as a specialty or discipline. [NIH] Otitis: Inflammation of the ear, which may be marked by pain, fever, abnormalities of hearing, hearing loss, tinnitus, and vertigo. [EU] Otosclerosis: A pathological condition of the bony labyrinth of the ear, in which there is formation of spongy bone (otospongiosis), especially in front of and posterior to the footplate of the stapes; it may cause bony ankylosis of the stapes, resulting in conductive hearing loss. Cochlear otosclerosis may also develop, resulting in sensorineural hearing loss. [EU] Presbycusis: Progressive bilateral loss of hearing that occurs in the aged. Syn: senile deafness. [NIH] Tinnitus: A noise in the ears, as ringing, buzzing, roaring, clicking, etc. Such sounds may at times be heard by others than the patient. [EU] Vertigo: An illusion of movement; a sensation as if the external world were revolving around the patient (objective vertigo) or as if he himself were revolving in space (subjective vertigo). The term is sometimes erroneously used to mean any form of dizziness. [EU] Viral: Pertaining to, caused by, or of the nature of virus. [EU]

89

CHAPTER 6. MULTIMEDIA ON CHIARI MALFORMATION Overview Information on Chiari malformation can come in a variety of formats. Among multimedia sources, video productions, slides, audiotapes, and computer databases are often available. In this chapter, we show you how to keep current on multimedia sources of information on Chiari malformation. 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. If you see an interesting item, visit your local medical library to check on the availability of the title.

Bibliography: Multimedia on Chiari Malformation 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 Chiari malformation (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 Chiari malformation. For more information, follow the hyperlink indicated: ·

Abnormal skull. Source: Department of Radiology, Temple University Hospital and School of Medicine; Year: 1965; Format: Slide; Philadelphia, Pa.: The Dept.; [Chicago: for sale by Micro X-Ray Recorder, inc, Medical Film Slide Division, 1965?].

90 Chiari Malformation

·

·

·

Anatomical correction of tetralogy of Fallot : by closure of ventricular septal defect and resection of infundibular pulmonary stenosis: utilizing the pump oxygenator. Source: [presented by] the University of Minnesota; Year: 1957; Format: Motion picture; [United States]: The University, 1957. Brain disorders. Source: a presentation of Films for the Humanities & Sciences; Year: 2000; Format: Videorecording; Princeton, N.J.: Films for the Humanities and Sciences, c2000. Congenital and acquired middle ear deformities. Source: American Academy of Ophthalmology and Otolaryngology; [produced by] the University of Texas Medical Branch, UTMBTV, Galveston; Year: 1976; Format: Videorecording; Rochester, Minn.: The Academy, c1976.

·

Congenital dislocation of the hip : innominate acetabuloplasty, iliac shelf and Chiari osteotomy. Source: American Academy of Orthopaedic Surgeons; Year: 1979; Format: Slide; [Chicago, Ill.]: The Academy, [197-].

·

Congenital dislocation of the hip : surgical treatment in the younger child. Year: 1979; Format: Videorecording; [Chicago, Ill.]: American Academy of Orthopaedic Surgeons, [1979].

·

Congenital malformations : external canal, middle ear, and inner ear. Year: 1987; Format: Videorecording; [Los Angeles, Calif.]: House Ear Institute, c1987. Congenital malformations of the heart. Source: [presented by] the University of Washington, School of Medicine; Year: 1950; Format: Motion picture; United States: The University, [1950]. Cryptic vascular malformation in the pediatric population. Source: the University of Texas Medical School at Houston; produced by UT-TV, Houston; Year: 1992; Format: Videorecording; [Houston, Tex.: UT/TV], c1992.

·

·

·

Ectopia cordis : (ectopic heart). Source: [produced by Eastman Teaching Films, Inc.]; Year: 1929; Format: Motion picture; [United States]: Eastman Teaching Films, c1929.

·

Embryology of the cloaca and its malformations . Year: 1988; Format: Videorecording; [United States?]: D. Kluth, W. Lambrecht, P. Reich, c1988. Encephalography in infants and children. Source: Harvey White and Sanat Bhagwati; Year: 1969; Format: Slide; Chicago: White: [for sale by Micro X-Ray Recorder, inc., Medical Film Slide Division, 1969?]. Gynecologic endocrinopathy. Source: Abraham E. Rakoff, and Misao Takeda; Year: 1974; Format: Slide; Chicago: Tutorials of Cytology, c1974.

·

· ·

Interventional thoracic radiology : vascular procedures. Source: Radiological Society of North America; Year: 1990; Format: Videorecording; Oak Brook, Ill.: The Society, c1990.

Multimedia 91

·

·

·

·

·

·

·

Laparoscopy in gynecology : trans-abdominal laparoscopies. Source: by Michel Cognat; Year: 1973; Format: Slide; Chicago: Micro X-ray Recorder: [for sale by its Medical Film Slide Division, 1973?]. Leg straightening surgery. Source: produced by Advanced Medical Education, Inc. for the Learning Channel; Year: 1993; Format: Videorecording; Princeton, N.J.: Films for the Humanities and Sciences, c1993. Lymphatic malformation in children. Source: produced and distributed by Health Video Dynamics; Year: 1991; Format: Videorecording; Washington, D.C.: Health Video Dynamics, c1991. Meso-atrial shunt for Budd-Chiari syndrome. Source: American Academy of Surgeons; produced by the University of Arkansas for Medical Sciences, Department of Campus Media Services, Instructional Television Unit; Year: 1991; Format: Videorecording; [Chicago, Ill.]: The College, c1991. Motor evaluation and treatment of a patient with rupture of A-V malformation. Source: George K. Makechnie Instructional Resource Center, Sargent College, Boston University; Year: 1985; Format: Videorecording; Boston, Mass.: The Center, 1985. Numerical abnormalities of human chromosomes. Source: [presented by] the U.S. Department of Health, Education, and Welfare, Public Health Service; a National Medical Audiovisual Center production; Year: 1970; Format: Motion picture; [United States]: The Center, 1970. Osteotomies around the hip for hip dysplasia. Source: [presented by] the American Orthopaedic Association, in conjunction with the Alfred I. DuPont Institute; Year: 1982; Format: Videorecording; [Chicago, Ill.]: American Academy of Orthopaedic Surgeons, [1982].

·

Percutaneous non-catheter brachial angiography : left panarteriography, right cerebral angiography. Source: T. R. Marshall and J. T. Ling; Year: 1969; Format: Slide; Chicago: Micro X-ray Recorder, inc.: [for sale by Micro X-ray Recorder, inc., Medical Film Slide Division, 1969?].

·

Radiologic diagnosis of congenital malformation syndromes. Source: Andrew K. Poznanski; Year: 1974; Format: Slide; New York: Medcom, c1974.

·

Radiological electronic atlas of malformation syndromes and skeletal dysplasias. Source: [Christine M. Hall and John Washbrook]; Year: 2000; Format: Electronic resource; Oxford; New York: Oxford University Press, [2000]. Repair of a complex cloacal malformation. Source: American College of Surgeons; Year: 1992; Format: Videorecording; [Woodbury, Conn.?: CinéMed?, 1992?].

·

92 Chiari Malformation

·

Spinal cord arteriovenous malformation. Source: by John L. Fox; Year: 1982; Format: Videorecording; [Morgantown, W. Va.]: Full Spectrum Video, c1982.

·

Surgical treatment of Epstein's malformation by prosthetic valve replacement. Source: University of Minnesota Medical Center and Variety Club Heart Hospital; Year: 1966; Format: Motion picture; Minneapolis: The University: [for loan by its Audio Visual Library Service], 1966.

·

Treatment of subacute Budd-Chiari syndrome with partial porto-caval shunt and intraoperative acute normovolemic hemodilution. Source: American College of Surgeons; from the Film Library and the Clinical Congress of ACS; Foundation for Liver Tr; Year: 1998; Format: Videorecording; [Woodbury, Conn.]: Ciné-Med, distributor, [1998]. Ultrasound of the abnormal fetus. Source: the Radiological Society of North America; Year: 1988; Format: Videorecording; Oak Brook, Ill.: The Society, c1988.

·

Vocabulary Builder Arteriography: Roentgenography of arteries after injection of radiopacque material into the blood stream. [EU] Arteriovenous: Both arterial and venous; pertaining to or affecting an artery and a vein. [EU] Cloaca: 1. in zoology, a common passage for faecal, urinary, and reproductive discharge in most lower vertebrates. 2. in mammalian embryology, the terminal end of the hindgut before division into rectum, bladder, and genital primordia. 3. in pathology, an opening in the involucrum of a necrosed bone. [EU] Ectopic: Pertaining to or characterized by ectopia. [EU] Gynecology: A medical-surgical specialty concerned with the physiology and disorders primarily of the female genital tract, as well as female endocrinology and reproductive physiology. [NIH] Hemodilution: Reduction of blood viscosity usually by the addition of cell free solutions. Used clinically l) in states of impaired microcirculation, 2) for replacement of intraoperative blood loss without homologous blood transfusion, and 3) in cardiopulmonary bypass and hypothermia. [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]

Multimedia 93

Orthopaedic: Pertaining to the correction of deformities of the musculoskeletal system; pertaining to orthopaedics. [EU] Osteotomy: The surgical cutting of a bone. [EU] Pulmonary: Pertaining to the lungs. [EU] 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] Subacute: Somewhat acute; between acute and chronic. [EU]

95

CHAPTER 7. PERIODICALS AND NEWS ON CHIARI MALFORMATION Overview Keeping up on the news relating to Chiari malformation can be challenging. Subscribing to targeted periodicals can be an effective way to stay abreast of recent developments on Chiari malformation. Periodicals include newsletters, magazines, and academic journals. In this chapter, we suggest a number of news sources and present various periodicals that cover Chiari malformation beyond and including those which are published by parent associations mentioned earlier. We will first focus on news services, and then on periodicals. News services, press releases, and newsletters generally use more accessible language, so if you do chose to subscribe to one of the more technical periodicals, make sure that it uses language you can easily follow.

News Services and Press Releases Well before articles show up in newsletters or the popular press, they may appear in the form of a press release or a public relations announcement. One of the simplest ways of tracking press releases on Chiari malformation is to search the news wires. News wires are used by professional journalists, and have existed since the invention of the telegraph. Today, there are several major “wires” that are used by companies, universities, and other organizations to announce new medical breakthroughs. 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.

96 Chiari Malformation

PR Newswire Perhaps the broadest of the wires is PR Newswire Association, Inc. To access this archive, simply go to http://www.prnewswire.com. Below the search box, select the option “The last 30 days.” In the search box, type “Chiari malformation” or synonyms. The search results are shown by order of relevance. When reading these press releases, do not forget that the sponsor of the release may be a company or organization that is trying to sell a particular product or therapy. Their views, therefore, may be biased.

Reuters Health The Reuters' Medical News and Health eLine databases can be very useful in exploring news archives relating to diseasex. While some of the listed articles are free to view, others can be purchased for a nominal fee. To access this archive, go to http://www.reutershealth.com/en/index.html and search by “diseasex” (or synonyms).

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 their 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.

Periodicals and News 97

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, log on to Market Wire’s Medical/Health channel at the following hyperlink http://www.marketwire.com/mw/release_index?channel=MedicalHealth. Market Wire’s home page is http://www.marketwire.com/mw/home. From here, type “diseasex” (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 Free-to-view news can also be found in the news section of your favorite search engines (see the health news page at Yahoo: http://dir.yahoo.com/Health/News_and_Media/, or use this Web site’s general news search page http://news.yahoo.com/. Type in “Chiari malformation” (or synonyms). If you know the name of a company that is relevant to Chiari malformation, you can go to any stock trading Web site (such as www.etrade.com) and search for the company name there. News items across various news sources are reported on indicated hyperlinks.

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 “Chiari malformation” (or synonyms).

Newsletter Articles If you choose not to subscribe to a newsletter, you can nevertheless find references to newsletter articles. We recommend that you use the Combined Health Information Database, while limiting your search criteria to “newsletter articles.” Again, you will need to use the “Detailed Search” option at 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.”

98 Chiari Malformation

By making these selections, and typing in “Chiari malformation” (or synonyms) into the “For these words:” box, you will only receive results on newsletter articles. You should check back periodically with this database as it is updated every 3 months. The following is a typical result when searching for newsletter articles on Chiari malformation: ·

Best of Fibromyalgia Frontiers 1999-2000, The Source: Linden, VA: National Fibromyalgia Partnership, Inc. (NFP). 2002. 190 p. Contact: Available from National Fibromyalgia Partnership, Inc. P.O. Box 160, Linden, VA 22642-0160. (866) 725-4404 toll-free. Fax (540) 622-2998. E-mail: [email protected]. Website: www.fmpartnership.org. Summary: This monograph, a compilation of articles on fibromyalgia syndrome (FMS), provides health professionals and people who have FMS with information on this chronic musculoskeletal syndrome. Part one focuses on fibromyalgia and related conditions. Articles provide an overview of FMS; offer new insights into the disease; and describe dysregulation spectrum syndrome, myofascial pain syndrome, chronic fatigue syndrome, carpal tunnel syndrome, restless legs syndrome, headaches, temporomandibular joint (TMJ) disorders, mycoplasma, cervical stenosis, Chiari malformation, vulvodynia, interstitial cystitis, and irritable bowel syndrome. Part two deals with managing fibromyalgia. Articles discuss a comprehensive care approach to the treatment of fibromyalgia, mediation management, fitness, thoracic pain and dysfunction, FMS self care, breathing techniques, management via water fitness, Watsu, electroencephalogram driven stimulation, and shoe selection by patients with FMS. Other articles offer lifestyle hints for FMS managers, present a central nervous system paradigm for fibromyalgia, and list questions people should ask about TMJ. The remaining articles focus on body structure and function, fibromyalgia and memory, chiropractic therapy, alternative medicine, photonic stimulation, infrared technology, magnet therapy, and cognitive behavioral therapy. Part three answers questions about medication and treatment issues. Part four focuses on coping with fibromyalgia. Topics include learning to live with fibromyalgia, being positive, making choices, dealing with FMS and denial, coping with guilt and fear, adjusting to life with FMS, attaining a sense of well being, developing better interpersonal relationships, being a parent with FMS, and caring for a child who has FMS. Part five addresses work and disability issues. Articles answer questions about the Americans with Disabilities Act; explain how to find a new job, undergo retraining, or start a home based business; identify emergency services

Periodicals and News 99

providers; and discuss disability benefits and insurance policies. 20 figures, 7 tables, and numerous references. ·

Headaches in Ehlers-Danlos Syndrome Source: Loose Connections. XV(3): 1,4-8. September-October 2000. Contact: Available from Ehlers-Danlos National Foundation. 6399 Wilshire Blvd., Suite 510, Los Angeles, CA 90048. (323) 651-3038. Summary: This newsletter article provides health professionals and people who have Ehlers-Danlos syndrome (EDS) with information on a study that investigated the occurrence of chronic headaches in this complex hereditary connective tissue disorder. Data were obtained from 18 patients with EDS and chronic headaches. All of the patients were seen in a rural practice setting and were followed for a minimum of 2 years. Procedures included clinical history taking, neurologic examination, computerized tomography of the head, magnetic resonance imaging of the brain, and electroencephalogram (EEG). Headaches were classified according to the International Headache Society. The study found that four patients had migraine with aura, four had migraine without aura, four had tension headaches, four had a combination of migraine and tension headaches, and two had posttraumatic headaches. Nine patients exhibited blepharoclonus, but none had a history of seizures and their EEGs were normal, ruling out eye closure epilepsy. Although one patient had a small right frontal angioma, a second had Arnold Chiari malformation type I, and a third had an old stroke, headaches did not clinically correlate with their central nervous system (CNS) lesions. The article concludes that chronic recurrent headaches may constitute the neurologic presentation of EDS in the absence of structural, congenital, or acquired CNS lesions that correlate with their symptoms. People who have EDS may be prone to migraine due to an inherent disorder of cerebrovascular reactivity or cortical excitability. Additional studies are needed to elucidate the pathogenesis of headaches in EDS. 1 table and 5 references. (AA-M).

Academic Periodicals covering Chiari Malformation Academic periodicals can be a highly technical yet valuable source of information on Chiari malformation. We have compiled the following list of periodicals known to publish articles relating to Chiari malformation and which are currently indexed within the National Library of Medicine’s PubMed database (follow hyperlinks to view more information, summaries,

100 Chiari Malformation

etc., for each). In addition to these sources, to keep current on articles written on Chiari malformation published by any of the periodicals listed below, you can simply follow the hyperlink indicated or go to the following Web site: www.ncbi.nlm.nih.gov/pubmed. Type the periodical’s name into the search box to find the latest studies published. If you want complete details about the historical contents of a periodical, visit http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi. Here, type in the 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.” The following is a sample of periodicals which publish articles on Chiari malformation: ·

Acta Neurochirurgica. (Acta Neurochir (Wien)) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Ac ta+Neurochirurgica&dispmax=20&dispstart=0

·

Acta Neurologica Scandinavica. (Acta Neurol Scand) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Ac ta+Neurologica+Scandinavica&dispmax=20&dispstart=0

·

Arthritis and Rheumatism. (Arthritis Rheum) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Ar thritis+and+Rheumatism&dispmax=20&dispstart=0

·

Intensive Care Medicine. (Intensive Care Med) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Int ensive+Care+Medicine&dispmax=20&dispstart=0

·

Journal of Neurology. (J Neurol) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Jo urnal+of+Neurology&dispmax=20&dispstart=0

·

Journal of Neurosurgery. (J Neurosurg) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Jo urnal+of+Neurosurgery&dispmax=20&dispstart=0

Periodicals and News 101

·

Magnetic Resonance Imaging. (Magn Reson Imaging) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=M agnetic+Resonance+Imaging&dispmax=20&dispstart=0

·

Pediatric Neurosurgery. (Pediatr Neurosurg) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Pe diatric+Neurosurgery&dispmax=20&dispstart=0

·

Pediatric Pulmonology. (Pediatr Pulmonol) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Pe diatric+Pulmonology&dispmax=20&dispstart=0

·

Plastic and Reconstructive Surgery. (Plast Reconstr Surg) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Pl astic+and+Reconstructive+Surgery&dispmax=20&dispstart=0

·

The Journal of Laryngology and Otology. (J Laryngol Otol) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Th e+Journal+of+Laryngology+and+Otology&dispmax=20&dispstart=0

·

The Journal of Rheumatology. (J Rheumatol) http://www.ncbi.nlm.nih.gov/entrez/jrbrowser.cgi?field=0®exp=Th e+Journal+of+Rheumatology&dispmax=20&dispstart=0

Vocabulary Builder Anemia: A reduction in the number of circulating erythrocytes or in the quantity of hemoglobin. [NIH] Aura: A subjective sensation or motor phenomenon that precedes and marks the of a paroxysmal attack, such as an epileptic attack on set. [EU] 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] Cortical: Pertaining to or of the nature of a cortex or bark. [EU] Cystitis: Inflammation of the urinary bladder. [EU] 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

102 Chiari Malformation

an opportunistic infection in AIDS. [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] Hematology: A subspecialty of internal medicine concerned with morphology, physiology, and pathology of the blood and blood-forming tissues. [NIH] Humoral: Of, relating to, proceeding from, or involving a bodily humour now often used of endocrine factors as opposed to neural or somatic. [EU] Interstitial: Pertaining to or situated between parts or in the interspaces of a tissue. [EU] Mycoplasma: A genus of gram-negative, facultatively anaerobic bacteria bounded by a plasma membrane only. Its organisms are parasites and pathogens, found on the mucous membranes of humans, animals, and birds. [NIH]

Particle: A tiny mass of material. [EU] Parvovirus: A genus of the family parvoviridae, subfamily parvovirinae, infecting a variety of vertebrates including humans. Parvoviruses are responsible for a number of important diseases but also can be nonpathogenic in certain hosts. The type species is mice minute virus. [NIH] 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] Proteins: Polymers of amino acids linked by peptide bonds. The specific sequence of amino acids determines the shape and function of the protein. [NIH]

Reactivation: The restoration of activity to something that has been inactivated. [EU] Transfusion: The introduction of whole blood or blood component directly into the blood stream. [EU] Viruses: Minute infectious agents whose genomes are composed of DNA or RNA, but not both. They are characterized by a lack of independent metabolism and the inability to replicate outside living host cells. [NIH] Vitex: A genus of trees in the LAMIACEAE family containing assorted flavonoids with possible analgesic and antineoplastic properties. The fruit of these trees is used in herbal preparations. [NIH]

103

CHAPTER 8. PHYSICIAN GUIDELINES AND DATABASES Overview Doctors and medical researchers rely on a number of information sources to help children with Chiari malformation. Many will subscribe to journals or newsletters published by their professional associations or refer to specialized textbooks or clinical guides published for the medical profession. In this chapter, we focus on databases and Internet-based guidelines created or written for this professional audience.

NIH Guidelines For the more common medical conditions, the National Institutes of Health publish guidelines that are frequently consulted by physicians. Publications are typically written by one or more of the various NIH Institutes. For physician guidelines, commonly referred to as “clinical” or “professional” guidelines, you can visit the following Institutes: ·

Office of the Director (OD); guidelines consolidated across agencies available at http://www.nih.gov/health/consumer/conkey.htm

·

National Institute of General Medical Sciences (NIGMS); fact sheets available at http://www.nigms.nih.gov/news/facts/

·

National Library of Medicine (NLM); extensive encyclopedia (A.D.A.M., Inc.) with guidelines: http://www.nlm.nih.gov/medlineplus/healthtopics.html

·

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

104 Chiari Malformation

NIH Databases In addition to the various Institutes of Health that publish professional guidelines, the NIH has designed a number of databases for professionals.20 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:21 ·

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

·

HIV/AIDS Resources: Describes various links and databases dedicated to HIV/AIDS research: http://www.nlm.nih.gov/pubs/factsheets/aidsinfs.html

·

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

·

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/

·

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

·

Cancer Information: Access to caner-oriented databases: http://www.nlm.nih.gov/databases/databases_cancer.html

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). 21 See http://www.nlm.nih.gov/databases/databases.html. 20

Physician Guidelines and Databases 105

·

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/

·

Chemical Information: Provides links to various chemical databases and references: http://sis.nlm.nih.gov/Chem/ChemMain.html

·

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

·

Space Life Sciences: Provides links and information to space-based research (including NASA): http://www.nlm.nih.gov/databases/databases_space.html

·

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

·

Toxicology and Environmental Health Information (TOXNET): Databases covering toxicology and environmental health: http://sis.nlm.nih.gov/Tox/ToxMain.html

·

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

While all of the above references may be of interest to physicians who study and treat Chiari malformation, the following are particularly noteworthy.

The NLM Gateway22 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.23 One target audience for the Gateway is the Internet user who is new to NLM’s online resources and does not know what information is available or how best to search for it. This audience may include physicians and other healthcare providers, Adapted from NLM: http://gateway.nlm.nih.gov/gw/Cmd?Overview.x. 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).

22 23

106 Chiari Malformation

researchers, librarians, students, and, increasingly, parents and the public.24 To use the NLM Gateway, simply go to the search site at http://gateway.nlm.nih.gov/gw/Cmd. Type “Chiari malformation” (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 Items Found Journal Articles 353520 Books / Periodicals / Audio Visual 2590 Consumer Health 294 Meeting Abstracts 2575 Other Collections 87 Total 359066

HSTAT25 HSTAT is a free, Web-based resource that provides access to full-text documents used in healthcare decision-making.26 HSTAT’s audience includes healthcare providers, health service researchers, policy makers, insurance companies, consumers, and the information professionals who serve these groups. HSTAT provides access to a wide variety of publications, including clinical practice guidelines, quick-reference 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.27 Simply search by “Chiari malformation” (or synonyms) at the following Web site: http://text.nlm.nih.gov. Other users may find the Gateway useful for an overall search of NLM’s information resources. Some searchers may locate what they need immediately, while others will utilize the Gateway as an adjunct tool to other NLM search services such as PubMed® and MEDLINEplus®. The Gateway connects users with multiple NLM retrieval systems while also providing a search interface for its own collections. These collections include various types of information that do not logically belong in PubMed, LOCATORplus, or other established NLM retrieval systems (e.g., meeting announcements and pre-1966 journal citations). The Gateway will provide access to the information found in an increasing number of NLM retrieval systems in several phases. 25 Adapted from HSTAT: http://www.nlm.nih.gov/pubs/factsheets/hstat.html. 26 The HSTAT URL is http://hstat.nlm.nih.gov/. 27 Other important documents in HSTAT include: the National Institutes of Health (NIH) Consensus Conference Reports and Technology Assessment Reports; the HIV/AIDS 24

Physician Guidelines and Databases 107

Coffee Break: Tutorials for Biologists28 Some parents may wish to have access to 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. To this end, we recommend “Coffee Break,” a collection of short reports on recent biological discoveries. Each report incorporates interactive tutorials that demonstrate how bioinformatics tools are used as a part of the research process. Currently, all Coffee Breaks are written by NCBI staff.29 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.30 This site has new articles every few weeks, so it can be considered an online magazine of sorts, and intended for general background information. You can access Coffee Break at 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/.

·

Medical World Search: Searches full text from thousands of selected medical sites on the Internet; see http://www.mwsearch.com/.

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. 28 Adapted from http://www.ncbi.nlm.nih.gov/Coffeebreak/Archive/FAQ.html. 29 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. 30 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.

108 Chiari Malformation

The Genome Project and Chiari Malformation With all the discussion in the press about the Human Genome Project, it is only natural that physicians, researchers, and parents want to know about how human genes relate to Chiari malformation. In the following section, we will discuss databases and references used by physicians and scientists who work in this area.

Online Mendelian Inheritance in Man (OMIM) The Online Mendelian Inheritance in Man (OMIM) database is a catalog of human genes and genetic disorders authored and edited by Dr. Victor A. McKusick and his colleagues at Johns Hopkins and elsewhere. OMIM was developed for the World Wide Web by the National Center for Biotechnology Information (NCBI).31 The database contains textual information, pictures, and reference information. It also contains copious links to NCBI’s Entrez database of MEDLINE articles and sequence information. Go to http://www.ncbi.nlm.nih.gov/Omim/searchomim.html to search the database. Type “Chiari malformation” (or synonyms) in the search box, and click “Submit Search.” If too many results appear, you can narrow the search by adding the word “clinical.” Each report will have additional links to related research and databases. By following these links, especially the link titled “Database Links,” you will be exposed to numerous specialized databases that are largely used by the scientific community. These databases are overly technical and seldom used by the general public, but offer an abundance of information. The following is an example of the results you can obtain from the OMIM for Chiari malformation: ·

Arnold-Chiari Malformation Web site: http://www.ncbi.nlm.nih.gov/htbinpost/Omim/dispmim?207950

Adapted from http://www.ncbi.nlm.nih.gov/. Established in 1988 as a national resource for molecular biology information, NCBI creates public databases, conducts research in computational biology, develops software tools for analyzing genome data, and disseminates biomedical information--all for the better understanding of molecular processes affecting human health and disease.

31

Physician Guidelines and Databases 109

·

Chiari Malformation Type I Web site: http://www.ncbi.nlm.nih.gov/htbinpost/Omim/dispmim?118420

Genes and Disease (NCBI - Map) The Genes and Disease database is produced by the National Center for Biotechnology Information of the National Library of Medicine at the National Institutes of Health. This Web site categorizes each disorder by the system of the body. Go to http://www.ncbi.nlm.nih.gov/disease/, and browse the system pages to have a full view of important conditions linked to human genes. Since this site is regularly updated, you may wish to re-visit it from time to time. The following systems and associated disorders are addressed: ·

Muscle and Bone: Movement and growth. Examples: Duchenne muscular dystrophy, Ellis-van Creveld syndrome, Marfan syndrome, myotonic dystrophy, spinal muscular atrophy. Web site: http://www.ncbi.nlm.nih.gov/disease/Muscle.html

·

Nervous System: Mind and body. Examples: Alzheimer disease, Amyotrophic lateral sclerosis, Angelman syndrome, Charcot-Marie-Tooth disease, epilepsy, essential tremor, Fragile X syndrome, Friedreich’s ataxia, Huntington disease, NiemannPick disease, Parkinson disease, Prader-Willi syndrome, Rett syndrome, Spinocerebellar atrophy, Williams syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Brain.html

·

Signals: Cellular messages. Examples: Ataxia telangiectasia, Baldness, Cockayne syndrome, Glaucoma, SRY: sex determination, Tuberous sclerosis, Waardenburg syndrome, Werner syndrome. Web site: http://www.ncbi.nlm.nih.gov/disease/Signals.html

110 Chiari Malformation

Entrez Entrez is a search and retrieval system that integrates several linked databases at the National Center for Biotechnology Information (NCBI). These databases include nucleotide sequences, protein sequences, macromolecular structures, whole genomes, and MEDLINE through PubMed. Entrez provides access to the following databases: ·

3D Domains: Domains from Entrez Structure, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=geo

·

Books: Online books, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=books

·

Genome: Complete genome assemblies, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Genome

·

NCBI’s Protein Sequence Information Survey Results: Web site: http://www.ncbi.nlm.nih.gov/About/proteinsurvey/

·

Nucleotide Sequence Database (Genbank): Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Nucleotide

·

OMIM: Online Mendelian Inheritance in Man, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM

·

PopSet: Population study data sets, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Popset

·

ProbeSet: Gene Expression Omnibus (GEO), Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=geo

·

Protein Sequence Database: Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Protein

·

PubMed: Biomedical literature (PubMed), Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed

·

Structure: Three-dimensional macromolecular structures, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Structure

·

Taxonomy: Organisms in GenBank, Web site: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=Taxonomy

Access the Entrez system of the NCBI at the following hyperlink: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=genom e, and then select the database that you would like to search. The databases

Physician Guidelines and Databases 111

available are listed in the drop box next to “Search.” In the box next to “for,” enter “Chiari malformation” (or synonyms) and click “Go.”

Jablonski’s Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes Database32 This online resource can be quite useful. It has been developed to facilitate the identification and differentiation of syndromic entities. Special attention is given to the type of information that is usually limited or completely omitted in existing reference sources due to space limitations of the printed form. You can search across syndromes using an alphabetical index at http://www.nlm.nih.gov/mesh/jablonski/syndrome_toc/toc_a.html. At http://www.nlm.nih.gov/mesh/jablonski/syndrome_db.html, search by keyword. The Genome Database33 Established at Johns Hopkins University in Baltimore, Maryland in 1990, the Genome Database (GDB) is the official central repository for genomic mapping data resulting from the Human Genome Initiative. In the spring of 1999, the Bioinformatics Supercomputing Centre (BiSC) at the Hospital for Sick Children in Toronto, Ontario assumed the management of GDB. The Human Genome Initiative is a worldwide research effort focusing on structural analysis of human DNA to determine the location and sequence of the estimated 100,000 human genes. In support of this project, GDB stores and curates data generated by researchers worldwide who are engaged in the mapping effort of the Human Genome Project (HGP). GDB’s mission is to provide scientists with an encyclopedia of the human genome which is continually revised and updated to reflect the current state of scientific knowledge. Although GDB has historically focused on gene mapping, its focus will broaden as the Genome Project moves from mapping to sequence, and finally, to functional analysis. To access the GDB, simply go to the following hyperlink: http://www.gdb.org/. Search “All Biological Data” by “Keyword.” Type Adapted from the National Library of Medicine: http://www.nlm.nih.gov/mesh/jablonski/about_syndrome.html. 33 Adapted from the Genome Database: http://gdbwww.gdb.org/gdb/aboutGDB.html#mission. 32

112 Chiari Malformation

“Chiari malformation” (or synonyms) into the search box, and review the results. If more than one word is used in the search box, then separate each one with the word “and” or “or” (using “or” might be useful when using synonyms). This database is extremely technical as it was created for specialists. The articles are the results which are the most accessible to nonprofessionals and often listed under the heading “Citations.” The contact names are also accessible to non-professionals.

Specialized References The following books are specialized references written for professionals interested in Chiari malformation (sorted alphabetically by title; hyperlinks provide rankings, information, and reviews at Amazon.com): ·

Atlas of Pediatric Physical Diagnosis by Basil J. Zitelli, Holly W. Davis (Editor); Hardcover, 3rd edition (March 1997), Mosby-Year Book; ISBN: 0815199309; http://www.amazon.com/exec/obidos/ASIN/0815199309/icongroupinter na

·

The 5-Minute Pediatric Consult by M. William Schwartz (Editor); Hardcover - 1050 pages, 2nd edition (January 15, 2000), Lippincott, Williams & Wilkins; ISBN: 0683307444; http://www.amazon.com/exec/obidos/ASIN/0683307444/icongroupinter na

·

The Behavioral Neurology of White Matter by Christopher M. Filley; Paperback - 279 pages; 1st edition (September 15, 2001), Oxford University Press; ISBN: 019513561X; http://www.amazon.com/exec/obidos/ASIN/019513561X/icongroupinter na

·

The Cerebellum and Its Disorders by Mario-Ubaldo Manto, Massimo Pandolfo; Hardcover - 1st edition (January 2002), Cambridge University Press; ISBN: 0521771560; http://www.amazon.com/exec/obidos/ASIN/0521771560/icongroupinter na

·

Clinical Neurology by David A. Greenberg, et al; Paperback - 390 pages; 5th edition (February 9, 2002), Appleton & Lange; ISBN: 0071375430; http://www.amazon.com/exec/obidos/ASIN/0071375430/icongroupinter na

·

Clinical Neurology for Psychiatrists by David M. Kaufman; Hardcover 670 pages, 5th edition (January 15, 2001), W. B. Saunders Co.; ISBN:

Physician Guidelines and Databases 113

0721689957; http://www.amazon.com/exec/obidos/ASIN/0721689957/icongroupinter na ·

Comprehensive Neurology by Roger N. Rosenberg (Editor), David E. Pleasure (Editor); 1280 pages, 2nd edition (April 1998), Wiley-Liss; ISBN: 0471169587; http://www.amazon.com/exec/obidos/ASIN/0471169587/icongroupinter na

·

Emergent and Urgent Neurology by William J. Weiner (Editor), Lisa M. Shulman (Editor); Hardcover - 571 pages; 2nd edition (January 15, 1999), Lippincott, Williams & Wilkins Publishers; ISBN: 0397518579; http://www.amazon.com/exec/obidos/ASIN/0397518579/icongroupinter na

·

Nelson Textbook of Pediatrics by Richard E. Behrman (Editor), et al; Hardcover - 2414 pages, 16th edition (January 15, 2000), W B Saunders Co; ISBN: 0721677673; http://www.amazon.com/exec/obidos/ASIN/0721677673/icongroupinter na

·

Neurology in Clinical Practice: Volume I: Principles of Diagnosis and Management, Volume II: The Neurological Disorders (2-Volume Set, Includes a 12-Month Subscription to the Online Edition) by W. G. Bradley, et al; Hardcover - 2413 pages, 3rd edition, Vol 1-2 (January 15, 2000), Butterworth-Heinemann; ISBN: 0750699736; http://www.amazon.com/exec/obidos/ASIN/0750699736/icongroupinter na

·

Neuroscience: Exploring the Brain by Mark F. Bear, et al; Hardcover 855 pages, 2nd edition (January 15, 2001), Lippincott, Williams & Wilkins Publishers; ISBN: 0683305964; http://www.amazon.com/exec/obidos/ASIN/0683305964/icongroupinter na

·

Office Practice of Neurology by Martain A. Samuels, Steven F. Feske; Hardcover, Churchill Livingstone; ISBN: 0443065578; http://www.amazon.com/exec/obidos/ASIN/0443065578/icongroupinter na

·

Patient-Based Approaches to Cognitive Neuroscience by Martha J. Farah (Editor), Todd E. Feinberg (Editor); Paperback - 425 pages (April 3, 2000), MIT Press; ISBN: 0262561239; http://www.amazon.com/exec/obidos/ASIN/0262561239/icongroupinter na

114 Chiari Malformation

·

Principles of Neural Science by Eric R. Kandel (Editor), et al; Hardcover - 1414 pages, 4th edition (January 5, 2000), McGraw-Hill Professional Publishing; ISBN: 0838577016; http://www.amazon.com/exec/obidos/ASIN/0838577016/icongroupinter na

·

Review Manual for Neurology in Clinical Practice by Karl E. Misulis, et al; Paperback, Butterworth-Heinemann Medical; ISBN: 0750671920; http://www.amazon.com/exec/obidos/ASIN/0750671920/icongroupinter na

Vocabulary Builder Molecular: Of, pertaining to, or composed of molecules : a very small mass of matter. [EU] Neural: 1. pertaining to a nerve or to the nerves. 2. situated in the region of the spinal axis, as the neutral arch. [EU]

115

PART III. APPENDICES

ABOUT PART III Part III is a collection of appendices on general medical topics relating to Chiari malformation and related conditions.

117

APPENDIX A. RESEARCHING ALTERNATIVE MEDICINE Overview Complementary and alternative medicine (CAM) is one of the most contentious aspects of modern medical practice. You may have heard of these treatments on the radio or on television. Maybe you have seen articles written about these treatments in magazines, newspapers, or books. Perhaps your child’s doctor or your friends have mentioned alternatives. In this chapter, we will begin by giving you a broad perspective on complementary and alternative therapies. Next, we will introduce you to official information sources on CAM relating to Chiari malformation. Finally, at the conclusion of this chapter, we will provide a list of readings on Chiari malformation from various authors. We will begin, however, with the National Center for Complementary and Alternative Medicine’s (NCCAM) overview of complementary and alternative medicine.

118 Chiari Malformation

What Is CAM?34 Complementary and alternative medicine (CAM) covers a broad range of healing philosophies, approaches, and therapies. Generally, it is defined as those treatments and healthcare practices which are not taught in medical schools, used in hospitals, or reimbursed by medical insurance companies. Many CAM therapies are termed “holistic,” which generally means that the healthcare practitioner considers the whole person, including physical, mental, emotional, and spiritual health. Some of these therapies are also known as “preventive,” which means that the practitioner educates and treats the person to prevent health problems from arising, rather than treating symptoms after problems have occurred. People use CAM treatments and therapies in a variety of ways. Therapies are used alone (often referred to as alternative), in combination with other alternative therapies, or in addition to conventional treatment (sometimes referred to as complementary). Complementary and alternative medicine, or “integrative medicine,” includes a broad range of healing philosophies, approaches, and therapies. Some approaches are consistent with physiological principles of Western medicine, while others constitute healing systems with non-Western origins. While some therapies are far outside the realm of accepted Western medical theory and practice, others are becoming established in mainstream medicine. Complementary and alternative therapies are used in an effort to prevent illness, reduce stress, prevent or reduce side effects and symptoms, or control or cure disease. Some commonly used methods of complementary or alternative therapy include mind/body control interventions such as visualization and relaxation, manual healing including acupressure and massage, homeopathy, vitamins or herbal products, and acupuncture.

What Are the Domains of Alternative Medicine?35 The list of CAM practices changes continually. The reason being is that these new practices and therapies are often proved to be safe and effective, and therefore become generally accepted as “mainstream” healthcare practices. Today, CAM practices may be grouped within five major domains: (1) alternative medical systems, (2) mind-body interventions, (3) biologicallybased treatments, (4) manipulative and body-based methods, and (5) energy 34 35

Adapted from the NCCAM: http://nccam.nih.gov/nccam/fcp/faq/index.html#what-is. Adapted from the NCCAM: http://nccam.nih.gov/nccam/fcp/classify/index.html.

Researching Alternative Medicine 119

therapies. The individual systems and treatments comprising these categories are too numerous to list in this sourcebook. Thus, only limited examples are provided within each. Alternative Medical Systems Alternative medical systems involve complete systems of theory and practice that have evolved independent of, and often prior to, conventional biomedical approaches. Many are traditional systems of medicine that are practiced by individual cultures throughout the world, including a number of venerable Asian approaches. Traditional oriental medicine emphasizes the balance or disturbances of qi (pronounced chi) or vital energy in health and illness, respectively. Traditional oriental medicine consists of a group of techniques and methods including acupuncture, herbal medicine, oriental massage, and qi gong (a form of energy therapy). Acupuncture involves stimulating specific anatomic points in the body for therapeutic purposes, usually by puncturing the skin with a thin needle. Ayurveda is India’s traditional system of medicine. Ayurvedic medicine (meaning “science of life”) is a comprehensive system of medicine that places equal emphasis on body, mind, and spirit. Ayurveda strives to restore the innate harmony of the individual. Some of the primary Ayurvedic treatments include diet, exercise, meditation, herbs, massage, exposure to sunlight, and controlled breathing. Other traditional healing systems have been developed by the world’s indigenous populations. These populations include Native American, Aboriginal, African, Middle Eastern, Tibetan, and Central and South American cultures. Homeopathy and naturopathy are also examples of complete alternative medicine systems. Homeopathic medicine is an unconventional Western system that is based on the principle that “like cures like,” i.e., that the same substance that in large doses produces the symptoms of an illness, in very minute doses cures it. Homeopathic health practitioners believe that the more dilute the remedy, the greater its potency. Therefore, they use small doses of specially prepared plant extracts and minerals to stimulate the body’s defense mechanisms and healing processes in order to treat illness.

120 Chiari Malformation

Naturopathic medicine is based on the theory that a medical condition is the manifestation of alterations in the processes by which the body naturally heals itself and emphasizes health restoration rather than treatment for the condition itself. Naturopathic physicians employ an array of healing practices, including the following: diet and clinical nutrition, homeopathy, acupuncture, herbal medicine, hydrotherapy (the use of water in a range of temperatures and methods of applications), spinal and soft-tissue manipulation, physical therapies (such as those involving electrical currents, ultrasound, and light), therapeutic counseling, and pharmacology.

Mind-Body Interventions Mind-body interventions employ a variety of techniques designed to facilitate the mind’s capacity to affect bodily function and symptoms. Only a select group of mind-body interventions having well-documented theoretical foundations are considered CAM. For example, patient education and cognitive-behavioral approaches are now considered “mainstream.” On the other hand, complementary and alternative medicine includes meditation, certain uses of hypnosis, dance, music, and art therapy, as well as prayer and mental healing. Biological-Based Therapies This category of CAM includes natural and biological-based practices, interventions, and products, many of which overlap with conventional medicine’s use of dietary supplements. This category includes herbal, special dietary, orthomolecular, and individual biological therapies. Herbal therapy employs an individual herb or a mixture of herbs for healing purposes. An herb is a plant or plant part that produces and contains chemical substances that act upon the body. Special diet therapies, such as those proposed by Drs. Atkins, Ornish, Pritikin, and Weil, are believed to prevent and/or control illness as well as promote health. Orthomolecular therapies aim to treat medical conditions with varying concentrations of chemicals such as magnesium, melatonin, and mega-doses of vitamins. Biological therapies include, for example, the use of laetrile and shark cartilage to treat cancer and the use of bee pollen to treat autoimmune and inflammatory conditions.

Researching Alternative Medicine 121

Manipulative and Body-Based Methods This category includes methods that are based on manipulation and/or movement of the body. For example, chiropractors focus on the relationship between structure and function, primarily pertaining to the spine, and how that relationship affects the preservation and restoration of health. Chiropractors use manipulative therapy as an integral treatment tool. In contrast, osteopaths place particular emphasis on the musculoskeletal system and practice osteopathic manipulation. Osteopaths believe that all of the body’s systems work together and that disturbances in one system may have an impact upon function elsewhere in the body. Massage therapists manipulate the soft tissues of the body to normalize those tissues. Energy Therapies Energy therapies focus on energy fields originating within the body (biofields) or those from other sources (electromagnetic fields). Biofield therapies are intended to affect energy fields (the existence of which is not yet experimentally proven) that surround and penetrate the human body. Some forms of energy therapy manipulate biofields by applying pressure and/or manipulating the body by placing the hands in or through these fields. Examples include Qi gong, Reiki and Therapeutic Touch. Qi gong is a component of traditional oriental medicine that combines movement, meditation, and regulation of breathing to enhance the flow of vital energy (qi) in the body, improve blood circulation, and enhance immune function. Reiki, the Japanese word representing Universal Life Energy, is based on the belief that, by channeling spiritual energy through the practitioner, the spirit is healed and, in turn, heals the physical body. Therapeutic Touch is derived from the ancient technique of “laying-on of hands.” It is based on the premises that the therapist’s healing force affects recovery and that healing is promoted when the body’s energies are in balance. By passing their hands over the patient, these healers identify energy imbalances. Bioelectromagnetic-based therapies involve the unconventional use of electromagnetic fields to treat illnesses or manage pain. These therapies are often used to treat asthma, cancer, and migraine headaches. Types of electromagnetic fields which are manipulated in these therapies include pulsed fields, magnetic fields, and alternating current or direct current fields.

122 Chiari Malformation

Can Alternatives Affect My Child’s Treatment? A critical issue in pursuing complementary alternatives mentioned thus far is the risk that these might have undesirable interactions with your child’s medical treatment. It becomes all the more important to speak with the doctor who can offer advice on the use of alternatives. Official sources confirm this view. Though written for women, we find that the National Women’s Health Information Center’s advice on pursuing alternative medicine is appropriate for everyone.36 Is It Okay to Want Both Traditional and Alternative or Complementary Medicine? Should you wish to explore non-traditional types of treatment, be sure to discuss all issues concerning treatments and therapies with your child’s healthcare provider, whether a physician or practitioner of complementary and alternative medicine. Competent healthcare management requires that the practitioner know of all conventional and alternative therapies that your child is taking. The decision to use complementary and alternative treatments is an important one. Consider before selecting an alternative therapy, the safety and effectiveness of the therapy or treatment, the expertise and qualifications of the healthcare practitioner, and the quality of delivery. These topics should be considered when selecting any practitioner or therapy.

Finding CAM References on Chiari Malformation Having read the previous discussion, you may be wondering which complementary or alternative treatments might be appropriate for Chiari malformation. For the remainder of this chapter, we will direct you to a number of official sources which can assist you in researching studies and publications. Some of these articles are rather technical, so some patience may be required.

36

Adapted from http://www.4woman.gov/faq/alternative.htm.

Researching Alternative Medicine 123

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 allow parents to search for articles that specifically relate to Chiari malformation and complementary medicine. To search the database, go to the following Web site: www.nlm.nih.gov/nccam/camonpubmed.html. Select “CAM on PubMed.” Enter “Chiari malformation” (or synonyms) into the search box. Click “Go.” The following references provide information on particular aspects of complementary and alternative medicine (CAM) that are related to Chiari malformation: ·

Acute deterioration in Chiari type 1 malformation after chiropractic cervical manipulation. Author(s): Leong WK, Kermode AG. Source: Journal of Neurology, Neurosurgery, and Psychiatry. 2001 June; 70(6): 816-7. No Abstract Available. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=11430298&dopt=Abstract

·

Chiropractic adjustment to the cervical spine and the Arnold-Chiari malformation. Author(s): Murphy DR, Goldstein D, Katz M. Source: Journal of Manipulative and Physiological Therapeutics. 1993 October; 16(8): 550-5. http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db= PubMed&list_uids=8263435&dopt=Abstract

·

Klinefelter's syndrome, cerebral germinoma, Chiari malformation, and syrinx: a case report. Author(s): Ellis SJ, Crockard A, Barnard RO. Source: Neurosurgery. 1986 February; 18(2): 220-2.

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/

124 Chiari Malformation

·

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

·

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

·

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

·

TPN.com: http://www.tnp.com/

·

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

·

WebMDÒHealth: http://my.webmd.com/drugs_and_herbs

·

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

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: 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. The following additional references describe, in broad terms, alternative and complementary medicine (sorted alphabetically by title; hyperlinks provide rankings, information, and reviews at Amazon.com): ·

Alternative and Complementary Treatment in Neurologic Illness by Michael I. Weintraub (Editor); Paperback - 288 pages (March 23, 2001), Churchill Livingstone; ISBN: 0443065586; http://www.amazon.com/exec/obidos/ASIN/0443065586/icongroupinter na

·

Healthy Child, Whole Child: Integrating the Best of Conventional and Alternative Medicine to Keep Your Kids Healthy by Stuart H. Ditchek, M.D. and Russell H. Greenfield; Paperback - 464 pages (June 2002), Harper Resource; ISBN: 0062737465; http://www.amazon.com/exec/obidos/ASIN/0062737465/icongroupinter na

·

Radical Healing: Integrating the World’s Great Therapeutic Traditions to Create a New Transformative Medicine by Rudolph Ballentine, M.D., Linda Funk (Illustrator); Paperback - 612 pages; Reprint edition (March 14, 2000), Three Rivers Press; ISBN: 0609804847; http://www.amazon.com/exec/obidos/ASIN/0609804847/icongroupinter na

Researching Alternative Medicine 125

·

The Review of Natural Products by Facts and Comparisons (Editor); CdRom edition (January 2002), Facts & Comparisons; ISBN: 1574391453; http://www.amazon.com/exec/obidos/ASIN/1574391453/icongroupinter na

For additional information on complementary and alternative medicine, ask your child’s doctor or write to: National Center for Complementary and Alternative Medicine Clearinghouse National Institutes of He alth P. O. Box 8218 Silver Spring, MD 20907-8218

127

APPENDIX B. RESEARCHING NUTRITION Overview Since the time of Hippocrates, doctors have understood the importance of diet and nutrition to health and well-being. Since then, they have accumulated an impressive archive of studies and knowledge dedicated to this subject. Based on their experience, doctors and healthcare providers may recommend particular dietary supplements for Chiari malformation. Any dietary recommendation is based on age, body mass, gender, lifestyle, eating habits, food preferences, and health condition. It is therefore likely that different patients with Chiari malformation may be given different recommendations. Some recommendations may be directly related to Chiari malformation, while others may be more related to general health. In this chapter we will begin by briefly reviewing the essentials of diet and nutrition that will broadly frame more detailed discussions of Chiari malformation. We will then show you how to find studies dedicated specifically to nutrition and Chiari malformation.

Food and Nutrition: General Principles What Are Essential Foods? Food is generally viewed by official sources as consisting of six basic elements: (1) fluids, (2) carbohydrates, (3) protein, (4) fats, (5) vitamins, and (6) minerals. Consuming a combination of these elements is considered to be a healthy diet:

128 Chiari Malformation

·

Fluids are essential to human life as 80-percent of the body is composed of water. Water is lost via urination, sweating, diarrhea, vomiting, diuretics (drugs that increase urination), caffeine, and physical exertion.

·

Carbohydrates are the main source for human energy (thermoregulation) and the bulk of typical diets. They are mostly classified as being either simple or complex. Simple carbohydrates include sugars which are often consumed in the form of cookies, candies, or cakes. Complex carbohydrates consist of starches and dietary fibers. Starches are consumed in the form of pastas, breads, potatoes, rice, and other foods. Soluble fibers can be eaten in the form of certain vegetables, fruits, oats, and legumes. Insoluble fibers include brown rice, whole grains, certain fruits, wheat bran and legumes.

·

Proteins are eaten to build and repair human tissues. Some foods that are high in protein are also high in fat and calories. Food sources for protein include nuts, meat, fish, cheese, and other dairy products.

·

Fats are consumed for both energy and the absorption of certain vitamins. There are many types of fats, with many general publications recommending the intake of unsaturated fats or those low in cholesterol.

Vitamins and minerals are fundamental to human health, growth, and, in some cases, disease prevention. Most are consumed in your child’s diet (exceptions being vitamins K and D which are produced by intestinal bacteria and sunlight on the skin, respectively). Each vitamin and mineral plays a different role in health. The following outlines essential vitamins: ·

Vitamin A is important to the health of eyes, hair, bones, and skin; sources of vitamin A include foods such as eggs, carrots, and cantaloupe.

·

Vitamin B1, also known as thiamine, is important for the nervous system and energy production; food sources for thiamine include meat, peas, fortified cereals, bread, and whole grains.

·

Vitamin B2, also known as riboflavin, is important for the nervous system and muscles, but is also involved in the release of proteins from nutrients; food sources for riboflavin include dairy products, leafy vegetables, meat, and eggs.

·

Vitamin B3, also known as niacin, is important for healthy skin and helps the body use energy; food sources for niacin include peas, peanuts, fish, and whole grains

·

Vitamin B6, also known as pyridoxine, is important for the regulation of cells in the nervous system and is vital for blood formation; food sources for pyridoxine include bananas, whole grains, meat, and fish.

Researching Nutrition 129

·

Vitamin B12 is vital for a healthy nervous system and for the growth of red blood cells in bone marrow; food sources for vitamin B12 include yeast, milk, fish, eggs, and meat.

·

Vitamin C allows the body’s immune system to fight various medical conditions, strengthens body tissue, and improves the body’s use of iron; food sources for vitamin C include a wide variety of fruits and vegetables.

·

Vitamin D helps the body absorb calcium which strengthens bones and teeth; food sources for vitamin D include oily fish and dairy products.

·

Vitamin E can help protect certain organs and tissues from various degenerative diseases; food sources for vitamin E include margarine, vegetables, eggs, and fish.

·

Vitamin K is essential for bone formation and blood clotting; common food sources for vitamin K include leafy green vegetables.

·

Folic Acid maintains healthy cells and blood; food sources for folic acid include nuts, fortified breads, leafy green vegetables, and whole grains.

It should be noted that it is possible to overdose on certain vitamins which become toxic if consumed in excess (e.g. vitamin A, D, E and K). Like vitamins, minerals are chemicals that are required by the body to remain in good health. Because the human body does not manufacture these chemicals internally, we obtain them from food and other dietary sources. The more important minerals include: ·

Calcium is needed for healthy bones, teeth, and muscles, but also helps the nervous system function; food sources for calcium include dry beans, peas, eggs, and dairy products.

·

Chromium is helpful in regulating sugar levels in blood; food sources for chromium include egg yolks, raw sugar, cheese, nuts, beets, whole grains, and meat.

·

Fluoride is used by the body to help prevent tooth decay and to reinforce bone strength; sources of fluoride include drinking water and certain brands of toothpaste.

·

Iodine helps regulate the body’s use of energy by synthesizing into the hormone thyroxine; food sources include leafy green vegetables, nuts, egg yolks, and red meat.

·

Iron helps maintain muscles and the formation of red blood cells and certain proteins; food sources for iron include meat, dairy products, eggs, and leafy green vegetables.

130 Chiari Malformation

·

Magnesium is important for the production of DNA, as well as for healthy teeth, bones, muscles, and nerves; food sources for magnesium include dried fruit, dark green vegetables, nuts, and seafood.

·

Phosphorous is used by the body to work with calcium to form bones and teeth; food sources for phosphorous include eggs, meat, cereals, and dairy products.

·

Selenium primarily helps maintain normal heart and liver functions; food sources for selenium include wholegrain cereals, fish, meat, and dairy products.

·

Zinc helps wounds heal, the formation of sperm, and encourage rapid growth and energy; food sources include dried beans, shellfish, eggs, and nuts.

The United States government periodically publishes recommended diets and consumption levels of the various elements of food. Again, the doctor may encourage deviations from the average official recommendation based on your child’s specific condition. To learn more about basic dietary guidelines, visit the Web site: http://www.health.gov/dietaryguidelines/. Based on these guidelines, many foods are required to list the nutrition levels on the food’s packaging. Labeling Requirements are listed at the following site maintained by the Food and Drug Administration: http://www.cfsan.fda.gov/~dms/lab-cons.html. When interpreting these requirements, the government recommends that consumers become familiar with the following abbreviations before reading FDA literature:37 ·

DVs (Daily Values): A new dietary reference term that will appear on the food label. It is made up of two sets of references, DRVs and RDIs.

·

DRVs (Daily Reference Values): A set of dietary references that applies to fat, saturated fat, cholesterol, carbohydrate, protein, fiber, sodium, and potassium.

·

RDIs (Reference Daily Intakes): A set of dietary references based on the Recommended Dietary Allowances for essential vitamins and minerals and, in selected groups, protein. The name “RDI” replaces the term “U.S. RDA.”

·

RDAs (Recommended Dietary Allowances): A set of estimated nutrient allowances established by the National Academy of Sciences. It is updated periodically to reflect current scientific knowledge.

37

Adapted from the FDA: http://www.fda.gov/fdac/special/foodlabel/dvs.html.

Researching Nutrition 131

What Are Dietary Supplements?38 Dietary supplements are widely available through many commercial sources, including health food stores, grocery stores, pharmacies, and by mail. Dietary supplements are provided in many forms including tablets, capsules, powders, gel-tabs, extracts, and liquids. Historically in the United States, the most prevalent type of dietary supplement was a multivitamin/mineral tablet or capsule that was available in pharmacies, either by prescription or “over the counter.” Supplements containing strictly herbal preparations were less widely available. Currently in the United States, a wide array of supplement products are available, including vitamin, mineral, other nutrients, and botanical supplements as well as ingredients and extracts of animal and plant origin. The Office of Dietary Supplements (ODS) of the National Institutes of Health is the official agency of the United States which has the expressed goal of acquiring “new knowledge to help prevent, detect, diagnose, and treat disease and disability, from the rarest genetic disorder to the common cold.”39 According to the ODS, dietary supplements can have an important impact on the prevention and management of medical conditions and on the maintenance of health.40 The ODS notes that considerable research on the effects of dietary supplements has been conducted in Asia and Europe where the use of plant products, in particular, has a long tradition. However, the overwhelming majority of supplements have not been studied scientifically. To explore the role of dietary supplements in the improvement of health care, the ODS plans, organizes, and supports conferences, workshops, and symposia on scientific topics related to dietary supplements. The ODS often works in conjunction with other NIH Institutes and Centers, other government agencies, professional organizations, and public advocacy groups.

This discussion has been adapted from the NIH: http://ods.od.nih.gov/showpage.aspx?pageid=46. 39 Contact: The Office of 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]. 40 Adapted from http://ods.od.nih.gov/showpage.aspx?pageid=2. The Dietary Supplement Health and Education Act defines dietary supplements as “a product (other than tobacco) intended to supplement the diet that bears or contains one or more of the following dietary ingredients: a vitamin, mineral, amino acid, herb or other botanical; or a dietary substance for use to supplement the diet by increasing the total dietary intake; or a concentrate, metabolite, constituent, extract, or combination of any ingredient described above; and intended for ingestion in the form of a capsule, powder, softgel, or gelcap, and not represented as a conventional food or as a sole item of a meal or the diet.” 38

132 Chiari Malformation

To learn more about official information on dietary supplements, visit the ODS site at http://ods.od.nih.gov/whatare/whatare.html. Or contact: The Office of 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]

Finding Studies on Chiari Malformation The NIH maintains an office dedicated to nutrition and diet. 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). 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.41 IBIDS is available to the public free of charge through the ODS Internet page: 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. We recommend that you start with the Consumer Database. While you may not find references for the topics that are of most interest to you, check back periodically as this database is frequently updated. More studies can be found by searching the Full IBIDS Database. Healthcare professionals and researchers generally use the third option, which lists peer-reviewed citations. In all cases, we suggest that you take advantage of the “Advanced Search” option that allows you to retrieve up to 100 fully explained references in a comprehensive format. Type “Chiari malformation” (or synonyms) into the search box. To narrow the search, you can also select the “Title” field.

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.

41

Researching Nutrition 133

The following information is typical of that found when using the “Full IBIDS Database” when searching using “Chiari malformation” (or a synonym): ·

Chiari malformation associated with vitamin D-resistant rickets: case report. Author(s): Division of Neurosurgery, Oregon Health Sciences University, Portland 97201-3098, USA. Source: Kuether, T A Piatt, J H Neurosurgery. 1998 May; 42(5): 1168-71 0148-396X

·

Use of caffeine in the treatment of apnea associated with the ArnoldChiari malformation. Author(s): Developmental Pharmacology and Perinatal Research Unit, Montreal Children's Hospital Research Institute, Que., Canada. Source: Davis, J M Zinman, R Aranda, J V Dev-Pharmacol-Ther. 1989; 12(2): 70-3 0379-8305

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&pag e=0

·

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

·

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

·

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

·

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/

·

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

134 Chiari Malformation

·

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/

·

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

·

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

·

WebMDÒHealth: http://my.webmd.com/nutrition

·

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

Vocabulary Builder The following vocabulary builder defines words used in the references in this chapter that have not been defined in previous chapters: 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-, poly- and heterosaccharides. [EU] Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils. [NIH] Degenerative: Undergoing degeneration : tending to degenerate; having the character of or involving degeneration; causing or tending to cause degeneration. [EU]

Researching Nutrition 135

Diarrhea: Passage of excessively liquid or excessively frequent stools. [NIH] Intestinal: Pertaining to the intestine. [EU] Iodine: A nonmetallic element of the halogen group that is represented by the atomic symbol I, atomic number 53, and atomic weight of 126.90. It is a nutritionally essential element, especially important in thyroid hormone synthesis. In solution, it has anti-infective properties and is used topically. [NIH]

Niacin: Water-soluble vitamin of the B complex occurring in various animal and plant tissues. Required by the body for the formation of coenzymes NAD and NADP. Has pellagra-curative, vasodilating, and antilipemic properties. [NIH] Overdose: 1. to administer an excessive dose. 2. an excessive dose. [EU] 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] Riboflavin: Nutritional factor found in milk, eggs, malted barley, liver, kidney, heart, and leafy vegetables. The richest natural source is yeast. It occurs in the free form only in the retina of the eye, in whey, and in urine; its principal forms in tissues and cells are as FMN and FAD. [NIH] Selenium: An element with the atomic symbol Se, atomic number 34, and atomic weight 78.96. It is an essential micronutrient for mammals and other animals but is toxic in large amounts. Selenium protects intracellular structures against oxidative damage. It is an essential component of glutathione peroxidase. [NIH] Thermoregulation: Heat regulation. [EU] Thyroxine: An amino acid of the thyroid gland which exerts a stimulating effect on thyroid metabolism. [NIH]

137

APPENDIX C. FINDING MEDICAL LIBRARIES Overview At a medical library you can find medical texts and reference books, consumer health publications, specialty newspapers and magazines, as well as medical journals. In this Appendix, we show you how to quickly find a medical library in your area.

Preparation Before going to the library, highlight the references mentioned in this sourcebook that you find interesting. Focus on those items that are not available via the Internet, and ask the reference librarian for help with your search. He or she may know of additional resources that could be helpful to you. Most importantly, 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. NLM’s interlibrary loan services are only available to libraries. If you would like to access NLM medical literature, then visit a library in your area that can request the publications for you.42

42

Adapted from the NLM: http://www.nlm.nih.gov/psd/cas/interlibrary.html.

138 Chiari Malformation

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 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)43: ·

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://www-med.stanford.edu/healthlibrary/

43

Abstracted from http://www.nlm.nih.gov/medlineplus/libraries.html.

Finding Medical Libraries 139

·

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/

·

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

140 Chiari Malformation

·

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 Library-Shreveport, 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/

·

Manitoba, Canada: Consumer & Patient Health Information Service (University of Manitoba Libraries), http://www.umanitoba.ca/libraries/units/health/reference/chis.html

Finding Medical Libraries 141

·

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://medlibwww.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

142 Chiari Malformation

·

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/

·

Nevada: Health Science Library, West Charleston Library (Las VegasClark 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

Finding Medical Libraries 143

·

Oklahoma: The Health Information Center at Saint Francis Hospital (Saint Francis Health System, Tulsa), http://www.sfhtulsa.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/

·

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

145

APPENDIX D. YOUR CHILD’S RIGHTS AND INSURANCE Overview Parents face a series of issues related more to the healthcare industry than to their children’s medical conditions. This appendix covers two important topics in this regard: your responsibilities and your child’s rights as a patient, and how to get the most out of your child’s medical insurance plan.

Your Child’s Rights as a Patient The President’s Advisory Commission on Consumer Protection and Quality in the Healthcare Industry has created the following summary of your child’s rights as a patient.44 Information Disclosure Consumers have the right to receive accurate, easily understood information. Some consumers require assistance in making informed decisions about health plans, health professionals, and healthcare facilities. Such information includes: ·

Health plans. Covered benefits, cost-sharing, and procedures for resolving complaints, licensure, certification, and accreditation status, comparable measures of quality and consumer satisfaction, provider network composition, the procedures that govern access to specialists and emergency services, and care management information.

44Adapted

from Consumer Bill of Rights and Responsibilities: http://www.hcqualitycommission.gov/press/cbor.html#head1.

146 Chiari Malformation

·

Health professionals. Education, board certification, and recertification, years of practice, experience performing certain procedures, and comparable measures of quality and consumer satisfaction.

·

Healthcare facilities. Experience in performing certain procedures and services, accreditation status, comparable measures of quality, worker, and consumer satisfaction, and procedures for resolving complaints.

·

Consumer assistance programs. Programs must be carefully structured to promote consumer confidence and to work cooperatively with health plans, providers, payers, and regulators. Desirable characteristics of such programs are sponsorship that ensures accountability to the interests of consumers and stable, adequate funding.

Choice of Providers and Plans Consumers have the right to a choice of healthcare providers that is sufficient to ensure access to appropriate high-quality healthcare. To ensure such choice, the Commission recommends the following: ·

Provider network adequacy. All health plan networks should provide access to sufficient numbers and types of providers to assure that all covered services will be accessible without unreasonable delay -including access to emergency services 24 hours a day and 7 days a week. If a health plan has an insufficient number or type of providers to provide a covered benefit with the appropriate degree of specialization, the plan should ensure that the consumer obtains the benefit outside the network at no greater cost than if the benefit were obtained from participating providers.

·

Access to specialists. Consumers with complex or serious medical conditions who require frequent specialty care should have direct access to a qualified specialist of their choice within a plan’s network of providers. Authorizations, when required, should be for an adequate number of direct access visits under an approved treatment plan.

·

Transitional care. Consumers who are undergoing a course of treatment for a chronic or disabling condition at the time they involuntarily change health plans or at a time when a provider is terminated by a plan for other than cause should be able to continue seeing their current specialty providers for up to 90 days to allow for transition of care.

·

Choice of health plans. Public and private group purchasers should, wherever feasible, offer consumers a choice of high-quality health insurance plans.

Your Child’s Rights and Insurance 147

Access to Emergency Services Consumers have the right to access emergency healthcare services when and where the need arises. Health plans should provide payment when a consumer presents to an emergency department with acute symptoms of sufficient severity--including severe pain--such that a “prudent layperson” could reasonably expect the absence of medical attention to result in placing that consumer’s health in serious jeopardy, serious impairment to bodily functions, or serious dysfunction of any bodily organ or part.

Participation in Treatment Decisions Consumers have the right and responsibility to fully participate in all decisions related to their healthcare. Consumers who are unable to fully participate in treatment decisions have the right to be represented by parents, guardians, family members, or other conservators. Physicians and other health professionals should: ·

Provide parents with sufficient information and opportunity to decide among treatment options consistent with the informed consent process.

·

Discuss all treatment options with a parent in a culturally competent manner, including the option of no treatment at all.

·

Ensure that persons with disabilities have effective communications with members of the health system in making such decisions.

·

Discuss all current treatments a consumer may be undergoing.

·

Discuss all risks, nontreatment.

·

Give parents the opportunity to refuse treatment for their children and to express preferences about future treatment decisions.

·

Discuss the use of advance directives -- both living wills and durable powers of attorney for healthcare -- with parents.

·

Abide by the decisions made by parents consistent with the informed consent process.

benefits,

and

consequences

to

treatment

or

148 Chiari Malformation

Health plans, health providers, and healthcare facilities should: ·

Disclose to consumers factors -- such as methods of compensation, ownership of or interest in healthcare facilities, or matters of conscience -that could influence advice or treatment decisions.

·

Assure that provider contracts do not contain any so-called “gag clauses” or other contractual mechanisms that restrict healthcare providers’ ability to communicate with and advise parents about medically necessary treatment options for their children.

·

Be prohibited from penalizing or seeking retribution against healthcare professionals or other health workers for advocating on behalf of their patients. Respect and Nondiscrimination

Consumers have the right to considerate, respectful care from all members of the healthcare industry at all times and under all circumstances. An environment of mutual respect is essential to maintain a quality healthcare system. To assure that right, the Commission recommends the following: ·

Consumers must not be discriminated against in the delivery of healthcare services consistent with the benefits covered in their policy, or as required by law, based on race, ethnicity, national origin, religion, sex, age, mental or physical disability, sexual orientation, genetic information, or source of payment.

·

Consumers eligible for coverage under the terms and conditions of a health plan or program, or as required by law, must not be discriminated against in marketing and enrollment practices based on race, ethnicity, national origin, religion, sex, age, mental or physical disability, sexual orientation, genetic information, or source of payment.

Confidentiality of Health Information Consumers have the right to communicate with healthcare providers in confidence and to have the confidentiality of their individually identifiable healthcare information protected. Consumers also have the right to review and copy their own medical records and request amendments to their records.

Your Child’s Rights and Insurance 149

Complaints and Appeals Consumers have the right to a fair and efficient process for resolving differences with their health plans, healthcare providers, and the institutions that serve them, including a rigorous system of internal review and an independent system of external review. A free copy of the Patient’s Bill of Rights is available from the American Hospital Association.45

Parent Responsibilities To underscore the importance of finance in modern healthcare as well as your responsibility for the financial aspects of your child’s care, the President’s Advisory Commission on Consumer Protection and Quality in the Healthcare Industry has proposed that parents understand the following “Consumer Responsibilities.”46 In a healthcare system that protects consumers’ rights, it is reasonable to expect and encourage consumers to assume certain responsibilities. Greater involvement by parents in their children’s care increases the likelihood of achieving the best outcome and helps support a quality-oriented, cost-conscious environment. Such responsibilities include: ·

Take responsibility for maximizing your child’s healthy habits.

·

Work collaboratively with healthcare providers in developing and carrying out your child’s agreed-upon treatment plans.

·

Disclose relevant information and clearly communicate wants and needs.

·

Use the insurance company’s internal complaint and appeal processes to address your concerns.

·

Recognize the reality of risks, the limits of the medical science, and the human fallibility of the healthcare professional.

·

Be aware of a healthcare provider’s obligation to be reasonably efficient and equitable in providing care to the community.

·

Become knowledgeable about health plan coverage and options (when available) including all covered benefits, limitations, and exclusions, rules regarding use of network providers, coverage and referral rules,

To order your free copy of the Patient’s Bill of Rights, telephone 312-422-3000 or visit the American Hospital Association’s Web site: http://www.aha.org. Click on “Resource Center,” go to “Search” at bottom of page, and then type in “Patient’s Bill of Rights.” The Patient’s Bill of Rights is also available from Fax on Demand, at 312-422-2020, document number 471124. 46 Adapted from http://www.hcqualitycommission.gov/press/cbor.html#head1. 45

150 Chiari Malformation

appropriate processes to secure additional information, and the process to appeal coverage decisions. ·

Make a good-faith effort to meet financial obligations.

·

Abide by administrative and operational procedures of health plans, healthcare providers, and Government health benefit programs.

Choosing an Insurance Plan There are a number of official government agencies that help consumers understand their healthcare insurance choices.47 The U.S. Department of Labor, in particular, recommends ten ways to make your health benefits choices work best for your family.48 1. Your options are important. There are many different types of health benefit plans. Find out which one your employer offers, then check out the plan, or plans, offered. Your employer’s human resource office, the health plan administrator, or your union can provide information to help you match your family’s needs and preferences with the available plans. The more information you have, the better your healthcare decisions will be. 2. Reviewing the benefits available. Do the plans offered cover preventive care, well-baby care, vision or dental care? Are there deductibles? Answers to these questions can help determine the out-of-pocket expenses you may face. Cheapest may not always be best. Your goal is high quality health benefits. 3. Look for quality. The quality of healthcare services varies, but quality can be measured. You should consider the quality of healthcare in deciding among the healthcare plans or options available to your family. Not all health plans, doctors, hospitals and other providers give the highest quality care. Fortunately, there is quality information you can use right now to help you compare your healthcare choices. Find out how you can measure quality. Consult the U.S. Department of Health and Human Services publication “Your Guide to Choosing Quality Health Care” on the Internet at www.ahcpr.gov/consumer.

More information about quality across programs is provided at the following AHRQ Web site: http://www.ahrq.gov/consumer/qntascii/qnthplan.htm. 48 Adapted from the Department of Labor: http://www.dol.gov/dol/pwba/public/pubs/health/top10-text.html. 47

Your Child’s Rights and Insurance 151

4. Your plan’s summary plan description (SPD) provides a wealth of information. Your health plan administrator can provide you with a copy of your plan’s SPD. It outlines your family’s benefits and your legal rights under the Employee Retirement Income Security Act (ERISA), the federal law that protects your family’s health benefits. It should contain information about the coverage of dependents, what services will require a co-pay, and the circumstances under which your employer can change or terminate a health benefits plan. Save the SPD and all other health plan brochures and documents, along with memos or correspondence from your employer relating to health benefits. 5. Assess your benefit coverage as your family status changes. Marriage, divorce, childbirth or adoption, and the death of a spouse are all life events that may signal a need to change your health benefits. You, your spouse and dependent children may be eligible for a special enrollment period under provisions of the Health Insurance Portability and Accountability Act (HIPAA). Even without life-changing events, the information provided by your employer should tell you how you can change benefits or switch plans, if more than one plan is offered. If your spouse’s employer also offers a health benefits package, consider coordinating both plans for maximum coverage. 6. Changing jobs and other life events can affect your family’s health benefits. Under the Consolidated Omnibus Budget Reconciliation Act (COBRA), you, your covered spouse, and your dependent children may be eligible to purchase extended health coverage under your employer’s plan if you lose your job, change employers, get divorced, or upon occurrence of certain other events. Coverage can range from 18 to 36 months depending on your situation. COBRA applies to most employers with 20 or more workers and requires your plan to notify you of your rights. Most plans require eligible individuals to make their COBRA election within 60 days of the plan’s notice. Be sure to follow up with your plan sponsor if you don’t receive notice, and make sure you respond within the allotted time. 7. HIPAA can also help if you are changing jobs, particularly if you have a medical condition. HIPAA generally limits pre-existing condition exclusions to a maximum of 12 months (18 months for late enrollees). HIPAA also requires this maximum period to be reduced by the length of time you had prior “creditable coverage.” You should receive a certificate documenting your prior creditable coverage from your old plan when coverage ends. 8. Plan for retirement. Before you retire, find out what health benefits, if any, extend to you and your spouse during your retirement years. Consult with

152 Chiari Malformation

your employer’s human resources office, your union, the plan administrator, and check your SPD. Make sure there is no conflicting information among these sources about the benefits your family will receive or the circumstances under which they can change or be eliminated. With this information in hand, you can make other important choices, like finding out if you are eligible for Medicare and Medigap insurance coverage. 9. Know how to file an appeal if a health benefits claim is denied. Understand how your plan handles grievances and where to make appeals of the plan’s decisions. Keep records and copies of correspondence. Check your health benefits package and your SPD to determine who is responsible for handling problems with benefit claims. Contact PWBA for customer service assistance if you are unable to obtain a response to your complaint. 10. You can take steps to improve the quality of the healthcare and the health benefits your family receives. Look for and use things like Quality Reports and Accreditation Reports whenever you can. Quality reports may contain consumer ratings -- how satisfied consumers are with the doctors in their plan, for instance-- and clinical performance measures -- how well a healthcare organization prevents and treats illness. Accreditation reports provide information on how accredited organizations meet national standards, and often include clinical performance measures. Look for these quality measures whenever possible. Consult “Your Guide to Choosing Quality Health Care” on the Internet at www.ahcpr.gov/consumer.

Medicaid Illness strikes both rich and poor families. For low-income families, Medicaid is available to defer the costs of treatment. In the following pages, you will learn the basics about Medicaid as well as useful contact information on how to find more in-depth information. Medicaid is a joint federal and state program that helps pay medical costs for some people with low incomes and limited resources. Medicaid programs vary from state to state. You can find more information about Medicaid on the HCFA.gov Web site at http://www.hcfa.gov/medicaid/medicaid.htm.

NORD’s Medication Assistance Programs Finally, the National Organization for Rare Disorders, Inc. (NORD) administers medication programs sponsored by humanitarian-minded

Your Child’s Rights and Insurance 153

pharmaceutical and biotechnology companies to help uninsured or underinsured individuals secure life-saving or life-sustaining drugs.49 NORD programs ensure that certain vital drugs are available “to those families whose income is too high to qualify for Medicaid but too low to pay for their prescribed medications.” The program has standards for fairness, equity, and unbiased eligibility. It currently covers some 14 programs for nine pharmaceutical companies. NORD also offers early access programs for investigational new drugs (IND) under the approved “Treatment INDs” programs of the Food and Drug Administration (FDA). In these programs, a limited number of individuals can receive investigational drugs that have yet to be approved by the FDA. These programs are generally designed for rare medical conditions. For more information, visit www.rarediseases.org.

Additional Resources In addition to the references already listed in this chapter, you may need more information on health insurance, hospitals, or the healthcare system in general. The NIH has set up an excellent guidance Web site that addresses these and other issues. Topics include:50 ·

Health Insurance: http://www.nlm.nih.gov/medlineplus/healthinsurance.html

·

Health Statistics: http://www.nlm.nih.gov/medlineplus/healthstatistics.html

·

HMO and Managed Care: http://www.nlm.nih.gov/medlineplus/managedcare.html

·

Hospice Care: http://www.nlm.nih.gov/medlineplus/hospicecare.html

·

Medicaid: http://www.nlm.nih.gov/medlineplus/medicaid.html

·

Medicare: http://www.nlm.nih.gov/medlineplus/medicare.html

·

Nursing Homes and Long-Term Care: http://www.nlm.nih.gov/medlineplus/nursinghomes.html

Adapted from NORD: http://www.rarediseases.org/cgibin/nord/progserv#patient?id=rPIzL9oD&mv_pc=30. 50 You can access this information at: http://www.nlm.nih.gov/medlineplus/healthsystem.html. 49

154 Chiari Malformation

·

Patient’s Rights, Confidentiality, Informed Consent, Ombudsman Programs, Privacy and Patient Issues: http://www.nlm.nih.gov/medlineplus/patientissues.html

·

Veteran’s Health, Persian Gulf War, Gulf War Syndrome, Agent Orange: http://www.nlm.nih.gov/medlineplus/veteranshealth.html

155

ONLINE GLOSSARIES The Internet provides access to a number of free-to-use medical dictionaries and glossaries. 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://www.graylab.ac.uk/omd/

·

Technology Glossary (National Library of Medicine) - Health Care Technology: http://www.nlm.nih.gov/nichsr/ta101/ta10108.htm

·

Terms and Definitions (Office of Rare Diseases): http://rarediseases.info.nih.gov/ord/glossary_a-e.html

Beyond these, MEDLINEplus contains a very user-friendly encyclopedia covering every aspect of medicine (licensed from A.D.A.M., Inc.). The ADAM Medical Encyclopedia can be accessed via the following Web site address: http://www.nlm.nih.gov/medlineplus/encyclopedia.html. ADAM is also available on commercial Web sites such as Web MD (http://my.webmd.com/adam/asset/adam_disease_articles/a_to_z/a) and drkoop.com (http://www.drkoop.com/). Topics of interest can be researched by using keywords before continuing elsewhere, as these basic definitions and concepts will be useful in more advanced areas of research. You may choose to print various pages specifically relating to diseasex and keep them on file. The NIH, in particular, suggests that patients with diseasex visit the following Web sites in the ADAM Medical Encyclopedia:

156 Chiari Malformation

Online Dictionary Directories The following are additional online directories compiled by the National Library of Medicine, including a number of specialized medical dictionaries and glossaries: ·

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

157

CHIARI MALFORMATION GLOSSARY The following is a complete glossary of terms used in this sourcebook. The definitions are derived from official public sources including the National Institutes of Health [NIH] and the European Union [EU]. After this glossary, we list a number of additional hardbound and electronic glossaries and dictionaries that you may wish to consult. Abdominal: Pertaining to the abdomen. [EU] Abscess: A localized collection of pus caused by suppuration buried in tissues, organs, or confined spaces. [EU] 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] 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]

Aerobic: 1. having molecular oxygen present. 2. growing, living, or occurring in the presence of molecular oxygen. 3. requiring oxygen for respiration. [EU] 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] Anemia: A reduction in the number of circulating erythrocytes or in the quantity of hemoglobin. [NIH] Anesthesiology: A specialty concerned with the study of anesthetics and anesthesia. [NIH] Angiography: Radiography of blood vessels after injection of a contrast medium. [NIH] Antibody: An immunoglobulin molecule that has a specific amino acid sequence by virtue of which it interacts only with the antigen that induced its synthesis in cells of the lymphoid series (especially plasma cells), or with antigen closely related to it. Antibodies are classified according to their ode of action as agglutinins, bacteriolysins, haemolysins, opsonins, precipitins, etc. [EU] Antihypertensive: An agent that reduces high blood pressure. [EU]

158 Chiari Malformation

Antioxidant: One of many widely used synthetic or natural substances added to a product to prevent or delay its deterioration by action of oxygen in the air. Rubber, paints, vegetable oils, and prepared foods commonly contain antioxidants. [EU] Apnea: A transient absence of spontaneous respiration. [NIH] Apnoea: Cessation of breathing. [EU] Arteries: The vessels carrying blood away from the heart. [NIH] Arteriography: Roentgenography of arteries after injection of radiopacque material into the blood stream. [EU] Arteriovenous: Both arterial and venous; pertaining to or affecting an artery and a vein. [EU] Arthropathy: Any joint disease. [EU] Assay: Determination of the amount of a particular constituent of a mixture, or of the biological or pharmacological potency of a drug. [EU] Asymptomatic: Showing or causing no symptoms. [EU] Ataxia: Failure of muscular coordination; irregularity of muscular action. [EU] Atrophy: A wasting away; a diminution in the size of a cell, tissue, organ, or part. [EU] Aura: A subjective sensation or motor phenomenon that precedes and marks the of a paroxysmal attack, such as an epileptic attack on set. [EU] Autopsy: Postmortem examination of the 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] Barotrauma: Injury following pressure changes; includes injury to the eustachian tube, ear drum, lung and stomach. [NIH] Benign: Not malignant; not recurrent; favourable for recovery. [EU] Biochemical: Relating to biochemistry; characterized by, produced by, or involving chemical reactions in living organisms. [EU] Buccal: Pertaining to or directed toward the cheek. In dental anatomy, used to refer to the buccal surface of a tooth. [EU] 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-, poly- and

Glossary 159

heterosaccharides. [EU] Cardiac: Pertaining to the heart. [EU] Cardiovascular: Pertaining to the heart and blood vessels. [EU] Catheter: A tubular, flexible, surgical instrument for withdrawing fluids from (or introducing fluids into) a cavity of the body, especially one for introduction into the bladder through the urethra for the withdraw of urine. [EU]

Cerebellar: Pertaining to the cerebellum. [EU] 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] Cerebrospinal: Pertaining to the brain and spinal cord. [EU] Cerebrovascular: Pertaining to the blood vessels of the cerebrum, or brain. [EU]

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] Cholesterol: The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils. [NIH] Chromosomal: Pertaining to chromosomes. [EU] Chronic: Persisting over a long period of time. [EU] Cloaca: 1. in zoology, a common passage for faecal, urinary, and reproductive discharge in most lower vertebrates. 2. in mammalian embryology, the terminal end of the hindgut before division into rectum, bladder, and genital primordia. 3. in pathology, an opening in the involucrum of a necrosed bone. [EU] 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] Contusion: A bruise; an injury of a part without a break in the skin. [EU] 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] Cortical: Pertaining to or of the nature of a cortex or bark. [EU] 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] Cyst: Any closed cavity or sac; normal or abnormal, lined by epithelium,

160 Chiari Malformation

and especially one that contains a liquid or semisolid material. [EU] Cystitis: Inflammation of the urinary bladder. [EU] 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] Degenerative: Undergoing degeneration : tending to degenerate; having the character of or involving degeneration; causing or tending to cause degeneration. [EU] 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] Dermatology: A medical specialty concerned with the skin, its structure, functions, diseases, and treatment. [NIH] Diarrhea: Passage of excessively liquid or excessively frequent stools. [NIH] Dislocation: The displacement of any part, more especially of a bone. Called also luxation. [EU] Disposition: A tendency either physical or mental toward certain diseases. [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] Dizziness: An imprecise term which may refer to a sense of spatial disorientation, motion of the environment, or lightheadedness. [NIH] Dysphagia: Difficulty in swallowing. [EU] Dysplasia: Abnormality of development; in pathology, alteration in size, shape, and organization of adult cells. [EU] Ectopic: Pertaining to or characterized by ectopia. [EU] Endocrinology: A subspecialty of internal medicine concerned with the metabolism, physiology, and disorders of the endocrine system. [NIH] Endogenous: Developing or originating within the organisms or arising from causes within the organism. [EU] Enzyme: A protein molecule that catalyses chemical reactions of other substances without itself being destroyed or altered upon completion of the reactions. Enzymes are classified according to the recommendations of the Nomenclature Committee of the International Union of Biochemistry. Each enzyme is assigned a recommended name and an Enzyme Commission (EC)

Glossary 161

number. They are divided into six main groups; oxidoreductases, transferases, hydrolases, lyases, isomerases, and ligases. [EU] Epidural: Situated upon or outside the dura mater. [EU] Epiphyseal: Pertaining to or of the nature of an epiphysis. [EU] Estrogens: A class of sex hormones associated with the development and maintenance of secondary female sex characteristics and control of the cyclical changes in the reproductive cycle. They are also required for pregnancy maintenance and have an anabolic effect on protein metabolism and water retention. [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] Fibrin: The insoluble protein formed from fibrinogen by the proteolytic action of thrombin during normal clotting of blood. Fibrin forms the essential portion of the blood clot. [EU] Fibrosis: The formation of fibrous tissue; fibroid or fibrous degeneration [EU] Fistula: An abnormal passage or communication, usually between two internal organs, or leading from an internal organ to the surface of the body; frequently designated according to the organs or parts with which it communicates, as anovaginal, brochocutaneous, hepatopleural, pulmonoperitoneal, rectovaginal, urethrovaginal, and the like. Such passages are frequently created experimentally for the purpose of obtaining body secretions for physiologic study. [EU] Gynecology: A medical-surgical specialty concerned with the physiology and disorders primarily of the female genital tract, as well as female endocrinology and reproductive physiology. [NIH] Hematology: A subspecialty of internal medicine concerned with morphology, physiology, and pathology of the blood and blood-forming tissues. [NIH] Hemodilution: Reduction of blood viscosity usually by the addition of cell free solutions. Used clinically l) in states of impaired microcirculation, 2) for replacement of intraoperative blood loss without homologous blood transfusion, and 3) in cardiopulmonary bypass and hypothermia. [NIH] Hemorrhage: Bleeding or escape of blood from a vessel. [NIH] Herpes: Any inflammatory skin disease caused by a herpesvirus and characterized by the formation of clusters of small vesicles. When used alone, the term may refer to herpes simplex or to herpes zoster. [EU] Humoral: Of, relating to, proceeding from, or involving a bodily humour now often used of endocrine factors as opposed to neural or somatic. [EU]

162 Chiari Malformation

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: A condition marked by dilatation of the cerebral ventricles, most often occurring secondarily to obstruction of the cerebrospinal fluid pathways, and accompanied by an accumulation of cerebrospinal fluid within the skull; the fluid is usually under increased pressure, but occasionally may be normal or nearly so. It is typically characterized by enlargement of the head, prominence of the forehead, brain atrophy, mental deterioration, and convulsions; may be congenital or acquired; and may be of sudden onset (acute h.) or be slowly progressive (chronic or primary b.). [EU]

Hypotension: Abnormally low blood pressure; seen in shock but not necessarily indicative of it. [EU] Hypothalamic: Of or involving the hypothalamus. [EU] Hypoxia: Reduction of oxygen supply to tissue below physiological levels despite adequate perfusion of the tissue by blood. [EU] Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents. [NIH] Implantation: The insertion or grafting into the body of biological, living, inert, or radioactive material. [EU] Infarction: 1. the formation of an infarct. 2. an infarct. [EU] 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 insulindependent diabetes mellitus. [NIH] Interstitial: Pertaining to or situated between parts or in the interspaces of a tissue. [EU] Intestinal: Pertaining to the intestine. [EU] Invasive: 1. having the quality of invasiveness. 2. involving puncture or incision of the skin or insertion of an instrument or foreign material into the body; said of diagnostic techniques. [EU] Iodine: A nonmetallic element of the halogen group that is represented by the atomic symbol I, atomic number 53, and atomic weight of 126.90. It is a nutritionally essential element, especially important in thyroid hormone synthesis. In solution, it has anti-infective properties and is used topically. [NIH]

Ischemia: Deficiency of blood in a part, due to functional constriction or

Glossary 163

actual obstruction of a blood vessel. [EU] Labyrinthitis: Inflammation of the inner ear. [NIH] Lesion: Any pathological or traumatic discontinuity of tissue or loss of function of a part. [EU] Lipoprotein: Any of the lipid-protein complexes in which lipids are transported in the blood; lipoprotein particles consist of a spherical hydrophobic core of triglycerides or cholesterol esters surrounded by an amphipathic monolayer of phospholipids, cholesterol, and apolipoproteins; the four principal classes are high-density, low-density, and very-lowdensity lipoproteins and chylomicrons. [EU] Lumbar: Pertaining to the loins, the part of the back between the thorax and the pelvis. [EU] Lumen: The cavity or channel within a tube or tubular organ. [EU] Malformation: A morphologic defect resulting from an intrinsically abnormal developmental process. [EU] Malignant: Tending to become progressively worse and to result in death. Having the properties of anaplasia, invasion, and metastasis; said of tumours. [EU] Medullary: Pertaining to the marrow or to any medulla; resembling marrow. [EU] Meningitis: Inflammation of the meninges. When it affects the dura mater, the disease is termed pachymeningitis; when the arachnoid and pia mater are involved, it is called leptomeningitis, or meningitis proper. [EU] Mental: Pertaining to the mind; psychic. 2. (L. mentum chin) pertaining to the chin. [EU] Metabolite: process. [EU]

Any substance produced by metabolism or by a metabolic

Microdialysis: A technique for measuring extracellular concentrations of substances in tissues, usually in vivo, by means of a small probe equipped with a semipermeable membrane. Substances may also be introduced into the extracellular space through the membrane. [NIH] Molecular: Of, pertaining to, or composed of molecules : a very small mass of matter. [EU] Morphogenesis: The development of the form of an organ, part of the body, or organism. [NIH] Mycoplasma: A genus of gram-negative, facultatively anaerobic bacteria bounded by a plasma membrane only. Its organisms are parasites and pathogens, found on the mucous membranes of humans, animals, and birds. [NIH]

164 Chiari Malformation

Myocardium: The muscle tissue of the HEART composed of striated, involuntary muscle known as cardiac muscle. [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] Neuroanatomy: Study of the anatomy of the nervous system as a specialty or discipline. [NIH] Neurology: A medical specialty concerned with the study of the structures, functions, and diseases of the nervous system. [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] Neurosurgery: A surgical specialty concerned with the treatment of diseases and disorders of the brain, spinal cord, and peripheral and sympathetic nervous system. [NIH] Neutrophil: Having an affinity for neutral dyes. [EU] Niacin: Water-soluble vitamin of the B complex occurring in various animal and plant tissues. Required by the body for the formation of coenzymes NAD and NADP. Has pellagra-curative, vasodilating, and antilipemic properties. [NIH] Nystagmus: An involuntary, rapid, rhythmic movement of the eyeball, which may be horizontal, vertical, rotatory, or mixed, i.e., of two varieties. [EU]

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] Orthopaedic: Pertaining to the correction of deformities of the musculoskeletal system; pertaining to orthopaedics. [EU] Orthopedics: A surgical specialty which utilizes medical, surgical, and physical methods to treat and correct deformities, diseases, and injuries to the skeletal system, its articulations, and associated structures. [NIH] Osteoporosis: Reduction in the amount of bone mass, leading to fractures after minimal trauma. [EU] Osteotomy: The surgical cutting of a bone. [EU] Otitis: Inflammation of the ear, which may be marked by pain, fever, abnormalities of hearing, hearing loss, tinnitus, and vertigo. [EU] Otolaryngology: A surgical specialty concerned with the study and treatment of disorders of the ear, nose, and throat. [NIH]

Glossary 165

Otorhinolaryngology: That branch of medicine concerned with medical and surgical treatment of the head and neck, including the ears, nose and throat. [EU]

Otosclerosis: A pathological condition of the bony labyrinth of the ear, in which there is formation of spongy bone (otospongiosis), especially in front of and posterior to the footplate of the stapes; it may cause bony ankylosis of the stapes, resulting in conductive hearing loss. Cochlear otosclerosis may also develop, resulting in sensorineural hearing loss. [EU] Overdose: 1. to administer an excessive dose. 2. an excessive dose. [EU] Paralysis: Loss or impairment of motor function in a part due to lesion of the neural or muscular mechanism; also by analogy, impairment of sensory function (sensory paralysis). In addition to the types named below, paralysis is further distinguished as traumatic, syphilitic, toxic, etc., according to its cause; or as obturator, ulnar, etc., according to the nerve part, or muscle specially affected. [EU] Paraplegia: Paralysis of the legs and lower part of the body. [EU] Particle: A tiny mass of material. [EU] Parvovirus: A genus of the family PARVOVIRIDAE, subfamily PARVOVIRINAE, infecting a variety of vertebrates including humans. Parvoviruses are responsible for a number of important diseases but also can be non-pathogenic in certain hosts. The type species is MICE MINUTE VIRUS. [NIH] Pediatrics: A medical specialty concerned with maintaining health and providing medical care to children from birth to adolescence. [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] Perindopril: An angiotensin-converting enzyme inhibitor. It is used in patients with hypertension and heart failure. [NIH] Physiologic: Normal; not pathologic; characteristic of or conforming to the normal functioning or state of the body or a tissue or organ; physiological. [EU]

Plasminogen: The inactive precursor of plasmin (=enzyme that catalyses the hydrolysis of peptide bonds at the carbonyl end of lysine or arginine residues). [EU] 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: Occurring after the menopause. [EU] Postoperative: Occurring after a surgical operation. [EU]

166 Chiari Malformation

Potassium: An element that is in the alkali group of metals. It has an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte and it plays a significant role in the regulation of fluid volume and maintenance of the water-electrolyte balance. [NIH] 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] Presbycusis: Progressive bilateral loss of hearing that occurs in the aged. Syn: senile deafness. [NIH] 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] Progressive: Advancing; going forward; going from bad to worse; increasing in scope or severity. [EU] Proteins: Polymers of amino acids linked by peptide bonds. The specific sequence of amino acids determines the shape and function of the protein. [NIH]

Proximal: Nearest; closer to any point of reference; opposed to distal. [EU] 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] Psychology: The science dealing with the study of mental processes and behavior in man and animals. [NIH] Pulmonary: Pertaining to the lungs. [EU] Radiology: A specialty concerned with the use of x-ray and other forms of radiant energy in the diagnosis and treatment of disease. [NIH] Reactivation: The restoration of activity to something that has been inactivated. [EU] Receptor: 1. a molecular structure within a cell or on the surface characterized by (1) selective binding of a specific substance and (2) a specific physiologic effect that accompanies the binding, e.g., cell-surface receptors for peptide hormones, neurotransmitters, antigens, complement fragments, and immunoglobulins and cytoplasmic receptors for steroid hormones. 2. a sensory nerve terminal that responds to stimuli of various kinds. [EU] Recombinant: 1. a cell or an individual with a new combination of genes not found together in either parent; usually applied to linked genes. [EU] Regeneration: The natural renewal of a structure, as of a lost tissue or part. [EU]

Glossary 167

Renin: An enzyme of the hydrolase class that catalyses cleavage of the leucine-leucine bond in angiotensin to generate angiotensin. 1. The enzyme is synthesized as inactive prorenin in the kidney and released into the blood in the active form in response to various metabolic stimuli. Not to be confused with rennin (chymosin). [EU] Resection: Excision of a portion or all of an organ or other structure. [EU] Rheumatology: A subspecialty of internal medicine concerned with the study of inflammatory or degenerative processes and metabolic derangement of connective tissue structures which pertain to a variety of musculoskeletal disorders, such as arthritis. [NIH] Riboflavin: Nutritional factor found in milk, eggs, malted barley, liver, kidney, heart, and leafy vegetables. The richest natural source is yeast. It occurs in the free form only in the retina of the eye, in whey, and in urine; its principal forms in tissues and cells are as FMN and FAD. [NIH] Rickets: A condition caused by deficiency of vitamin D, especially in infancy and childhood, with disturbance of normal ossification. The disease is marked by bending and distortion of the bones under muscular action, by the formation of nodular enlargements on the ends and sides of the bones, by delayed closure of the fontanelles, pain in the muscles, and sweating of the head. Vitamin D and sunlight together with an adequate diet are curative, provided that the parathyroid glands are functioning properly. [EU] 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] Selenium: An element with the atomic symbol Se, atomic number 34, and atomic weight 78.96. It is an essential micronutrient for mammals and other animals but is toxic in large amounts. Selenium protects intracellular structures against oxidative damage. It is an essential component of GLUTATHIONE PEROXIDASE. [NIH] Semantics: The relationships between symbols and their meanings. [NIH] Shunt: 1. to turn to one side; to divert; to bypass. 2. a passage or anastomosis between two natural channels, especially between blood vessels. Such structures may be formed physiologically (e.g. to bypass a thrombosis) or they may be structural anomalies. 3. a surgically created anastomosis; also, the operation of forming a shunt. [EU] Skeletal: Pertaining to the skeleton. [EU] Skull: The skeleton of the head including the bones of the face and the bones enclosing the brain. [NIH]

168 Chiari Malformation

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] Stenosis: Narrowing or stricture of a duct or canal. [EU] Subacute: Somewhat acute; between acute and chronic. [EU] Subarachnoid: Situated or occurring between the arachnoid and the pia mater. [EU] 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] Syncope: A temporary suspension of consciousness due to generalized cerebral schemia, a faint or swoon. [EU] Systemic: Pertaining to or affecting the body as a whole. [EU] Thermoregulation: Heat regulation. [EU] Thoracic: Pertaining to or affecting the chest. [EU] Thrombolytic: 1. dissolving or splitting up a thrombus. 2. a thrombolytic agent. [EU] Thrombosis: The formation, development, or presence of a thrombus. [EU] Thyroxine: An amino acid of the thyroid gland which exerts a stimulating effect on thyroid metabolism. [NIH] Tinnitus: A noise in the ears, as ringing, buzzing, roaring, clicking, etc. Such sounds may at times be heard by others than the patient. [EU] Tolerance: 1. the ability to endure unusually large doses of a drug or toxin. 2. acquired drug tolerance; a decreasing response to repeated constant doses of a drug or the need for increasing doses to maintain a constant response. [EU]

Tomography: The recording of internal body images at a predetermined plane by means of the tomograph; called also body section roentgenography. [EU]

Transfusion: The introduction of whole blood or blood component directly into the blood stream. [EU] Tremor: An involuntary trembling or quivering. [EU] Venereology: A branch of medicine which deals with sexually transmitted disease. [NIH] Ventricular: Pertaining to a ventricle. [EU] Vertigo: An illusion of movement; a sensation as if the external world were

Glossary 169

revolving around the patient (objective vertigo) or as if he himself were revolving in space (subjective vertigo). The term is sometimes erroneously used to mean any form of dizziness. [EU] 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] Viral: Pertaining to, caused by, or of the nature of virus. [EU] Viruses: Minute infectious agents whose genomes are composed of DNA or RNA, but not both. They are characterized by a lack of independent metabolism and the inability to replicate outside living host cells. [NIH] Vitex: A genus of trees in the LAMIACEAE family containing assorted flavonoids with possible analgesic and antineoplastic properties. The fruit of these trees is used in herbal preparations. [NIH] Warfarin: An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide. [NIH]

General Dictionaries and Glossaries While the above glossary is essentially complete, the dictionaries listed here cover virtually all aspects of medicine, from basic words and phrases to more advanced terms (sorted alphabetically by title; hyperlinks provide rankings, information and reviews at Amazon.com): ·

Dictionary of Medical Acronymns & Abbreviations by Stanley Jablonski (Editor), Paperback, 4th edition (2001), Lippincott Williams & Wilkins Publishers, ISBN: 1560534605, http://www.amazon.com/exec/obidos/ASIN/1560534605/icongroupinter na

·

Dictionary of Medical Terms : For the Nonmedical Person (Dictionary of Medical Terms for the Nonmedical Person, Ed 4) by Mikel A. Rothenberg, M.D, et al, Paperback - 544 pages, 4th edition (2000), Barrons Educational Series, ISBN: 0764112015, http://www.amazon.com/exec/obidos/ASIN/0764112015/icongroupinter na

·

A Dictionary of the History of Medicine by A. Sebastian, CD-Rom edition (2001), CRC Press-Parthenon Publishers, ISBN: 185070368X,

170 Chiari Malformation

http://www.amazon.com/exec/obidos/ASIN/185070368X/icongroupinter na ·

Dorland’s Illustrated Medical Dictionary (Standard Version) by Dorland, et al, Hardcover - 2088 pages, 29th edition (2000), W B Saunders Co, ISBN: 0721662544, http://www.amazon.com/exec/obidos/ASIN/0721662544/icongroupinter na

·

Dorland’s Electronic Medical Dictionary by Dorland, et al, Software, 29th Book & CD-Rom edition (2000), Harcourt Health Sciences, ISBN: 0721694934, http://www.amazon.com/exec/obidos/ASIN/0721694934/icongroupinter na

·

Dorland’s Pocket Medical Dictionary (Dorland’s Pocket Medical Dictionary, 26th Ed) Hardcover - 912 pages, 26th edition (2001), W B Saunders Co, ISBN: 0721682812, http://www.amazon.com/exec/obidos/ASIN/0721682812/icongroupinter na/103-4193558-7304618

·

Melloni’s Illustrated Medical Dictionary (Melloni’s Illustrated Medical Dictionary, 4th Ed) by Melloni, Hardcover, 4th edition (2001), CRC PressParthenon Publishers, ISBN: 85070094X, http://www.amazon.com/exec/obidos/ASIN/85070094X/icongroupintern a

·

Stedman’s Electronic Medical Dictionary Version 5.0 (CD-ROM for Windows and Macintosh, Individual) by Stedmans, CD-ROM edition (2000), Lippincott Williams & Wilkins Publishers, ISBN: 0781726328, http://www.amazon.com/exec/obidos/ASIN/0781726328/icongroupinter na

·

Stedman’s Medical Dictionary by Thomas Lathrop Stedman, Hardcover - 2098 pages, 27th edition (2000), Lippincott, Williams & Wilkins, ISBN: 068340007X, http://www.amazon.com/exec/obidos/ASIN/068340007X/icongroupinter na

·

Tabers Cyclopedic Medical Dictionary (Thumb Index) by Donald Venes (Editor), et al, Hardcover - 2439 pages, 19th edition (2001), F A Davis Co, ISBN: 0803606540, http://www.amazon.com/exec/obidos/ASIN/0803606540/icongroupinter na

171

INDEX A Abdominal..............................................91 Abscess .................................................84 Adenosine................................40, 71, 157 Adolescence ....................15, 75, 157, 165 Angiography.....................................50, 91 Antibody.................................................52 Antihypertensive ....................................46 Antioxidant .......................................42, 47 Apnea ............................................68, 133 Apnoea ............................................58, 64 Arteries ......................50, 72, 92, 158, 159 Arteriography .........................................91 Arteriovenous.........................................92 Arthropathy ............................................64 Assay .....................................................52 Ataxia.............................................85, 109 Atrophy ......................16, 57, 63, 109, 162 Aura .......................................................99 Autopsy..................................................63 B Bacteria................. 93, 102, 128, 163, 168 Barotrauma ............................................85 Benign....................................................85 Biochemical ...........................................48 Buccal ......................................43, 72, 158 C Capsules..............................................131 Carbohydrate .......................................130 Cardiac ....................................48, 74, 164 Cardiovascular...........................46, 62, 81 Catheter ...........................................50, 91 Cerebellar ..................................30, 57, 85 Cerebellum ..............................36, 85, 159 Cerebrospinal ..........................15, 55, 162 Cerebrovascular ..................43, 45, 53, 99 Chemoreceptor ......................................65 Cholesterol.....................74, 128, 130, 163 Chromosomal ........................................82 Chronic .... 16, 45, 47, 85, 93, 98, 99, 146, 162, 168 Cloaca....................................................90 Collapse.................................................62 Contusion.........................................58, 85 Coronary ................................................49 Cortical.....................................16, 99, 167 Cues ......................................................47 Cyst..................................................30, 56 Cystitis ...................................................98 D Degenerative .........................76, 129, 167

Depolarization ....................................... 40 Diarrhea .............................................. 128 Dislocation ...................................... 85, 90 Distal ....................................... 63, 76, 166 Dizziness................................. 85, 88, 169 Dysphagia ............................................. 61 Dysplasia .................................. 58, 61, 91 E Ectopic .................................................. 90 Endocrinology ......................... 83, 92, 161 Endogenous.................................... 48, 52 Enzyme ....... 51, 73, 75, 76, 160, 165, 167 Epidural................................................. 84 Epiphyseal ............................................ 58 Estrogens.............................................. 53 F Fatigue .................................................. 98 Fibrin ..................................................... 45 Fibrosis ................................................. 69 G Gynecology ........................................... 91 H Hemodilution ......................................... 92 Hemorrhage .............................. 43, 45, 85 Herpes .................................... 85, 88, 161 Hybridization ......................................... 52 Hydrocephalus .................................. 4, 14 Hypotension .......................................... 68 Hypothalamic ........................................ 47 Hypoxia ........................................... 41, 44 I Immunohistochemistry.......................... 52 Infarction ............... 43, 45, 48, 77, 85, 169 Insulin...................................... 44, 74, 162 Interstitial............................................... 98 Intestinal.............................................. 128 Invasive................................................. 42 Ischemia............ 41, 44, 47, 48, 49, 51, 85 L Labyrinthitis........................................... 85 Lesion ..................................... 16, 42, 165 Lipoprotein .............................. 46, 74, 163 Lumbar.................................................. 55 Lumen ................................................... 42 M Malignant ................................ 85, 87, 158 Medullary .............................................. 85 Meningitis................................ 63, 74, 163 Mental 16, 49, 73, 76, 102, 118, 120, 148, 160, 161, 162, 166 Metabolite ........................................... 131

172 Chiari Malformation

Microdialysis ..........................................52 Molecular 71, 76, 104, 107, 108, 157, 166 Morphogenesis ......................................80 Mycoplasma...........................................98 N Neural ............................16, 102, 161, 165 Neuronal ..............................40, 41, 44, 47 Neurons .....................51, 74, 77, 164, 168 Neurosurgery .........................................83 Niacin...................................................128 Nystagmus.................................56, 65, 66 O Osteoporosis..........................................59 Osteotomy .............................................90 Otitis.......................................................85 Otolaryngology.......................................84 Otosclerosis .............................85, 88, 165 Overdose .............................................129 P Paralysis ........................................16, 165 Perindopril..............................................46 Physiologic.................40, 76, 88, 161, 166 Plasminogen ..........................................45 Posterior ............. 15, 60, 65, 88, 159, 165 Postmenopausal ....................................52 Postoperative.........................................69 Potassium ......................................48, 130 Precursor .......................................75, 165 Presbycusis ...........................................85 Prevalence.............................................44 Proteins..........................73, 128, 129, 162 Proximal...................................50, 73, 160 Psychiatry ............................................166 Pulmonary................................77, 90, 169 R Radiology...............................................90 Receptor ..................................41, 72, 159

Recombinant......................................... 45 Regeneration ........................................ 69 Renin..................................................... 46 Resection ........................................ 69, 90 Riboflavin ............................................ 128 Rickets .......................................... 59, 133 S Seizures ...................... 14, 16, 44, 99, 167 Selenium ............................................. 130 Semantics ............................................. 70 Shunt........................... 16, 67, 91, 92, 167 Skeletal ................................... 75, 91, 164 Skull ............................ 16, 30, 54, 89, 162 Spectrum............................................... 98 Stenosis .............................. 50, 52, 90, 98 Subacute............................................... 92 Subarachnoid.................................. 43, 60 Synaptic ................................................ 40 Syncope ................................................ 44 Systemic ................................. 49, 77, 169 T Thermoregulation................................ 128 Thoracic .................................... 67, 90, 98 Thrombolytic ........................... 45, 77, 168 Thrombosis ............... 16, 45, 77, 167, 169 Thyroxine ............................................ 129 Tinnitus ................................... 85, 88, 164 Tomography.................................... 70, 99 Transfusion ................................... 92, 161 Tremor .......................................... 66, 109 V Ventricular....................................... 48, 90 Vertigo....................... 84, 85, 88, 164, 169 Vestibular .............................................. 57 Viral ....................................................... 85 W Warfarin .......................................... 50, 52

Index 173

174 Chiari Malformation

The Official Parent's Sourcebook on Chiari Malformation: Updated Directory for the Internet Age

The Official Patient's Sourcebook on Botulism: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Psoriasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Genital Candidiasis: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Ataxia Telangiectasia: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Shigellosis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Brainerd Diarrhea: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Adrenoleukodystrophy: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Balantidiasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Coccidioidomycosis: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Psoriasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Toxocariasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Intraocular Melanoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Opsoclonus Myoclonus: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Shingles: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Penile Cancer: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Hansen's Disease: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Cerebral Atrophy: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Prolactinoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Macular Pucker: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Sjgren's Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Dry Mouth: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Prostatitis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Ketamine Dependence: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Mdma Dependence: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Tourette Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Hyperopia: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Pheochromocytoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Bladder Cancer: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Sjgren's Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Kaposi's Sarcoma: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Chiari Malformation: Updated Directory for the Internet Age

The Official Patient's Sourcebook on Botulism: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Psoriasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Genital Candidiasis: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Ataxia Telangiectasia: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Shigellosis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Brainerd Diarrhea: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Adrenoleukodystrophy: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Balantidiasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Coccidioidomycosis: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Psoriasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Toxocariasis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Intraocular Melanoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Opsoclonus Myoclonus: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Shingles: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Penile Cancer: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Hansen's Disease: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Cerebral Atrophy: A Revised and Updated Directory for the Internet Age

The 2002 Official Patient's Sourcebook on Prolactinoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Macular Pucker: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Sjgren's Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Dry Mouth: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Prostatitis: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Ketamine Dependence: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Mdma Dependence: A Revised and Updated Directory for the Internet Age

The Official Parent's Sourcebook on Tourette Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Hyperopia: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Pheochromocytoma: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Bladder Cancer: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Sjgren's Syndrome: A Revised and Updated Directory for the Internet Age

The Official Patient's Sourcebook on Kaposi's Sarcoma: A Revised and Updated Directory for the Internet Age

Recommend Documents