Parkinson's Disease and Parkinsonism in the Elderly

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Parkinson's Disease

Paikinsonism Elderly £

Parkinson's diseaseand andparkinsonism parkinsonism in in the the elderly Parkinson's disease

disease and and parkinsonism parkinsonism are particular problems in the Parkinson's disease the elderly, elderly, causing disability disability the clincial clincial features, features, diagnosis diagnosisand and management management covers the and impairing quality quality of life. This book covers in elderly elderlypeople, people, dealing dealingnot not only onlywith withthe themotor motor symptoms but also of Parkinson's disease in also probproblems such as depression and autonomic autonomic nervous nervous system system dysfunction. dysfunction. ItIt stresses stresses particularly the importance of rehabilitation and total patient care. Topics covered include: Topics Differential diagnosis diagnosis and and epidemiology •• Differential • Drug-induced Drug-induced parkinsonism parkinsonism and and gait gait apraxia apraxia • The The drug treatment of of elderly elderly patients • Cognitive Cognitive impairment in in elderly elderly patients with Parkinson's Parkinson's disease • The The multidisciplinary rehabilitation team

A comprehensive, comprehensive, practical practical and and enlightened enlightenedaccount accountofofParkinson's Parkinson'sdisease diseasein in the theelderly, elderly, drawing drawing on international experts, experts, this this book book will will appeal appeal to to clinicians clinicians in in the thevarious variousdisciplines disciplines involved involved with neurological disease disease and rehabilitation in in elderly elderly people. people.

Jolyon Meara is is Senior Lecturer Lecturer in Geriatric Geriatric Medicine Medicine at at the the University University of ofWales Wales College College of Medicine. William C. Koller Neurology at the University of Kansas KansasMedical MedicalCenter. Centet Koller isis Professor Professor of Neurology University of

Parkinson's disease and parkinsonism parkinsonism in the in the elderly elderly Edited by

Dr Jolyon Meara University Department of Geriatric Medicine (North Wales) University Department of Geriatric Medicine (North Wales)

and and

Professor William Professor William C C Koller Koller University of Kansas Kansas Medical Medical Center, Center, Kansas Kansas City City University

I

CAMBRIDGE j CAMBRIDGE UNIVERSITY UNIVERSITY PRESS PRESS

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Information on this title: www.cambridge.org/9780521628846 © Cambridge University Press 2000 publication is in copyright. copyright. Subject Subject to statutory statutory exception exception This publication and to the provisions of relevant collective collective licensing licensing agreements, agreements, of any any part part may may take take place place without without no reproduction of of Cambridge Cambridge University University Press. Press. the written permission of

First published 2000 Reprinted 2004 catalogue record is available from thethe British Library A catalogue recordfor forthis thispublication publication is available from British Library

Library data Library of Congress CongressCataloguing CataloguingininPublication Publication data Meara and and Parkinson's disease and parkinsonism in the elderly / edited by Jolyon Meara William C. C. Koller. Koller. cm. p. ;; cm. Includes bibliographical references and index. index. ISBN 0 521 62884 9 (pb :: alk. paper) 1. Parkinson's disease. disease. 2. Aged Aged—- Diseases. I. Meara, Meara, Jolyon, Jolyon,1956— 1956- . II. II. Koller, William .

C., 1945— 1945- . [DNLM: [DNLM: 1. Parkinson Disease Disease —Aged. -Aged. WL 359 P2467 2000] RC382.P258 2000 2000 618.97'6833-dc21 99-057614 618.97'6833—dc2l 99—057614 ISBN-13 978-0-521-62884-6 ISBN-13 978-0-521-62884-6 paperback ISBN-b 0-521-62884-9 0-521-62884-9 paperback ISBN-10 Transferred to digital printing 2006 Transferred

Every effort effort has has been been made made in in preparing preparing this this publication publication to to provide provide accurate accurate and and up-to-date up-to-date information information Every which is in accord with accepted standards and practice at the time of publication. Nevertheless, the authors, authors, publisher can make make no no warranties warranties that that the the information information contained contained herein herein isis totally totally free free from from error, error, editors and publisher not least because clinical standards standards are constantly changing through research and regulation. The The authors, authors, editors and publisher publisher therefore therefore disclaim disclaim all liability liability for for direct direct or consequential damages resulting from from the the use use publication. The reader reader is is strongly strongly advised advised to to pay pay careful careful attention to information of material contained in this publication. information provided by the manufacturer manufacturer of of any any drugs drugs or or equipment equipment that that they they plan plan to touse. use.

Contents

contributors List of contributors Foreword Foreword 1

1

A A glossary of terms

page vii ix 11

Jolyon Meara 2

Diagnosis of parkinsonism parkinsonism in the elderly Diagnosis of

4

Robert L. Rodnitzky

3

Parkinson's disease and and parkinsonism parkinsonism in in the elderly Parkinson's disease

22

Jolyon Meara Meara and and Bimal K. Bhowmick Jolyon

4

Drug-induced parkinsonism in the elderly Drug-induced parkinsonism

64

P.. Hubble Hubble Jean P

5

Essential tremor in the elderly Essential tremor

80

Rajesh Pahwa and William C. C. Koller Koller

6

Gait apraxia apraxia and and multi-infarct multi-infarct states states

98

Richard Liston Tallis Liston and andRaymond RaymondC.C.Talus

7

The epidemiology of Parkinson's and parkinsonism parkinsonism in elderly Parkinson's disease disease and elderly subjects

111 111

Jolyon Meara and Peter Hobson 8

Health and social needs needs of people with with Parkinson's Parkinson's disease disease and the organization of of their their care care worldwide organization

122 122

Peter Hobson

9

The drug treatment of Parkinson's Parkinson's disease in elderly people Theresa A. Zesiewicz and Robert A. Hauser

V

134 134

vi vi

10 10

Contents

and parkinsonism Rehabilitation in in Parkinson's Parkinson's disease and

165

Christopher D. D. Ward Ward 11 11

and elderly patients with Rehabilitation, nursing and with Parkinson's Parkinson's disease disease

185

Sally Roberts 12 12

and elderly patients with Rehabilitation, physiotherapy and with Parkinson's Parkinson's disease

198

Hilary Chatterton and and Brenda Brenda Lovgreen Lövgreen 13 13

occupational therapy therapy and and elderly elderly patients patients with with Rehabilitation, occupational Parkinson's disease

217

Jackie Hughes Hughes

14 14

and language language therapy therapy and and elderly elderly patients patients with Rehabilitation, speech and with Parkinson's disease

226

Sheena Round

Index

240

Contributors

Bimal K. K. Bhowmick MD MD FRCP F RCP Consultant Physician Physician in in Geriatric Medicine, Clwyd District General Hospital, Rhyl, Glan Clwyd Denbighshire, North NorthWales Wales LL18 LL18 5UJ, UK. UK.

HilaryChatterton Hilary ChattertonMSC MSC MCSP MCSP Lecturer, School of Physiotherapy, Infirmary, Manchester Manchester Manchester Royal Royal Infirmary, M13 9WL, UK. Robert A. Robert A. Hauser Hauser MD MD Associate Professor, College College of Medicine, Medicine, University of South Florida, Department of University of South Florida, Department of Neurology, Harbour HarbourSide, Side,Medical MedicalTower, Tower, 44

Columbia Drive, Drive, Suite 410, Tampa, Florida 33606, USA. BSC Peter Hobson HobsonBSC University Department Department of Geriatric University Medicine, Glan Clwyd District General Hospital, Rhyl, Denbighshire, North North Wales Wales LL18 5UJ, UK. Hubble MD P. Hubble MD Jean P. Co-Medical Director, Co-Medical Director, The Ohio State 1581 University, University Medical Center, 1581 Dodd Drive, McCampbell Hall, Hall, Suite 371, Columbus, Ohio Ohio 43210, 43210, USA. USA.

Jackie Hughes dip COT Occupational Therapist, Colwyn Colwyn Bay Bay Community Hospital, Community Hospital,Colwyn ColwynBay, Bay,Conwy, Conwy, North NorthWales WalesLL29 LL298AY, 8AY, UK. UK. VII vii

William C. Koller MD PhD MD PHD Professor and and Chairman, Chairman, Department Department of Professor Neurology, The The University University of ofKansas Kansas Medical Center, 3901 Rainbow Boulevard, Boulevard, Kansas City, Kansas 66160, 66160, USA. USA. Richard Liston F R C P1 RichardListon FRCPI

Consultant Physician Physician in in Geriatric Medcine, Tralee Tralee General General Hospital, Tralee, Tralee, County County Kerry, Eire Msc MCSP Brenda LOvgreen Lövgreen MSC MCSP Lecturer, School of Physiotherapy, Infirmary, Manchester Manchester Manchester Royal Royal Infirmary, M13 9WL, UK.

JolyonMeara Jolyon Meara MD MD FRCP FRC P Lecturer in Geriatric Medicine, Senior Lecturer University of Wales College of Medicine, Clwyd District General Hospital, Rhyl, Glan Clwyd Denbighshire, North NorthWales Wales LL18 LL18 5UJ, UK. UK. Rajesh Pahwa Pahwa MD MD Assistant Professor, Professor, Department of of Medicine, The The Neurology, School of University of Kansas Medical Center, 3901 Rainbow Boulevard, Boulevard, Kansas Kansas City, City, Kansas 66160, USA. GN Sally Roberts RobertsitRGN Disease Nurse, Nurse, Health Health Care Care of of Parkinson's Disease Elderly Department, Department, Glan Glan Clwyd Clwyd District District the Elderly Hospital, Rhyl, Denbighshire, North North Wales Wales LL18 5UJ, UK.

viii viii

List of contributors contributors Robert L. Rodnitzky MD L. Rodnitzky MD Professor of Neurology, Neurology, The The University University of of Professor Iowa Hospitals Hospitals and and Clinics, Clinics,Department Department of of Iowa Neurology, 200 Hawkins Drive, Drive, Iowa Iowa City, Iowa 522242—1053, 522242-1053, USA.

SheenaRoundBsc dipCCS c c sMCSLT MCSLT Sheena Round BSCDip Speech and Language Therapist, Glan Glan Clwyd Clwyd District General Hospital, Rhyl, Rhyl, Denbighshire, North Denbighshire, NorthWales WalesLL18 LL185UJ, 5UJ, UK. UK. C. Tallis Talus D D lLitt i t t FRCP FRCP Raymond C. Professor of Geriatric Medicine, The Professor University of of Manchester, Manchester, Hope Hospital, University Manchester M6 M6 8HD, 8HD, UK. UK.

Christopher CP WardMD MDF RFRCP ChristopherD.D.Ward

Profess ofof of Rehabilitation Rehabilitation Medicine, Professof University of of Nottingham, Nottingham, Faculty of of University Medicine and and Health Health Sciences, Sciences, Rehabilitation Research Unit, Derby City General Hospital, Uttoxeter Road, Derby DE22 3NE, UK. Theresa A. Zesiewicz Zesiewicz MD MD TheresaA.

Associate Professor of Neurology, College of Florida, Medicine, University of South Florida, Department of Neurology, Neurology, Harbour Side Medical Tower, 4 Columbia Drive, Drive, Suite Suite 410, 410, Florida 33606, 33606,USA. USA. Tampa, Florida

Foreword

No illness illness can can be be contained contained in a vacuum and and Parkinson's Parkinson's disease disease is is no no exception. exception.

Legislation by by Governments, Governments, awareness awareness and and real real understanding understanding of Legislation of this challenging challenging illness profile illness by by the the healthcare healthcare professionals, professionals,and and the the change change in in the the demographic profile of our nation will will have major implications for for the families families impacted by this illness. The population of of the the world world isis living living longer and with old age age there are increasing numbers of of chronic chronic neurological neurological illness illness such such as as stroke, stroke, Alzheimer's Alzheimer's and ing numbers Parkinson's - and yet these people attract scant attention. attention. Parkinson's Parkinson's in in the the elderly elderly is aa common Failure to manage manage itit well well results in distress and guilt guilt common clinical problem. Failure family and expenditure of untargeted resources resources within the community. community. within aa family This book examines the diagnosis diagnosis and and the the management management of of Parkinson's Parkinson's in in both both examines the The editors editors are are distinguished distinguished not not only by their expertise the UK UK and USA. USA. The expertise in the management of of Parkinson's but also also well well known known for for their their compassion and desire to improve services. Our elderly population should should benefit benefit from from the the contents contents of of this this book book and and I elderly population rejoice rejoice that that such such eminent people have have given given their their time time and and expertise expertise to to contribute to such a worthwhile project. Mary G Baker MBE President of the the European European Parkinson's President Parkinson's Disease Disease Association Association (EPDA) (EPDA)

ix ix

A glossary of terms Jolyon Meara Jolyon Meara

As disease and and parkinsonism increases As our knowledge of Parkinson's disease increases considerable

confusion confusion can arise in relation to the the terms terms used used to to describe describe these these conditions, conditions. To To maintain consistency consistency in the text the following following definitions will be be used. used.

Parkinsonism A A clinical syndrome of akinesia accompanied by rigidity and often tremor. Akinesia includes difficulty difficulty with with voluntary voluntary motor actions, includes actions, difficulty difficulty performing sequential sequential concurrent motor motor actions, actions, slowness slowness of voluntary voluntary movement, movement, and abnormal or concurrent and abnormal fatigability actions. Rigidity, Rigidity, or 'stiffness', 'stiffness', can fatigability of of repetitive repetitive motor actions. can be be defined defined as the resistance an examiner examiner when when passively passively stretching relaxed relaxed muscles muscles resistance encountered by an Rigidity in parkinsonism parkinsonism can can often often be be detected detected in in the the axial axial skeleton skeleton around aa joint. Rigidity and upper limbs by the examiner performing passive flexion/extension flexion/extension movements Tremor is is often often present at at rest rest when when the the muscles muscles are are of the neck and wrist joint. Tremor fully fully relaxed relaxedand and isis usually usuallyfirst firstnoted notedininthe the upper upper limb limb involving involvingthe the hand hand and and fingers. occur. Parkinson's Parkinson's disease disease is the fingers. Leg Leg and and jaw jaw tremor tremor may less commonly occur. most cause of parkinsonism and arises arises sporadically sporadically and most common cause and is is of of unknown cause. Known Known causes causes of ofparkinsonism parkinsonism include drugs, cerebrovascular disease, other sporadic and inherited neurodegenerative neurodegenerative disease, disease, infections, head trauma, hydrodiseases, amongst cephalus, and metabolic diseases, amongst others.

Parkinson's disease (PD) Levodopa-responsive parkinsonism parkinsonism resulting resulting in in aa characteristic clinical picture and When present, a typical 'pill rolling' rolling' tremor tremor involving the thumb natural history. When typical 'pill involving the and is almost almost pathognomic pathognomic for for PD PD or drug-induced and index finger finger is drug-induced parkinsonism. parkinsonism. The primary neuropathological neuropathological findings findings consist consist of of degeneration of of cells cells in the substantia nigra pars compacta resulting in striatal dopamine dopamine deficiency deficiency and the presLewy bodies. ence in surviving cells of inclusions called Lewy bodies. Other Other discrete areas of the also demonstrate demonstratecell cellloss loss and and Lewy Lewy bodies. brain also

2

J. Meara

Late stage PD PD Late stage PD PD is associated associated with with significant significant functional fnnctional disability disability and and the the onset of Late stage

increasing increasing dependency. dependency. The The clinical clinical picture picture in in late late stage stage disease disease isis dominated dominated by features that do not respond respond to to dopaminergic dopaminergic drugs, drugs, such such as as poor poor mobility, mobility, falls, falls, features confusion, communicaconfusion, drowsiness, drowsiness, dementia, dementia, sialorrhoea, sialorrhoea, dysarthria, dysarthria, impaired impaired communicadysphagia, and and weight weight loss, loss. tion, dysphagia, Late onset PD PD

onset PD can be arbitrarily defined PD presenting for the first time in subLate onset defined as PD jects who are aged 70 years jects who years or older. older. Even Even after after adjusting adjusting for age, age, late onset PD PD appears to progress faster faster and to be associated with the earlier development of of cogcognitive possibly depression. depression. In late onset disease disease gait nitive impairment impairment and possibly gait and balance appear early early in the course course of of the illness. illness. The pattern of of disease disease at presenproblems appear tation in in elderly elderly subjects may may reflect the more widespread loss loss of of nigral nigral cells cells due to tation aging aging in in addition addition to the more circumscribed loss due to PD. PD. Drug-induced parkinsonism (DIP) (DIP) Parkinsonism resulting from exposure resulting from exposure to antidopaminergic drugs, drugs, usually usually due to

receptors. The clinical picture can be indistinguishable from from blocking of dopamine receptors. cause of of DIR DIP. PD. Neuroleptic drugs are by far far the most common cause Vascular parkinsonism Parkinsonism resulting from of the the brain. The clinical from vascular vascular disease disease of clinical picture is

dominated dominated by by gait gait disturbance, disturbance, truncal truncal ataxia ataxia with with relative relative sparing sparing of of the the upper absence of tremor. There may be associated limb and the absence associated evidence evidence of of upper upper motor involvement (pseudobulbar (pseudobulbar palsy, palsy, pyramidal history of neurone involvement pyramidal deficits) deficits) and and aa history stroke events, events. A imaging may may stroke A history history of of hypertension hypertension is often often present present and brain imaging demonstrate extensive extensive deep white matter ischaemic changes. Akinesia Akinesia of ofthe the upper upper is absent in in most most cases. cases. However, However, basal ganglia infarcts may rarely give rise limb is rise to to a clinical from PD. PD. clinical picture picture indistinguishable from Parkinsonism in multisystem neurodegenerative neurodegenerative disease disease Parkinsonism can arise from from sporadic disease such as multiple system atrophy, pro-

gressive supranuclear supranuclear palsy, palsy, corticobasal corticobasal degeneration, degeneration, Alzheimer's Alzheimer's disease disease and gressive dementia with Lewy Lewy bodies. bodies. Inherited degenerative degenerative diseases such as Huntington's Huntington's

3

Glossary Glossary of terms

parkinsonism and familial can canse cause parkinsonism familial parkinsonism, although very rare, is well described. described. Progressive Progressivesupranuclear supranuclear palsy palsy and and multiple multiple system system atrophy now well maybe mistaken mistaken for for PD PD early early in the natural history history of of the the disease disease as both these conditions may initially initially respond respond to to levodopa levodopa treatment. disease

Dementia with with Lewy Lewy bodies bodies (DLB) (DLB) A A progressive fluctuant dementia dementia associated associated with with variable degrees degrees of parkinsonism, parkinsonism, visual hallucinations, transient loss hallucinations, falls, falls, transient loss of consciousness and neuroleptic neuroleptic sensitivsensitivity. limbic ity. Cortical Cortical Lewy Lewybodies bodiesare areprominent prominent at at postmortem, postmortem, particularly in the limbic areas of Subcortical changes areas of the mesial mesial temporal temporal lobe. Subcortical changes identical identical to to those those found found in PD may also be present.

Gait apraxia Gait apraxia results from a failure failure of integration of of cerebral activity involving highlevel sensorimotor sensorimotor systems. walking cannot cannot be explained explained by by motor systems. The difficulty difficulty in walking or sensory abnormalities that can be detected detected by bybedside bedsideneurological neurological examination. examination. The term 'high level level gait gait disorder' disorder' is often often preferred. preferred. Lesions Lesions of ofthe the premotor premotor area, the supplementary supplementary motor area, area, the basal ganglia ganglia and connections appear to and their their connections development of of gait gait apraxia. apraxia. be related to the development

Essential tremor tremor (ET) (El) most common movement The most movement disorder disorder in in elderly elderly people. people. A A bilateral, bilateral, persistent persistent postural the hands and postural tremor involving involving the and forearms forearms of of longstanding longstanding duration is is Akinetic kinetictremor tremor on on movement may also be required for a definite diagnosis of ET. ET. A family history present. A family history of of tremor tremor is elicited, elicited, as as isis aa short short term term improvement improvement of tremor after after alcohol. alcohol. The The head, head, voice voice and and legs legs may may also also be involved involved with decreasdecreasing frequency. frequency. In elderly subjects ET is commonly misdiagnosed as as PD. PD.

Diagnosis of parkinsonism parkinsonism in the elderly Diagnosis of Robert LL Rodnitzky Rodnitzky

The diagnosis of parkinsonism depends depends on on recognizing recognizing its its component componentclinical clinicalfeafea-

tures. Parkinsonism includes the varied varied conditions includes Parkinson's disease (PD) and all the with clinical features features resembling resembling those those of of PD. PD. To Toidentify identifypatients patientswith with parkinsonparkinsonto be able to recognize the cardinal clinical clinical features ism correctly it is is important important to features of of namely akinesia, akinesia, lead-pipe instability. The PD, namely lead-pipe rigidity, rigidity, rest rest tremor, tremor, and postural instability. next critically step is is to determine determine whether whether they they suggest suggest PD PD or one one of of the the critically important important step other non-PD causes causes of of parkinsonism parkinsonism (see (see Table Table 2.1). This latter distinction distinction will will enable enable effective effectivetreatment treatment strategies strategiesto to be be devised devisedand and aa meaningful meaningful discussion discussion of of genetic implications to be undertaken. The The entire entire process process of of idenidenprognosis and genetic andassigning assigningaaspecific specific clinical clinical diagnosis is particularly particularly chalchaltifying parkinsonism parkinsonism and lenging in the elderly because because many many of of the the motor changes associated associated with with normal ageing ageing resemble resemble parkinsonism. parkinsonism. Additionally, Additionally, several several medical medical conditions conditions that that are common in this age group can result may incorrectly be conresult in parkinsonism that may evidence of of PD. PD. sidered evidence

The clinical signs signs of of PD PD Of the cardinal motor signs of Of motorsigns of PD, PD, akinesia akinesia is is perhaps the the most most disabling. disabling.Slowness, Slowness, difficultyinin initiation, initiation, and and aa reduction reduction in in the the amount amount or amplitude difficulty amplitude of of voluntary voluntary movement (Rodnitzky (Rodnitzky and and Uc Uc 1997) 1997) characterize characterize akinesia akinesia (see (seeTable Table 2.2), 2.2). A A great great variety of clinically recognizable recognizable signs result from akinesia. A lack of facial facial expresexpresreduced blink rate is one manifestations of sion attended by reduced one of the most apparent manifestations akinesia. sides of the akinesia. Additional Additional findings findings are are diminished diminished arm swing on one or both sides difficulty arising from body, difficulty from a chair, chair, aa slow, slow,short short stepped stepped gait, gait, en enbloc blocturning, turning, and soft, poorly articulated speech speech (hypophonia). (hypophonia). The The clinical clinical signs signs of of akinesia akinesia are are so so soft, their presence presence alone alone has been considered considered by some to be sufficient sufficient to striking that their establish 1995). The more usual view is that additional additional establish aa diagnosis diagnosis of of PD PD (Quinn 1995). findings are are necessary necessary to establish establish a diagnosis diagnosis of PD. PD. Rigidity Rigidity is another another motor findings common finding finding in in patients patients with withPD PD(see (seeTable Table 2.3). Rigidity is felt felt by by an an examiner examiner as an resistance to passive passive movement in the the fully fully relaxed relaxed limb. limb. an increased increased resistance movement of joints in 4

5

Diagnosis

Table 2.1 Stages in the diagnosis of parkinsonism Stages in parkinsonism

Stage 11 •• Is there there clinical clinical evidence evidence of of parkinsonism? • Akinesia must be present for for this this diagnosis. diagnosis. Stage 2 • V/hat What type of parkinsonism is is present?

Table 2.2 Clinical Clinical features features of of PD PD Akinesia

• Reduction with Reduction in in spontaneous spontaneous voluntary voluntary motor motoractivity, activity, slow slow movements, difficulty difficulty with sequential and and concurrent concurrent motor acts, abnormal early early fatiguability fatiguabiity and reduction in sequential amplitude of movements

Table 2.3 Clinical features of PD PD

Lead-pipe rigidity • Abnormal resistance resistance which remains constant throughout throughout the therange rangeof ofmovement movement and andisisfelt felt when passively stretching muscles muscles around around a joint in passively stretching in aa relaxed relaxed subject subject Cog-wheel rigidity rigidity Cog-wheel • A ratchet type of fluctuating resistance felt felt at at the the wrist wrist in in synchrony synchrony with with tremor tremor bursts

Resistanceisisunchanged unchangedthroughout throughout the the range range of of movement movement of of the the joint joint and can Resistance be distinguished in which which resistance greatest atat the the onset of distinguished from from spasticity, spasticity, in resistance isis greatest of passive movement and then suddenly gives way (clasp (claspknife knifephenomenon). phenomenon). Often, passive movement gives way is present as as the joint is is moved especially when a ratchet like quality (cog-wheeling) is tremor is is present. Subtle rigidity can be enhanced by utilizing utilizing reinforcement reinforcement techniques such as instructing the patient patient to to execute execute repetitive repetitive forceful forceful movements in the contralateral limb. distinguished from from gegenhalten gegenhalten in the contralateral limb. True True rigidity rigidity must must be distinguished in patients with diffuse diffuse encephalopathy or frontal lobe dysfunction exert exert a force which patients passive movement. opposite in direction to the examiner's attempted passive Rest tremor is 2,4), It It is one of of the the most most easily easily recognizable recognizable signs of PD (see (see Table Table 2.4). usually appears appears at aa frequency Hzwhen whenthe the limb limb is is fully fully supported supported and usually frequency of of 3—6 3-6 Hz and appears in the hands when the arms motionless. It also also appears arms are are suspended suspended at at the the sides sides during walking. Typically, Typically,the thetremor tremor isisreduced reducedor ortotally totallydisappears disappearsduring during action. action. In PD, tremor is often often unilateral illness and asymmetunilateral at the onset of the illness and remains remains asymmetrical presence or rical even even though though ultimately ultimately spreading spreading to the contralateral limbs. The presence absence of major consideration consideration when attempting attempting to to determine determine absence of rest rest tremor tremor is a major

6

R. L. L Rodnitzky R. Rodnitzky

Table 2.4 Clinical Clinical features of of PD PD

Rest tremor 4—5 oscillationinvolving involving distal portionofofthe theupper upperlimb limb of of 'pill 'pill rolling' rolling' • Classically Classically a 4-5 HzHzoscillation thethedistal portion Atypical rest rest tremor tremor can can occur and rest tremor is often often accompanied by postural postural and type. Atypical head and trunk are usually spared kinetic tremors. The head usually spared

whether a patient has PD or another form form of of parkinsonism. parkinsonism. Rest Rest tremor is is present in the great majority of of patients with PD, PD, but in in only only aa smaller percentage of those with other forms of parkinsonism. parkinsonism. The The distribution of tremor is also also important important in helping to establish diagnosis of of PD. PD. The The tremor tremor of PD commonly begins begins in the establish a diagnosis and is is slightly less hands and less common common in the lower extremities extremities and mandible. It almost never never affects affectsthe thehead head or or the the muscles muscles of of articulation. articulation. When When present, present, a 'pill rolling' at rest rest involving involving the the thumb thumband andindex indexfinger fingervery verystrongly stronglysuggests suggests aa diagdiagtremor at nosis of PD or drug-induced parkinsonism. Postural instability has a great number nosis of PD or drug-induced has a great number of causes other than PD, PD, particularly in the elderly. elderly. Patients of potential potential causes Patients manifesting this dysfunction dysfunction are at at increased increased risk risk of of falling falling since since they generate they are unable to generate normal reflex movement even the slightest perturbation their normal reflex movement toto counter counter even the slightest perturbation to to their posture. The The clinician clinician can can safely safely demonstrate an an absence absence of of postural postural reflexes reflexes by and applying applying a brisk backward backward directed standing behind behind the patient and directed push push on on the sternum. Episodes of of freezing, freezing, also also referred referred to to as as 'motor 'motor blocks', most commonly Episodes commonly involve involve gait. The The patient's patient's feet feet appear appear 'glued' 'glued' to to the the floor floor when when attempting attempting to to initiate initiate gait, gait, gait. during turns, or or when when approaching approaching aa real real or or imagined imagined obstacle obstacle such as as aa narrow narrow passageway ororan room. Whether Whether this phenomenon phenomenon represents represents a passageway an entrance entrance to to aa room, severe form form of akinesia akinesia or is physiologically physiologicallyseparate separateisisnot notknown. known. ItIt isis common common in late stage PD, but in some other forms of parkinsonism it can be an early, stage PD, but in some forms it can an early, or even a presenting presenting clinical sign (Giladi et al. 1991). Several guidelines guidelines have been been suggested for utilizing clinical signs to establish a diagnosis of clinically probable for signs to establish diagnosis of clinically probable PD. PD. Definite Definite diagnosis PD. Most Most guidelines guidelines diagnosis strictly strictly requires requires postmortem postmortem confirmation confirmation of of PD. require a certain certain number number of the cardinal cardinal motor signs of of parkinsonism parkinsonism to to be be present present of PD PD in in life. life. For example, the UK UK Parkinson's Disease Disease Society Society to make a diagnosis of Brain Bank criteria (Hughes (Hughes et et al. al. 11992a) 992a) requires requires the presence of akinesia plus one other clinical sign instability. Koller Koller sign from from among rigidity, rest rest tremor, and postural instability. (1995), on the other hand, suggested that any two of three motor findings from (1995), on hand, suggested motor findings from among rigidity, akinesia, is sufficient sufficient to establish a clinical akinesia, and and tremor is clinical diagnosis diagnosis of of PD. While these criteria increase increase diagnostic diagnostic accuracy, accuracy, they they are are far far from from infallible. infallible. Several Several studies studies have have suggested suggestedaahigh high level levelof of diagnostic diagnostic inaccuracy inaccuracy compared compared to to postmortem findings, findings, even even when when the the clinical clinical diagnosis diagnosis of of PD PD is is made made by by experiexperienced al. 1991a, 1991a, Hughes et al. al. 1992a, 1992a, de Rijk et al. 1997). 1997). enced neurologists neurologists (Rajput et al.

7

Diagnosis

Rajput et al. (1991a) Rajput (199 1a) found that that only only 76% 76% of of patients patients with with aafinal final clinical clinical diagnosis diagnosis of PD during life had evidence of the disease when examined at autopsy. Hughes et al. (1992a) ( 1992a) examined 100 100 brains of patients with a final clinical diagnosis of PD and could confirm such aa diagnosis in only only 76% 76% of cases. cases. The diagnosis in the remainremainder included included conditions conditions such such as as progressive progressive supranuclear supranuclear palsy palsy (PSP), (PSP), multiple multiple system system atrophy (MSA), and Alzheimer's Alzheimer's disease disease (AD). (AD). The The clinicians clinicians in in this this study study had utilized clinical clinical criteria criteria of of their their choice choice in in arriving arriving at a diagnosis diagnosis of of PD. PD. When When systematized systematized diagnostic clinical criteria were retrospectively applied to cases in in this study diagnostic accuracy improved to 82%. In clinical practice diagnostic accustudy diagnostic accuracy improved clinical practice diagnostic accuracy suggest since racy is is likely likely to to be be much much lower lower than these figures suggest since these these studies studies only only 10 years looked at final diagnosis. However, all patients in these studies died at least patients at 10 interim diagnostic diagnosticawareness awareness may may have have improved improved as as is is suggested by ago and in the interim the most recent recent clinicopathological clinicopathological series series (Ansorge (Ansorge et al. 1997). 1997). When applying When applying generated criteria criteria with with aa high high predictive predictive value value for for diagnostic diagnostic additional computer generated accuracy (asymmetrical onset, no atypical features and no other possible etiology), features the diagnostic accuracy accuracy was increased to 93%, but 32% 32% of of pathologically pathologically was further further increased confirmed cases were rejected on this basis. A further study compared eight confirmed cases were rejected on this basis. A further study compared eight different sets applied to to prevalence prevalence studies of different sets of of diagnostic diagnostic criteria criteria that might be applied PD and found some some sets sets too inclusive inclusive and others others too too restrictive restrictive (de (deRijk Rijk et et al. al. 1997). 1997). These authors concluded that that the the most most reasonable reasonable inclusion inclusioncriteria criteriafor for PD PD was was two of the three cardinal features (tremor, akinesia and rigidity) in the absence of features (tremor, akinesia and rigidity) in the absence ofother other apparent causes of parkinsonism. It is clear clear from from these these studies studies that that PD PD can can be be disdistinguished from other forms of parkinsonism on clinical grounds alone with high, from clinical alone with high, not total, total, accuracy. accuracy. Using the most stringent diagnostic diagnostic criteria criteria reduces reduces misdimisdibut not agnosis, but misclassifying aa significant cases of agnosis, but at the expense of misclassifying significant number number of of true cases of PD. PD.

Controversy still still exists exists over overthe thecondition condition of of 'tremor 'tremor dominant' dominant' PD. PD. In In this situsituation tremor tremor isis an an isolated isolated finding finding and and this this can can easily easily lead to misdiagnosis with the most common common cause cause of of isolated isolated tremor, tremor, essential essential tremor. Rest Rest tremor, accompanyaccompanying the more usual postural and kinetic tremor, tremor, can can be a late manifestation of of essential tremor in elderly subjects (Rajput et al. 1993). Tremor dominant tial tremor in elderly subjects (Rajput et 1993). Tremor dominant cases cases of of presumed usefully be challenge test presumed PD could usefully be assessed assessedwith with an an apomorphine apomorphine challenge test to to help distinguish PD PD from from other other causes causes of of isolated isolated tremor. tremor.

Atypical features suggesting suggesting diagnoses other than than PD PD Despite the caveat that diagnostic diagnostic criteria criteria can can be be made made so so specific specific as to reduce their clinical utility, careful search features clinical utility, the the usefulness usefulness of of incorporating incorporating a careful search for for atypical atypical features as an exclusionary criterion for PD deserves special mention. Atypical features an exclusionary special mention. Atypical features not only alert the clinician clinician to the possibility possibility that that the diagnosis may may not be PD, but but in aa

8

R. L Rodnitzky R. L. Rodnitzky

positive sense may may suggest suggest another another form form of parkinsonism and and lead lead the the clinician clinician to to

the correct diagnosis. In this this regard regard itit is is important important to understand which diagnosis. In which facets facets of the natural history atypical for for history or or clinical clinical examination are are to be considered highly atypical strongly suggest suggest another anotherclinclinPD as well as as which which of of them them singly singly or or in combination strongly ical ical diagnosis diagnosiswithin within the the spectrum spectrum of of parkinsonism. reasonably well well defined defined body body of of clinical clinicalsigns signsand and symptoms symptoms that that are There is a reasonably distinctly these signs signs appear distinctlyunusual unusualinin PD. PD.When Whenone one or or more more of of these appear in in the akinetic-rigid akinetic—rigidorortremulous tremulouspatient, patient,they theyshould shouldprompt prompt the the clinician clinician to to question diagnosis of PD. PD. The following following discussion of the diagnosis discussion describes describesthe the most most important important of atypical signs signs and indicates indicates which alternative alternative diagnoses diagnoses each one suggests. suggests. these atypical Several likely to occur in in elderly elderly subjects, subjects, Several of of these these atypical atypical features features are are much more likely in this this making their identification identification and and proper proper interpretation even more important in age group. Early dementia

Dementia is is common common in PD, having having been been found found in as many as 65% of of patients by

the age age of of 85 85 years years (Mayeux (Mayeux et al. al. 1990). 1990). However, However, the dementia of of PD PD seldom seldom appears at the onset appears onset of of the theillness. illness. Early Early dementia dementia ininthe theakinetic—rigid akinetic-rigid patient consideration of of aa variety variety of other syndromes syndromes with parkinsonian parkinsonian should prompt consideration features, including dementia dementia with withLewy Lewy bodies bodies (DLB), (DLB), PSP, PSP, corticobasal ganglionic ganglionic degeneration (CBGD), (CBGD), normal normal pressure pressurehydrocephalus, hydrocephalus,Creutzfeldt—Jacob Creutzfeldt-Jacob disease, disease, or AD. appear AD. The The confusion confusion with with AD AD arises arises from from the the fact fact that that parkinsonism can appear in some patients with with AD AD (Hughes (Hughes etet al, al. 1992a, 1992a, Hulette Hulette et et al, al. 1995), 1995). These These signs signs are are usually thought the illness illness and are most likely likely to seen in the usually thought to occur late in the to be be seen elderly severe cognitive (Lopez et et al. al. 1997). 1997). However, However, in elderly patient patient with severe cognitive impairment (Lopez of Hughes Hughes et et al. al. (1992a) (1992a) AD was a common cause cause of diagnostic error error in in the study of PD and and itit appears appears from from this this study study that thatAD, AD,particularly particularly when when AD AD pathology pathology PD involves the parkinsonism sufficient sufficient to involves the corpus corpus striatum, striatum, can can present present with parkinsonism to lead lead to to diagnostic confusion confusion with PD. The The development development of of mild mild parkinsonian parkinsonian features one or more years after after the onset onset of of otherwise otherwise clinically clinically typical AD, especially especially in in an an elderly elderly patient, patient, should should be be considered considered to be be aa case case of ofAD ADwith withparkinsonism, parkinsonism, rather rather PD with with dementia. dementia. In In the the elderly elderly patient, patient, the the possibility possibility of of the the concurrent concurrent than PD appearance and AD AD should should also also be be given given consideration, consideration, taking taking into into appearance of of both both PD and account prevalence of age group. In this this account the high prevalence of both both of these conditions in this age circumstance, the presence presence of of aa rest resttremor tremor or or aa significant significantimprovement improvement in in parkinparkinsonism after after treatment with levodopa levodopa lends some support to to aadiagnosis diagnosis of of PD, PD, sonism rather than parkinsonism due due to to AD AD alone. alone.

Certain characteristics of of the the dementia Certain clinical clinical characteristics dementia may may also also be atypical atypical for for PD. PD. Marked Marked fluctuations fluctuations in cognitive cognitive impairment consisting consisting of periods of of confusion confusion alternating with lucidity, lucidity, for for example, example, should should suggest suggest DLB DLB (Byrne (Byrne et et al, al. 1989, 1989, Mega Mega

9

Diagnosis

1996). The The differentiating differentiating characteristics characteristics of the dementia associated al. 1996). associated with AD and those fonnd found in in PD PD have have been been well well stndied. studied. The dementia dementia of of AD AD is is more more likely likely to be associated associated with language and presence of with abnormalities abnormalities of of memory memory and langnage and the presence of anosognosia while anosognosia while that that of of PD PD is is more more commonly commonly characterized characterized by by impairment impairment of of visuospatial and executive functions (Mohr et al. al. 1990, 1990, Starkstein Starkstein et et al. al. 1996, 1996, visnospatial and execntive fnnctions (Mohr Mahieuxetal. Mahienx et al. 1998). Early hallncinations have have implications implications similar similar toto those those of Early hallucinations of early early dementia. dementia. Hallncinations occnrring illness and and Hallucinations occurring in in PD PD typically typically appear late in the conrse course of the illness are almost almost always always associated antiparkinsonian drngs. drugs. are associatedwith withthe the chronic chronic use nse of antiparkinsonian Hallucinations the first first Hallncinationsoccurring occnrringprior priortoto the the initiation initiation of of such snch drugs drngs or or with with the administration of of these these agents agents strongly strongly snggests suggests another another diagnosis, diagnosis, particnlarly particularly administration DLB. out the the importance importance of ofaccnrately accurately distingnishing distinguishing DLB. This This phenomenon phenomenon points points ont between PD and DLB. DLB. Compared Compared to PD, PD, DLB DLB patients are mnch much more more likely likely to between patients are suffer snffer behavioural behavionral side side effects effectsfrom fromdopaminergic dopaminergic or or anticholinergic anticholinergic agents. agents. et

Early falls and and postural postural instability

typically the the result resnlt of of impaired impaired postural postnral reflexes, reflexes,postural postnral hypotenFalling in PD is typically sion or severe large amplitnde amplitude dyskinesias. dyskinesias. Severe Severe freezing freezing with with inability inability to to check forward upper trnnk trunk isis another another possible possible canse. cause. These These causes forward propulsion propnlsion of the npper canses of of falling falling typically typicallyappear appear in in late late stage stage PD PD and and seem seem to to occur occnr earlier earlier with with late late onset PD. However, features may present However,aavariety varietyof ofother otherconditions conditions with with parkinsonian parkinsonian featnres early falling. falling. with early The condition among among those those most most likely likely to present present with with falling falling is PSP (Jankovic (Jankovic et al. 1990). 1990). In In these these patients, patients, the the gait gait abnormality abnormality is is quite qnite different different from from that that seen seen in PD. In PSP patients there is is akinesia akinesia associated associated with with axial axial rigidity rigidity and and nuchal nnchal supranuclear gaze gaze palsy and impaired postnpostudystonia, often often in extension, vertical snprannclear ral reflexes. This frequent and early early falling. falling. The The early early ral reflexes. This combination combination results resnlts in in freqnent appearance of gait freezing freezing in appearance of gait in PSP, PSP,which whichsometimes sometimesantedates antedatesthe the other other motor motor signs of occurrence of of early early of this this condition, condition, is also also aa major major contributing contribnting factor to the occnrrence falling. early in the conrse course of MSA than falling. Postural Postnral instability instability is is much mnch more common early in PD. These These patients may have marked akinesia akinesia with aa loss loss of of postnral postural reflexes, reflexes, sometimes sometimes associated associated with with truncal trnncal dystonia. dystonia. Wenning Wenning et et al. al. (1997) (1997) in in a review review of of ataxia. 203 pathologically proven cases of of MSA MSA found fonnd that that 38% had presented with ataxia. The term 'lower body parkinsonism'has hasbeen beennsed usedtotodescribe describe aasevere severe isolated 'lowerbodyparkinsonism' isolated gait gait associated with diffnse diffuse cerebral cerebral vascnlar vascular disease disease (Fitzgerald (Fitzgerald and Jankovic Jankovic disorder associated 1989). 1989). This This form form of ofvascular vascnlarparkinsonism parkinsonism is characterized characterized by by isolated isolated involvement involvement severe freezing freezing of gait, often leading leading to falls. falls. Normal of the lower extremities and severe pressure pressnre hydrocephalus hydrocephalns can can present present with with an an early early and and predominant predominant gait disorder associated with associated with frequent freqnent falls. falls. In In this this condition condition the gait is characterized characterized by by inabilinability lift the feet feet from from the the floor, floor, short short shnffling shuffling steps, steps, imbalance imbalance while while walking, walking, ity to lift

10 10

R. L. L. Rodnitzky R.

difficulty turning, and difficulty and gait gait ignition failure failure (Marsden (Marsden and Thompson Thompson 1997, 1997, GraffGraffRadford and Godersky Godersky 1997, 1997, and and see see Chapter Chapter 6). 6). Severe autonomic dysfunction dysfunction

Severe autonomic autonomic dysfunction dysfunction early earlyin inthe thecourse courseofofthe theillness illnessisisnot nottypical typicalof ofPD. PD. Late Late in in the the course course of of PD PDpatients patients may may develop developmild mildto tomoderate moderate symptoms symptoms of autoinsufficiency such orthostatic hypohyponomic insufficiency such as as constipation, constipation, urinary urinary incontinence, orthostatic impotence,or orimpaired impairedlacrimation lacrimation(Goetz (Goetzetetal.al.1986, 1986,Beattie Beattieetetal. al.1993). 1993). tension, impotence, Anticholinergic drugs to the the appearance appearance of of conconAnticholinergic drugs used used to to treat PD may contribute to or bladder bladder dysfunction, dysfunction, while while dopaminergic dopaminergic agents agents can can cause cause or or exacerexacerstipation or bate hypotension hypotension and, to to aalesser lesser extent, extent, constipation. constipation. The The possibility possibility of of MSA MSA bate evidence of early autonomic dysfunction dysfunction in in should be considered in patients with evidence absence of other diseases diseases and effect the the absence and drug drug treatments known to effect the autonomic (Magalhaes 1995). In system (Magalhaes In MSA MSAautonomic autonomic dysfunction dysfunction can predate signs of of parparseveral years. kinsonism by several years. In In one one study study of of MSA MSAautonomic autonomicsigns signsantedated antedated motor motor cases (Wenning (Wenningetetal. al.1994). 1994).Two Two techtechsymptoms by by one one to to two two years years in in aa quarter quarter of ofcases symptoms niques, (Pramstellar et al. 1995) 1995) and niques, electromyography electromyography of of the the urethral urethral sphincter sphincter (Pramstellar formal studies (Bonnett (Bonnett etetal. al. 1997) 1997) are are available available to objectively objectively distinformal urodynamic studies guish dysfunction of MSA MSA from guish the the autonomic dysfunction from that that of PD. PD. The The urethral urethral sphincter is is invariably invariably denervated denervated in in MSA MSApatients patientswith withincontinence, incontinence, but but not in PD patients with similar similar symptoms. symptoms. In PD, PD, urodynamic urodynamic studies studies reveal reveal an urgency urgency to void void with retention, associated associated with detrussor hyperreflexia hyperreflexia and without chronic retention, and normal normal urefunction. In In MSA MSA there thral sphincter function, there is is often often chronic chronic urinary urinary retention, a hypoactive active detrusor detrusor muscle muscle and lower lower urethral pressures. drug treatment Poor or transient transientbenefit benefitfrom from drug treatment Dopaminergic drugs, especially levodopa, usually improve the the signs of PD. The vast majority of of PD patients benefit benefit from from levodopa levodopa therapy. therapy. In In one one series series of pathologically proven proven cases casesofofPD, PD,94% 94%had had responded responded to to levodopa levodopa during during life life (Rajput (Rajput et et cally al.1990). The The response response rate rate to to levodopa levodopa in in other causes of parkinsonism parkinsonism is much al.1990). causes of respond, at at least least iniinilower. As As many many as as 65% 65% of ofMSA MSApatients patients have havebeen beenreported reported to respond, tially, to tially, to levodopa levodopa (Hughes (Hughes et et al. al. 1992b), 1992b), although although in in most studies the response is closer (Rajput et et al, al. 1990), 1990). Even Even among those with an initial response, response, closer to to one one third (Rajput fewer may continue continue to benefit benefit in in the the advanced advanced stages stages of of the the illness illness fewer than than 5% 5% may (Wenning et al. 1997). In In PSP, PSP,aalevodopa levodoparesponse responserate rate of of 38% 38% has hasbeen been reported reported Golbe 1993). 1993). Patients with multisystem degenerative disease who are (Nieforth and Golbe initially initially responsive responsivetoto levodopa levodopa commonly commonly experience experience aa rapid rapid disappearance disappearance of of benefit within one to to two two years. years. In CBGD CBGD early benefit agents benefit benefit from from dopaminergic agents is that seen seen in in PSP PSP or MSA. In other causes of parkinsonparkinsonis much much less less common common than that ism pressure hydrocephalus, hydrocephalus, lack ism such such as vascular vascular parkinsonism parkinsonism and and normal pressure lack of of

11 11

Diagnosis

significant response to levodopa levodopa is the the rule, rnle, but bnt rare exceptions have have been been reported significant (Market (Mark etal. al. 1995). 1995). On On balance, balance, these these observations observations suggest suggest that total total refractoriness refractoriness to levodopa therapy, therapy, in absence of malabsorption, malabsorption, is a to therapeutic doses of levodopa in the absence other hand, hand, responsiveness responsiveness to to 1evlevstrong point against against the diagnosis diagnosis of of PD. On the other onset of of illness illness can be considered a mild point in in favour favour of of aa diagnodiagnoodopa at the onset sis several other sis of of PD, PD, but but does does not reliably reliably distinguish distinguish between between PD PD and several other forms forms of parkinsonism. Striking asymmetry of motor signs signs

Marked asymmetry is unusual unusual in PD, PD, although although mild mild asymmetry asymmetry is is quite quite common. When mild mild to to moderate moderateasymmetry asymmetrydoes doesexist existininPD, PD,itit tends tends to to become become less less apparapparent as the the illness illnessadvances. advances.Profound Profound tremor tremor or rigidity on one side of of the the body body with with minimal or no no symptoms symptoms on on the thecontralateral contralateralside sidesuggests suggests aa variety variety of diagnoses diagnoses PD. Marked unilateral limb rigidity rigidity is often often seen in CBGD CBGD (Schneider (Schneider other than PD. et al. al, 1997). 1997). A Asevere severeand andstrictly strictlyunilateral unilateralrest resttremor tremor raises raisesthe the question question of of strucstructural pathology such as an infarction involving involving the cerebellar outflow the contralateral cerebellar outflow localization is is further suggested suggested when when the the tremor tremorisisworse worse pathways. This anatomic localization upon assuming aa posture during action action than is at rest, rest. The syndrome syndrome of upon assuming posture and and during than it is of hemiparkinsonism levodopa-responsive parkinhemiparkinsonism hemiatrophy results in unilateral levodopa-responsive parkinsonism associated with contralateral brain atrophy, atrophy, sonism associated with ipsilateral ipsilateral body body atrophy atrophy and contralateral presumably early in life life (Giladi presumably related related to to brain injury early (Giladi et et al. al. 1990). 1990).This This condition condition should major source source of of diagnostic diagnostic confusion confusion in the elderly elderly since should not be aa major since itit typitypically appears onset cally appears before before the age age of of 50 50 years yearsold. old,Unilateral Unilateral parkinsonism parkinsonism of acute onset associated with pyramidal tract signs signs should suggest the possibilor parkinsonism associated ity of an isolated isolated infarction involving the contralateral brain brain stem stem or or basal basal ganglia ganglia Fernandez 1995). 1995). (de la Fuente Fernandez

Absence of rest tremor failure to to demonstrate demonstrate rest tremor does not exclude of PD, PD, but but The failure exclude the diagnosis diagnosis of rest tremor tremor is more more common common in in PD PD than than in in most most other other forms forms of of parkinsonism. parkinsonism. In In rest two two series series of of pathologically pathologically confirmed confirmed cases cases of of PD, PD, rest rest tremor tremor was was found found to have 76% (Hughes (Hughes et et al. al. 1992c) 1992c) and 100% 100% (Rajput et et al. al. 1991b) 1991b) of patients occurred in 76% life. However, during life, However,the theincidence incidenceofofrest resttremor tremorininparkinsonism parkinsonism not not due due to to PD PD in these studies was between cases of MSA MSA only between 31% 31% and 50%. In autopsy proven cases only (Wenning et et al. al. 1997), 1997), while in 11 autopsy 39% had rest tremor (Wenning autopsy confirmed confirmed cases cases of of CBGD only life (Schneider et al. 1997). It is imporCBGD only 18 18 had had resting resting tremor tremor during life tant to mention that that aa tremor tremor of of the the head head should should not not be be considered considered aa rest tremor. More commonly, a head head tremor tremor is is aa postural postural tremor, reflecting reflecting the the continued continued activity of of axial axial postural postural muscles muscles when when in in the the upright upright position, or a dystonic tremor. In the former instance, a diagnosis diagnosis of of essential essentialtremor tremor should should be be considered considered and and in in the the

12 12

R. L. L Rodnitzky R.

latter instance, cervical dystonia is the likely likely cause cause of of tremor. tremor. In those patients sussuspected of having PD, but who do do not not manifest manifest rest resttremor, tremor,ititisisuseful useful to toengage engage the the patient in such as as difficult difficultmental mental arithmetic, arithmetic, in in order order to in mildly mildly stressful stressful activities, activities, such tremor. uncover a latent rest tremor. Other features less common in PD

A variety of other findings cast doubt on on aa presumptive presumptive diagnosis diagnosisof ofPD. PD. The The prespresence of of more than one while not impossible ence one similarly similarly affected affected first degree relative, while in PD, is unusual. Families with autosomal autosomal dominant dominant parkinsonism been in Families with parkinsonism have have been reported are exceedingly exceedingly rare (Golbe et al. 1996). 1996). Rather, Rather, conditions reported but are rare (Golbe conditions with with a genetic basis for familial occurrence should should be be considered, considered, such suchas asWilson's Wilson's known genetic disease, Machado-Joseph Hallervorden-Spatz disease. Machado—Josephdisease diseaseand and adult adult onset Hallervorden—Spatz disease. In In aa tremor dominant dominant disease disease (see (see above) above) aapositive positive history history of of familial familial patient with tremor may suggest suggest the diagnosis diagnosis of tremor may of essential essential tremor tremor (see (see Chapter Chapter 5). Other Other than essential essential tremor, tremor, these these heritable heritable causes causes of of parkinsonism parkinsonism are unlikely unlikely to to present present in elderly. Patients the elderly. Patientswith with Huntington's Huntington's disease, disease,an anautosomal autosomaldominant dominant condition, can manifest manifest signs signs of ofparkinsonism parkinsonism at at any anyage, age,although althoughisolated isolatedparkinsonism parkinsonism (the (the Westphal variant) typically appears age of 25 years years Westphal variant) typically appears as as aa juvenile juvenile form form prior prior to the age old. Recent neuroleptic neuroleptic drug therapy precludes precludes a definite definite diagnosis diagnosis of PD. PD. old. Recent drug therapy Although drug-induced drug-induced parkinsonism typically typically remits of weeks weeks Although remits within within a matter of after after withdrawal withdrawal of the the offending offending agent, itit can can sometimes sometimes persist persist for for up to one year (see Chapter A history history of of oculogyric oculogyric crisis crisis suggests suggests the possibility possibility of post post (see Chapter 4). A encephalitic PD. encephalitic parkinsonism parkinsonism rather than PD.

There are several several other other clinical clinical signs signsthat that may may appear appear in in aa patient patient with with parkinThere sonism which which are not not only only atypical atypical for for PD, PD, but but by bythemselves themselves strongly strongly suggest suggest sonism another specific specific diagnosis. they suggest, suggest, include include diagnosis. These These signs, signs, and and the conditions they gaze palsy or eyelid eyelid apraxia cerebellar supranuclear gaze apraxia (PSP), ataxia ataxia or or other signs of cerebellar dysfunction (MSA), (MSA), prominent prominent myoclonus myoclonus(Creutzfeldt—Jakob (Creutzfeldt-Jakob disease), alien alien limb limb or limb limb apraxia apraxia (CBGD) (CBGD) and and aa marked marked response response to to anticholinergic anticholinergic phenomenon, or therapy (drug-induced parkinsonism).

commonly mimicking PD Conditions commonly PD in the elderly Certain conditions mimic mimic PD PD so commonly in the elderly elderly that their differentiating differentiating features deserve special mention. These These conditions conditions have have several several overlapping overlapping cliniclinical features features with with PD, PD, but but for most of cal of them, them, the the relative relative severity severity of these these signs signs and and of appearance appearance in in the the course course of of the the illness illness differ differ from from that seen in PD. their time of More importantly importantly the nature nature of of associated associated neurological neurological signs symptoms in More signs and and symptoms usually allow the correct diagnosis to be made. those conditions usually

13 13

Diagnosis

Vascular Vascular parkinsonism symptoms can can occur occnr as a resnit Parkinsonian symptoms result of aa nnmber number of of different different vascnlar vascular

pathologies. Riley Riley and and Lang Lang (1996) (1996) identified identified four fonr categories of cerebral vascnlar pathologies. categories of cerebral vascular disease that disease that have have this this potential: potential: multi-infarct mnlti-infarct disease, disease, etat etat crible crible (multiple (mnltiple dilated dilated perivascnlar disease (snbcortical (subcorticalarterioscleroticencephaloparteriosclerotic encephalopperivascular spaces), Binswanger's disease athy) and single focal focal infarctions infarctions or or haemorrhage. haemorrhage. These pathologic states states become become increasingly increasingly common common with with advancing advancing age. age.ItItisisimportant importantto to distinguish distingnishthem them from from PD so therapy can can be be considered. considered. For For vascnlar vascular parkinsonism, risk risk so that that appropriate therapy factors factors such snch as as hypertension, hypertension, hyperlipidemia, hyperlipidemia, and and coagulation coagnlation abnormalities abnormalities must mnst identified and reversed, reversed, whereas whereas in trne true PD, PD, specific specific antiparkinsonian antiparkinsonian drng drug be identified therapy will be indicated. A A variety of clinical clinical signs and several several facets facets of the clinical course conrse help help distinguish distingnish vascular vascnlar parkinsonism parkinsonism from PD. In vascular vascnlar parkinsonism, a subacute acute onset onset of of symptoms symptoms isis sometimes sometimes seen, seen, and and similarly, similarly, the the snbacnte or acnte progression illness may stepwise fashion fashion (Hnrtig (Hurtig 1993). 1993). Rest Rest progression of of the the illness may occur occnr in aa stepwise tremor is rare (Inzelberg (Inzelberg et et al. al. 1994) 1994) and and isis almost almost never neverthe thepredominant predominant motor sign findings snch such as as hyperrefiexia hyperreflexia and sign as as can can be the case in PD. Pyramidal tract findings Babinski laughing Babinski signs signs are are common common in vascular vascnlar parkinsonism, as are pathological langhing Rigidity, when likely and crying. Rigidity, when present present in vascular vascnlar parkinsonism, parkinsonism, is much mnch more likely to be clasp clasp knife knife in in nature natnre secondary secondary to to spasticity, spasticity,rather rather than than the the typical typical lead-pipe lead-pipe variety associated associated with PD. In patients patients with with widespread widespread bilateral bilateralvascnlar vascular disease, disease, especially that especially that involving involvingthe the frontal frontal lobes, lobes,paratonic paratonic rigidity rigidity (gegenhalten) (gegenhalten) may may be be present and can be distingnished distinguished from the rigidity rigidity of PD. Many patients with vasvascular parkinsonism characteristic isolated ambulacnlar parkinsonism present present with with a characteristic isolated impairment impairment of of ambnlation consisting of a hesitant, shnffling shuffling gait with aa preserved arm arm swing swing (Elbe (Elbe et al. 1996). frequent falls falls are 1996). These These patients patients have have extremely extremelypoor poor postural postnral stability and freqnent much more common than in PD (Trenkwalder et al. 1995). Parkinsonian sympmnch common than in PD (Trenkwalder et al. 1995). Parkinsonian symptoms such as hypophonia facial masking toms snch hypophonia or facial masking are are seldom seldom present present (Thompson (Thompson and Marsden 1987). Patients with vascular parkinsonism demonstrating Marsden 1987). Patients with vascnlar parkinsonism demonstrating this this predomipredominant involvement of gait and balance balance with less less involvement involvement of and face, face, nant involvement of the arms and have been described as manifesting the syndrome of 'lower body parkinsonism' have been described as manifesting the syndrome of 'lower body parkinsonism' (Quinn 1995). 1995). One One of of the the most most important important differentiating differentiating featnres features between between PD PD and and (Qninn vascular parkinsonism is that there is seldom a significant clinical response to levvascnlar parkinsonism is that there is seldom a significant clinical response to 1evodopa in in patients patients with with the the vascnlar vascular syndrome. syndrome. Occasional Occasional striking, odopa striking, though thongh rare, rare, exceptions to this observation have been reported (Mark et al. 1995). As will be disexceptions to this observation have been reported (Market al. 1995). As will be discussed below, confirm the the diagnosis diagnosis of of parkinsonism parkinsonism cnssed below, brain brain imaging imaging may may help help confirm related to isolated cerebral infarctions or widespread subcortical vascular related to isolated cerebral infarctions or widespread snbcortical vascnlar insults. insnlts. Normal pressure pressure hydrocephalus clinical triad triad of of dementia, dementia, urinary nrinary inconThis condition typically typically presents with the clinical incontinence, and a gait disorder (Graff-Radford (Graff-Radford and and Godersky Godersky 1997). 1997). It can occnr occur at any

14 14

R. L. L Rodnitzky R.

age, but but iu age, in adults the majority majority of of cases cases preseut present after after the the age age of of 60 60 years years (Krauss (Krauss et et usually the the gait gait disorder disorder of uormal al. 1997). Although it is usually normal pressure hydrocephahydrocephalus (NPH) that variety of of other other parkinthat results results iu in diaguostic diagnostic confusiou confusion with with PD, PD, a variety NPH, sonian features features cau can also be seen In aa study of of 90 adults with NPH, souiau seeu in this this condition. couditiou. lu Krauss Krauss et et al. al. 1997 1997found found that that 81% 81% had had some some evidence evidence of of akinesia. akinesia. In In the the same same study, study, rest tremor relatively rare, tremor and rigidity were relatively rare, each each occurring occurring in only only 14% 14% of of patients. patients. is commonly referred referred to to as as an an apraxia apraxia of of gait, gait, although although The gait disorder of NPH is this label is is physiologically physiologicallyincorrect, incorrect,given giventhe theunderlying underlyingmotor motorabnormality abnormality and and akinesia Typically, patients akinesia seen seen in in this this condition. Typically, patients with with NPH NPH walk walk with with slow slow short short steps and demonstrate demonstrate impaired impaired ability ability to to lift lift their their feet feet off off the thewalking walking surface surface steps (Sudarksy and Simon 1987). 1987). Like Like vascular patients (Sudarksy vascular parkinsonism, parkinsonism, the gait in these patients is proportion to to upper upperextremity extremity and andfacial facial dysfunction. dysfunction. is often often impaired impaired out out of proportion Aside from symptoms, differentiation differentiation from from PD PD is is aided Aside from this this unusual unusual distribution of symptoms, antecedent events events known by a history history of antecedent known to to predispose predispose to to NPH NPH (meningitis, subarachnoid haemorrhage, serious cranial trauma) and and the the early early appearance of both dementia dysfunction. Whereas improvement with levodopa levodopa therapy therapy dementia and bladder dysfunction, is mild removal of of40—50 40-50 ml mild and infrequent, removal mlofofCSF CSFbybylumbar lumbarpuncture puncture may may result result of symptoms. symptoms. in a marked, albeit transient, improvement of

The diagnosis of NPH NPH should not The diagnosis of not be be seriously seriously considered considered unless unless ventriculoventriculomegaly in the absence megaly in absence of cortical cortical atrophy is demonstrated by by brain brain imaging. imaging. In In properly selected selected patients, the neurological neurological abnormalities abnormalities associated associated with NPH, NPH, including the gait disorder, the generalized generalized akinesia akinesia and and cognitive cognitive impairment, impairment, can of aa cerebrospinal cerebrospinal fluid fluid shunt shunt(Black (Blacketetal. al.1985). 1985). be improved by placement of Essential tremor

This condition is is characterized by a postural tremor that that worsens worsens with action action (see (see

predominantly in Chapter 5). It appears predominantly in the the arms arms and and to to aa lesser lesser extent in the lower extremities. involved and associated voice extremities. The The head head is commonly involved and there may be an associated voice tremor. Essential Essential tremor common disorder disorder with with a prevalence prevalence in tremor. tremor (ET) (ET) isis a common in the general estimated to be as as high as as 1.7% 1.7% (Larsson (Larsson and Sjogren Sjogren 1960). 1960). general population population estimated more common common with with advancing advancing age. age. Prevalence Prevalence rates More importantly, it is much more rates as high as 13% 13% in individuals between the ages of have been been reported to be as of 70 70 and 79 1982). In 79 years years old old (Rautakorpi (Rautakorpi et al. 1982). In individuals individuals experiencing experiencing the onset earlier in life, amplitude becomes becomes progressively progressively greater over time. Accordingly, Accordingly, the life, tremor tremor amplitude itself becomes much more more apparent apparentininthe thelater lateryears yearsof oflife. life. tremor itself Because this this condition condition is so so common in the elderly, and because it presents with Because ET is frequently frequently misdiagnosed as as PD. PD. Several Several clinical tremor, ET clinical features features help help distinguish differentiating feature guish itit from from PD. PD. Perhaps Perhapsthe the most most important important differentiating feature isisthe the nature nature of in the the two two conditions. conditions. The tremor of of ET ET appears appears preof the the predominant tremor in dominantly fixed posture. posture. It dominantly when when the the involved involvedbody bodypart part isis maintained maintained in in a fixed

1 5 15

Diagnosis persists or may tremor of PD may be be accentuated accentuated during during movement. movement. The The classical classical tremor PD

occurs at rest. the opposite opposite form form of of tremor occurs at rest. In both both conditions, conditions, however, however, the tremor may may appear concurrently, but but is is almost almost never never the the predominant predominant tremor type. appear concurrently, type. Another Another feature is that in in ET, ET, tremor commonly commonly affects affects the head and and voice, voice, distinguishing feature distribution isisvirtually virtually never never seen. seen. Aside Aside from from tremor, tremor, the the assoassowhile in PD this distribution ciated neurological ciated neurological findings findings of of ET ET and and PD PD help help distinguish distinguish the the two two conditions. conditions, ET ET is aa monosymptomatic being the the sole sole manifestation, whereas whereas in monosymptomatic condition, tremor being fully fully developed developed PD, PD, there there may maybe be coexistent coexistentbradykinesia, bradykinesia,postural postural imbalance, and rigidity. In those maybe the the sole sole preprethose cases casesof oftremor tremor dominant dominant PD in which tremor maybe senting diagnosis depends on on identifying identifying the the tremor's tremor's classical classical senting sign, sign, the correct diagnosis (sparing the the head head and and voice). voice). circumstance of occurrence (at rest) and distribution (sparing source of diagnostic uncertainty uncertainty in in the the elderly elderly patient patient isis the possibilpossibilAn additional source ity that ET and PD PD may may both be be present. present. Koller Koller et et al. al. (1994), (1994), in in aa study study of of 678 678 ET ET concurrent PD PD in in 6.1%, 6.1%, an an association association that that isis slightly slightly higher than patients, found concurrent predicted by by the the relatively relatively high prevalence of the two conditions conditions alone. alone. would be predicted In regard, itit should appearance of In this regard, should be be noted noted that that the late appearance of rest rest tremor tremor in in an as having ET is not sufficient elderly patient patient previously diagnosed as sufficient to diagnose conRajput et al. (1993) current PD. Rajput (1993) report report the finding finding of of resting resting tremor tremor with with no other signs parkinsonism in three three patients patients with with confirmed confirmed essential essential tremor who who signs of of parkinsonism underwent postmortem study. study. All underwent postmortem All three three patients patients had had additional additional postural postural and/or kinetic tremor tremor of of at at least least 10 10 years years duration duration before before the the development development of of resting resting kinetic tremor. Resting Resting tremor first developed developed in these three subjects subjects when they were all 60 years. years. Similarly, Similarly, cogwheel cogwheel rigidity in ET ET reflecting reflecting the the older than 60 rigidity may may appear in presence of ongoing tremor in muscles that may not be properly relaxed. When presence of ongoing tremor muscles that be properly relaxed. When failed to distinguish ET from from PD, a trial of therapies other diagnostic criteria have failed that are specific specific for other of of these these two two conditions conditions may may prove prove useful. useful. for one one or the other Levodopa remarkably, while Levodopa has has the the potential potential to to improve improve PD PD tremor remarkably, while itit has has virtuvirtually no effect on the tremor of ET. On the other hand, alcohol may dramatically ally no effect on the tremor of ET. On the other hand, alcohol may dramatically improve postural tremor tremorof ofET ET(Koller (Koller and and Biary Biary 1984) 1984) but has has aa less consistent improve the the postural consistent and less impressive effect on the rest tremor of PD. and less impressive effect on the rest tremor of PD, Senile gait

Significant changes changes in in the the mechanics mechanics of of ambulation ambulation appear appear in in the the normal normal elderly Significant elderly individual (Sudarsky (Sudarsky 1990, Elble Elble et et al. al. 1992). 1992).The Theresultant resultant pattern pattern of walking walking has has referred to by by aa variety variety of of terms, terms, including including senile senile gait, gait, cautious cautious gait, gait, and and been referred marche pas. This This condition condition is is characterized characterized by slow short stepped gait gait marche àa petit pas. by a slow short stepped on aa slightly slightly widespread base. Nutt and Horak Horak (1997) (1997) have have likened likened this this carried out on of a normal person person walking walking on on aa slippery surface, and at risk for gait pattern to that of falling. and falling. Elble Elble (1997) (1997) reviewed reviewed the the kinematics kinematics of of gait gait in older, healthy adults and found that that the the major major changes changes were were aa slower velocity, aa shorter reduced shorter stride length, reduced

16

R. L. L Rodnitzky R.

arm swing, swing, aa more more flexed flexed knee knee position position and and reduced reduced toe toe clearance clearance at at initial initial heel—floor contact,He Healso alsoobserved observedthat thatthese theseindividuals individualsspend spend aa greater greater proporproporheel-floor contact. of time in in aa double double limb limb support support stance stance than than younger younger subjects. subjects. In In this this popupopution of lation, arm swing and lower lower extremity extremity rotations rotations are are reduced reduced in inproportion proportion to stride to these these abnormalities, abnormalities, Sudarsky Sudarsky and and Tideiksaar Tideiksaar (1997) (1997) noted noted length. In addition to other kinematic kinematic investigations investigations of the gait in this population revealed revealed a reducthat other healthy tion in velocity and a wider stride. These These data data clearly clearlyindicate indicatethat that the the gait gaitof ofhealthy aged aged individuals individuals isis characterized characterized by by aa variety variety of of features features such such as as reduced reduced stride length, diminished diminished arm swing and and axial axial flexion flexion that overlaps with several of length, arm swing that overlaps with several of the the typical clinical features features of of PD. PD. The The clinician clinician must must therefore therefore not succumb to the to diagnose diagnose PD PD or or parkinsonism parkinsonism in in an an elderly elderly individual individual solely solely on temptation to on the basis of mobility. Rather, basis of an an abnormal gait and impaired mobility. Rather, additional additional confirmatory disorder must besought, sought,such suchasasaadecrease decreaseininfacial facial expression, expression, signs of aa movement movementdisordermustbe reduced blink rate, significant rest tremor, tremor, rigidity rigidity and and freezing, freezing, singly singly significant hypophonia, hypophonia, rest or in combination. The presence presence of gait abnormalabnormalof parkinsonism parkinsonism in addition to aa gait ity needed to confidently confidently establish parkinsonism in an an ity is is needed establish aa diagnosis diagnosis of of PD PD or or parkinsonism elderly elderly patients presenting presenting with aa elderly individual. individual. AA corollary corollary of of this this is is that elderly disorder of of gait gait should should not not be be assigned assigned aa diagnosis diagnosis of of senile senile gait if any of primary disorder of these ancillary clinical findings findings are present.

and neurophysiological neurophysiological tests tests in the diagnosis of parkinsonism Neuroimaging and diagnosis of The differentiation differentiation of of PD from based on from other causes causes of parkinsonism is is largely largely based

careful However, in some instances neurocareful clinical clinical observation observation and and examination. However, some instances imaging or neurophysiologic studies may enhance the certainty certainty of of the clinical clinical diagnosis. conditions for which the causative causative gene identified and can be nosis, In In conditions gene has has been been identified assayed, clinassayed,the thelaboratory laboratory may may provide provide absolute absolute confirmation confirmation of the presumptive clindiagnosis. ical diagnosis. Neurodiagnostic aids are Neurodiagnostic aids are almost almost never never of of significant significant benefit benefit in confirming confirming aa diagnosis comdiagnosis of of PD. PD. Neither Neither magnetic magnetic resonance resonance imaging (MRI) of the head nor com(CT) of of the the head, head, reveal reveal any any consistent findings. Subtle Subtle abnorputed tomography (CT) malities are MRI of the malities are sometimes sometimes found found in in the region of the substantia nigra in MRI in late late stage PD (Stern et al. 1989) 1989) but they are not sufficiently sufficiently common brain in common or definitive great practical practical benefit benefit in in everyday everyday practice. practice. Similarly, Similarly, neurodefinitive to to be be of great physiologic tests electroencephalography, evoked physiologic tests such such as electroencephalography, evoked potentials, potentials, and and blink reflex reflex studies, studies, while while revealing revealingsubtle subtle abnormalities, abnormalities, are seldom useful useful in establishing a diagnosis diagnosis of of PD. PD. Neuroimaging Neuroimaging is occasionally occasionally useful useful in in evaluation evaluation of patients forms of of degenerative degenerative parkinsonism. parkinsonism.This Thisisis especially especially true of advanced advanced with other forms cases. in cases.Therefore, Therefore,the theprimary primary rationale rationale behind behind obtaining a neuroimaging study in a patient with parkinsonism is is not to to confirm confirm aa diagnosis diagnosis of PD, but to determine determine

11 17

Diagnosis

if a multisystem degenerative or or vascular vascular form form of of parkinsonism parkinsonism is is present. present. In a if multisystem degenerative snrvey of of 49 49 movement movement disorder specialists, Anonti and Koller (1996) found fonnd that survey specialists, Anouti Koller (1996) CT to 100% CT or MRI MRI scans scans were were ordered ordered between between 76% 76% to 100% of the the time time to to evalnate evaluate patients with snspected suspected non-PD non-PD akinetic-rigid akinetic-rigid syndromes. syndromes. Snrprisingly, Surprisingly, in the patients in the same same survey, snrvey, these thesestudies stndieswere wereordered ordered 50% 50%toto 75% 75%of ofthe thetime time in in patients patients with with aa clinical diagnosis of PD. In the late stages of PSP, PSP,MRI MRI and and CT CT may may reveal revealatrophy atrophy of of the the dorsal mid brain brain with associated associated dilation dilation of of the the cerebral cerebral aqnednct aqueduct(Schonfeld (Schonfeldetetal.1987, al.1987,Yagishita Yagishita and Oda 1996). higher instance instance of of multiple mnltiple cerebral cerebral infarcts infarcts has has also alsobeen been noted noted 1996). A higher in the scans of patients with with PSP, PSP, raising the possibility that ischaemia ischaemia may may play play an an aetiological role (Dubinsky and Jankovic Jankovic 1987). aetiological role in in this this condition (Dnbinsky imaging in mnltiple A variety of abnormalities can can be nncovered uncovered by by nenro neuroimaging multiple system system atrophy, again again largely largely in in the the advanced advanced stages stagesof ofillness. illness.InInthe thestriatonigral striatonigral form form of of MSA MSA signal signalabnormalities abnormalities in in the the striatum striatnm can be seen, which which helps helps distinguish distingnish the syndrome from PD. Most typical typical is putaminal hypodensity on on T2 T2 weighted weighted MRI MRI syndrome from pntaminal hypodensity scans 1992). In scans (Olanow (Olanow 1992), In addition, addition, a narrow band of hyperintensity can be seen in the lateral putamen, aa finding finding not not seen seen in in PD PD (Konagaya (Konagaya et et al. al. 1994). 1994). In the olivoolivoof MSA MSA brain CT CT or or MRI MRI may may reveal reveal cerebellar cerebellar atrophy pontocerebellar type of atrophy and enlargement of the fourth fourth ventricle ventricle (Mark (Mark and andSage Sage 1993). 1993). In In cases cases of of suspected suspected enlargement diagnosis can can be be strongly strongly supported supported by by CT CT or or MRI MRI findings findings vascular parkinsonism diagnosis discrete infarctions, diffuse deep white matter signal signal abnormalities. abnormalities. of discrete infarctions, lacunes or diffuse The latter two abnormalities are much better demonstrated demonstrated by by MRI MRI than than CT. CT. The The in elderly elderly patients with clinically typical appearance of these imaging abnormalities in PD, however, however, cannot cannot be be given given too too much weight since cerebral vascular vascular disease disease and and PD are are both both common in this age group and may appear in the same patient indeof one one another. another. pendent of In normal pressure hydrocephalus a definitive definitive diagnosis diagnosis cannot cannot be be made made without without neuroimaging. Therefore, in patients in in whom whom this this diagnosis diagnosis isis being being considered, considered, surgical intervention, CT or MRI must be performed performed and who are candidates for surgical to for evidence evidence of of enlarged enlarged ventricles ventricles in the the absence absence of of significant significant brain brain to look for atrophy. The atrophy. The diagnosis diagnosis of of several several other other less less common common causes causes of of parkinsonism parkinsonism in in elderly subjects CBGD, CT elderly subjects is is occasionally occasionally aided aidedby by neuroimaging. neuroimaging. In In CBGD, CT or or MRI MRI can reveal (Riley et al. 1990). reveal frontoparietal frontoparietal atrophy, which which is sometimes asymmetric (Riley In Hallervorden-Spatz Hallervorden—Spatzsyndrome syndrome pallidal pallidal hypointensity hypointensity on T2 weighted MRI scans is greater normal individuals individuals (Rodnitzky (Rodnitzky1993). 1993).In Inadvanced advanced cases cases greater than than that seen in normal the 'eye of tiger' sign sign is is seen seen in the the globus globus pallidus, pallidus, consisting consisting of central the 'eye of the tiger' of a central increased (Sethi et al. 1988). increased signal signal surrounded surrounded by a zone of hypointensity (Sethi Positron emission emission tomography (PET) (PET) can can provide provide aa powerful powerful research research tool to to define selectivepatterns patterns of of disruption disruption of regional cerebral metabolism that helps define selective cerebral metabolism helps distinguish between other forms forms ofofneurodegenerative neurodegenerative disease disease causing causing distinguish between PD PD and other

18

R. L. L Rodnitzky R. Rodnitzky

parkinsonism (Brooks (Brooks 1993). 1993). PET PET can can also also detect detect early early sub-clinical snb-clinical PD PD in in at-risk at-risk snbjects. subjects.

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Mark MH, Binswanger's disease disease presenting presenting MH, Sage Sage JI, JI, Walters AS, Duvoisin RC, Miller DC (1995) Binswanger's as levodopa-responsive parkinsonism: Clinicopathologic levodopa-responsive parkinsonism: Clinicopathologic study of of three threecases. cases.Movement Movement Disorders, 450—S. Disorders,10,10, 450-5. Marsden CD, Thompson PA (1997) of gait Marsden CD, Thompson PA (1997) Toward Toward aa nosology nosology of gait disorders: descriptive classification. andand Therapeutic Strategies, eds. eds. Masdea JD, JD, classification. In: Gait GaitDisorders DisordersofofAgeing: Ageing:Falls Falls Therapeutic Strategies, Masdeu Sudarsky L, Philadelphia: Lippincott-Raven. Lippincott-Raven. L, Wolfson Wolfson L, L, pp. pp. 135—46. 135-46. Philadelphia:

Mayeux R, Chen J, Mirabello E, Marder K, K, Bell Bell K, Dooneief G, Cote Cote L, L, Stern Y Y (1990) (1990) An An estiestimate of disease. Neurology, 40,40, 1513—16. Neurology, 1513-16. of the the incidence incidenceof ofdementia dementiaininidiopathic idiopathicParkinson's Parkinson's disease. Mega MS, Masterman DL, DL, Benson Benson DF. DF.Vinters Vinters HV, HV, Tomiyasu Tomiyasu U, U, Craig Craig AH, AH, Foti FotiDJ, DJ,Kaufer Kaufer D, D, Scharre DW, Fairbanks L, Cummings JL JL (1996) Dementia with with Lewy Lewy bodies: Reliability Reliability and 47,47, 1403—9. validity of clinical clinical and andpathologic pathologiccriteria. criteria.Neurology, Neurology, 1403-9. Mohr E, Chase TN (1990) Selective deficits in in Alzheimer Alzheimer and and parE, Litvan Litvan I,I, Williams J, Fedio P, Chase Selective deficits kinsonian dementia: dementia: Visuospatial Visuospatial function. function. Canadian CanadianJournal Journal Neurological Sciences, ofofNeurological Sciences, 17,17, 292-7. 292—7. Nieforth KA, Golbe LI LI (1993) (1993) Retrospective Retrospectivestudy studyof ofdrug drug response response in in 87 87patients patients with with progresprogresKA, Golbe sive supranuclear supranuclear palsy. 16, 16, 338—46. palsy.Clinical ClinicalNeuropharmacology, Neuropharmacology, 338^6. Nutt JD, Movement Neurologic JD, Horak Horak FB FB(1997) (1997) Gait Gaitand andbalance balancedisorders. disorders.In:In: MovementDisorders: Disorders: Neurologic Principles eds. Watts RL,RL, Keller WC, p. p. 658. New York: McGraw-Hill. Principlesand andPractice, Practice, eds. Watts Keller WC, 658. New York: McGraw-Hill. Olanow CW (1992) Neurologic Clinics, 10,405—20. Neurologic Clinics, 10,405-20. OlanowCW (1992) Magnetic Magneticresonance resonanceimaging imagingininParkinsonism. Parkinsonism. Pramstellar Quinn NP, Fowler CJ (1995) Nerve conducPramstellar PP, PP, Wenning GK, GK, Smith SJM, SJM, Beck RO, Quinn tion studies, studies, skeletal skeletal muscle EMG, and sphincter EMG in multiple system system atrophy. Journal Journalof of Neurology, andand Psychiatry, 58, 618—21. Neurology,Neurosurgery, Neurosurgery, Psychiatry, 58, 618-21. Quinn Quinn NN(1995) (1995)Parkinsonism Parkinsonism—- Recognition and differential differential diagnosis. diagnosis. British BritishMedical MedicalJournal, Journal, 310, 310,447—52. 447-52. Rajput AH, AR, Rozdilsky B, Ang Ang LL (1991b) (1991b) Occurrence Occurrence of of resting tremor in Rajput Rozdilsky B, in Parkinson's Parkinson's disease. disease. Neurology, Neurology,41, 41,1298—9. 1298-9. Rajput AH, Rozdilsky B, Ang Ang L, L,Rajput RajputAA(1993) (1993)Significance SignificanceofofParkinsonian Parkinsonianmanifestations manifestations in Rozdilsky B, essential Journal of Neurological Sciences, 20, 114—17. essential tremor. tremor.Canadian Canadian Journal of Neurological Sciences, 20, 114-17. Rajput AH, Rozdilsky Rajput A (1991a) Accuracy of clinical diagnosis in Rajput Rozdilsky B, Rajput in parkinsonism parkinsonism— - aa prospective study. Journal of Neurological Sciences, 18, 275—8. prospective study.Canadian Canadian Journal of Neurological Sciences, 18, 275-8. Rajput AH, AR, Rozdilsky Rajput A, Ang L (1990) Levodopa efficacy and pathological basis of Rajput Rozdilsky B, Rajput efficacy and Parkinson Neuropharmacology, 13, 553—8. Clinical Neuropharmacology, 13, 553-8. Parkinson syndrome. syndrome.Clinical Rautakorpi tremor in RautakorpiI,I,Takala Takala J, Martilla Martilla RJ, RJ, Sievers Sievers K, Rinne UK (1982) Essential tremor in aa Finnish Finnish poppopulation. 66, 66, 58—67. ulation.Acta ActaNeurologica NeurologicaScandinavica, Scandinavica, 58-67. Riley Riley DE, DE, Lang Lang AE (1996) (1996) Non-Parkinson Non-Parkinson akinetic-rigid akinetic-rigid syndromes. syndromes. Current Current Opinion Opinion in in Neurology, 32321-6. 1—6. Neurology,9, 9, Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewicz 0, O,Black Black5S(1990) (1990)Cortical—basal Cortical-basal ganglionic 40,40, 1203—12. Neurology, 1203-12. ganglionic degeneration. degeneration.Neurology, eds. Stern MB, Rodnitzky RL (1993) (1993) Hallervorden—Spatz Hallervorden-Spatz syndrome. syndrome.In: In:Parkinsonian ParkinsonianSyndromes, Syndromes, eds. Stern MB, Koller 1—58. New New York: Marcel-Dekker. Marcel- Dekker. Koller WC, WC, pp. pp.34 341-58.

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Diagnosis RL, Uc Uc EY EY (1997) (1997) Approach Approach to to the hypokinetic Neurology, ed.ed. Rodnitzky RL, hypokinetic patient. patient.In: In:Practical Practical Neurology, Philadelphia: Lippincott-Raven. Lippincott-Raven. 299-311. Philadelphia: Biller J, pp. 299—311. Schneider Schneider JA, JA, Watts Watts RL, RL, Gearing Gearing M, M, Brewer Brewer RP, RP, Mirra Mirra SS SS (1997) (1997) Corticobasal Gorticobasal degeneration: Neuropathologic 48,48, 959—69. Neurology, 959-69. Neuropathologicand andclinical clinicalheterogeneity. heterogeneity.Neurology, Schonfeld SM, SM, Golbe Golbe LI, LI, Sage SageJI, JI,Safer SaferJN, JN,Duvoisin DuvoisinRC RGetetal. al.(1987) (1987)Computed Gomputed tomographic tomographic Schonfeld findings in progressive findings progressive supranuclear supranuclear palsy: palsy: Gorrelation Correlation with with clinical clinical grade. grade.Movement Movement Disorders, 2,2, 263—78. Disorders, 263-78. Sethi KD, Adams RJ, Loring DW, El Gammal TT (1988) (1988) Hailervorden—Spatz Hallervorden-Spatz syndrome: syndrome: Glinical Clinical and magnetic Annals ofof Neurology, 24,24, 692—4. Neurology, 692-4. magneticresonance resonanceimaging imagingcorrelations. correlations. Annals Starkstein SE, SE, Sabe Sabe L, L, Petracca Petracca G, G, Chemerinski Ghemerinski E, E, Kuzis G, Merello Merello M, Leiguarda Starkstein Kuzis G, Leiguarda R (1996) (1996) Neuropsychological and psychiatric psychiatric differences differences between Alzheimer's disease and Parkinson's Parkinson's disease with dementia. Neurosurgery, andand Psychiatry, 61, 381—7. dementia.Journal JournalofofNeurology, Neurology, Neurosurgery Psychiatry, 61, 381-7. Stem MB, MB, Braffman BE,Hurtig Hurtig HI, HI, Grossman Stem Braffman BH, Skohalck Skolnick BE, Grossman RI RI (1989) (1989) Magnetic Magnetic resonance resonance 39,39, 1524—6. imaging in Parkinson's Parkinson's disease disease and andparkinsonian parkinsoniansyndromes. syndromes.Neurology, Neurology, 1524-6. Sudarsky LL (1990) Gait Gait disorders disordersininthe theelderly. elderly.New NewEngland England Journal of Medicine, 322, 1441-6. Journal of Medicine, 322, 1441—6. Sudarsky L, Simon 5S (1987) (1987) Gait Gait disorder disorderininlate latelife lifehydrocephalus. hydrocephalus.Archives ArchivesofofNeurology, Neurology,44,44, 263—7. 263-7. Tideiksaar R (1997) (1997) The The cautious cautious gait, fear of falling falling andpsychogenic and psychogenic gait disorders. Sudarsky L, Tideiksaar andand Therapeutic Strategies, eds.eds. Masdeu JD, Sudarsky L, L, In: Gait GaitDisorders DisordersofofAgeing: Ageing:Polls Falls Therapeutic Strategies, Masdeu JD, Sudarsky Wolfson L, Philadelphia: Lippincott-Raven. L, pp. pp.283—95. 283-95. Philadelphia: Lippincott-Raven. Thompson PD, PD, Marsden GD CD (1987) Gait disorder of subcortical arteriosclerotic encephalopathy: 1—8. Disorders,2, 2, 1-8. Binswanger's disease. disease.Movement MovementDisorders, Trenkwalder C, G, Paulus Paulus W, W, Krafczyk KrafczykS,5,Hawken HawkenM, M,Oertel Oertel WH, WH, Brandt T (1995) Postural Trenkwalder Postural stabilstability differentiates differentiates 'lower ‘lower body' body' from from idiopathic idiopathicparkinsonism. parkinsonism.Acta ActaNeurologica NeurologicaScandinavica, Scandinavica, 91, 444—52. 91,444-52. Wenning GK, Ben Shlomo Y, Magalhaes Magalhaes M, M, Daniel Daniel SE, SE,Quinn Quinn NP (1994) Glinical features and Clinical features natural history Brain,117, 117,835—45. 835-45. natural historyof ofmultiple multiplesystem systematrophy: atrophy:An Ananalysis analysisofof100 100cases. cases.Brain, Wenning GK, Daniel SE, SE, Quinn Quinn NP (1997) GK, Tison F, F, Ben Shlomo Y, Daniel (1997) Multiple Multiple system system atrophy: atrophy:A A 12,12, 133—47. review of 203 203 pathologically pathologicallyproven provencases. cases.Movement MovementDisorders, Disorders, 133-47. Yagishita A, Oda Oda M (1996) supranuclear palsy: Yagishita A, (1996) Progressive Progressive supranuclear palsy: MRI and and pathological pathological findings. findings. Neuroradiology, 38, 1, 1, 560—6. Neuroradiology, 38,Suppl. Suppl. 560-6.

Parkinson's disease and parkinsonism in the elderly Jolyon Meara and Bimal K Bhowmick

Introduction The syndrome of parkinsonism, defined as akinesia accompanied by rigidity, and

often tremor, occurs more frequently as people grow older and may be present in a significant proportion of elderly people (Mayeux et al.1992, Bennett et al. 1996, Meara et al. 1997). Two-thirds of patients with the diagnosis of Parkinson's disease (PD) will be over the age of 70 years, though the proportion of parkinsonism not due to PD increases with age. A further important issue in the care of the elderly with PD is that many patients, particularly women, will be living alone or will be cared for by a spouse who may also suffer from limitations imposed by health problems. The frailty of the diagnosis of parkinsonism and PD is addressed in Chapter 2, the epidemiology of parkinsonism in Chapter 7 and the drug treatment of PD in elderly subjects in Chapter 9.

The neuropathological basis of PD PD is characterized by cell loss and gliosis in a paired brain stem nucleus called the

substantia nigra (Jellinger 1986, Forno 1996). The substantia nigra is a pigmented dopamine rich nucleus that forms part of five closely related deep-seated subcortical brain nuclei, collectively called the basal ganglia (caudate, putamen, globus pallidus, subthalamic nucleus and substantia nigra). The substantia nigra consists of a densely cellular pars compacta, and a less cellular pars reticulata. The neurones in the substantia nigra pars compacta project to the caudate and putamen (together called the striatum) formingthe nigrostriatal tract. The substantia nigra pars reticulata forms a major output nucleus ofthe basal ganglia projecting to specific thalamic nuclei and receiving afferent input from the striatum and subthalamic nucleus. The cell loss in the substantia nigra occurs in a different distribution to that seen simply as a result of aging changes (Gibb and Lees 1991). Tretiakoff first suggested the causal link between degenerative changes in the substantia nigra and PD in 19 19. His hypothesis was not widely accepted until after PD was found to be asso22

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with a dopamine deficiency state in the striatum and the dopamine rich nigrostriatal tract linking the substantia nigra with the the striatum had been ciated

located (Bernheimer et al. 1973). The remaining, but compromised nerve cells in the substantia nigra pars corn-

pacta contain typical inclusions in the cytoplasm called Lewy bodies (Jellinger 1986, Forno 1996). Lewy bodies have a dense eosinophilic core and pale halo and demonstrate characteristic staining reactions. Other inclusions have also been described in P1) (Gibb et al. 1991). There is still debate about whether the diagnosis of PD can be made in clinically typical cases that fail to show typical neuropathological features. At postmortem, PD that was clinically typical in life may fail to show Lewy body degenerative disease in the substantia nigra (Rajput et al. 1989). In PD, Lewy bodies may be found in other pigmented and nonpigmented nuclei that provide ascending dopaminergic, serotonergic, noradrenergic and cholinergic projections to the cortex and basal ganglia (Agid et al. 1987). Lewy bodies also appear to occur in the cerebral cortex in most cases of PD (Hughes et al. 1992). Pathology outside the nigrostriatal tract presumably accounts for the clinical manifestations in PD of cognitive impairment, dementia, autonomic failure, postural imbalance, freezing, dysphagia and dysarthria.

Large numbers of cortical Lewy bodies in association with senile plaques, a feature of Alzheimer's disease, may suggest the diagnosis of dementia with Lewy bodies or a variant of Alzheimer's disease. Interestingly, cases that fulfil pathological criteria for PD but which have atypical clinical features, often including a poor response to levodopa treatment, tend to have prominent numbers of cortical Lewy bodies or additional vascular disease in the striatum (Hughes et al. 1993). Lewy

bodies can also be found in other neurodegenerative conditions such as motor neurone disease and may be present in up to 10% of elderly subjects dying without evidence of PD in life (presumably disease-in-evolution). Two important clinicopathological brain bank studies have demonstrated that diagnostic accuracy for P1) at death is at best only around 76% (Rajput et al. 1991, Hughes et al. 1992). Neuropathological findings in cases misdiagnosed as PD consisted, not unexpectedly, of multisystem degenerative diseases that cause parkinsonism, such as progressive supranuclear palsy and multiple system atrophy, but

more surprisingly, a significant number of cases of Alzheimer's disease and Alzheimer type changes. The use of stringent clinical diagnostic criteria can improve the specificity for the diagnosis of PD at the expense of sensitivity. Diagnostic accuracy in more recent cases referred to a brain bank was shown to have improved to around 84% (Ansorge et al. 1997). The second striking finding from these studies is that co-existing neuropathology both within and outside the striatum is extremely common in the brains of elderly subjects with histologically confirmed PD (Hughes et al. 1993).

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Motor loop Limbic loop Complex (frontal) loop

[ Thalarnus Fig. 3.1

Anatomy of cortical—basal ganglia—cortical loops: motor loop, limbic loop, complex

(frontal) loop.

The neurophysiological basis of PD The loss of dopaminergic modulation in the striatum causes a profound distur-

bance of voluntary motor control leading to akinesia, tremor and rigidity. Restoring dopamine levels by the oral administration of the dopamine precursor levodopa can, at least temporarily, improve the motor impairments of PD. Our knowledge of the complex neurochemical, neuroanatomical and neurophysiolog-

ical relationships in the basal ganglia in man and animal models has greatly increased in the past decade (Alexander et al. 1986, Alexander and Crutcher 1990, DeLong 1990, ElbIe 1998, Graybiel 1990, McRae 1998). Widespread cortical areas project to the striatum and pass from there to the globus pallidus/substantia nigra

pars reticulata (the output nuclei of the basal ganglia) before passing back to specific cortical areas via the thalamus. Several loops exist linking the cortex, the basal ganglia, the thalarnus, and cortex (see Fig. 3.1). The basic output from the thalamus is excitatory to the cortex, though this can be altered by the inputs from the output nuclei of the basal ganglia, which cause tonic inhibition of the thalamus (see Fig. 3.2). The motor signs of PD appear to result from a failure of the motor loop that links the sensorimotor cortex, putamen, globus pallidus/substantia nigra pars reticulata, thalarnus, and the supplementary motor area in the cortex. Normally, tonic thalarnic inhibition from the basal ganglia is reduced in preparation for a voluntary movement so that the excitatory thalamic output can increase to the relevant area in the supplementary motor area of the motor cortex. This is

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Sensorimotor

Fig. 3.2

Functional aspects of the motor loop — there is a resting tonic inhibition of VA/VI (ventroanterior and ventrolateral thalamic nuclei) by GPi (globus pallidus pars interna) and SNpr (substantia nigra pars reticulata).

achieved by cortical inputs to the putamen that cause inhibition of the output nuclei (direct pathway). Cortical inputs can also increase the inhibition of the thaiamus from the output nuclei by the involvement of a loop between the globus pal-

lidus, subthalamic nucleus and globus pallidus/substantia nigra pars reticulata (indirect pathway). The flow of activity between the direct and indirect pathways is modulated by dopaminergic input from the substantia nigra pars compacta via the ascending nigrostriatal pathway. Normally, dopamine in the striatum excites the

direct pathway and inhibits the indirect pathway. In PD the loss of dopamine increases activity iii the indirect pathway, thereby increasing thalamic inhibition and reducing cortical excitability (see Fig. 3.3). This provides an explanation, albeit rather simplistic, for the akinesia of PD and the failure of the proper execution of automatic learned motor plans (Delwaide and Gonce 1998). Neurophysiological

data broadly support this type of explanation for some aspects of akinesia. However, the simple model of PD provides no satisfactory explanation for tremor or rigidity and the neurophysiogical basis for these motor signs is controversial (Meara and Cody 1992, Hua et al. 1998). The subthalamic nucleus appears to play a critical part in the pathophysiology of PD by providing powerM control over the inhibitory output activity of the basal ganglia (globus pallidus pars interna/substantia nigra pars reticulata). Surgical

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1— Putamen Direct

Indirect

VA/VL

GPe

I Fig. 3.3

Direct and indirect basal ganglia pathways modulated by dopamine from the nigrostriatal tract. GPi, globus pallidus pars externa; SNpc, substantia nigra pars compacta; SNPr, substantia nigra pars reticulata; STN, subthalamic nucleus; VA/VL, ventroanterior and

ventrolateral thalamic nuclei.

lesions

of the subthalamic nucleus or subthalamic stimulation can improve and

sometimes even abolish all the motor features of P1.) (Limousin et al. 1995). Vascular damage to this area in elderly subjects without P1) has been known for a long time

to cause the hyperkinetic movement disorder of hemiballism. In the future, pharmacological manipulation of the excitatory glutamatergic pathway between the subthalamic nucleus and globus pallidus may also offer therapeutic potential.

A case history The following history gives some account of the difficulties faced by many elderly

patients with PD and the stress faced by some caregivers. Mr R. W. is an 80-year-old retired solicitor and past captain of a golf club, who had contin-

ued to work until over 70 years old. His problem first began when he gave up playing golf about eight years ago. He refused to leave the house, saying that he had a bad back and complained to his children that the visits of his young grandchildren made him feel too tired and irritable. One year later he was diagnosed as having 'mild' PD by his general practitioner. He had been made to consult by his wife, who was worried about a tremor of his right hand that she had noticed for about six months and his difficulty with buttoning clothes and drawing cheques. He was commenced on selegiline. Due to lack of benefit, levodopa was added, with improvement of manual dexterity and some improvement of tremor. Still he did not engage in social activities.

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Parkinson's disease and parkinsonism Within two years he was requiring increasing doses of levodopa to control stiffness and loss of mobility. A tremor of the left hand developed. His wife noticed that his memory was getting worse. He fell twice, once in the house and once outside in the garden. Since that time he has become reluctant to go outside or venture upstairs. Dopamine agonist therapy with bromocriptine was started with reduction in akinesia and rigidity. However, this drug had to be stopped due to recurrent complex visual hallucinations

at night. The hallucinations consisted of a dog that had been a pet 30 years before. At present he remains disabled by akinesia, dysarthria, tremor and impairment of postural reflexes. Oxybutynin has been prescribed for nocturia. He self medicates with levodopa

and often takes doses in excess of that prescribed though remains dependent on his wife

for elements of self-care. He is distressed and agitated at night, calling to his wife, who sleeps in a separate room, several times throughout the night to help him to go to the toilet. At night his wife has found him wandering in the bedroom looking for burglars. His wife notices that he becomes drowsy later in the day, so much so that on occasion he has fallen asleep into his dinner. He has refused to attend at a Day Centre because he believes his wife

is having affairs behind his back when he is away.

The effects of aging and comorbidity on PD Aging directly influences the clinical expression of PD and other diseases that

appear with the passage of time. The resulting multiple pathology and polypharmacy of old age act to magnify and modify the disability and handicap of PD (see Table 3.1). In aging, cells from widespread areas of the substantia nigra projecting into the striatum are lost as well as dopamine receptors in the striatum. In PD, cells are primarily lost more specifically from the ventral tier nigral zona compacta cells projecting into the posterior striatum (Gibb and Lees 1991). Aging changes involving muscle, connective tissue, balance, gait, vision and the autonomic nervous system will compound and interact with impairment due to PD.

Clinical heterogeneity with age in PD As

with many diseases, in PD clinical complexity increases with the age of the group

of patients studied. Elderly patients with PD will include several groups at very differing stages in the natural history oftheir disease, posing different problems and challenges (see Table 3.2). In young patients with PD the clinical challenge is that of dealing with a disorder of motor control typified by tremor, akinesia, involun-

tary movements and drug-induced motor fluctuations. However, in elderly patients akinesia, poor mobility and impaired balance are the most disabling motor

features of PD. Additional problems in elderly patients arise from coexisting depression, dementia, autonomic dysfunction and the neuropsychiatric side effects of drug treatment.

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Table 3.1 Factors influencing the clinical expression of PD in elderly subjects

Aging changes in the nervous system

motor pathways sensory pathways proprioreception balance syslcms gait generation

• Aging changes in other organ systenis

muscle connective tissue joints vision

•

• Concurrent disease: specific

cerebrovascular disease

degenerative brain disease peripheral neuropathy arthritis/niyopathy depression nonspecilic •

Elfects of concurrent drug therapy: nervous system

cardiovascular

immobility/frailty

cognitive impairment confusion sedation drug-induced parkinsonism

orthostatic hypotension

Late stage PD

of the most difficult management challenges are encountered in this group of elderly patients. The clinical picture in late stage disease is dominated by features that do not respond to dopaminergic drugs, such as poor mobility, falls, confusion, drowsiness, dementia, sialorrhoea, dysarthria, impaired communication, dysphagia, and weight loss. With time, palliative care becomes increasingly appropriate for survivors at this stage of the disease. Some

Subtypes of PD and age at diagnosis There has been considerable interest in the existence of subtypes of PD (see Table 3.3). Clinical impression suggests that patients with late onset PD (symptoms first starting after the age of 70 years) tend to progress more quickly than patients with early onset disease (symptoms before the age of 40 years). To a large extent clinical

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Table 3.2 Clinical heterogeneity of elderly subjects with PD

Elderl)' subjects with longstanding Pt) • Elderly subjects with symptoms developing after 70 yrs old (late onset P1)) • Elderly subjects with P1) of onset before 70 yrs old and of short duration • Elderly subjects with associated moderate/scverc disability/handicap (late stage P1)) •

Table 3.3 Subtypes of

PD

Early onset <50 yrs old Tremor dominant Benign slow progression Unilateral with or without axial disease

vs. Late onset >70 yrs old vs. Postural imbalance and gait disorder vs. Malignant rapid progression vs. Bilateral disease with or without impaired balance

impressions have been supported by most studies investigating the existence of subtypes in PD (Zetusky et al. 1985, Goetz et al. 1988). The large DATATOP study

has analysed the initial clinical findings in a cohort of 800 patients at diagnosis (Jankovic et al. 1990). This study showed that patients with late onset disease progressed at a greater rate and were more cognitively impaired than those with early onset disease. Patients classified as having rapidly progressive PD were older and had more severe bradykinesia and postural imbalance and less tremor when compared to the group with slowly progressive disease. These motor features remained even after adjustment for age. The group of patients with tremor dominant disease had less disability, less cognitive impairment and less depressive symptomatology compared to the group with marked gait and balance impairment. These findings are supported by most (Zetusky et al. 1985, Goetz et al. 1988, Diamond et al. 1989), but not all (Gibb and Lees 1988, Friedman 1994) studies. Late onset PD The pattern of disease at presentation in elderly subjects may reflect the more wide-

spread loss of nigral cells due to aging in addition to the more circumscribed loss due to PD. The failure of gait and balance difficulties to respond to levodopa also suggests involvement of other non-dopaminergic neural pathways. The faster progression of PD in late onset disease has been explained by the independent and additive effects of age and disease duration on the natural history of PD. Nigral cell loss due to PD appears to be most rapid in the early stages of disease evolution. There appears to be a clear association of late onset disease with depression and cognitive impairment. The extent to which this association is attributable to age alone is unclear. The DATATOP analysis suggested that cognitive function and motor deterioration were relatively independent once adjustment for age had taken

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place (Jankovic et al. 1990). However, patients with late onset disease become demented sooner than patients with early onset disease of similar age (Tanner et al. 1985).

Comprehensive assessment of PD in elderly patients Optimal management of P1) and parkinsonism in elderly patients requires an approach based on the comprehensive assessment of the impact of PD on the phys-

ical, mental and social functioning of the individual (Rubenstein et al. 1991, Stuck et al. 1993). Quality of care ultimately depends on timely functional assessment and

reassessment from diagnosis, through disease progression and finally to palliative care in advanced PD. The needs and views of the carer should also be addressed in the assessment process.

Specialist clinics with access to a wide range of health and social care expertise can provide this level of assessment, can organize or provide treatment and can monitor outcomes of care. Standardized assessment can be used as the basis for management decisions and for determining treatment outcomes. Assessment in PD should be structured (see Fig. 3.4). The baseline assessment of patients can be undertaken by medical, nursing and therapy staff. In reality, most elderly patients

will require referral for specialist assessment by the multidisciplinary team. Swallowing difficulties require assessment by speech therapists, who can also aid communication and language. Dieticians can advise on strategies to improve levodopa absorption, to maintain weight, to maintain safe swallowing and to alleviate constipation. Occupational therapists can assess the physical environment in the

home and the patient's functional ability in their normal surroundings. The financial burden and social isolation of PD can be assessed by a social worker who

can advise on any financial benefits available and measures to improve social contact. Some patients may require formal psychological and psychiatric assessment.

Elderly patients should initially be assessed by a full clinical history and review of

current medication, supported by a general medical and neurological examination. Lying and standing blood pressure and pulse rate are important measures of auto-

nomic function and risk of postural syncope with anti-parkinsonian medication. Selected investigations indicated by the history and examination may be required. Diagnosis should be made based on recommended clinical diagnostic criteria for PD, such as those developed as a result of clinicopathological Brain Bank studies (Hughes et al.1992). The Unified Parkinson's Disease Rating Scale (UPDRS — see

Fahn et al. 1987) covers mood, memory, activities of daily living, motor impairment and complications of drug therapy and is used in most large drug research studies in PD. However, the UPDRS takes around 15 minutes to administer in

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Activities of Daily Living (Barthel ADL)

(Nottingham Extended ADL Index) Fig. 3.4

Domains in the assessment of Parkinson's disease.

routine clinical practice. One approach is to select the motor section and cornplications of therapy from the UPDRS and to use more generic measures for the other

domains. Assessment of motor function needs to take place at a defined time in relation to drug treatment, usually when the patient is maximally 'on' in terms of drug response. A simple measure of motor function is a timed ten metre walk with step count and a measure of upper limb akinesia such as the two touch test. The two

touch test involves sitting the patient in a straight backed chair with arms. The patient is then asked to touch each arm of the chair in turn with first the right then

the left hand. Full excursions over one minute are counted for each hand. Normative values in elderly subjects need to be established. Activities of daily living, memory and mood are well covered by generic measures, such as the Barthel ADL Scale (Collin et al. 1988), the Nottingham Extended ADL Index (Noun and Lincoln 1987), the Mini Mental State Examination (MMSE) (Folstein et al. 1975), the CAMCOG assessment of cognitive function (Roth et al.

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and the short form of the Geriatric Depression Scale, the GDS-15 (D'Ath et

a!. 1994).

The GDS-15, has been validated in elderly community dwellers and elderly patients in hospital settings (D'Ath et al. 1994, Lesher and Berryhill 1994). Out of a maximum score of 15 a score of five or more on the GDS-15 has a sensitivity and specificity of around 90% and 70% respectively for depression when compared to formal psychiatric diagnosis (Jackson and Baldwin 1993, D'Ath et al. 1994). The CAMCOG assessment is more sensitive to early cognitive decline than the MMSE

(Hobson and Meara 1998) and could be used to assess patients with borderline scores on the MMSE. Health related quality of life measures are being increasingly used in health care

to measure outcome and quality of care. A widely used generic health status measure, the Short-Form 36 (Brazier et al. 1992), contains items inappropriate for elderly subjects and also suffers fro in missing data when administered by self report

in elderly patients with stroke and PD (Hobson and Meara 1997). The Health Status Questionnaire 12 (HSQ- 12) is a new measure, derived from the SF-36, which appears to be acceptable to elderly subjects as a brief core measure of health related

quality of life (Bowling and Windsor 1997). In addition to generic measures two

disease specific tools exist; the PDQL (de Boer et al. 1996) and the PD-39 (Jenkinson et al. 1995). Frequency of assessment visits The frequency of clinic visits is determined by the severity and number of func-

tional problems identified and how rapidly patients progress to late stage disease. Patients with stable disease who are managing well may need review only on a yearly basis. Open access to the clinic for patients experiencing difficulties can be an important feature in promoting confidence in the patient and carer. In late stage disease a time will arise when, due to frailty and immobility, clinic visits may no longer be appropriate. In these circumstances home assessment can be performed by the nurse specialist of the team. The need for palliative care can be evaluated and treatment plans agreed with the patient, carer and family doctor. Patients failing to attend at clinic visits are easily lost to follow-up, with resulting fragmentation of care at the terminal stage of their disease.

Specific problems encountered in elderly patients with PD Motor syndromes Unlike younger patients with PD, elderly patients are more troubled by gait and

balance problems. Dyskinesia and motor fluctuations, apart from 'end of dose' deterioration or 'wearing off', before the next medication is due, are rarely seen in

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The two most common motor syndromes seen in elderly patients are, firstly, appar-

ent drug resistant parkinsonian motor signs and secondly, increasing gait and balance problems. Non-drug rehabilitative approaches are an important part of the

management of motor disability in elderly patients with PD. Electroconvulsive therapy (ECT) has been found to improve the motor symptoms of PD in elderly patients who are not depressed and ongoing benefit has been reported for nearly three years in some patients (Pridmore and Pollard 1996). Despite evidence of benefit in the majority of the very small number of patients treated with ECT and reported in the literature, it is unlikely that ECT would be widely acceptable to many elderly patients. Drug resistant motor parkinsonism

Failure of motor signs to respond to levodopa would indicate a diagnosis other than

PD. However, motor signs in patients with late onset disease do not appear to respond as well to levodopa as in younger patients. This cannot be explained simply by an inability to tolerate a full therapeutic dose. Patients with long standing PD

remain responsive to levodopa, though the duration of response is reduced. Patients with late onset PD should be treated with maximally tolerated doses of 1evodopa to obtain the best control of symptoms. Patients with long standing PD who

have not been under regular medical review may well have been significantly undertreated for several years. In elderly patients standard formulations of levodopa (co-careldopa 25/100 or co-beneldopa 25/100) should be used in a trial of treatment to obtain the best control of disease symptoms. A minimum daily dose of 600mg levodopa should be reached. A few patients will require higher doses to maximize motor response, though this is unusual. Commonly, a maximally therapeutic levodopa dose may not be tolerated due to side effects of drowsiness, dizziness on standing, and hallucinosis. Patients with late onset disease tend to respond even less well to levodopa and are often poorly tolerant of the nausea and vomiting induced by dopamine and the neuropsychiatric effects of this drug. Poor tolerance of levodopa raises the possibility of parkinsonism not due to PD. The peripheral

doparnine antagonist, domperidone, at a dose of 20mg three times daily taken

30 minutes before levodopa, can be used to control nausea and vomiting. Domperidone may also help postural hypotension, though this requires further study. Depending on the pattern of motor response, dispersible or slow release forms of levodopa can be substituted for standard forms. Motor fluctuations in elderly patients

With time, levodopa-induced motor complications develop in all patients, though these tend to be less obvious in late onset disease compared to younger patients

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with PD. How levodopa induces these changes is unknown, but the mechanism probably involves altered dopamine mediated gene regulation in the basal ganglia, coupled with central changes in the pharmacokinetic handling of levodopa. End of dose fluctuations may respond to the use of tolcapone, a drug that inhibits the peripheral and central metabolism of levodopa, thereby increasing the bio-

availability of oral doses, or the use of sustained release levodopa (Sinemet CR, Madopar CR). Elderly patients with a short duration of response to levodopa who remain parkinsonian for long periods of the day may improve with the introduction of oral dopamine agonist drugs (bromocriptine, pergolide, ropinirole, cabergoline) as adjunctive therapy to levodopa (Hindle et al. 1998). The dopamine agonist apomorphine may be useful in selected elderly patients with PD who respond to levodopa, to control severe 'off' period symptoms (Hughes et a!. 1993). Apomorphine can also be used perioperatively in elderly patients undergoing surgery, particularly after femur fracture. Those patients with significant cognitive impairment, levodopa-induced hallucinosis, or who are unresponsive to levodopa, will not benefit from the introduction of dopamine agonist therapy. Severe 'on' period dyskinesia in patients with long standing PD that impairs function may necessitate a reduction in dopaminergic drug treatment. Dopamine agonists and selegiline tend to exacerbate levodopa-induced dyskinesia and should be withdrawn before finally reducing the dose of levodopa itself. Painful 'off' period dystonia, often appearing in the early morning as cramps in the feet and legs, can respond to rapidly absorbed dispersible formulations of levodopa (Madopar dispersible) taken on waking. Balance and gait problems In elderly patients mobility deteriorates as a result of impaired gait and balance.

Falls inevitably occur, further eroding confidence and increasing immobility. Antiparkinsonian drugs do not help this problem and may make matters worse by impairing cognitive function and increasing postural hypotension. Good multidisciplinary team working remains the most important aspect in managing this problem. Physiotherapists can work to maintain muscle condition and increase confidence and can instruct patient and carer on the best way to get up after a fall. The team can provide an important source of support, motivation and encouragement to patients facing these difficulties. Neurosurgery Neurosurgical approaches to the treatment of PD, involving highly selective lesion-

ing, fetal grafting and deep brain stimulation, are undergoing resurgence at the present time. Unfortunately, it is unlikely that many elderly patients with PD would be suitable candidates for neurosurgical treatment. However, some physiologically

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fit elderly individuals, particularly if troubled by severe tremor and who respond

well to levodopa, may benefit from neurosurgical procedures (Obeso et al. 1997). The major benefit of neurosurgery is to improve 'off' period disability. The quality of the 'on' response to doparninergic drugs is not improved. Depression The most common neuropsychiatric complication of PD is depression, occurring

in around 40% of subjects studied (cummings 1992). However, depression is also the most common psychiatric disorder in older people and community studies of elderly people without P1) over 65 years old indicate a prevalence of significant depression of around 15% (Livingstone et al. 1990, Saunders et al. 1993). Furthermore, studies of elderly inpatients suggest a prevalence for depression of around 35% in this group (Jackson and Baldwin 1993). Diagnostic criteria for depression in PD The prevalence of depression detected in any study will depend upon the criteria

used to diagnose depression. Depression remains, in essence, a clinical judgement based on assessment, observation and clinical experience. Self report 'paper and pencil tests' such as the GDS, structured interviews such as the present state examination and computerized psychiatric assessment schedules have been used in an attempt to standardize and refine this diagnosis. The profile and severity of depressive symptoms has been used in diagnostic systems such as the Diagnostic and Statistical Manual of Mental Disorders (American Psychiatric Association 1994) to define 'major depressive disorder' and a persistent but less severe condition called 'dysthymic disorder'. There can be no doubt that self rated questionnaires used to assess mood disorder in PD will inevitably confuse somatic problems due to physical disease with mood disorder and thus will over report mood disturbance. To some extent this has been addressed by the development of self rating scales such as the GDS. The GDS avoids over emphasis on the somatic/vegetative symptoms of depressive illness that could also arise from physical illness and functional disability. Both autonomic failure, which is well recognized in PD, and emotionalism can

be mistaken for depression in PD and lead to over diagnosis of this condition (Madeley et al 1992). Epidemiology of depression in PD

using standardized criteria for both depression and PD in hospital-based 1992) and community populations (Tison et al. 1995, Tandberg et al. 1996, Meara et al. 1999) report a prevalence for depression of around 40%, though much lower figures have also been reported (Brown and Maccarthy 1990, Madeley et al. 1993). Depression appears to be more common in elderly patients with PD Studies

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than would be expected from age alone, though any medical illness is also asso-

ciated with increased levels of depression. One study comparing a sample of patients with arthritis and P1) found that depression scores were similarly high in both disease groups compared with healthy elderly subjects (Gotham et al. 1986). The overall incidence of depression in PD patients over the age of 40 years old has been estimated as around 1 .86% per year, with age specific incidence increasing with age of subjects (Dooniefet al. 1992). This compares with rates in the general population of 0.17%. Nature of depression in PD Although elderly patients with PD express high levels of depressive symptoms the

prevalence of major affective disorder, as diagnosed on L)SM-1II criteria, and other formal psychiatric diagnoses of 'caseness', appears to be low (Madeley et al. 1993, Hantz et al. 1994, Tandberg et al. 1996). The nature of depression in PD may also be characterized qualitatively by pessimism, hopelessness and poor motivation and

the absence of guilt, self blame and worthlessness, compared to elderly subjects with primary depressive disease (Gotham et al. 1986). The profile of depressive symptoms may also alter with disease progression (Huber et al. 1990). Psychotic features (hallucinations and delusional ideas) in major depression in P1) appear to be rare though have not been specifically studied in depressed patients with PD (Brown and MacCarthy 1990). Features of PD associated with depression

A complex relationship exists between depression and the disease variables of age at onset, cognitive impairment and the pattern and severity of motor involvement. Patients with early onset disease, starting before the age of 50 years, may have a greater prevalence of depression compared with late onset disease starting after the age of 70 years (Starkstein et al. 1989). The relationship between disease severity and depression is unclear, with some (Gotham et al. 1986, Meara et al. 1999) but not all studies (Huber et al. 1988) supporting a positive association between the two. However, the DATATOP analysis (Jankovic et al. 1990) revealed that patients with unilateral disease were less likely to be depressed than patients with more severe bilateral disease. Depression in PD appears to be associated with disability, particularly at times in the natural history of the disease when disability is rapidly changing (Brown et al. 1988). Stress and social support are also predictors of the risk of depression in PD (Fleminger 1991). Cognitive impairment appears to be more consistently linked with the expression of depression in PD (Starkstein et al. 1992, Troster et al. 1995, Tandberg et al. 1996) though this may reflect both the impact of depression on cognitive and the depressing effect of the awareness of impaired cognition. Some evidence

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that the cognitive function of depressed patients with PD deteriorates more rapidly than in nondepressed patients (Starkstein et al. 1992). The self rated questionnaires used in many studies to diagnose depression may be inappropriate for patients with cognitive impairment and dementia.

suggests

The basis of depression in PD There is no simple

mechanism to explain the relationship between depression and PD. It is likely that depression has its origin both in the neurobiology of PD and in the 'reactive' psychological response to living with the unpredictable dis-

ability of a progressive neurological disease. However, motor disability is not strongly linked to depression in PD and the treatment of motor disability with anti— parkinsonian drugs is not usually associated with resolution of coexisting depressive symptoms. Evidence exists supporting a link between depression and biological mechanisms

that cause PD. Patients may present with depression before the onset of motor symptoms in PD (Mayeux et al. 1981) and mood swings are clearly linked to 'on—off' motor fluctuations in PD (Cantello et al. 1986, Menza et al. 1990). Brain serotonin is reduced in PD and has been implicated in the pathophysiology of depression in PD (D'Amato et al. 1987). An improvement in mood and drive has been reported after the addition of the serotonin precursor !7tryptophan to levodopa (Coppen et al. 1972). Lumbar CSF levels of 5-hydroxyindoleacetic acid have

been claimed to be reduced in depressed patients with PD compared with PD patients without depression and normal subjects (Mayeux et al. 1984, Kostic et al. 1987, Mayeux et al. 1988), though values between groups overlap and the reproducibility of these findings is uncertain (Sano et a!. 1989). Serotonin precursors have been shown to improve depression in PD patients with concomitant rise in CSF metabolite levels (McCance-Katz et al. 1992). Data from studies of platelet imipramine binding and fenfluramine induced prolactin release, support a greater serotonergic deficit in depressed PD patients compared with nondepressed PD patients and normal age matched subjects without PD (Schneider et al. 1988, Kostic et al. 1993).

A strong case can be made for the involvement of dopamine itself as depression in PD has been shown to be associated with more severe loss of cells in the meso-

corticolimbic projection. Furthermore, mood swings are known to complicate rapid changes in striatal dopamine transmission ('on—off' swings) in PD (Menza et al. 1990). Attempts to define a neuroanatomical basis for depression in PD have produced conflicting results with both right- and left-sided hemiparkinsonism being claimed to be associated with more severe symptoms of depression and anxiety (Starkstein et al. 1990, Fleminger 1991). Studies using positron emission tomography

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comparing depressed and nondepressed PD patients and depressed and nonde-

pressed age matched controls suggest that depression in PD and primary depressive illness both reflect abnormal dysfunction in the medial prefrontal cortex (Ring et al. 1994). Treatment of depression in PD Depression in PD will respond to the general psychotherapeutic support of a spe-

cialist therapy team. More specifically, antidepressant therapy and ECT have been shown to be effective in treating depression in PD (Balldin et al. 1981, Holcomb et al. 1983). Cognitive therapy is also likely to be effective but has not been formally evaluated. ECT can also improve motor symptoms (Fall et al. 1995). There have been only four double-blind studies of the drug treatment of depression in PD, spanning over 30 years, and involving a total of around 100 PD patients of all ages (Strang 1965, Laitinen 1969, Andersen et al. 1980, Goetz et al. 1984). The primary goal of three of these studies was to confirm the utility of tricyclic antidepressants (Strang 1965, Laitinen 1969) and bupropion, an indirect dopamine agonist (Goetz et al. 1984), in treating the motor symptoms of PD. The criteria used both for the diagnosis of PD and depression in the two early reports are unclear. The only study using standardized criteria for PD and depression did not report the proportion of patients whose depression clinically responded to drug treatment (Andersen et al. 1980). These studies taken together suggest that drug treatment is

effective in the treatment of depression in PD, though side effects to the antidepressant drugs were reported. The selective serotonin reuptake inhibitors (SSRI5) are better tolerated than tricyclic antidepressant drugs in elderly subjects and are equally effective in treating primary depressive illness. Evidence linking serotonin to depression in PD also indicates that this class of drug should be effective in this group of patients. Some concerns have been raised over evidence indicating that two drugs of this class, fluoxetine and paroxetine, can cause drug-induced parkinsonism (Steur 1993, Jiminez-Jiminez et al. 1994). The MAO-B inhibitor selegiline, used to treat PD, in combination with SSRI's may cause the toxic serotonin syndrome. However, the combination of selegiline and fluoxetine, despite these con-

cerns, does not appear to be associated with any significant problems in daily clinical use (Waters 1994, Richard et al. 1996). There have been two recent reports

of the efficacy and safety of the SSRI sertraline in depressed patients with PD (Meara et al. 1996, Hauser and Zesiewicz 1997). Sertraline has not been reported to cause drug-induced parkinsonism and is in fact a weak dopamine reuptake blocker. We have also studied the long-term use of the drug in a further group of 41 patients with PD reporting high levels of depressive symptoms based on the self report GDS scale. After three months treatment, 39% of this group showed a reduction of 50% or more in the score on the GDS. No deterioration in motor function

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observed in any of the patients in the study (unpublished observations). There

would appear to be considerable opportunity costs of using the SSRIs to treat depression in P1) compared to the older tricyclic antidepressant drugs, although the potential economic advantage remains to be formally evaluated. Carer distress in PD The majority of elderly patients with FL) living at home receive help from their

spouse, or other close family members. An early study found evidence of an association between carer distress and functional impairment in the patient (Calder et al. 1991). However, two recent studies have suggested that distress in carers was best predicted by the level of depression, rather than physical disability, in the recipient of care (Miller eta!. 1996, Meara et al. 1999). The former study also demonstrated for the tirst time that carers of subjects with PD showed significantly elevated levels

of distress and strain compared to age matched controls with spouses in good health. The detection and aggressive treatment of depression in patients with PD may be important in treating the distress of their carers. Carer distress could be an

important and valid outcome measure for the treatment of depression in the patient. Detecting and treating depression in

PD

Despite the high level of depressive symptomatology in PD and evidence for the

efficacy of drug therapy few patients appear to receive specific treatment. In our community-based study of 132 patients over the age of 60 years with PD, only 7% were in receipt of antidepressant medication despite the fact that 64°A of this group scored in the depressed range on the GDS-15 (Meara et al. 1999). In this study 79 carer spouses were identified and 35% of this group of carers had significant depressive symptoms. Only around 7% of carers scoring in the depressed range on the GDS were being treated with antidepressant drugs. Dementia

Dementia is a major factor in the management of PD in elderly subjects, limiting both the drug therapy that can be offered and the quality of life that can be achieved for patient, carer and family. Cognitive impairment in PD, particularly if complicated by hallucinations and delusional ideas, is the most potent risk factor for admission to nursing home care. Epidemiology of dementia in PD Dementia is common in patients over the age of 65 years with PD although preva-

lence figures vary between 10—44% depending on the diagnostic criteria used for dementia, PD, and the nature of the study population (Cummings 1988, Mayeux

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et al. 1995, Hobson and NIeara 1999). Operational criteria for dementia resulting from AD are usually used to define dementia in PD (American Psychiatric Association 1994), though specific criteria have also been suggested et al. 1992, Tison

(Cummings and Benson 1983). In simple terms dementia can be defined as global cognitive impairment of sufficient severity to impair social function and employment in the absence of delirium and other general medical disease known to cause

cognitive impairment. The risk of developing dementia is at least doubled in patients with PD compared to age matched subjects without the diagnosis of PD. In one study (Biggins etal. 1992) there was a cumulative incidence of dementia of 19% in surviving members of a cohort of 87 patients with PD followed up for over four years. The risk of dementia increases exponentially with age such that 65% of surviving members of a cohort of patients with PD over the age of 85 years are likely to be demented (Mayeux et al. 1990). Risk factors for dementia

Depression, cognitive impairment and age interact in a complex manner and most studies have shown depression to be a risk factor for subsequent dementia (Troster

et al. 1995). Risk factors for dementia in PD apart from age and depression have been shown to be: late onset disease, postural imbalance and gait disorder subtype, severe motor deficits, rapid progression of PD, and severe facial akinesia/hypomimia (Ebmeier et al. 1990, Jankovic et al. 1990, Stern et al. 1993, Viitanen et al. 1994, Marder et al. 1995). Causes of dementia in PD

Elderly patients with dementia predating parkinsonism by at least one year should probably be considered to have a primary dementia, such as AD, or dementia with Lewy bodies complicated by parkinsonism. Parkinsonism predating dementia by

at least one year can be considered to be 'PD with dementia'. In this situation dementia can arise from the same causes as for primary dementia as well as from typical brainstem PD pathology alone. Dementia in PD is usually never definitively

diagnosed in elderly patients as so few brains are examined in detail after death. There are many causes of dementia in PD. The striking relationship of age to dementia in PD indicates the importance of aging changes and the impact of other concurrent age related dementing disease in the aetiology of dementia in PD. These include the primary changes of PD as well as concurrent pathology due to AD and cerebrovascular disease.

Dementia with Lewy bodies

There remains considerable uncertainty over the nosology of the condition called dementia with Lewy bodies (Okazaki et al. 1961, Kosaka 1978, Gómez-Tortosa et al.

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Holmes eta! 1999). Some investigators claim that dementia with Lewy bodies is the second commonest cause of dementia and can be detected in life using agreed clinical diagnostic criteria (Byrne eta!. 1989, McKeith et al. 1992, McKeith eta!. 1995). The 1998,

primary finding at post mortem in this condition are plentiful Lewy bodies in the limbic areas of the temporal lobe and other cortical areas. Lewy bodies may also be present in subcortical regions. The pathology in older patients with this condition is

nearly always complicated by the additional presence of senile plaques and neurofibrillary tangles, similar or identical to those found in AD. This condition is variously felt to be an extension of PD (Gibb eta!. 1985, Lieberman 1997), to be a type of AD (Weiner et a!. 1996), or to represent a specific type of primary Lewy-body disease (McKeith et a!. 1995). As a result the commonest cause of dementia in PD is claimed

by some to be AD, and by others to be dementia with Lewy bodies. Cortical Lewy bodies, especially in limbic areas, appear to be present in many if not all PD patients even in the absence of cognitive impairment (Hughes eta!. 1992). Furthermore, the density of Lewy bodies in the cortex does not appear to be related to the degree of regional brain atrophy found at post mortem (Mann and Snowden 1995). Neuropsychological features of dementia and pre-dementia in PD

The characteristics of the neuropsychological deficits in PD patients with dementia or pre-dementia and the relationships of such impairments to the underlying disease processes causing dementia are poorly understood (Brown and Marsden 1990). A classification of dementia into cortical and subcortical dementia has been proposed, based on the results of neuropsychological tests in cortical diseases such as AD and subcortical diseases such as Huntington's chorea. The concept of cortical and subcortical cognitive impairment in PD remains controversial (Starkstein eta!. 1996, Lieberman 1997). Many patients with PD demonstrate cognitive deficits that tend to be of subcortical type and may be relatively nonprogressive (Cooper et at. 1991), though a minority of such patients will progress to a full subcortical dementia. Dementia in PD is much more commonly of cortical than subcortical type. Lieberman's (1997) review suggests that two-thirds of dementia in PD is due to AD related changes with or without cortical Lewy bodies, the remaining third being due to Lewy-body pathology. This latter group appears to consist largely of patients with cortical and brainstem Lewy bodies. Patients with presumed dementia with Lewy bodies may show more evidence of impaired attention, executive function and visuospatial difficulty compared to patients with AD. On the whole, most investigators have found little difference between the neuropsychologicat

deficits of AD and PD with dementia. However, impaired visual reasoning (Starkstein et a!. 1996, Mahieux eta!. 1998) and verbal fluency (Jacobs et a!. 1995) have been proposed to be neuropsychological deficits that may serve to distinguish PD with dementia from concurrent AD.

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Management of dementia in PD

Elderly patients and their carers need to be told about the risks of cognitive impair-

ment in PD and when cognitive impairment appears to be developing or accelerating, opportunity should be given to address matters such as living wills and advance directives. All drug treatment should be critically reviewed, particularly with regard

to any anticholinergic actions of drugs prescribed for other concurrent disease. Most antiparkinsonian drugs further impair cognitive function in dementia, either by impairing alertness or increasing confusion. Patients should be managed on simple regimes of standard levodopa whenever possible. The dose of levodopa should be reduced to the minimum amount that maintains parkinsonian symptoms at levels acceptable to the patient and carer. Reducing antiparkinsonian drug treatment can improve cognitive function by relieving drug-induced delirium and psychosis and increasing alertness. There is no specific drug therapy for dementia in PD. However, the acetylcholinesterase inhibitors tacrine and donepezil may be effective in treating certain types of dementia in PD, though there are no data as yet to support such use. Psychosis In both late onset and long standing PD neuropsychiatric complications related to

drug therapy are common and result in acute confusional states, sleep abnormal-

ities, hallucinations and paranoid delusions (Celesia and Wanamaker 1972, Nausieda et al. 1984, Factor et al. 1995). Delusions of sexual infidelity are common and may result in elderly patients refusing to attend day centres or be admitted for respite admissions for fear of leaving their partner. A classification of psychosis in PD has recently been suggested (Peyser et al. 1998), which emphasizes the distinc-

tion between psychosis associated with and without coexisting delirium and dementia. Although all antiparkinsonian drugs can provoke these reactions, anticholinergic drugs, dopamine agonists and selegiline appear to cause problems much more commonly than levodopa itself. A strong case can be made for avoiding the use of anticholinergic drugs in P1) as these drugs cause cognitive impairment in addition to psychiatric side effects. However, the ability of such drugs to alleviate sialorrhoea in late stage disease, albeit at the expense of a dry mouth, may make patients reluc-

tant to stop such therapy. A balance has to be maintained in late stage disease between mobility and mental clarity. Simple hallucinosis that does not distress the patient or carer may be tolerated without reduction in drug treatment (HaeskeDewick 1995). Atypical neuroleptic drugs such as clozapine or olanzapine that act on limbic rather than striatal dopamine receptors may be useful in selected patients to specifically control dopaminometic psychosis and allow a larger dose of levodopa to be prescribed to preserve mobility (Kahn et al. 1991, Meltzer et al. 1995,

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patients with psychosis not clearly related to dopaminergic drug therapy do not tol-

erate atypical neuroleptic drugs, due to the sedation and delirium such drugs induce.

Autonomic function in elderly patients with PD Autonomic function involving both the sympathetic and parasympathetic system can be impaired in PD and can lead to a number of prominent and disabling symp-

toms. These include bladder dysfunction, constipation, dizziness on standing, abnormal sweating, sexual dysfunction and breathlessness. Although tests of autonomic function are normal in early PD, with disease progression increasing evidence of autonomic dysfunction becomes apparent (Gross et al. 1972, Sandyk and Awerbuch 1992, van Dijk et al. 1993, Martin et al. 1993). However, autonomic function also declines with age and is influenced by the presence and drug treatment of concurrent diseases such as diabetes and hypertension. Drugs used to treat PD may also adversely affect the autonomic nervous system. As a result, nearly all elderly patients with PD demonstrate some abnormalities of autonomic fimction. Symptoms usually attributed to autonomic failure, such as sweating and dizziness on standing, appear to correlate poorly with documented tests of autonomic function (Berrios et al. 1995). The extent of autonomic failure compatible with a diagnosis of PD has not been established, though the development of severe symptoms of autonomic dysfunction in a patient with parkinsonism within two years of the onset of syniptoms suggests a diagnosis of multiple system atrophy. Orthostatic hypotension

Blood pressure and pulse rate need to be carefully monitored in elderly patients with PD and should be assessed lying and standing under controlled conditions with the patient resting supine for ten minutes before standing. The blood pressure and pulse rate should be taken supine and again after two minutes and five minutes standing. A fall in systolic blood pressure of 2OmmHg or more and/or a fall of lOmmHg or more in the diastolic blood pressure is the most commonly used standard definition for the presence of orthostatic hypotension, whether or not there are any symptoms. Using this definition, orthostatic hypotension was detected in nearly 60% of a group of patients with PD attending a specialist clinic (Senard Ct al. 1997). Falls in blood pressure occur particularly on rising in the morning and getting up from the meal table. At these times patients are at most risk of syncope and falls. Large drops in systolic and diastolic blood pressure on standing or after exercise do not always cause symptoms, but will do so under any conditions of stress such as the development of any intercurrent infection or prolonged bed rest. The most common cause of orthostatic hypotension is the drug therapy of

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patients

with PD rather than PD itself, or age related autonomic dysfunction.

Levodopa and dopamine agonists cause falls in postural blood pressure by central

and peripheral dopaminergic effects. Selegiline may potentiate this effect (Churchyard et al. 1997). Domperidone appears to improve orthostatic hypotension secondary to diabetes and other diseases (Montastruc et a!. 1985, Lopes de Faria et al. 1988) though its place in PD remains unclear. As a result of multiple pathology elderly patients with PD are often receiving other hypotensive drugs such as antihypertensive drugs, antianginal drugs, diuretics, sedatives, antidepressants, and anticholinergic drugs. Many of these prescriptions will be unwarranted and can be withdrawn to good effect. It is surprising how many elderly patients with PD and low resting blood pressure are on active treatment with antihypertensive drug therapy. Diuretic d rugs are often used inappropriately to treat ankle swelling resulting from immobility rather than cardiac failure. Elderly patients with PD admitted into hospital with a diagnosis of collapse due to 'transient ischaemic attack' or 'stroke' very often turn out to have syncope due to

drug and disease induced orthostatic hypotension. Careful history taking with witness accounts coupled with appropriate assessment of autonomic function, help resolve the diagnostic difficulty in most cases. As prolonged syncope can result in reflex anoxic seizures, a history of 'limb twitching' in the 'funny turn' should not lead to the automatic diagnosis of fits and prescription of inappropriate anticonvulsant therapy. in frail patients with late stage disease orthostatic hypotension can

become manifest on transfer from bed to commode or wheelchair even in the seated position. Orthostatic hypotension should initially be treated by simple measures. Raising of the head of the bed at night can help early morning problems, as can drinking strong coffee before rising. Elasticated garments should compress the lower abdomen to be of any value and are rarely practicable. The patient and carer need to be told about the importance of making changes in posture slowly, and the times of greatest risk of postural syncope, such as getting out of bed at night or in the morning and standing up after meals. In reality patients have usually come to such realisations on their own. Critical review of all prescribed medications can suffice to control symptoms. If this fails to control symptoms a change in antiparkinsonian drug therapy may be necessary, particularly the withdrawal of selegiline and reduction of dopamine agonist drugs. In frail patients on monotherapy with levodopa, reduction of this drug may cause increasing parkinsonism and immobility. In this situation the use of specific drugs to combat orthostatic hypotension such as fludrocortisone (0.1 mg to 1 mg per day) and nonsteroidal anti-inflammatory drugs such as flurbiprofen

may be necessary. Most elderly patients with orthostatic hypotension can be managed without resorting to the use of specific drugs.

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Bladder symptoms

Symptoms of urge, urge incontinence, frequency and distressing nocturia are common in late stage and longstanding PD. These problems result from bladder detrusor muscle hyperreflexia coupled with abnormalities of the external urethral sphincter. There is still uncertainty about how often urinary dysfunction reflects a specific PD related urinary dysfunction as opposed to the effects of aging on the bladder and external sphincter (Gray et al. 1995). The clinical picture in male patients is often complicated by additional obstructive symptoms suggestive of

prostatism. Indeed, many patients may have previously undergone prostatic surgery, which has usually resulted in worsening of urinary symptoms. Urodynamic studies do not support the existence of specific abnormalities in PD and are rarely useful in directing treatment (Khan etal. 1989). An exception to this is in patients referred with prostatism when the demonstration of external sphincter abnormalities should preclude surgery. It is unclear how documented urinary

problems respond to antiparkinsonian drug therapy. In most elderly patients bladder dysfunction does not respond predictably to antiparkinsonian drug treatment (Fitzmaurice et al. 985), though some recent work published only in abstract form suggests that the dopamine agonist pergolide may decrease detrusor instability due to a Dl receptor agonist action. Voiding difficulty in the 'off' state can improve after a subcutaneous apomorphine injection by relaxing the striated urethral sphincter (Christmas et al. 1988, but see Gray et al. 1995). Urological examination of the patient is necessary before embarking on treat-

ment and this should include a rectal examination, a urinary flow rate and a bladder ultrasound scan to determine residual volume. A bladder specimen of urine by the use of disposable 'lofric' catheter should be obtained for culture. In the absence of a significant residual volume, oxybutynin can help nocturia and urge due to an unstable bladder but at the risk of central and peripheral anticholinergic side effects. In some patients nocturia is so disruptive and tiring that this risk is justified. Desmopressin given intranasally at night may also benefit some patients though blood urea and electrolytes need to be measured. The guidance and support of a specialist nurse continence advisor can be an important part of the long-term

management of this problem. Despite the frequency of urinary problems few patients require intermittent self catheterization or suprapubic indwelling urinary catheters. Bowel symptoms

Constipation, a reduction in normal bowel movements for an individual, is very

common in elderly patients with PD (Edwards et al. 1993, Byrne et al. 1994). Immobility, poor diet, drug therapy, poor pelvic floor contractions and autonomic dysfunction with delayed transit time all contribute to the problem of constipation.

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Constipation needs to be addressed vigorously to avoid the development of faecal

impaction. Loss of the gastrocolic reflex due to constipation may impair levodopa

absorption by delaying gastric emptying. Exercise and a good fluid intake are important aspects of bowel maintenance although in most patients aperients will also be required. Osmotic laxatives, such as lactulose, can be effective in some patients, but stimulant laxatives, such as senna, are likely to be needed in most elderly patients. A combination of stool softener and stimulant such as codanthramer can be particularly useful in elderly patients. Co-danthramer discolours the urine and in the presence of urinary incontinence can cause an irritative dermatitis. Polyethylene glycol, a novel laxative agent that hydrates the stool by

osmotic effect, results in a more normal bowel action and has recently become available in the UK. Bulking the stool with fibre in elderly patients can easily provoke faecal incontinence. Cisapride may also be useful in managing constipation in some patients (Jost and Schimrigk 1993). Faecal impaction can occur in frail patients with late stage disease and can present with confusion, nausea and vomiting and overflow diarrhoea. If rectal examination does not demonstrate impacted hard stool then a plain abdominal radiograph will show the level of impaction. A

combination of oral laxatives, manual evacuation and phosphate enemas are usually required to resolve the situation. The importance of bowel maintenance in late stage PD needs to be emphasized to the patient, nursing home staff, communit)' nurses and the family. Speech and swallowing difficulties in PD With disease progression problems with speech and swallowing soon become apparent in elderly patients (Stroudley and Walsh 1991, Edwards et al. 1993, 1994, Byrne et al. 1994, Bine et al. 1995, and see Chapter 14). Speech problems appear to develop more quickly in late onset PD and communication difficulties are a major source of disadvantage to elderly patients. Response to antiparkinsonian medica-

tion is disappointing but evidence exists to support the clinical effectiveness of speech therapy (Ramig et al. 1995). In elderly subjects akinesia and muscle rigidity

commonly affect swallowing mechanisms, presenting first as pooling of saliva before progressing in time to cause embarrassing sialorrhoea and choking and coughing at meals. Likewise, response to drug treatment is disappointing, but drug therapy, particularly in previously undiagnosed patients, has been reported to improve swallowing problems (Fonda and Schwarz 1995, Thomas and Haigh 1995). Although causing distress and handicap it is rare for a patient with PD to become anarthric or to require percutaneous gastrostomy feeding. Specialist support is needed from speech and language therapists and from dieticians to rnininiize disability and handicap and to maintain nutritional status (Davies et al. 1994).

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Balance and falls

Impaired balance and an increasing risk of falls is an early feature of late onset PD

and of PD in older patients (Klawans and Topel 1974). The pathogenesis of impaired postural reflexes is unknown but may involve neurotransmitter systems other than dopamine as well as age related changes in the brain and peripheral nervous system. Falls are common in elderly patients with PD and such patients are at increased risk of fracture (Johnell eta!. 1992). Bone mineral density appears to

be lower in PD than that expected from the effects of age alone (Taggart and Crawford 1995). There is also a suggestion that femur fracture in PD is associated with a poorer outcome than in age matched subjects without PD (Gialanella et al. 1990). FaIls are life threatening and also further severely restrict mobility due to loss of confidence. This sets in motion a spiral of immobility leading to muscle deconditioning, further immobility and increased risk of falls. Falls are a major cause of institutionalization in PD. Falls due to akinesia should respond to increasing dopaminergic drug therapy and likewise falls due to drug-induced involuntary movements can be helped by reducing antiparkinsonian drugs. More general gait and balance deterioration is worsened rather than improved by increasing medication because of drug-induced exacerbation of cognitive impairment and drowsiness. Freezing can also result in falls and although noradrenergic drugs have been claimed to reduce freezing epi-

sodes in experimental trials no drug therapy has been found to be generally effective.

The best approach is to review drug prescriptions critically and to use a general preventive strategy (Tinetti et a!. 1994) coupled with exercise and physiotherapy, (Campbell et al. 1997). Problems with vision and foot care that can be remedied should be urgently addressed. Sleep disturbances Most elderly patients with PD complain of disturbed nights though only around a

third complain of poor quality of sleep (Lees et al. 1988). In several instances careful questioning can reveal the cause to be motor problems associated with PD (difficulty turning over in bed, painful leg cramps, dystonia, restless legs, leg jerks), more general musculoskeletal problems (back pain), nocturia, vivid dreams, visual hallucinations and mental restlessness or depression. Some of these problems are clearly due to PD and may respond to dopaminergic treatment last thing at night (controlled-release levodopa in the form of Sinemet CR or a dopamine agonist

such as pergolide) or antidepressant therapy. Other symptoms such as vivid dreams, dyskinesia and hallucinations will require a reduction in dopaminergic drug treatment later in the day. Hypnotics are best avoided but the use of short acting hypnotics used every few days, or at most in weekly cycles separated by a

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fortnight, may be justified. In reality at least a third of elderly patients will have already been taking prescribed hypnotic drugs when first assessed and have developed dependency on them to sleep, albeit poorly. There is increasing evidence to suggest that sleep abnormalities are common in

treated PD patients and the term 'sleep fragmentation' has been used to describe this (Nausieda et al. 1984). These abnormalities are in excess of those found simply due to age alone. There is a close association between rapid eye movement sleep (REM sleep) abnormalities in PD, vivid dreams and the presence of dopaminergic drug related hallucinations (Comella et al. 1993). Although polysomnographic sleep measures may be abnormal in untreated PD patients, significant REM abnormalities appear to be related to duration of disease and length of exposure to dopaminergic drug therapy. Comella et al. (1993) found that half of the patients in their study had evidence of REM behaviour disorder. In REM behaviour disorder instead of atonia there is purposeful movement of the limbs and vocalization in REM sleep. This can even amount to sleep violence, as has been reported in a recent study of REM sleep behaviour disorder in which 15% of 61 patients with PD were

reported by their caregivers as having kicked and punched the caregiver while asleep (C. Comella, unpublished observations). Sexual dysfunction Erectile problems and impotence has been reported by 60% of male patients with

PD (Brown et al. 990). This study took place in a small number of younger patients

(mean age 52 years). Sexual dysfunction in older patients has not undergone detailed study. Although motor problems due to PD and autonomic dysfunction may play a small part, it seems likely that the bulk of sexual difficulty expressed by patients and partners is due to psychological factors and stress. Elderly patients are often not approached about sexual problems because of fear of offending a senior citizen, embarrassment on the part of the health care professional or an unjustified

assumption that this is not a relevant area for assessment. Enquiries into sexual difficulties in elderly subjects need careful and sensitive handling. Elderly patients are often denied the privacy that such enquiries demand in their encounters with health and social services. An assessment of how important such sexual problems are to the patient and partner is vital to further management. Drug-induced sexual dysfunction should not be overlooked. Commonly prescribed drugs causing this problem are most antidepressant drugs and beta-blockers. After discussion, some patients and their partners will need referral for specialist urological advice. Palliative care In late stage disease the time will come when it is evident that a terminal phase of

the disease is approaching, as frailty and dependency increase. Often patients will

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Parkinson's disease and parkinsonism be in nursing homes, particularly if cognitive impairment is advanced. At this time

good communication between hospital services, primary health care teams and the general practitioner is essential to optimize management. Treatment plans need to be established in the event of any sudden medical deterioration. These need to be understood and agreed by the patient, family and all the health care professionals involved. Antiparkinsonian drug treatment will need to be reduced if side effects, such as confusion, outweigh any obvious benefit in the patient confined to bed and needing all nursing care. Levodopa can be administered via nasogastric tube in terminal care to control severe rigidity and tremor. Active treatment ofbladder, bowel,

respiratory and painful symptoms are usually required to relieve distress and suffering.

Parkinsonism not due to PD The differential diagnosis of parkinsonism has already been discussed (see Chapter

2). The commonest cause of parkinsonism in this group is drug-induced (Gershanik 1994 and see Chapter 4). Excluding long standing prescription of neuroleptic drugs for psychiatric illness, drug-induced parkinsonism (DIP) is most often caused by prochlorperazine prescribed for dizziness and metclopramide for gastrointestinal symptoms. A proportion of elderly patients with presumed DIP will, despite stopping the offending drug, still show signs of parkinsonism and progress to PD. With the exception of DIP no specific treatment exists for these conditions. Some patients with confirmed multiple system atrophy and progressive nuclear palsy do appear to have responded to levodopa, at least over the initial course of the illness (Hughes et al. 1992). Given this finding and the known difficulties in accurate diagnosis, it would seem reasonable that a trial of levodopa therapy should be undertaken in most cases of parkinsonism even though the presence of PD is thought to be unlikely. In the absence of benefit treatment can be withdrawn. Levodopa is often poorly tolerated by patients with parkinsonism due to multisystem degenerations. Nonpharmacological approaches are the mainstay of treatment and patients in this group need regular assessment, therapy and support.

The specific difficulties faced by elderly patients with parkinsonism are effectively the same as those discussed above, though are usually more severe and progress more rapidly. Parkinsonism due to multisystem degenerations (multiple system atrophy, progressive supranuclear palsy) in elderly subjects is usually a very unpleasant and rapidly progressive disease lasting on average six years from first symptoms. In progressive supranuclear palsy (Litvan et al. 1996) falls and balance problems were the presenting feature in 63% of patients. Falls seemed particularly common in older patients at onset of symptoms. Complaints of disturbed vision

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are

frequentJy reported due to the characteristic impairment of the voluntary

control of eye movements. In this disease there is early involvement of speech and swallowing. Early signs of memory loss and frontal lobe impairment are common. In multiple system atrophy the clinical picture can be very varied, though severe dysarthria, dysphonia, respiratory stridor and autonomic dysfunction is typical of the striato nigral variant (Quinn 1989). Rehabilitative principles are similar to those used in the management of PD but, in multisystem degenerations, the clinical situation reaches the stage of palliation

much more quickly than in PD. Depression needs to be detected and treated, although depression in multisystem degenerations has received little attention (but see Pilo et al. 1996). Patients with parkinsonism due to multisystem degenerative disease are much more likely than patients with PD to be wheelchair bound and to

require additional support with gastrostomy feeding, tracheostomy and suprapubic catheterization. Before embarking on life sustaining measures in diseases such as MSA and PSP, careful ethical consideration needs to be given to the implications of such a course of action. Detailed discussion with the patient and carer must take place before a medical decision can be reached and the patient's consent is needed for life sustaining measures to be undertaken or to be withheld. Support for the patient and caret is needed throughout the course of the illness. Elderly patients with gait disorders due to vascular disease (see Chapter 6) are

difficult to treat effectively. Most patients should receive a trial of levodopa, although the drug will not help if the diagnosis is correct. Attempts to retrain gait by treadmill walking are undergoing evaluation. The blood pressure of patients on

antihypertensive medication should be carefully monitored to avoid over and under treatment. Antiplatelet drugs are routinely prescribed but are unlikely to be helpful in preventing further gait deterioration, although giving modest protection against vascular events.

Apomorphine treatment in elderly patients with PD In the UK the dopamine agonist apomorphine has been licensed for the treatment

of PD since 1993 and is also widely available in Europe. Neither apomorphine nor domperidone is currently licensed for use in the US, but may be obtained for individual patients. Apomorphine was first reported to improve the motor symptoms of PD in 1951 (Schwab et al. 1951), but oral administration was associated with uraemia (Cotzias

et al. 1970). It was not until the parenteral administration of apomorphine was adopted, coupled with the use of domperidone to reduce the peripheral dopaminergic effects of nausea, vomiting and postural hypotension (Corsini et al. 1979) that apomorphine became a clinically useful drug with which to treat PD (Hardie

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al. 1984). Currently apomorphine is administered subcutaneously by intermittent injection (a preloaded adjustable dose pen system for self injection is now available in the UK) or by continuous waking day subcutaneous infusion via a syringe driver system (Stibe et al. 1988). The benefit from apomorphine is maintained over five years' follow-up (Hughes et al. 1993). The major advantage of apomorphine is that, given by intermittent subcutaneous injection, it can rapidly turn a patient back to the 'on' state from an 'off' state that is refractory to oral medication. In addition to open studies, the reduction in 'off' time has been demonstrated et

in two recent double-blind placebo-controlled studies (van Laar et al. 1993, Ostergaard et al. 1995). Open studies have also reported the ability of continuous infusion of apomorphine to reduce total daily 'off' time (Hughes et al. 1993). The mean age of patients in all these studies has been around 60 years. Like levodopa, apomorphine stimulates both Dl and D2 receptors and the magnitude, though not the duration, of the motor response in PD to these two drugs appears to be very similar (Kempster et al. 1990, Rodriguez et al. 1994). Subcutaneous apomorphine has a rapid onset of action (usually under 20 minutes), which lasts for 20—40 minutes. Patients with 'off' periods lasting longer than this are likely to require con-

tinuous infusion therapy. Intermittent injection of apomorphine is usually an adjunctive therapy to levodopa and other oral dopaminergic drugs, though in patients on continuous infusion oral levodopa therapy can be considerably reduced. Apomorphine is clearly the most potent of all the dopamine agonist drugs and also appears to be less likely than other agonist drugs to cause neuropsychiatric side effects (Ellis et al. 1997) and drug-induced dyskinesia. Continuous apomorphine

infusion also appears to cause much less dyskinesia than oral levodopa therapy (Colzi et al. 1997). However, in clinical practice long-term administration of apomorphine is associated with increasing 'on period' dyskinesia (Hughes et al. 1993), which may be attributable to larger coprescribed doses of levodopa in late stage disease.

Despite evidence of clinical effectiveness, apomorphine in the UK still appears to be underused, possibly reflecting the expense of the drug and the need for parenteral administration (Chaudhuri and Clough 1998). This is likely to be particularly true for elderly patients with PD in whom the efficacy and tolerability of apomorphine has not been studied. Criteria for the selection of patients for apomorphine therapy are shown in Table 3.4. Sadly, the majority of patients with late onset PD, and many elderly patients with late stage disease will not meet the suggested criteria for apomorphine use. A combination of factors is usually responsible, including: a variable or poor levodopa response, the absence of identified 'off' periods,

significant cognitive impairment/dementia, general physical frailty, concurrent

medical disease, and the presence of disabling symptoms such as postural

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Table 3.4 Suggested criteria for the selection of elderly patients for apomorphine

a good response to levodopa that is not niaintaincd by oral therapy throughout the day • clearly identifiable 'off motor periods in relation to medication • 'off' period dystonia and restless legs • distressing nonrnotor 'off symptoms such as anxicty, panic, breathlessness, abdominal pain, urinary retention • severe and disabling levodopa-induced dyskinesia • absence of signiicant cognitive impairment/dementia • absence of disabling symptoms such as postural instability and dysarthria that do not respond to dopaminergic drug therapy • absence of concurrent specific disabling medical disease and general frailty • ability of the patient, carer or health care support in the community to administer the drug by injection • availability of resources to fund the drug/administration system •

instability and dysarthria that do not respond to doparninergic drugs. Elderly patients with psychosis clearly related to dopaminergic therapy may benefit from apomorphine as this will allow a reduction to be made in the daily levodopa dose (Ellis et al. 1997). Suitable elderly patients will need an apomorphine challenge test to establish the

effective dose of apomorphine and the tolerability of this treatment. Despite pretreatment with domperidone for three days prior to the test, significant postural hypotension can still occur in frail subjects and preclude the regular use of apo-

morphine. Elderly patients without a carer may be unable to self administer apomorphine, even with the new pen delivery system, particularly if 'off' periods occur suddenly with little warning. Some elderly patients may have difficulty recognizing when their oral medication is beginning to fail, and again, may rely on a carer to identify this situation. Frail elderly patients may prefer the option of con-

tinuous waking day infusion to repeated injections of apomorphine. Clinical impression suggests that apomorphine confers considerable benefit in terms of improved quality of life, though this has not been formally studied. The PD nurse specialist, recently developed in the UK, appears to have an important role in initiating, establishing and monitormg apomorphine therapy. Apomorphine can also be useful in other situations such as the preoperative management and postoperative rehabilitation in elderly patients with PD. An apomorphine challenge test can also be useful in late stage P1) to demonstrate the

continuing presence of dopaminergic responsiveness. This can encourage the physician and patient to strive for improved disease control using oral drug therapy and can confirm the diagnosis of PD.

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Parkinson's disease and parkinsonism The long-term use of apomorphine results in the development of nodules at the

injection site in nearly all patients. These nodules can become painful, and can break down into ulcers with secondary infection. This is usually only seen in patients on continuous infusions, but can occur with intermittent injection regimes. Rotation of injection sites, massage of the skin before and after injection, reduction of apomorphine dose and good injection technique can reduce the incidence of complicated nodules. It is recommended that hepatic and renal function

should be monitored periodically. As with levodopa, autoimmune haemolytic anaemia has been reported as a rare occurrence with the long-term use of apomorphine. Apomorphine, although expensive, is effective in maintaining independence in elderly patients with PD and can prevent institutionalization (with considerably greater financial implications) and should not be denied to patients with PD based on age alone. The proportion of elderly patients with late onset and late stage PD that would benefit from and tolerate apomorphine is unknown. In our own specialist clinic based population over the age of 70 years, around 5% are currently prescribed apomorphine.

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Drug-induced parkinsonism in the elderly Drug-induced parkinsonism Jean Jean P Hubble

Introduction Drug-induced parkinsonism (DIP) (DIP) is is probably the most common form form of of parkinparkinsonism after after Parkinson's Parkinson's disease disease (PD) (PD) in in terms terms of of overall overall prevalence (Hubble (Hubble 1993). 1993). Elderly subjects subjects have have the the greatest greatest risk risk of of developing developing DIP. DIP. The The reason reason for this susElderly ceptibility ceptibility isisunknown. unknown, In In some some older older individuals individuals DIP DIP may mayrepresent represent pharmacologpharmacological (preexisting nigrostriatal degeneration). degeneration). Alternatively, Alternatively, ical exposure exposure of of latent latent PD (preexisting less specific specific pathological pathological changes changes in in the the aging aging brain brain may simply increase increase the risk of DIP and other types of drug-induced side side effects. effects. While various classes classes can While drugs drugs of ofvarious can produce DIP, DIP, the antipsychotic or neuroleptic drugs drugs are most most often often the the agents agents produce the antipsychotic or neuroleptic responsible (Montastruc et al. 1994). DIP pharmacoresponsible DIP is is attributed attributed to the primary pharmacological action logical action of of this this class class of of drugs, drugs, i.e. i.e.dopamine dopamine receptor receptor blockade. blockade. Other Other medications cations without without overt antidopaminergic action only rarely produce parkinsonism (Hubble 1997). 1997). These single case case reports (Hubble These occurrences occurrences are are so so infrequent infrequent that that single reports are often surprisingly, DIP often the the only only documentation of these phenomena. Not surprisingly, DIP due to drugs class of drugs falling falling outside outside the the antidopaminergic antidopaminergic class of medications medications isis not not well well underunderextensively with stood. This chapter will deal first first and most extensively with DIP DIP secondary secondary to to antidopaminergic subsequently, will other causative causative dopaminergic drugs drugs and, and, subsequently, will review review reports reports of of other drugs. It serve as a complete It is is important important to emphasize that this chapter cannot serve complete or or final final treatise treatise on on DIP DIP because because of of the the ongoing ongoing development development of of new new medications medications and evolving recognition side effects effects (Marti-Masso the constantly evolving recognition of drug-induced side (Marti-Masso et al. al. 1996).

Neuroleptic-induced parkinsonism

1950s,following followingits itsintroduction introduction for for the the treatment treatment of psychiatric illness, illness, In the early 1950s, reports were issued neurologissued linking linking the the neuroleptic neuroleptic chlorpromazine with various neurological side side effects effects including including parkinsonism (Anton-Stephens 1954, 1954, Lehmann ical parkinsonism (Anton-Stephens Lehmann and Hanrahan 1954). In these early early series, Hanrahan 1954). In these series, the the incidence incidenceof of DIP DIPwas wasinin the the range range of of 4 ^ 0 % (Kinross-Wright 1955, Hall Hall et et al. al.1956). investigators 4—40% (Kinross-Wright 1954, Goldman Goldman 1955, 1956). Whilst investigators 64

65

Drug-induced parkinsonism agreed on the clinical manifestations manifestations of the syndrome, they varied varied in in their their opinions

regarding causative causative mechanism mechanism and and identification regarding identification of at at risk risk individuals. individuals. A A clear clear occurrence of of parkinsonian parkinsonian signs and and dose dose of of chlorpromrelationship between the occurrence azine administered (Hall et et al. al. 1956). 1956). It was initially initially azine administered could could not not be demonstrated (Hall suggested may be related related to to chlorpromazine chlorpromazine induced induced liver liver damage. damage. suggested that that DIP DIP may However, of liver However, there there did did not not appear to be any any relationship relationship between abnormalities of function tests and the the occurrence occurrence of of this this syndrome syndrome (Hall (Hall etet al. al.1956). 1956). Similarly, Similarly, function tests these others that these investigators investigatorswere wereunable unabletoto corroborate corroborate the the claim claim made made by by others patients whose whose psychosis psychosis responded prone to to patients respondedwell welltoto drug drug benefit benefit were were most most prone develop DIP. DIR In subsequent years, years, as additional neuroleptic compounds compounds were were developed developed and and as the number of of individuals individuals exposed exposed to to this this class class of of drugs drugs grew, grew, several several distinct adverse reactions tone were were described. described. adverse reactions involving involving abnormalities abnormalities in in movement and tone including DIP, DIP, dyskinesia dyskinesia and akaakaIn 1961, Ayd Aydreported reported extrapyramidal reactions including thisia in 39% of (Ayd 1961). In of 3775 3775 patients patients on on neuroleptic medication (Ayd In searching searching for clues to to the the cause of of DIP, DIP,Ayd Aydfound foundthat that the the syndrome syndrome developed over a shorter time period in individuals individuals treated fluorinated phenothiatreated with with the piperazine piperazine and fluorinated zine zine compounds. Specific neuroleptic agents agents

now recognized recognized that that parkinsonism parkinsonism can result from the use of numerous drugs It is now drugs (see Table Table 4.1). among the various types of neuroleptics (see 4.1). Certain Certain neuroleptics, such effects and as thioridazine, have fewer fewer reported reported extrapyramidal side effects and as a result result the risk of DIP may be be less lesswith with their their use. use. However, However,well wellcontrolled controlled comparison comparison studies substantiating this notion are are lacking. In one series series thioridazine thioridazine was was the the third third most most commonly offending offending drug following haloperidol amitriptyline/perphenazine commonly drug following haloperidol and and amitriptyline/perphenazine followed for disorders. The atypical atypical among 125 patients followed for drug-induced drug-induced movement disorders. neuroleptic clozapine causes causes less less extrapyramidal extrapyramidal side side effects, effects, including including DIP DIP neuroleptic clozapine Frankenburg 1991), 1991), aa characteristic which is attributed to the the relrel(Baldessarini and Frankenburg attributed to ative specificity ative specificityof of clozapine's clozapine'sdopamine dopamine receptor receptor blockade. blockade.The The introduction introduction of clozapine offered of psychosis psychosis in PD (Scholz (Scholz clozapine offered particular particular promise promise for for the treatment of Dichgans 1985, 1985, Friedman 1989). Confusion, and Dichgans Friedman and and Lannon Lannon 1989). Confusion, agitation agitation and and halluhalludrug therapy, therapy, the the primary primary disease disease cinations can occur in PD due to dopaminergic drug process, or as antipsychotas aa result resultof ofunrelated unrelated psychiatric psychiatric disorders. disorders. Conventional antipsychotics PD due due to to their theirdopamine dopaminereceptor receptorblocking blockingeffects. effects. ics are are poorly poorly tolerated tolerated in PD Clozapine may Clozapine may also alsoreduce reducetremor tremorand and motor motor fluctuations fluctuations in in PD PD (Friedman (Friedman and and Lannon 1990, 1990, Bennett Bennett et et al. al. 1993). 1993). However, However, clozapine clozapine at at doses doses of ofaround around75—250 75-250 mg daily in mg daily in elderly elderly PD PD patients patients has has been been associated associated with with sedation, sedation, delirium delirium and and signs and and symptoms symptoms (Wolters (Wolters et et al. al. 1990). 1990). The association of worsening of motor signs this with haematological haematological abnormalities abnormalities including including fatal fatal agranulocytosis agranulocytosis has has this drug with

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Table 4.1 Common neuroleptic neuroleptic drugs drugs and and related related agents agents Trade names

Generic names

Phenothiazines Phenothiazines

Compazine Compazine Triavil Mellaril Phenergan Prolixin Norzine Serentil Sparine Stelazine Thorazine Torecan Trilafon

prochiorperazine prochlorperazine amitriptyline/perphenazine thioridazine promethazine fiuphenazine fluphenazine thiethylperazine mesoridazine promaxine trifluoperazine trifluoperazine chiorpromazine chlorpromazine thiethylperazine perphenazine

Butyrophenones

Haldol Fentanyl

haloperidol droperidol

Thioxanthenes

Navane Taractran

thiothixene chiorprothixene chlorprothixene

Benzamides

Reglan

metoclopramide

Dihydroindolone

Moban

molindone

Dibenzoxazepthe Dibenzoxazepine

Loxitane

loxapthe loxapine

Thienobenzodiazepine

Zyprexa

olanzapine

Benzisoxazole

Risperdal

risperidone

Dibenzodiazepine Dibenzodiazepine

Clozaril

clozapine

necessitated weekly weeklyblood blood counts counts in in all all treated treated individuals (Kane et al. 1988). The necessitated concern concern over over the the potential potential for for neutropenia neutropenia coupled coupled with the cost cost and and inconveninconvenience frequent blood has limited limited the the use use of ofthis thisagent. agent.Several Several atypical atypical ience of of frequent blood tests has neuroleptic agents are currently available available in in the the UK UK (risperidone, (risperidone, sertindole, sertindole, olanzapine and quetiapine) quetiapine) which, which, like like clozapine, clozapine, rarely rarely cause cause acute acute extrapyramidal extrapyramidal zapine effects, but, clozapine, are are less less likely likely to side effects, but, unlike clozapine, to have have an an adverse adverse influence influence on the neutrophil count count (Kerwin (Kerwin and and Taylor Taylor 1996). High risperidone neutrophil High dose dose treatment treatment with risperidone cause DIR DIP. Other atypical atypical neuroleptic neuroleptic drugs drugs (ziprasidone, (ziprasidone, zotezotehas been found to cause amisulpride) have have not not yet yet been licensed licensed for use in the UK, pine and amisulpride) UK, but but both both ziprasidone and amisulpride amisulpride can can at at high dosage result in DIP. While While neuroleptics neuroleptics are used primarily as antipsychotic agents, it is to recognize recognize that these these drugs drugs are are is important to

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Drug-induced parkinsonism

prescribed for depression, anxiety, sometimes prescribed anxiety, and insomnia. insomnia. Although Although typically typically used to control nausea and vomiting, prochlorperazine and used and related related agents agents belong to the neuroleptic to neuroleptic class class of drugs drugs and and can can produce produce DIP DIP (Bateman (Bateman et et al. al.1986). 1986). Metoclopramide, an an atypical atypical neuroleptic neuroleptic belonging belonging to to the thebenzamide benzamideclass, class,isis used used to ameliorate gastric gastric stasis employed as antiemetic and various extraextrato ameliorate stasis and and is employed as an an antiemetic pyramidal reactions, reactions, including includingDIP, DIP, have been associated with its use (Grimes et al, al. 1982, 1982, Sethi Sethi etet al. al. 1989, 1989, Miller Miller and and Jankovic Jankovic 1989). 1989). In In one one series, series, five five out out of of 2557 2557 metoclopramide treated treated patients patients developed developed parkinsonism. parkinsonism. All All such such affected affected indiindiet al. al. 1989). 1989). viduals were over 40 years old (Bateman et Clinical features of neuroleptic-induced parkinsonism parkinsonism

Clinical descriptions descriptions of neuroleptic-induced neuroleptic-induced parkinsonism date date back to the original reports in the 1960s. 1960s. In 1961, 1961, Ayd Aydreported reported on on DIP's DIP's responsiveness responsivenessto to anticholanticholinergic discontinuation of the the inergic medications, medications, noted noted its its usual usual abatement abatement with with the the discontinuation offending drug, distinguished itit clinically clinically from from PD PD (Ayd (Ayd 1961). 1961). Ayd Ayd also also offending drug, and distinguished reported it to be be more more common common in inwomen women and andininthe theelderly. elderly.ItItwas was initially initially sugsuggested that resembled postencephalitic parkingested that neuroleptic-induced neuroleptic-induced parkinsonism resembled sonism than PD PD (Steck (Steck 1954). 1954). However, However, subsequent sonism rather than subsequent work suggests suggests that that the clinical manifestations clinical manifestationsofofthis this syndrome syndromecan canbe be identical identicaltoto those those seen seen in in PD 1975). Akinesia, may (Marsden et al. 1975). Akinesia, rigidity, rigidity, postural postural abnormalities, abnormalities, and tremor may occur. Akinesia manifestaoccur. Akinesia isisthe the earliest, earliest,most most common, common, and and frequently frequently the the only only manifestation of DIP, DIP, accounting for for the the expressionless expressionless face, face, loss loss of associated associated movements, slow activity, and disturbed speech. Rigidity of the slow initiation initiation of motor activity, the extremities, extremities, neck, or trunk, usually without a cogwheel cogwheel phenomenon, may occur after the onset phenomenon, may maybe of bradykinesia. bradykinesia. While While the the characteristic characteristic parkinsonian parkinsonian pill pill rolling rolling tremor may be present, resembling essential essential tremor present, postural postural tremor resembling tremor may may also also be be seen seen (Indo (Indo and and 1982, Hershey Hershey et et al. al. 1982). 1982). Ando 1982,

indistinguishable from from PD, some differentiating Although DIP may be clinically clinically indistinguishable differentiating characteristics may occur occur (see Table Table 4.2). 4.2). The The signs signs and and symptoms symptoms of PD usually characteristics may have a unilateral onset before before involving, involving, over time, the opposite opposite side, side. The The disease disease persistent asymmetry asymmetry with with the the first first side side affected affected always always being demonstrates mild persistent being more severely severely impaired (Klawans et 1973). Postural instability with episodic impaired (Klawans et al. al. 1973). Postural instability with episodic freezing frequently occurs occurs in in late late stage stage PD but rarely rarely complicomplifreezing and and start hesitation frequently cates DIP cates DIP (Giladi (Giladi et et al. al. 1997). 1997). The The manifestations manifestations of of neuroleptic-induced neuroleptic-induced DIP are frequently often develop develop acutely acutely or subacutely. subacutely. In frequently bilateral bilateral and and symmetrical symmetrical and and often In series, the emerged within a few few days days of one series, the signs signs of of DIP emerged of neuroleptic neuroleptic treatment gradual increase increase in in incidence incidence so sothat that50—70% 50-70% of cases cases appeared appeared by one one with aa gradual month and and 90% 90% of of cases cases within three months months (Marsden (Marsden et et al. al. 1975). 1975). It It is is often often stated with time time neuroleptic-induced neuroleptic-induced DIP DIP can can spontaneously spontaneously diminish. diminish. stated that with However, lacking. The The only only However, prospective prospectivestudies studiestoto verify verifythis this phenomenon phenomenon are lacking.

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Table 4.2 Distinguishing drug-induced parkinsonism from PD Drug-thduced Drug-induced parkinsonism

Parkimson's disease Parkinson's

Symptom onset onset

Bilateral Bilateral and symmetric

Unilateral or asymmetric Unilateral

Course

Acute or subacute

Insidious, chronic

type Tremor type

Bilateral symmetric postural Bilateral symmetric or rest rest tremor

Unilateral or asymmetric Unilateral rest tremor

Freezing or or start start hesitation Freezing

Rare

Frequent feature with disease progression

Anticholinergic drug response response

May be be pronounced pronounced May

Usually mild—moderate mild-moderate

Withdrawal of suspected offending offending drug

Remittance within weeks weeks to months

Symptoms and and signs signs slowly progress

evidence for for this this assumption assumption isis the the observation observation that that withdrawal withdrawal of of anticholinergic anticholinergic evidence drugs coadministered for several months with neuroleptics drugs coadministered for several months neuroleptics is is followed followed by the the DIR appearance of of relatively relatively few few cases of DIP. After After the discontinuation of neuroleptic medication the majority majority of of patients are are free of of parkinsonian parkinsonian signs last longer, weeks. However, the effects effects may last free signs within within aafew few weeks. years (Klawans et al. al. 1973). 1973). Metoclopramide-induced in some cases cases up to several years (Klawans et parkinsonism has been reported reported to to take take several several months months totoresolve resolve completely completely parkinsonism has been (Weiden et al. 1987, 1987, Yamamoto of neuneu(Weiden Yamamoto et et al. al. 1987). 1987).The Thepotentially potentially long long duration duration of roleptic-induced appreciate so avoid roleptic-induced parkinsonism parkinsonism isis important important to to appreciate so that that one can avoid diagnostic error. DIP will will usually usually improve improve slowly slowlyover overtime timewith withreduction reduction or or discontinuation offending drug whereas whereas the signs and symptoms of PD PD will will continuation of the offending worsen. progressively worsen. Neuroleptic-induced movement movement disorders disorders including including DIP DIP frequently frequently go unrecognized. nized. Miller Miller and Jankovic Jankovic (1989) (1989) in their their review review of of metoclopramide-induced metoclopramide-induced movement disorders found found that that the drug movement disorders drug was was continued continued for for an an average average of of six six after the the onset onset of of extrapyramidal extrapyramidal symptoms. symptoms. Other Other investigators investigators found found months after physicians in 11 % of of neuroleptic neuroleptic treated treated that psychiatry physicians in training diagnosed DIP in 11% prevalence to patients, while researchers researchers determined determined the prevalence to be be 26% 26% in in the the same same pop(Hansen et et al. al. 1992), 1992). Approximately Approximately 32% of 192 192 individuals newly diagulation (Hansen nosed parkinsonism were have DIP DIP using using aa large large neurology neurology nosed with with parkinsonism were found found to to have outpatient referral referral registry registry in in Madrid Madrid (Perez (Perez Gilabert Gilabert et et al. al. 1994). 1994). ItIt has has been estiestimated 4% of of all all neurology neurology outpatient outpatient clinic clinic patients patients have have DIP DIP (Gershanik (Gershanik mated that 4% and cases cases 1994). The The correct diagnosis of DIP depends on a high index of suspicion, and have been described where covert administration have been described where DIP DIP has has resulted resulted from from the the covert administration of of drugs by by aa spouse to their partner partner(Albanese (Albanese etetal. al. 1992). 1992). neuroleptic drugs

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Drug-induced parkinsonism

Pathogenesis of neuroleptic-induced neuroleptic-induced parkinsonism Pathogenesis of determinant of of PD The primary primary neurochemical neurochemical determinant PD is striatal dopamine dopamine depletion depletion (Ehringer and Hornykiewicz 1960), Sinceneuroleptic nenroleptic drugs drngs function fnnction as as dopamine 1960). Since receptor surprising that clinical clinical featnres features of receptor blockers, blockers, itit is is not not snrprising of parkinsonism parkinsonism can resnlt from from their nse. result use. However, However, DIP cannot simply be a resnlt result of dopamine recepreceptor blockade. If this were true, incidence and and severity severity of DIP DIP shonld should correlate correlate trne, the incidence to drug drng dosage and length of exposure. exposnre. Attempts Attempts to to link link total total drug drng dosage dosage with with the the occurrence of occnrrence of parkinsonism parkinsonism have have failed failed to to show show such snch aa relationship relationship (Hall (Hall et et al. al. 1956). drug levels levels do 1956). Furthermore, Fnrthermore, plasma neuroleptic nenroleptic drng do not not appear to correlate correlate with the severity severity of DIP (Crowley (Crowley et al. 1978). Parkinsonism appearing within within several several relatively small drng drug doses is a common clinical clinical experience, days of of treatment treatment with relatively but other other patients patientsare aresnccessfnlly successfully maintained maintained on onrelatively relatively high high doses doses for for several several bnt developing parkinsonism. It has has been snggested suggested that DIP DIP is is simply simply years without withont developing idiopathic PD occurring by chance chance in nenroleptic neuroleptic treated individnals. individuals. The reported occnrringby prevalence varies, bnt but clinically clinically significant significant prevalence of of neuroleptic-induced nenroleptic-indnced parkinsonism varies, reportedly occnrs occurs inin10—15% 10-15% of individuals (Korczyn (Korczyn and and parkinsonism reportedly of treated individnals underestiGoldberg 1976, Moleman Moleman et al. 1986). These These rates of DIP are probably nnderestimates (McClelland (McClelland 1976). Thus, could not acconnt account for for all all cases cases of Thns, coincidental PD conld DIP since individuals is since the the occurrence occnrrence rate rate of of parkinsonism parkinsonism in neuroleptic nenroleptic treated individnals much estimates of PD in the general general populace. mnch greater than estimates popnlace.

The mechanisms determining individnal individual snsceptibility susceptibility to DIP DIP remain remain nnclear. unclear. Some Some studies stndies suggest snggestthat that women women are are at at an an increased increased risk risk for for the the development development of of neuroleptic-induced disorders, inclnding including tardive nenroleptic-indnced movement movement disorders, tardive dyskinesia dyskinesia and and DIP DIP (Ayd 1961, Korczyn related (Ayd Korczyn and and Goldberg Goldberg 1976, Kane Kane and and Smith 1982). Oestrogen related dopamine receptor blockade has been offered offered as as the explanation explanation for for this this female female dopamine preponderance (Glazer et 1983). Others found aa gender gender infinence influence preponderance (Glazer et al. al. 1983). Others have have not not fonnd (Kennedy et al. 1971, 1971, Moleman et et al, al. 1982). 1982). Differences Differences in in case case ascertainment, ascertainment, (Kennedy et al, medication prescription prescription and and drng drugnsage usage based based on onsex sex in in these these stndies studies may may explain explain these report low low nrinary urinarylevels levels of of free free dopamine dopamine were were associated associated these findings. findings. In one report subsequent development development of phenothiazine-indnced phenothiazine-induced parkinsonism, parkinsonism, sngsugwith the snbseqnent gesting that metabolic defect defect may increase the risk of DIP (Crowley (Crowley et gesting that an inherent metabolic et suggesting a genetic infinence influence al. 1976). HLA HLA B44 B44isisreported reported to to be be common common in DIP snggesting (Metzer et 1989). In series, five (Metzer et al. 1989). In one series, five out ont of of 16 patients patients with with metoclopramideinduced movement disorders disorders had family family members members with reported parkinsonism, parkinsonism, indnced movement tremor, or chorea chorea (Miller (Miller and and Jankovic Jankovic 1989). 1989). However, However, the precise role of genetics in DIP remains remains uncertain. nncertain. The possibility snsceptibility to to DIP might be related to snbclinpossibility that increased susceptibility subclinical PD PD has also been considered ical considered (Delay (Delay and Deniker Deniker 1968, 1968, Dnvoisin Duvoisin 1977). 1977). In some instances overt during nenroleptic some instances itit appears appears that PD PD becomes becomes clinically clinically overt neuroleptic therapy subsides when is discontinued, only only to reappear reappear years years therapy and then subsides when the drug is

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J. P. P. Hubble Hubble

later. At least least nine nine such cases been documented later. At cases have have been documented in the the medical medical literature literature

(Goetz Stephen and Williamson (Goetz 1983, Stephen Williamson 1984, 1984, Hardie and Lees Lees 1988). 1988). In addition, addition, two instances instances of DIP that completely completely remitted upon upon drug drugwithdrawal withdrawal have have been been described which postmortem postmortem examination examination ultimately ultimately revealed revealed pathological pathological described in in which 18 changes (Rajput et al. al. 1982). 1982). Reduced Reduced putaminal putaminal '8F-dopa F-dopa changes consistent consistent with with PD PD (Rajput uptake on positron emission emission tomography has been reported in in two two out out of of seven seven uptake individuals with (Brooks 1991). 1991). Four five patients PET individuals with DIP (Brooks Four of of the five patients with with normal PET scans recovered recovered fully fullywith with cessation cessationof ofdrug drug therapy. therapy.The Thetwo twopatients patientswith with abnormal persistent evidence evidence of parkinsonism requiring requiring levodopa levodopa therapy, therapy, mal scans scans had had persistent underlying PD. PD. suggesting underlying

The relationship The relationship of of DIP DIP to to other otherneuroleptic-induced neuroleptic-induced extrapyramidal extrapyramidal synsyndromes is intriguing. Like tardive dyskinesia dyskinesia isis reported reported to to occur more dromes Like DIP, DIP, tardive more frefrequently in the elderly elderly (Jus (Jus et al. al. 1976, 1976, Pineau et et al. al. 1976, 1976, Crane and and Smeets Smeets 1974, 1974, over the age age Jeste and Wyatt Jeste Wyatt 1982, Woerner Woerner et al. 1991). In a study of 215 patients over of 55 was 55 years yearsafter afterthe theinitiation initiation of of neuroleptic neuroleptic therapy evidence of parkinsonism parkinsonism was found in 103 103 patients by by week 43 of drug therapy therapy (Saltz (Saltzet al. 1991). 1991). Tardive dyskiet al, nesia 12% of nonparnonparnesia developed developed in in 40% 40% of ofthe theparkinsonian parkinsonian patients patients compared to 12% kinsonian subjects. subjects. The coexistence of DIP and and tardive tardive dyskinesia dyskinesia has been been kinsonian The coexistence of DIP reported byothers others(Fann (Fannand andLake Lake1974, 1974,De DeFraites Fraitesetetal. al. 1977, 1977, Rao et al. 1987). The reportedby occurrence hypokinetic and hyperkinetic hyperkinetic drug-induced drug-induced side side effects effects in a occurrence of of both both hypokinetic common patient population population isisdifficult difficult to to explain. explain. A A possible explanation may be blockade is related that presynaptic receptor blockade related to to the the development development of of both both DIP and tardive dyskinesia (Seeman et al. 1974). Differential Differential effects effects of of neuroleptics neuroleptics on dopamine subtypes may may also also play play aa part. part.Elderly Elderly subjects subjects may may be beespecially especially amine receptor subtypes vulnerable to and tardive tardive dyskinesia dyskinesia due to diminished diminished drug drug metabolism, metabolism, vulnerable to DIP and dopaminergic alterations in dopamine dopamine receptors receptors (Finch (Finch 1973, 1973, dopaminergic neuronal neuronal loss, loss, or or alterations Glazer Glazer et al. 1983, Goetz et al. 1982). Treatment of neuroleptic-induced parkinsonism The first first line line treatment treatment of of DIP DIP is, is, whenever whenever possible, possible, the withdrawal withdrawal of the the offending was initially initially preoffending drug. drug. The The underlying underlying condition condition for for which which the the drug was predifficulty in the instance of scribed may subsequently worsen, which is a particular particular difficulty psychosis associated psychosis associated with with schizophrenia. schizophrenia. ItIt is is prudent prudent to weigh weigh the the benefits benefits that that the derives from the drug drug against against the the severity severity and and disability disability of of DIP. DIP. Mild nonnonpatient derives disabling individuals who have achieved psydisabling DIP DIP occurring occurring in in individuals who have achieved good good control control of of psychotic symptoms symptoms on a stable stable dose dose of of neuroleptic neuroleptic may mayrequire require only onlyobservation observation and and no intervention. Alternatively, Alternatively, the patient can be switched to an atypical neurolepneurolepmotor side side effects effects such as olanzapine or tic with less potential for extrapyramidal motor risperidone. used to Domperidone and betahistine can be substituted for neuroleptic drugs used

71 71

Drug-induced parkinsonism

control nausea nausea and dizziness dizziness (Parkes (Parkes 1986). 1986). Domperidone is is not not commercially commercially available in the the US. Drug-induced movement available in movement disorders disorders have have occasionally occasionally been associated with the the use use of of domperidone domperidone (Debontridder 1980). which associated with 1980). Antiemetics, which hydrodo not act via via dopamine dopamine blockade, blockade, such such as asondansetron ondansetron and benzquinamide benzquinamide hydroinstances. chloride, can also be used in some instances. Standard antiparkinsonian DIP. Anticholinergic Anticholinergic drugs drugs antiparkinsoniandrugs drugscan canbe beused used to to treat treat DIP. reportedly decrease decrease the the signs signsand andsymptoms symptomsof ofneuroleptic-induced neuroleptic-induced parkinsonism parkinsonism to a greater degree degree than in PD. PD. This drug action action has has been been suggested suggested to be of of use use as aa means means ofofdistinguishing distinguishing between between DIP DIP and andPD PD(Hornykiewicz (Hornykiewicz 1975). 1975). as Dopaminergic neuroleptic Dopaminergic drugs drugs may provoke provoke the the very very symptoms symptoms for for which which the neuroleptic was first first prescribed prescribed (Hauser 1980). 1980). In aa double-blind double-blind crossover crossover study, study, the the drug was effects of apomorphinewere wereassessed assessed in in 12 12 effects oflevodopa levodopa and and the the dopaminergic dopaminergic agonist apomorphine schizophrenic (Merello et al. 1996). No schizophrenic patients with DIP (Merello No clear clear benefit benefit from from antiantiparkinsonian drug treatment was was detected, detected, though though no no acute acute exacerbation exacerbation of of parkinsonian drug treatment occurred. This This seeming seeming lack lack of of effect effect may may be be explained explained by by psychiatric symptoms occurred. the and relatively relatively persistent persistent dopamine receptor blockade neuroleptic the robust and dopamine receptor blockade of of neuroleptic does not not benefit benefit tremor tremor in inDIP DIP(Metzer (MetzeretetaL al. 1993). 1993). In In aa single drugs. Propranolol does case report, tardive dyskinesia dyskinesia in an case report, pyridoxine pyridoxine was was reported to improve DIP and tardive individual neuroleptic treated schizophrenia schizophrenia (Sandyk (Sandyk and Pardeshi 1990). 1990). individual with with neuroleptic and Pardeshi While electroconvulsive electroconvulsive shock safe in PD, PD, it While shock therapy therapy isis usually usually considered considered to to be be safe reportedly reportedly caused caused worsening worsening of of immobility immobility in in a psychiatric psychiatric patient patient with with DIP DIP and tardive et al. al. 1995). 1995). tardive dystonia dystonia (Hanin et

Dopamine storage storage and and transport transport inhibitors causing causing parkinsonism brain dopaReserpine, an antihypertensive that is is now rarely prescribed, depletes depletes brain mine and other other biogenic biogenic amines amines by by interfering interfering with with presynaptic presynaptic vesicular vesicular storage storage mechanisms. It can can produce produce both bothclinical clinical and andexperimental experimental parkinsonism parkinsonism al. 1957). 1957). Reserpine is still sometimes used to treat treat tardive tardive dyskinesia dyskinesia (Carlsson et al, 1983, Klawans Klawans et Since individuals dyskinesia may (Fahn 1983, et al. 1983). Since individuals with with tardive dyskinesia is warhave an increased risk of developing DIP, close close monitoring monitoring of these patients is ranted if treatment with reserpine reserpine is is employed. Tetrabenazine, aa synthetic synthetic analogue analogue reserpine, also also depletes depletes amines amines and may may also also block block postsynaptic postsynaptic dopamine dopamine of reserpine, receptors tetrabenazine receptors (Reches (Reches etet al. aL 1983). 1983).Not Not currently currently marketed marketed in in the US, tetrabenazine appears to be useful useful in the the treatment treatment of ofhyperkinetic hyperkineticdisorders disorders(Jankovic (Jankovic 1983). 1983). appears Parkinsonism side effect effect of Parkinsonism has has been been reported reported as the most common side of tetrabenazine tetrabenazine affecting 53 exposure, affecting 53 of of 217 217patients patients receiving receivingthe the drug drug for for hyperkinesia hyperkinesia (Jankovic andOrman 1988). and Orman 1988). falseneurotransmitter neurotransmitter for for dopamine, has has been been reported reported to to Alpha-methyldopa, a false cause DIP to exacerbate exacerbate existing existing PD (Rosenblum (Rosenblum and Montgomery Montgomery 1980, 1980, cause DIP and to

12 72

J. P. R Hubble

Giliman and Sandyk 1984). Alpha-methyldopa Aipha-methyldopa has has been been used nsed as as an an antihypertenGillman Sandyk 1984). sive drng and and has even been employed employed in in the the treatment treatment of PD sive drug even been PD (Fermaglich (Fermaglich and Chase 1973). The significance significance of of the the few few reported reported cases cases of of DIP DIP with with this drng Chase 1973). The drug is is unclear. nnclear.

channel blockers parkinsonism Calcium channel blockerscausing causing parkinsonism Available in Europe Enrope and Latin America, America, the the piperazine piperazine derivatives, finnarizine and derivatives, flunarizine

calcinm entry entry blockers blockers and and have havebeen beenprescribed prescribed for forvarions cinnarizine, act as calcium various disorders et al. al. 1982, 1982, Holmes orders including inclnding vertigo, vertigo, migraine, migraine, and and tinnitus tinnitns (Godfraind et Holmes et et al. 1984). Extrapyramidal exacerbation of PD PD have have al. 1984). Extrapyramidal reactions reactions including inclnding DIP DIP and exacerbation associated with use (Marti (Marti Masso Masso et et al. al. 1987, 1987, Micheli Micheli et al, al. 1989). 1989). A been associated with their nse primate model model of of cinnarizine-indnced cinnarizine-induced parkinsonism parkinsonism has has also also been been described described primate (Fadda et 1989). Parkinsonism antidopaminergic (Fadda et al. al. 1989). Parkinsonismisisthought thonght to to be be due dne to to the the antidopaminergic effects Vries and 1984). effects of ofthese these compounds componnds at pre or postsynaptic sites (De Vries and Beart Beart 1984). persistence of parkinsonian signs, particularly after The persistence particnlarly in the aged, many months after drug has been reported reported (Garcia-Rniz (Garcia-Ruizetal.1992). DIP the cessation of the offending drng et al.1992). DIP due calcium channel channel blockers blockers may have have a genetic genetic component as as aa relatively relatively dne to calcinm occurrence of other movement movement disorders disorders among among relatives relatives of affected affected indihigher occnrrence mdividuals has been fonnd found (Negrotti (Negrotti et et al. al. 1992). 1992). Calcium Calcium channel channel blockers blockers available available vidnals (Dick and and Barold Barold 1989). 1989). in the US are rarely associated associated with clinical clinical parkinsonism parkinsonism (Dick The antidopaminergic antidopaminergic effect such agent, agent, nimodipine, nimodipine, has been demondemonThe effect of of one one such experimentally (Pileblad (Pileblad and Carlsson Carlsson 1986). 1986). strated experimentally

drugs reported to cause Other drugs cause parkinsonism been associated Other drugs of of various various classes classes have occasionally occasionally been associated with parkinsonparkinsoncausal relationship relationship is is often often difficult difficult to to establish establish (see (see Table Table 4.3). 4.3). ism, though aa causal can cause cause several several neurological side effects Amiodarone can effects including including aa tremor tremor resemHowever, DIP DIP has has also also been described described (LeMaire (LeMaire etetal. al.1982, 1982, bling essential tremor. However, et al. al. 1987, 1987, Werner and Olanow 1989). 1989). Palakurthy et In single single case case reports, parkinsonism parkinsonism has has been been ascribed ascribed to to cholinergic cholinergic drugs drugs including bethanechol bethanechol and and the cholinesterase including cholinesterase inhibitor pyridostigmine pyridostigmine that does does not penetrate penetrate the the blood—brain blood-brain barrier (Iwasaki (Iwasaki et et al. al. 1988, 1988, Fox Fox et al. 1989). 1989). In the the case may result result from from cholinergic overactivity overactivity in case of of bethanacol, bethanacol, parkinsonism parkinsonism may in the although in in one one case case a postmortem demonstrated demonstrated pathological pathological changes changes striatum, although consistent (Fox et 1989). Lithium Lithium commonly commonly produces postural consistent with with PD PD (Fox et al. 1989). produces a postural but itit isis unclear unclear as as to to whether whether itit can can cause cause DIP. DIP. Cogwheel Cogwheel rigidity tremor but rigidity has has been found in aa small small percentage of patients taking lithium (Kane (Kane et et al. found on examination in 1978). Two develop parkinsonian parkinsonian symptoms symptoms after after 1978). Two patients patients were were reported reported to develop

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Drug-induced parkinsonism Drug-induced

Table 4.3 Miscellaneous drugs drugs causing causing parkinsonism parkinsonism Reserpine

Tetrabenzaine Aipha-methyldopa Alphamethyldop a Calcium channel blockers e.g., e.g., cinnarizine, fiunarizine flunarizine Amiodarone Bethamechol Pyridostigmine Lithium Diazepam Fluoxetine Phenelzine Procaine Meperidine Amphotericin B Cephaloridine 5-fluorouradil 5-fluorouracil Vincristine—adriamycin Vincristine-adriamycin

lithium lithium therapy therapy but both both had had prior prior exposure exposure to to neuroleptic neuroleptic drugs drugs (Tyrer (Tyrer et et al. al. 1980). 1980).

DIP was was reported reported in in four four patients patients on high high dose dose ((>100mg 100 mgdaily) daily)diazepam diazepambeing being used in the treatment treatment of of schizophrenia schizophrenia (Suranyi-Cadotte (Suranyi-Cadotteetetal. al.1985). 1985). The The selective selective serotonin reuptake reuptake inhibitor inhibitor antidepressants antidepressantsfluoxetine fluoxetine (Bouchard (Bouchard etetal. al. 1989, 1989, Tate Tate (Jiminez-Jiminez et et al. al. 1994) 1994) 1989, Gernaat et al. 1991, 1991, Steur Steur 1993), and and paroxetine paroxetine (Jiminez-Jiminez associated with have have been been associated with parkinsonism. Isolated instances of parkinsonism have also (Teusink et al. 1984), 1984), proalso been been ascribed ascribed to to other agents including phenelzine (Teusink procaine 1971), meperidine (Lieberman and Goldstein 1985), amphotericin caine (Gjerris (Gjerris 1971), meperidine (Lieberman and Goldstein B (Fisher and Dewald Dewald 1983), 1983), cephaloridine cephaloridine (Mintz al. 1971), 1971), 5-fluorouracil 5-fluorouracil B (Fisher (Mintz et al. (Bergevin et adriamycin (Boranic (Boranic and (Bergevin et al. al. 1975), 1975), and and vincristine vincristine combined combined with with adriamycin Raci 1979). Summary DIP is common, particularly in the elderly. elderly. Dopamine receptor blockers, used primarily as as antipsychotics antipsychotics and and antiemetics, are most frequently marily frequently responsible responsible for this this condition. Other pharmacological of medications condition. pharmacological classes classes of medications have have also also been impliimplicated, of DIP DIP in in such such instances instances isis unclear. unclear. The The susceptisuscepticated, although the mechanism of bility of the individual to develop DIP has not been established, but may, in some

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represent latent latent PD PD or a heritable cases, represent heritable trait. Further scrutiny of the occurrence occurrence characteristics of of DIP DIPwould wouldprovide provideaabetter betterunderstanding understanding of ofthis this phenomephenomeand characteristics greater insight insight into into the cause cause and and pathogenesis pathogenesis of PD. PD. non and may may also give give aa greater REFERENCES Albanese A, Colosimo Colosimo C, Bentivoglio AR, Bergonzi BergonziPp (1992) (1992) Unsuspected, Unsuspected, surreptitious surreptitious drugBentivoglio AR,

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after acute acute parkinsonism in schizophrenic patients: Motor response and psychiatric changes changes after challenge with Neuropharmacology, 19, 439—43. L-Dopa and andapomorphine. apomorphine.Clinical Clinical Neuropharmacology, 19, 439-43. with L-Dopa Newton JE, JE, Steele Steele RW, RW, Claybrook M, M, Paige Paige SR, SR, McMillan McMillan DE, DE,Hays Hays SS(1989) (1989)HLA HLA Metzer WS, Newton antigens in drug-inducedparkinsonism. Movement Disorders, 4, 121—8. Disorders, 4, 121-8. drug-induced parkinsonism. Movement Metzer Paige SR, SR, Newton Newton JEO JEO (1993) (1993) Inefficacy Inefficacyofofpropranolol propranolol in in attenuation of Metzer WS, Paige of drugdruginduced 8, 43—6. induced parkinsonian parkinsoniantremor. tremor.Movement MovementDisorders, Disorders, 8, 43-6. Michei MicheliFE, FE, Fernandez Fernandez Pardal Pardal MM, MM, Giannaula GiannaulaR, R,Gatto GattoM, M,Casas Casas Parera Parera I,I, Paradiso Paradiso G, G, Torres Torres M, Pilcielny R,Fernandex Fernandex Pardal PardalJJ(1989) (1989)Movement Movement disorders disorders and depression due due to to fiunarizine flunarizine Pikielny R, and 4, 139—46. 4, 139^6. and cinnarizine. cinnarizine.MovementDisorders, Movement Disorders, Miller LG, Jankovic JankovicJJ(1989) (1989)Metoclopramide-induced Metoclopramide-induced movement movement disorders. disorders.Archives Archivesof of Internal Internal Medicine, Medicine,149, 149,2486—92. 2486-92. Mintz U, Liberman UA, Vries Vries Ade A de(1971) (1971)Parkinsonism Parkinsonism syndrome syndrome due to cephaloridine. Journal MintzU, Journal the American American Medical of the MedicalAssociation, Association,216, 216,1200. 1200. Moleman L, Schmitz Schmitz PI P1(1986) Moleman P, Janzen Janzen G, von Bargen Bargen BA, BA, Kappers Kappers EJ, EJ, Pepplinkhuizen Pepplinkhuizen L, (1986) Relationship between between age and incidence incidence of parkinsonism in psychiatric psychiatric patients treated treated with with haloperidol. American of of Psychiatry, 143, 232—4. AmericanJournal Journal Psychiatry, 143, 232-4. haloperidol. Moleman P, (1982) Extrapyramidal side effects haloperidol: An P, Schmitz Schmitz PJM, PJM, Ladee GA (1982) effects and oral haloperidol: analysis of explanatory patient ofof Clinical 43,43, patient and andtreatment treatmentcharacteristics. characteristics.Journal Journal ClinicalPsychiatry, Psychiatry, 492—6. 492-6. Montastruc ME, RascolO, Rascol 0, Senard SenardJM JM(1994) (1994)Drug-induced Drug-induced parkinsonism. parkinsonism. Fundamental Fundamental Montastruc JL, JL, Llau LlauME, and Clinical 8, 293—306. ClinicalPharmacology, Pharmacology, 8, 293-306. and Negrotti A, Calzetti 5, (1992) Calcium-entry blockers-induced parkinsonism: S, Sasso Sasso E (1992) parkinsonism: possible possible role of 13,13, 261—4. Neurotoxicology, 261-4. of inherited inheritedsusceptibility. susceptibility.Neurotoxicology, Palakurthy PR, PR, Iyer V, Meckler Meckler RJ RJ (1987) (1987) Unusual Unusual neurotoxicity neurotoxicity associated associated with with amiodarone Palakurthy Iyer V, therapy. Archives 147, 881—4. therapy. ArchivesofofInternal InternalMedicine, Medicine, 147, 881^. Parkes JD (1986) Domperidone Neuropharmacology, 9,517—32. Domperidoneand andParkinson's Parkinson'sdisease. disease.Clinical Clinical Neuropharmacology, 9, 517-32. Perez Gilabert Perez Gilabert Y, Y,Mateo MateoD, D,Gimenez-Roldan Gimenez-RoldanS5(1994) (1994)Patient Patientcare careinin aahospital-based hospital-based unit unit for for yearprospective prospectivestudy. study.Neurologica, Neurologica, treating Parkinson's Parkinson's disease disease and and movement movementdisorders: disorders:aa33year 9, 9, 317—23. 317-23. Pileblad E, Carlsson A (1986) nimodipine on dopamine (1986) In In vivo vivo effects effects of of the the Ca2+-antagonist nimodipine dopamine metabolism of of Neural Transmission, 66,66, 171—87. metabolism ininmouse mousebrain. brain.Journal Journal Neural Transmission, 171-87. Villenueve R R (1976) (1976) Epidemiology Epidemiology of of Pineau R, Lachance R, Pelchat G, Jus K, Pires P, Pires P, Villenueve tardive dyskinesia. of of thethe Nervous System, 37,37, 210—14. Diseases Nervous System, 210-14. tardive dyskinesia.Part PartI.I.Diseases Rajput AH, Rozdilsky Hornykiewicz O, 0, Shannak Seeman P (1982) Reversible Rozdilsky B, Hornykiewicz Shannak K, K, Lee Lee T, Seeman Reversible druginduced study of two cases. Archives of Neurology, 39, 39, 644—6. induced parkinsonism: parkinsonism:clinicopathologic clinicopathologic study of two cases. Archives of Neurology, 644-6. Rao JM, JM, Cowie Cowie VA, VA,Mathew Mathew BB (1987) (1987) Tardive Tardivedyskinesia dyskinesiaininneuroleptic neuroleptic medicated medicated mentally mentally Rao handicapped subjects. Sandinavicu, 76,76, 507—13. handicapped subjects.Acta ActaPsychiatrica Psychiatrica Sandinavica, 507-13. Reches A, Burke Burke RE, RE, Kuhn Kuhn CM, Hassan Reches A, Hassan MN, Jackson Jackson VR, Fahn Fahn 5S(1983) (1983) Tetrabenazine, Tetrabenazine, an an amine-depleting drug, JournalofofPharmacology Pharmacology drug, also also blocks blocks dopamine dopamine receptors receptors in inrat ratbrain. brain.Journal and Experimental 225, 515—21. and ExperimentalTherapeutics, Therapeutics, 225, 515-21. AM, Montgomery Montgomery EB EB (1980) (1980) Exacerbation Exacerbationof ofparkinsonism parkinsonism by by methyldopa. methyldopa. Journal Journal Rosenblum AM, the American American Medical 244, 2727—8. of the MedicalAssociation, Association, 244, 2727-8. Saltz BL, Woerner Woerner MG, Kane JM, Leiberman JA, JA, Alvir JM, Bergmann KJ, KJ, Blank Blank K, K, Koblenzer Koblenzer J,

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Drug-induced parkinsonism (1991) Prospective Prospective study of tardive dyskinesia incidence in the Kahaner K (1991) the elderly. elderly. Journal Journal of of theAmerican Associ ation, 266, 2402—6. the AmericanMedical Medical Association, 266, 2402-6. Sandyk Sandyk R, R, Pardeshi Pardeshi RR(1990) (1990)Pyridoxine Pyridoxineimproves improvesdrug-induced drug-induced parkinsonism parkinsonism and psychosis in a schizophrenic Journal of Neuroscience, 52, 52, 225—30. International Journal of Neuroscience, 225-30. schizophrenic patient. patient.International Scholz E, E, Dichgans Dichgans JJ (1985) (1985) Treatment Treatment of of drug-induced exogenous Scholz exogenous psychosis in parkinsonism parkinsonism with Archives of Psychiatry andand Neurological Sciences, 235, 235, with clozapine clozapineand andfiuperlapine. fluperlapine.European European Archives ofPsychiatry Neurological Sciences, 60—4. 60-4. Seeman P, P, Staiman StaimanA, A,Lee LeeTT(1974) (1974)The Themembrane membraneaction actionof oftranquilizers tranquilizersinin relation relationto to neuneuSeeman roleptic-induced parkinsonism parkinsonism and and tardive Advances in Biochemical Biochemical roleptic-induced tardive dyskinesia. In: Advances Psychopharmacology, The Structurally Related Drugs, eds.eds. Forrest IS, Carr Psychopharmacology, ThePhenothiazines Phenothiazinesand and Structurally Related Drugs, Forrest IS, Carr CJ, Usdin E, E, pp. pp.137—48. 137-48. New York: Raven Press. Press. KD, Patel PatelB, B,Meador MeadorKJ KJ(1989) (1989)Metoclopramide-induced Metoclopramide-inducedparkinsonism. Sethi KD, parkinsonism. Southern SouthernMedical Medical Journal, 1581—2. Journal,82,82, 1581-2. Steck H H (1954) (1954) Le Lesyndrome syndromeextrapyramidal extrapyramidal etet diencephalique diencephalique au au cours cours des destraitements traitements au au forforSteck 737—43. AnnalesMedico-Psychologiques, Medico-Psychologiques, 737^3. gactil au au Serpasil. Serpasil.Annales 2, 2, 1082—3. Stephen PJ, Williamson J (1984) Drug-induced Drug-inducedparkinsonism parkinsonismininthe theelderly. elderly.Lancet, Lancet, 1082-3. Steur EN (1993) Steur (1993) Increase Increase of of Parkinson Parkinson disability disability after after fluoxetine fluoxetine medication. medication. Neurology, Neurology,43, 43, 211—13. 211-13. Nestoros JN, JN, Nair Nair NP, NP, Lal LalS,5,Gauthier Gauthier SS (1985) (1985) Parkinsonism Parkinsonism induced by Suranyi-Cadotte BE, Nestoros high Psychiatry, 20, 20, 455—7. high doses dosesofofdiazepam. diazepam.Biological Biological Psychiatry, 455-7. Tate and fluoxetine fluoxetine (letter). (letter). Tate JL JL(1989) (1989)Extrapyramidal Extrapyramidalsymptoms symptoms in in aa patient patient taking haloperidol and American Psychiatry, 146, 399—400. Journalofof Psychiatry, 146, 399-400. AmericanJournal Teusink JP, JP, Alexopoulos Alexopoulos GS, GS,Shamoian Shamoian CA CA (1984) (1984) Parkinsonian Parkinsonian side effects Teusink effects induced by a monomonoamine of of Psychiatry, 141,118—19. amine oxidase oxidase inhibitor. inhibitor.American AmericanJournal Journal Psychiatry, 141,118-19. P, Alexander Alexander MS, MS, Regan ReganA, A,Lee LeeI 1(1980) Anextrapyramidal extrapyramidal syndrome syndrome after lithium Tyrer P, (1980) An lithium therapy. therapy. British Journal of British of Psychiatry, Psychiatry, 136, 136,191—4. 191^.

Weiden PJ, PJ, Mann Frances A Weiden Mann JJ, JJ, Haas Haas G, G, Mattson Mattson M, M, Frances A (1987) (1987) Clinical Clinical nonrecognition nonrecognition of of neuneuroleptic-induced movement ofof Psychiatry, 144, cautionarystudy. study.American AmericanJournal Journal Psychiatry, 144, roleptic-induced movementdisorders: disorders:aacautionary 1148—53. 1148-53. Werner EG, Olanow CW (1989) Parkinsonism Parkinsonism and andamiodarone amiodaronetherapy. therapy.Annals AnnalsofofNeurology, Neurology,25, 25, 630—2. 630-2. Woerner NR, Woerner MG, Kane Kane JM, JM, Lieberman Lieberman JA, JA, Alvir Alvir J, Bergmann Bergmann KJ, KJ, Borenstein Borenstein M, Schooler Schooler NR, Mukherjee 5, Rotrosen J, Rubinstein Rubinstein M (1991) (1991) The The prevalence prevalence of oftardive Journal of tardive dyskinesia. Journal of MukherjeeS, Clinical harmacology, 11, 11, 34—42. ClinicalPsychop Psychopharmacology, 34-42. Wolters EC, Hurwitz Hurwitz TA, Mak E, E, Teal Teal P, P, Peppard Peppard FR, FR, Remick RemickR, R,Calne Cake 5, Wolters EC, TA, Mak S, Cake CalneDB DB(1990) (1990) Clozapine in in the treatment Clozapine treatment of of parkinsonian parkinsonian patients patients with with dopaminomimetic dopaminomimetic psychosis. psychosis. Neurology, 40, Neurology, 40,832—4. 832-4. Yamamoto M, Ujilce H, Ogawa Yamamoto M, Ujike H, Ogawa N (1987) (1987) Metoclopramide-induced Metoclopramide-induced parkinsonism. parkinsonism. Clinical Clinical Neuropharmacology, 10,10, 287—9. Neuropharmacology, 287-9.

Essential tremor in the elderly Essential tremor Rajesh Pahwa and and William C Rajesh Pahwa C Koller Koller

Introduction Essential tremor (ET) (ET) is the most common common movement movement disorder disorderininthe theelderly. elderly. Senile Senile tremor, tremor and and benign benign tremor tremor are are some some of of the the other terms used to tremor, familial familial tremor describe ET. ET.Although Although ET ET could could be be aa trivial trivial disorder, disorder, in in some some individuals it can be describe disabling disabling and and progressive progressive and and could could be be confused confusedwith withother other neurodegenerative neurodegenerative disdiseases. eases. In In this this chapter, chapter, we we will willdiscuss discussthe the epidemiology, epidemiology, clinical clinical features features and and therapy therapy ofET. of ET. Epidemiology ET ET is reported from all all the the regions regions of of the the world, world, indicating indicating aa global global occurrence occurrence

(Hornabrook (Hornabrook and Nagurney Nagurney 1976, 1976, Haerer Haerer et al. al. 1982, 1982, Rautakorpi Rautakorpi et et al, al. 1982, 1982, Moretti et Rajput et al. 1984, Bharucha et al. 1988, et al. al. 1983, 1983, Aiyesiloju Aiyesiloju et al. 1984, Rajput Salemi 1994). Depending study methods, methods, prevalence prevalence estimates estimates vary vary Salemi et et al. al. 1994). Depending on the study widely. The incidence and and prevalence prevalence of ET ET increases increases with with advancing advancing age. age. widely. The incidence Hornabrook and Nagurney (1976) (1976) examined a region in New Guinea Guinea for for characprevalence of of ET. ET. They reported no no cases cases of ET ET under 30 years old, but but teristics and prevalence under 30 in the 50—59 50-59 year increased to to 41 41 yearold oldage agegroup groupthere there were were 17 cases casesper per 1000, which increased cases 1000 over cases per per 1000 over the the age age of of 60 60 years. years. Louis Louis etet al. al, (1995) (1995) studied studied a randomized sample of 2117 2117 Medicare Medicare recipients 65 years) years) in in the the Washington Washington sample recipients (older (older than than 65 Heights New York. York. They prevalence Heights Inwood Inwood area area in Manhattan, New They reported reported a crude prevalence of 39.2 per 1000 1000 subjects over the age age of of 65 65 years. years. Age Age is a risk factor for for ET. ET. Data Data multiple studies studies show show aa dramatic dramatic increase increase in in the theprevalence prevalence of of ET ET with with aging aging from multiple (see Fig. 5.1). Genetics The tendency of of ET ET to run run in in families families has has been been recognized recognized for for many manyyears. years. Family Family

history positivity varies by report from 17 17 to 96% (Marshall 1962, Hornabrook and and 80

81 81

Essential Essential tremor 8000 8000

Men —

Women

6000 a)

0

4000 Based on data from: from: and Homabrook and Nagumey (1976) Nagurney(1976) Haerer et aI.(1982) Haereretal.(1982) Rautakorpi (1978) Bharucha et aL(1988) Bharuchaetal.(1988) Salemi et aL(1 994) Salemietal.(1994)

Q.

2000

0

0-29

30-39

40-49

50-59

60-69

70-79

80 +

Years Fig. Fig. 5.1 5.1

Crude age specific specificprevalence prevalenceofofETEl per per 100000 100000 population. Crude age

Nagurney 1976, Rautakorpi 1978, 1976, Rautakorpi 1978, Rajput et et al. al. 1984, 1984, Aiyesiloju Aiyesiloju et al. al. 1984). 1984). The The genealogical reports published published to to date date support support the assumption that genealogical reports that familial familial ET ET is inherited in an an autosomal autosomal dominant dominantmanner manner(Dana (Dana1887, 1887,Buckley Buckley1938, 1938, Jager Jager and King 1955, Bain 1994). Genetic earlier age age of King 1955, Bain et et al. al. 1994). Genetic anticipation, anticipation, defined defined as as an earlier disease onset in successive successive family families with disease family generations, generations, is is recognized recognized in in some families ET (Critchley 1949, 1949, Larsson Sjogren 1960, 1960, Bain Bain et al. al. 1994, 1994, Jankovic Jankovic et al. al. ET (Critchley Larsson and and Sjogren 1997). CAG repeats 1997). Kaneko Kanekoetet al. al. (1993) (1993)reported reported an an increased increased number number of GAG repeats in in the the gene in a subject subject with late onset ET. androgen receptor gene ET.AAtremor tremor indistinguishable indistinguishable from from ET may may be be observed observedininpatients patientswith withautosomal autosomaldominant dominant idiopathic idiopathic torsion dystonia (Ozelius et al. al. 1989). 1989). Similarly, Similarly, individuals genetic disorders disorders dystonia individuals with with other genetic such as X-linked atrophy (SBMA) (SBMA) (LaSpada (LaSpada et al. 1993) 1993) and X-linked spino spino bulbar muscular atrophy familial linked to to chromosome chromosome4q21—q23 4q21-q23 (Polymeropoulos (Polymeropoulos et et al. al. familial parkinsonism parkinsonism linked 1996) 1996) have have tremor tremor similar to ET. Recently, Recently,Higgins Higginsetetal. al.(1997) (1997)reported reported the results of linkage linkage analysis in a large American family of Czech descent analysis in large American family of Gzech descent with with dominantly inherited genetic anticipation. anticipation. Genetic Genetic loci on on chromosome chromosome 2p22-p25 2p22-p25 inherited ET and genetic established linkage to this region. Their finding suggested that a single highly penestablished linkage to gene is sufficient sufficient to cause at least ET. Their etrant gene least one form of ET. Their results results suggest suggest that X-linked SBMA SBMA and ET may share a similar tremorgenic mechanism. and ET may share a similar tremorgenic mechanism.

Gulcher et al. (1997) performed aa genome genome wide wide scan scan for for familial familial ET ET in Iceland. Iceland. The scan scan revealed one locus locus on on chromosome The revealed one chromosome 3q13 when the data data were were analysed analysed either parametrically, parametrically, assuming assuming an anautosomal autosomal dominant dominant model, model, or or non non parametrically. This 80% of of the the Icelandic Icelandic families families cally. This gene gene appears appears to to account account for for ET in about 80% they studied. A more definite assessment on on how how the the linked linked gene gene affects affects these famfamilies ilies may may be be possible possible only only after after cloning of the gene.

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R. Pahwa Pahwa and W. C. Koller R.

Clinical manifestations Clinical Tremor is usually usually the the sole sole manifestation manifestation of ET. Tremor Tremor is is defined defined as as an an involuntary

oscillation oscillation of of aabody bodypart part produced produced by by alternating alternating or or synchronous synchronous contractions contractions of of reciprocally 1980). Although reciprocally innervated innervated antagonistic muscles (Jankovic and Fahn 1980). mild gait may be present, neurological neurological examination mild abnormalities abnormalities of of tone and gait examination is is usually otherwise (Larsson and and Sjogren Sjogren 1960). 1960). Essential Essential tremor isis usually otherwise unremarkable (Larsson is the tremor tremor isis best seen with maintenance of of aa fixed fixed posture usually postural; that is (Koller 1984a). movement of the limbs limbs towards towards a (Koller 1984a).The Thetremor tremor isis attenuated attenuated with with movement (Critchley 1949, 1949, Larsen Larsen and Calne Calne 1983, 1983, Elble Elble 1986). 1986). However, However, a recent recent target (Critchley community-based emphasized the presence othercommunity-based study has emphasized presence of of kinetic kinetic tremor in otherwise untreated previously unknown cases cases of of ET ET (Louis (Louis et al, al. 1998). 1998). Resting Resting wise untreated and previously is rare but may may be more common common in in elderly elderly subjects. subjects. tremor is The most frequent are the hands (Bain The frequent body parts parts affected affected by ET are (Bain et et al. al. 1994; 1994; Borges et et al. al. 1994; 1994;Koller Kolleretetal. al.1994). 1994).Usually Usuallythe thetremor tremorisisunilateral unilateral at at the the onset, Borges but with with time time both both hands handsdevelop develop tremor tremor(Critchley (Critchley1949, 1949,Larsson Larsson and and Sjogren Sjogren 1960, Longe adduction-abduction movement of of 1960, Longe 1985). 1985). Tremor Tremor usually usually causes causes an adduction—abduction fingers and aa flexion—extension flexion-extension movement (Critchley 1949, 1949, Koller Koller the fingers movement of the hands (Critchley 1984a). Handwriting deteriorates, but true parkinsonian micrographia, when on 1984a). Handwriting deteriorates, but micrographia, on attempting to write handwriting handwriting progressively progressively decreases decreases in size size to to almost a single affected body part. Head Head line, does not occur. The head is the next most frequently affected tremor isis usually associated with hand tremor tremor(Longe (Longe1985, 1985,Herskovits Herskovits et et al, al. 1988). 1988). tremor parts associated associated with hand tremor tremor include include voice, voice, legs, legs, trunk and and The other body parts chin (Larsson and Sjogren Sjogren 1960, 1960, Rautakorpi Rautakorpi 1978, 1978, Findley Findley and and Gresty Gresty 1981, 1981, chin (Larsson Gerstenbrand et et al. al. 1982, 1982, Longe Longe 1985, 1985, Massey Massey and Paulson 1985, 1985, Herskovits et al. see Fig. Fig. 5.2). 5.2). 1988 and see Tremor progression Over time time tremor increaseand and tremor tremor can can spread tremor amplitude amplitude can can slowly slowly increase spread to

unaffected unaffected body parts. Critchley Critchley (1949) (1949) suggested that for for the first first couple couple of of years there is is slow progression of of tremor tremor followed followed by by little little or or no nochange changefor forseveral severalyears. years. Finally, with Finally, with advancing advancing age age tremor tremor may suddenly worsen. Factors influencing influencingET ET Age influences influences the the expression The tremor frequency expression of ET. The frequency usually usually declines declines and and

also amplitude increases increases with age age (Marshall 1962, 1962, Herskovits et al, al. 1988), 1988). There is also increasing involvement of multiple body areas Since functional functional disability areas with age. Since amplitude, elderly elderly subjects with ET are more disin ET is mainly mainly related related to to tremor tremor amplitude, abled subjects (Rajput (Rajput et et al. al. 1993, 1993, Elble Elble 1986). Other factors factors abled compared to younger subjects influence tremor severity severity include fatigue, fatigue, temperature extremes, extremes, emotional emotional that influence

83

Essential Essential tremor

120

100

Hands

80-

Head

s,

bO Ct

£3 0 2 o 0)

Yoke Voice

60-

Legs

Trunk Trunk

8.

Chin

40

Clinical Studies: Studies: 55 == Massey 1= = Larsson & & Sjogren Sjogren (1960) (1960) Massey and and Paulson Paulson (1985) (1985) 2 == Rautakorpi 6 Rautakorpi (1978) (1978) 6 == Findley Findley and and Gresty Gresty (1981) (1981) 985) 77 == Herskovits 3= = Longe Longe (1 (1985) Herskovits etal. et al.(1988) (1988) 4= = Gerstenbrand etal. etal. (1982) (1982)

Fig. Fig. 5.2 5.2

Percentage of body body parts affected by Percentage of by El. ET.

upset, sexual sexual arousal, arousal, central central nervous nervous system system stimulants stimulants and and diurnal fluctuations upset, fluctuations Wake et al. al. 1974, 1974, Larsen Larsen and and Calne Calne 1983). in catecholamine levels levels (Critchley 1949, 1949, Wake Disability

was once once known known as as 'benign 'benign essential essential tremor' tremor' because life expectancy isis normal normal ET was life expectancy et al. al. 1984). 1984). However, it is well (Rajput et well known known that ET can cause significant psychodisability (Rajput (Rajput et etal. al. 1984, 1984, Busenbark Busenbark et et al. al. 1991, 1991, Bain et logical and and functional disability et 1994). In fact, referred to to aa university university al. 1994). fact, approximately approximately 15% 15% of of patients patients with ET referred (Rautakorpi 1978). 1978).Busenbark Busenbark et etal. al. (1991) (1991) clinic retired retired from from work due to tremor (Rautakorpi oflife life measured measured by bythe the Sickness Sickness Impact Impact Profile Profile in in753 753 patients patients compared quality of ET, 145 patients subjects. Quality Quality of of life life was impaired with ET, patients with PD and 87 control subjects. in patients with ET compared compared to controls, but but to a lesser lesser degree degreethan thanthat that found found in in patients Communication, work, emotional behaviour, behaviour, home managemanagepatients with with PD. Communication, ment, recreation and and pastimes pastimes were were particularly particularly impaired impaired in inET ET (Busenbark (Busenbark etetal. al. 1991). have major functional disability disability that includes includes 1991). Patients Patients with with hand hand tremors tremors have major functional impaired writing, drinking from a cup, feeding and manipulating fine objects impaired writing, drinking from a cup, feeding and manipulating fine objects (Rajput et et al. al. 1975, 1975, Rajput Rajput et etal. al.1984, 1984, Busenbark Busenbark et etal. al.1991, 1991,Bain Bain et etal. al.1994). 1994). (Rajput Severe voice tremor can make speech very difficult to understand, especially in Severe voice tremor speech difficult to understand, especially in elderly patients. the major disability disability in in patients with with elderly patients. Although Although embarrassment embarrassment is the

84

R. Pahwa Pahwa and W. C. Koller R.

head tremor (Pahwa patient with with head (Pahwa et al. al. 1995), 1995), Rajput Rajput (1997) (1997) described described aa patient

tremor who who could could not not get get aa haircut haircut at at aa barber barber shop! shop! Differential diagnosis ET most commonly misdiagnosed in elderly elderly subsubET is most misdiagnosed as as PD PD (Koller (Koller 1984a), 1984a), especially in

jects with gait gait abnormality abnormality and bradykinesia related to aging. Resting Resting tremor tremor can can jects with bradykinesia related occur in ET though though its significance significance isisunknown. unknown. One One autopsy autopsy study study of of nine nine cases casesof of reported resting resting tremor in in six six cases cases in in life. life. Despite Despite the the presence presence of of resting resting ET reported Lewy bodies any of these these cases cases in the substantia nigra nigra tremor Lewy bodies were were not not found found in any (Rajput et al. 1993). All Allthe thepatients patients with with resting resting tremor tremor had a clinical history of ET of more than 10 10 years, years, all were over 60 developed, 60 years years old oldwhen when the the resting resting tremor developed, and in all the the subjects subjects resting resting tremor tremor was was preceded precededby bypostural postural and and kinetic kinetic tremor tremor degenerative diseases diseases in in early early stages can also be mis(Rajput et al. 1993). Cerebellar degenerative lithium, valproic valproic acid acid and and taken for ET in the elderly. elderly. Drug-induced Drug-induced tremor, due to lithium, neuroleptics, can can also also be confused confused with with ET. ET. Clinical variants

In ET the degree of postural and kinetic degree of kinetic tremor tremor may may vary. vary. In In cases cases of kinetic predominant tremor tremor aa marked marked dissociation dissociation occurs with the postural component being being minimal or absent absent (Biary (Biary and and Koller Koller 1987). 1987). Although Although cerebellar cerebellar signs minimal signs are are not not disability may be severe tremor. found, disability severe due due to to the the marked degree of intention tremor. Task specific specifictremor tremor (see (see Table Table5.1) 5.1)isisproduced produced mainly mainly by by the the act act of performTask performing certain motor activities, The most most common task ing certain specific specific motor activities. The task specific specific tremor is is primary writing writing tremor. tremor. Pronation Pronation of the arm arm while while writing produces produces a pronapronation/supination tremor not seen seen during during other other movements movements of arm tion/supination tremor that that is is not of the the arm (Rothwell et 1979, Klawans 1982, Ravits 1985). Other activities activities (Rothwell et al. al. 1979, Klawans etet al. al. 1982, Ravits et et al. 1985). involving major involvingpronation pronation also also result result inin tremor, tremor, though though impaired impaired writing writing isis the the major source of disability. disability. It not associated associated with with other other neurological neurological abnormalities. abnormalities. source It is not Other task specific specific tremors associated with golf club swinging, swinging, fist tremors include those associated fist clenching, screwdriver, sewing, cutting with with scissors, scissors, clenching, shaving, shaving, combing, combing, using aa screwdriver, glass or protruding the the tongue tongue(Rothwell (Rothwell etet al. al. 1979, 1979, Kachi Kachi et holding a glass or cup and protruding al. 1985, Ravits 1985, Rosenbaum Jankovic 1988). 1988). Patients Patients with al. 1985, Ravits et et al. al. 1985, Rosenbaum and and Jankovic with task specific tremor tremor during duringaaspecific specific task taskinvolving involving repetitive repetitive specific tremor usually manifest tremor and frequently performed performed movements. movements. Some Some investigators investigators believe believe task task specific specific and frequently ET, whereas others have have suggested suggested it may be a form of may be be aa variant variant of of ET, tremor may dystonia. has some some characteristics characteristics similar to ET because dystonia. Primary writing tremor has because it clinical characteristics (Kachi (Kachi et al. 1985, shares some of the electrophysiologic and clinical 1985, 1986). However, However, there there are are other other investigators investigators(Ravits (Ravitsetetal. al.1985, 1985, Koller and Martyn 1986). Elble et Elble et al. al. 1990) 1990)who whoregard regardprimary primary writing writing tremor tremor as a manifestation manifestation of writer's cramp.

85

Essential Essential tremor

Table 5.1 tasks associated associated with with tremors 5.1 Specific tasks

Writing Golf club swinging Gutting with scissors Cutting Holding cup or or glass glass Protruding tongue Shaving Gombing Combing Using a screwdriver Sewing

ET can can be be restricted restricted to to one one body body part. Isolated tremors of the tongue, chin and Isolated tremors voice may occur. Tongue Tongue tremor tremor is found if the tongue is tested in the postural postural posiposition. Often patients patients are are unaware unaware of of any any difficulty difficulty with the the tongue tongue tremor. tremor.Rarely, Rarely, tongue tremor can can affect affect speaking and eating. An isolated chin tremor not not involvinvolving the lips may also also occur. occur, An An isolated isolated chin chin tremor, tremor, which is stimulus stimulus sensitive, has described in in families families (Grossman (Grossman 1957, 1957,Lawrence Lawrence et et al. al. 1968). 1968). It It may may begin at at been described birth and and itit usually usually improves improves with age. age. Speech birth Speech involvement involvement can can be bethe the predominant predominant 1967, Hachinski et al. 1975). or sole symptom symptom in ET (Brown (Brown and Simonson 1967, 1975). Truncal Truncal ET is rare. Heilman (1984) described tremor occurring occurring as as a presenting symptom of of ET three the three patients patients with with the the sole sole symptom symptom of of orthostatic orthostatic tremor tremor of of the the trunk and the proximal legs. legs. Tremor increases with Tremor is is present present after after standing for several seconds, increases with time and can can lead lead to to falling falling (Koller (Koller et al, al. 1986).

Treatment Although there is no cure for ET, pharmacological pharmacological and surgical surgical therapies therapies presently available can help help control control tremor tremor in the majority of 5.2). Tremor Tremor available can of patients patients (see (see Table 5.2). of different body parts and different body and various various tremor tremor variants variants may may have have different different levels levels of pharmacological responsiveness pharmacological responsiveness and and some some patients patients might might not not respond respond to the currently available available therapies. Alcohol

A majority patients with with ET ET report report aa dramatic dramatic but but short short term term improvement improvement of of majority of patients tremor with with alcohol alcohol (Growdon (Growdonetetal. al.1975, 1975,Koller Koller and and Biary Biary 1984). 1984). However, However, tolertolerance to alcohol alcohol and and fear fear of of addiction addiction make many clinicians clinicians avoid avoidthe the routine routine use use of of treatment of ofET. ET. Schroeder Schroeder and and Nasrallah Nasrallah (1982) (1982) in in aa retrospective retrospective ethanol in the treatment survey alcohol abuse abuse was patients survey of of records records reported reported that that alcohol was present present in in 67% of patients ET. However, with ET. However, Koller Koller(1983) (1983)conducted conducted aaprospective prospectivestudy studyand and found found that that the

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R. Pahwa Pahwa and and W. C. Koller R. W. C.

Table Therapies for for ET El Table 5.2 5.2 Therapies Medications: Medications: definite benefit

Beta adrenergic blockers Beta Primidone Alcohol

Medications: Medications: possible benefit benefit Benzodiazepines Phenobarbital Carbonic anhydrase inhibitors Gabapentin

Medications: questionable benefit benefit Levodopa Anticholinergics Amantadthe Amantadine Clonidine Trazadone Verapamil Nicardipine Flunarizine Surgical procedures Thalamotomy Thalamic stimulation

prevalence pathological drinking in ET did not differ prevalence of pathological differ from that of of patients patients with with other tremor disorders disorders or or chronic chronic neurological neurological disease disease without tremor. The mechanism mechanism of action action of ethanol is unknown. Growdon et al. (1975) (1975) found found The that orally administered ethanol produced a dramatic reduction in in essential tremor but intra arterial concluded that tremor is arterial infusion infusion had had no no effect. effect. They concluded is reduced by a central mechanism. mechanism. Boecker Boecker et et al. al. (1996) (1996) used used PET PET scans scans to to investigate investigate the the effect effect of flow in six patients with alcohol-responsive alcohol-responsive of ethanol on regional cerebral blood flow controls. Ethanol Ethanol ingestion ingestion led led to tobilateral bilateraldecreases decreases of of cerecereET and age matched controls. bellar blood control groups. groups. In bellar blood flow flow in in both both patient patient and and control In the patient group addiincrease of regional regional cerebral cerebral blood blood flow flow was was observed observed in in inferior inferior olivary olivary tional increase suppressed tremor via a reduction of of afferent afferent nuclei. They concluded concluded that alcohol suppressed input to the inferior inferior olivary olivary nuclei caused caused by by abnormal abnormal cerebellar cerebellar input. input. ItIt can can be be concluded occasional use alcohol before contraindicated concluded that that the occasional use of alcohol before meals meals isis not not contraindicated and the risk risk of of alcoholism alcoholism isis low. low.

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Beta adrenergic blockers was among the first investigators Marshall (1968) (1968) was investigators to suggest suggest that beta adrenergic adrenergic

blockers might be helpful blockers might helpful in the the treatment treatment of of ET. ET. This This was was supported supported by by some some not all all studies studies(Foster (Fosteretetal. al.1973, 1973,Sweet Sweet (Sevitt 1971, Winkler and Young 1974), but not et al. al. 1974). 1974).Numerous Numerous investigations have since since confirmed confirmed the efficacy efficacyofofpropranpropran(Murray 1972, 1972, Tolosa and Loewenson 1975). 1975). It is generally estiolol in treating ET (Murray estimated 50-70% ofofpatients symptomatic mated that 50—70% patientswith with ET ET involving involvingthe the hands hands gain gain symptomatic propranolol. ItIt is is not known why some benefit from treatment with propranolol. some patients patients do do not respond Some reports suggest suggest that treatrespond to this treatment. Some that chronic propranolol treatment is better in younger of disease, though younger patients patients and and those those with with short short duration of suggest that propranolol is is more more effective effective in other reports suggest in older older patients. patients. The mechanism of of action action of of beta beta adrenergic adrenergic blockers blockers in in ET ET is is unknown. unknown. Young Young because he he noticed noticed no no effect effect of intravenous intravenous (1982) proposed a central site of of action actionbecause or intra arterial arterial propranolol propranolol and and aa delay delay in in the the effect effect of of chronic chronic oral oral therapy. therapy. However, However, other other investigators investigators have haveproposed proposed aa peripheral peripheral site site of of action action (Jefferson (Jefferson et et 1979, Huttunen al. 1979, Huttunen et al. 1984). ET ET can can also alsobe behelped helpedby bybeta-blockers beta-blockersthat thatdo do not not readily blood-brain barrier, However, readily penetrate the blood—brain barrier, such such as as atenolol atenolol and sotalol. However, the concentration of of atenolol in the cerebrospinal fluid fluid has has been been noted noted to to be be higher higher than that that of of propranolol propranolol(Taylor (Tayloretetal. 1981).Specific Specific beta2 beta 2 antagonists antagonists (ICI (ICI118551 al. 1981). 118551 LI 32—468) 32-468) which are effective effective in decreasing decreasing and LI whichpredominantly predominantly act act peripherally are of action action (Jefferson (Jefferson tremor, which further supports supports aa peripheral peripheral beta2 beta 2 mechanism of et al. al. 1984). 1984). et al. 1979, Huttunen Huttunen et Although propranolol has the greatest on upper extremity Although greatest effect effect on extremity tremor, it also also suppresses head, head, voice voice and and tongue tremor (Koller Biary and and suppresses (Koller 1984b, 1984b, Koller Koller 1985, Biary Koller propranolol, 240—320mg/day 240-320 mg/day was Koller 1987). 1987). In In dose dose response studies of propranolol, was found found to be the upper limit of the optimal dose dose range. range. However, However, older patients will not be able to to tolerate tolerate such such high high doses. doses. In In some some subjects subjects sustained sustained release releasepreparations preparations of of propranolol can can be more effective effective than (Koller 1985). 1985). than normal formulations (Koller Other orally orally active beta-adrenergic blocking drugs such as metoprolol, nadolol, atenolol, timolol and and pindolol pindololhave havealso also been been shown showntotobe bebeneficial beneficial in inET. ET. A A comcomtimolol (5mg (5mg twice twice daily) daily) and atenolol atenolol (100mg (100mg daily) daily) demondemonparison study of timolol strated strated that atenolol, atenolol, unlike unlike timolol, timolol, was was not not effective effective at the the dose dose employed employed (Dietrichson and Espen 1981). 1981). A double-blind study study with metoprolol metoprolol (150mg (150mg (Dietrichson and Espen A double-blind daily) and propranolol (120mg (120 mgdaily) daily) revealed revealed that that both both were were effective effective in the treatdaily) ment of tremor (Calzetti (Calzetti et al. 1981). 1981). Another metoprolol, Another study study with with the the use use of of metoprolol, atenolol and sotalol sotalol found found that only only sotalol sotalol was superior to placebo placebo (Leigh (Leigh et al. atenolol 1983). antagonists are better than placebo in the treatment of ET ET 1983), In In general, general, beta beta1 antagonists are better than placebo in the of 1 are not notasaseffective effective as as beta2 beta 2 antagonists antagonists (Calzetti (Calzetti etetal. al.1981, 1981,Leigh Leigh etet al. al.1981, 1981, but are Larsen Larsen and Teravainen 1981).

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Beta-blockers should be used with caution in in elderly elderly subjects and in in all all patients with with bronchospastic bronchospastic disease. disease. Relative Relative contraindications contraindications include include heart heart failure, failure, second and second or third degree degree heart block, asthma asthma or other other bronchospastic bronchospastic disease disease and insulin dependent dependent diabetes. diabetes. Most adverse effects effects are related to betabetainsulin Most of of the adverse are related adrenergic adverse effects effects in elderly elderly subjects adrenergic blockage. blockage. Common adverse subjects include fatigue, rash, impotence impotence and and mental mentalstatus statuschanges. changes.IfIfaaside side weight gain, nausea, diarrhoea, rash, effect changing to aa different different betabetaeffect such such as as impotence impotence occurs occurs with with propranolol, propranolol, changing of the the adverse adverse effect effect without loss loss of of benefit. benefit. blocker may result in disappearance of Primidone

When primidone primidone was administered administered to an an epileptic epileptic patient with with ET, ET, tremor was was

noticeably reduced reduced (O'Brien et al. 1981). This This led led to to aa study study with with primidone primidone in 20 20 patients with with ET ET at an initial dose of 125 125 mg/day, increasing to 750 mg/day (O'Brien (O'Brien patients et al. 1981). Twelve al. 1981). Twelvepatients patientshad hadaagood goodclinical clinicalresponse, response,but butsix sixpatients patientsdid did not not drug even even at at the the starting starting dose. dose. Findley Findley and and Caizetti Calzetti (1982) (1982) studied studied 11 11 tolerate the drug that tremor tremor was was reduced reduced by by 66% 66% and over over 90% in patients with ET and reported that 590 mg/day. In In aa placebo-controlled placebo-controlled study using two patients patients at aa mean mean dose dose of of 590mg/day. two accelerometry, Koller 1986) reported primidone(50—1000mg/day) (50-1000 mg/day) Koller and and Royse( Royse (1986) reported that primidone reduced untreated and and propranolol propranolol treated treated reduced the the amplitude amplitude of of tremor tremor in in both untreated decreased tremor Control of tremor patients. Primidone Primidone decreased tremor more more than than propranolol. propranolol. Control was lost lost when when primidone primidone was was replaced replaced by phenobarbital. phenobarbital. The The most most dramatic dramatic was response the first first week week of of primidone primidone therapy therapy response frequently frequently occurs occurs during during the Espen 1987), 1987). Although the long-term effect effect of primidone on on ET ET (Dietrichson and Espen has not been studied, a few studies studies suggest suggestthat that tolerance tolerance to to primidone primidone may develop (Crystal 1986, 1986, Shale the (Crystal Shale and and Fahn Fahn 1987). However, However, other other investigators investigators found found that the antitremor effect effect of for aa one one year year period (Koller (Koller and and of primidone was maintained for Vetere-Overfield 1989, Sasso in small small doses doses Vetere-Overfield Sassoetet al. al. 1990). 1990).Primidone Primidone should be used in 25 mg at at bedtime bedtime and andvery very gradually gradually increasing increasingto to250 250 mg. mg. in the elderly, starting at 25mg can cause cause nausea and impaired impaired balance. balance. In elderly subjects subjects primidone can The mechanism mechanism of of action action of of primidone primidone is unknown. Primidone is converted converted to to The active metabolites: metabolites: phenyethylmalonamide (PEMA) and The two active phenyethylmalonamide (PEMA) and phenobarbital. The administration ofhigh highdoses dosesof of PEMA PEMA had no no effect effect on tremor tremor (Calzetti (Calzettietetal. al.1981) 1981) administration of and only minimal antitremor antitremor action action (Koller (Koller and and Royse Royse 1986). 1986). and phenobarbital has only Primidone unrecognized metabolite, responsible Primidone itself, itself, or or an unrecognized metabolite, appears appears to to be be the responsible agent. Benzodiazepines

Although there have been very very few fewstudies studies of ofbenzodiazepines benzodiazepines in in ET, ET, they have been used clinically for for a long long time time in in the the mistaken belief belief that that tremor was due to anxiety. Thompson et al. al. (1984) (1984) studied studied the effect effect of clonazepam clonazepam in ET and and found found no no Thompson in ET

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improvement in tremor, thongh improvement though sedation sedationwas was aa common commonside sideeffect. effect. However, However, subsequently clonazepam clonazepamhas has been been found found to be sequently be effective effective in kinetic kinetic predominant predominant tremor (Biary (Biary and Koller Koller 1987) (Heilman 1984, 1984, Papa and 1987) and and in in orthostatic orthostatic tremor (Heilman Gershanik 1988). Huber Huber and and Paulson Paulson (1988) (1988) studied studied alprazolam aiprazolam in in aa double-blind, double-blind, placebo-controlled 24 patients patients with with ET. ET.Significant Significant improvement improvement placebo-controlledstudy studyinin 24 occurred, though half the patients in the study complained of fatigue fatigue and and sedation. The antitremor effect of of benzodiazepines is is likely to reducantitremor effect to be due to aa secondary reduction in anxiety. anxiety. In In animal animal models models of of tremor, diazepam diazepam probably suppresses suppresses tremor through enhancement of of GABAergic GABAergic neurotransmission et al. 1984). neurotransmission (Rappaport et Benzodiazepines commonly falls in elderly elderly subjects subjects and and Benzodiazepines commonly cause cause confusion confusion and falls need to be used used with with great great caution. caution. Unfortunately low doses are are unlikely unlikelyto to improve improve tremor. tremor,

Phenobarbital has been Phenobarbital has been used used for for many many years years for for ET, ET, though though there there have have been been conflicting benefit. Some Somestudies studieshave havereported reportedthat that phenobarbiphenobarbiconflicting reports regarding benefit. tal is more effective effective than placebo (Baruzzi (Baruzzi et et al. al. 1983, 1983, Findley Findley and and Cleeves Cleeves 1985). 1985). However, phenobarbital (90mg/day) However, Koller Kollerand andRoyse Royse(1986) (1986)found foundnonoeffect effectofofphenobarbital (90mg/day) in in 12 patients patients with with ET. ET,In Inaadouble-blind double-blind comparison comparison of of primidone primidone and phenobarbital, Sasso Sassoetetal. al.(1988) (1988)found foundprimidone primidonesuperior superior to toboth both placebo placebo and and phenobarbiphenobarbital in reducing tremor in in 13 13patients. patients. The The effectiveness effectiveness of phenobarbital in in ET ET is is its use in elderly elderly subjects. controversial and drowsiness often limits its Carbonic anhydrase anhydrase inhibitors inhibitors inhibitor methazolamide has been been reported to be highly The carbonic anhydrase anhydrase inhibitor methazolamide has highly effective ET,based basedon on the the results results of of an open label effective inin ET, label trial trial involving involving 28 patients patients 1991). A improvement, four four (Muenter et al. 1991). A total total of of 12 patients patients reported marked improvement, reported moderate improvement, improvement, four reported mild mild improvement improvement and and eight eight reported moderate four reported no improvement. improvement. Head Head and voice voice tremor patients reported no tremor responded responded particularly well. Although Busenbark et al. (1992) (1992) also also found found in in an an open open label label trial trial that that the caracetazolamide decreased bonic anhydrase inhibitor acetazolamide decreased tremor, tremor, patients patients did did not not report improvement functional disability. disability. A later later double-blind double-blind placebo-controlled placebo-controlled improvement in functional study (Busenbark et al. 1993) 1993) failed failedtoto demonstrate demonstrate any any superiority superiority of of methazolamide over placebo placebo in in any any of of the the measures measures tested. tested. Only Only two two patients patients continued continued the appears to to have have limited limited efficacy efficacy in the treatment treatment of ofET. ET. drug. Methazolamide appears

Other drugs Although antiparkinsonian antiparkinsonian medications medications such such as levodopa levodopa and anticholinergic anticholinergic treatment have been used in ET ET there there have have been been no no formal formal studies. studies. Similarly, Similarly, treatment have been used Critchley (1972) be helpful helpful for for ET. ET. Other Other studies studies have have Critchley (1972) reported reported amantadine amantadine to be

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confirmed that an may respond respond to to this this drug (Manyam confirmed an occasional occasional patient with ET may 1981, Obeso et al. 1986, Koller Koller 1984a). 1 984a).Other Other drugs drugs that that have been been reported reported to to have have questionable benefit (Caccia and Mangoni 1985, 1985, Koller Koller et benefit in ET include clonidine (Caccia et al. 1986), thymoxamine Olsen 1981), 1981), trazadone (McLeod and White al. 1986), thymoxamine (Mai (Mai and and Olsen trazadone (McLeod and White 1986), mephenesin (Critchley 1972), 1972), nifedipine (Topaktas (Topaktas et et al al 1987), 1987), verapamil verapamil 1986), mephenesin (Critchley (Topaktas et 1987), nicardipine nicardipine (Jimenez-Jimenez (Jimenez-Jimenez et al. al. 1994), 1994), flunarizine flunarizine (Topaktas et al. 1987), and Lang Lang 1993) 1993) and gabapentin gabapentin (Pahwa (Pahwa et et al. al. 1998). 1998). (Curran and Botulinum toxin injections injections

Botulinum toxin intramuscular intramuscular injection, injection, which whichisis mainly mainly used used for for focal focal dystonias, has been used for the treatment treatment of of ET. ET. Botulinum Botulinum toxin toxin causes causes muscle muscle paresis by acting on the peripheral nerve endings to block release release of of acetylcholine. acetylcholine. In In an an open open injections label trial, Jankovic Jankovic and and Schwartz Schwartz(1991) (1991)reported reportedthat that botulinum botulinum toxin injections reduced various 67% of of treated treated patients. patients. Pahwa Pahwa et et al. al. (1995) (1995) reduced various body body tremors tremors in 67% studied botulinum toxin in double-blind study studied botulinum toxin in a double-blind study in in head head tremor tremor and and found found that of their tremor. tremor. 40% of the patients had improvement of

Thalamotomy thalamotomy isis an procedure for for the the treatment Stereotaxic thalamotomy an effective effective procedure treatment of of ET. ET. Improvement in technical aspects and advances location of of Improvement advances in neurophysiological neurophysiological location The ventralis ventralis oralis oralis posposthe lesion site have have renewed renewed the interest in thalamotomy. The (VOP) and and the the ventralis ventralis intermedius intermedius (VIM) (VIM)thalamic thalamicnuclei nucleireceive receive cerebelcerebelterior (VOP) lar afferents afferents and the most most common common targets targets for treatment of tremor tremor lar and are are the for the treatment (Bertrand et al. al. 1969, 1969, Narabayashi Narabayashi and Ohye Ohye 1980). 1980). Stereotaxic Stereotaxic thalamotomy (Bertrand thalamotomy is is with the the use use of of mild sedation and and local local anaesthesia anaesthesia (Goldman (Goldman and and Kelly Kelly performed with 1992). The 1992). The stereotaxic stereotaxic coordinates coordinates are are generated generated by by use use of of the the computerized computerized tomographic and computerized computerized programs. programs. Goldman Goldman and and Kelly Kelly (1992) (1992) ographic scanning scanning and reported outcome after after thalamotomy reported outcome thalamotomy in in seven seven patients patients who who underwent underwent unilateral unilateral and in in one one patient patient who who underwent underwent bilateral bilateral thalamotomy. thalamotomy. At At followfollowthalamotomy and (mean 17.3 17.3 months) months) all all patients patients had had marked marked improvement improvement of of the the targeted targeted up (mean after a mean follow-up of 53.4 months, tremor. Jankovic et al. (1995) (1995) reported reported that after 83% 83% of of ET ETpatients patients had had cessation cessationof ofor or moderate moderate to to marked marked improvement improvement in their contralateral improvement in in function, function. Shahzadi Shahzadi et et al. al. contralateral tremor with a concomitant improvement that 86% 86% of the ET patients had significant postoperative improveimprove(1995) reported that in tremor, tremor, though though 42% 42% of of the the patients patients had had recurrence recurrence of of tremor tremor after after five five ment in years. Unilateral Unilateral thalamotomy in ET has a mortality rate of less than 0.3%, mostly related hemiparesis, dysdysrelated to to postoperative postoperative complications. Confusion, contralateral hemiparesis, arthria seizures can occur occur in in the the postoperative postoperative period, period, but butusually usually resolve resolve arthria and seizures rapidly. Bilateral Bilateral thalamotomy thalamotomy is associated with more severe and long lasting complications such as severe cognitive impairment. severe dysarthria dysarthria and and permanent permanent cognitive impairment,

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Thalamic stimulation Thalamic

observed that during a thalamotomy procedure stimulation It had long been observed stimulation of of the the as its its destruction. destruction, This paradoxical effect related target site had the same effects effects as effect is related 100 Hz to stimulus frequency frequency and and cannot be induced induced under 100 Hz stimulation. stimulation. The The main advantages advantages of of stimulation stimulation compared compared to thalamotomy thalamotomy include include reversibility, reversibility, the parameterstotoincrease increaseefficacy efficacy or or reduce reduce side side effects, effects, and ability to change stimulus parameters the fact dysfact that that bilateral bilateral operations operations can be be performed performed without causing permanent permanent dys(Benabid et et al. al. 1989). 1989). The mechanics of lead arthria (Benabid lead placement placement are similar similar to to thalamotomy, though connected to amotomy, though the the lead lead is is internalized internalized under under the the skin skin and and connected to an implantable pulse generator, which which is is placed under under the the clavicle. clavicle. The disadvantages disadvantages system, the of stimulation include the cost of the system, the presence presence of of foreign foreign material material and and the future need need to to replace replace the energy energy source. source. Thalamic Thalamic stimulation the future stimulation results results in in aa significant and aa marked marked reduction reduction ininglobal global disability disability significantreduction reductioninin tremor tremor and 1991,1993, (Benabid et al. 1991, 1993, Koller Kolleretal. et al.1997). 1997).Complications Complicationsrelated relatedtotostimulation stimulation include paraesthesia, paraesthesia, headache, disequilibrium, gait disorare usually usually minor and include der, dysarthria localized pain (Benabid (Benabid et et al. al. 1991, 1991, 1993, 1993, Koller Koller et al. 1997). 1997). der, dysarthria and localized Thalamotomy and thalamic thalamic stimulation stimulation should shouldbe be reserved reserved for for patients patients with with severe severe causing marked functional disability. disability. drug resistant tremor causing

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Essential Essential tremor by behaviour therapy. keigaku therapy. Use Use of of Jacobson's Jacobson's progressive progressive relaxation relaxation method. method.Seishin SeishinShin Shinkeigaku

Zasshi, Psychiatrica (Tokyo), 76, 7,76, 509—17. Zasshi, PsychiatricaetNeurologiaJaponica et Neurologia Japonica (Tokyo), 7, 509-17. action tremors tremors of ofthe the Winkler GF, Young RR RR (1974) (1974) Efficacy Efficacyofofchronic chronicpropranolol propranolol therapy in action of of Medicine, 290, 984—8. EnglandJournal Journal Medicine, 290, 984-8. familial, senile or or essential essentialvarieties. varieties.New NewEngland Young RR (1982) Essential—familial tremor and and other action tremors. Essential-familial tremor tremors.Seminars SeminarsininNeurology, Neurology,2, 2, 386—91. 386-91.

Gait apraxia and and multi-infarct multi-infarct states Gait apraxia states Richard Liston and Talus and Raymond RaymondCCTallis

Introduction There is an an extensive extensiveliterature literature on gait gait apraxia apraxia or 'higher-level gait disorders' as they

have been called (Nutt et al. have recently recently been called (Nutt al. 1993). 1993). The The term term encompasses encompasses walking walking difficultiesthat that are are out out of of proportion proportion to those that would be expected on the basis difficulties of the the bedside bedside neurological neurological examination examination and that are best explained by by disorders disorders of of integration of cerebral activity. activity. There There have have been been few few attempts attempts to to formulate formulate unifyout by by the large number number ing theories regarding these gait disorders and this is borne out of synonyms used to describe what are essentially the same or very similar gait disorders (see Table orders (see Table 6.1). 6.1).In In this this chapter, chapter,we weshall shallattempt attempt to to unravel unravel the the conceptual conceptual that surrounds surroundsthese thesegait gaitdisorders. disorders.Firstly, Firstly, we we shall shall review review the historical historical muddle that accounts the literature. literature. Secondly, Secondly, we shall shall describe describe a recent, accounts of of such such disorders in the Thirdly, we we shall shall propose own though unsatisfactory unsatisfactory classification. Thirdly, propose our our own classification neuroanatomical and and neurophysiological neurophysiological correlations correlations classificationbased based on on neuroanatomical drawn largely especially in largely from from the the existing existing literature literature on on gait gait initiation, initiation, especially in relation relation to to disease (PD). Finally, Finally, we Parkinson's disease we will will suggest suggesthow how aa clearer clearer understanding understanding of disease processes processes may developing rational approaches to therapeutic the disease may aid aid in in developing rational approaches to therapeutic interventions. this chapter chapter we we shall shall focus particularly on the higherhigherinterventions. Throughout this level gait level gait disorders disorders associated associated with with cerebral cerebral multi-infarct multi-infarct states (CMIS) and refer to (vascular HLGDs). HLGDs). It must must however however be be these as vascular higher-level higher-level gait disorders (vascular remembered that, that,along alongwith withCMIS, CMIS,many manyother otherdisease diseaseprocesses processes can can lead lead to to these these remembered gait gait disorders: disorders: many many of of the the original original descriptions were were of of frontal frontal lobe tumours.

and the the frontal lobes Gait apraxia apraxia and lobes It has been recognized for well over over aa century century that that frontal lobe lesions can cause gait impairment, particularly in elderly elderly patients. patients. Bruns Bruns (1892) (1892) described describedimpairment impairment of of gait lesions and named this condition condition 'ataxia 'ataxia of of gait'. gait'. He He gait secondary secondary to to such such lesions and named described frontal described two two patients patients with with frontal frontal lobe lobe tumours, tumours, and and two two patients patients with with frontal of aamore morediffuse diffuse disease disease process. process. Originally Originally many many authors authors lobe damage as part of 98

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Gait apraxia apraxia and and multi-infarct multi-infarct states Gait

Table 6.1 6.1 Synonyms Synonyms for for HLGDs HLGDs

Gait apraxia Frontal ataxia Frontal disequilibrium ataxia and disequilibrium Frontal ataxia gait disorder Frontal gait Subcortical disequilibrium Subcortical Marche àa petit pas Vascular Vascular pseudoparkinsonism Lower body body parkinsonism parkulsonism Lower Arteriosclerotic parkinsonism Gait disorder of Binswanger's Binswanger's disease Cautious gait gait ignition failure Isolated gait

thought that that these these 'ataxic 'ataxic gaits' gaits' were were secondary secondary to to cerebellar cerebellar lesions, lesions, the the precise precise location location of direct or indirect indirect damage damage to to the the cerebellum cerebellum being unknown. Bruns Bruns himself the 'cerebellar' 'cerebellar' signs were himself thought that the were due due to to compression compression of of the cerebelcerebellum from from frontal frontal tumours tumours or or disruption disruption ofofthe thefronto-ponto-cerebellar fronto-ponto-cerebellar tract. tract. Gerstmann and Schilder Schilder (1926) (1926) described Gerstmann described two two similar similar cases, cases, one one of of whom whom had had aa frontal 'gait apraxia'. apraxia'. Van Bogaert Bogaert and Martin Martin frontal glioma, and they first used the term 'gait (1929) subsequently 'frontal disequilibrium' and described described the gait gait (1929) subsequently used used the the term term 'frontal patient with with aa frontal lobe abscess and in another with a frontal lobe disorder in one patient glioma. Austregesilo cumbersome term term glioma. Austregesiloand and Fortes Fortes (1936) (1936) suggested suggestedthat that the the cumbersome 'frontal ataxia and disequilibrium' be used, suggesting suggesting two two distinct distinct components components of of the disorder. Earlier, frontal lobe lobe is the the disorder, Earlier, Gordon Gordon (1917) (1917) had had concluded concluded that that the the frontal centre for their seminal seminal descriptions, descriptions, Meyer Meyer and centre for equilibrium equilibrium and and orientation. In their and Barron (1960) subsequently described described seven seven cases casesusing usingthe the term term 'apraxia 'apraxia of of gait', ataxia'. Their aim was which they preferred preferred to 'frontal ataxia'. was to to describe describethe the deterioration deterioration in gait that occurs from cerebral cerebral damage damage particularly frontal particularly in the region of the frontal although they they did did concede concede that thatsome someof oftheir theircases cases suffered suffered from from diffuse diffuse cerelobes, although authors clearly clearly documented documented that thatthe thedisease diseaseprocess process was was not cerecerebral damage. The authors bellar in nature. nature. They They defined defined 'apraxia 'apraxia of of gait' gait' as as an inability to use the lower limbs in the act of walking, walking, which which could could not not be accounted accounted for for by by demonstrable demonstrable sensory weakness or motor impairment. They speculated that any lesion such as as tumour, tumour, weakness or impairment. They speculated that any lesion such abscess or abscess or arteriosclerosis, arteriosclerosis, which which involved involved mesial mesialaspects aspectsofofthe the frontal frontal and and pariepanetal lobes, could produce the syndrome. They argued that the primary difficulty tal lobes, could produce the syndrome. They argued primary difficulty appeared to be in the initiation of of movement, movement, particularly particularly in the abstract abstract perforperforappeared mance of motor movement movement such such as as taking taking the the first first step, step, kicking kicking an an imaginary imaginary ball ball

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concinded that that apraxia of gait gait is is aa 'transor writing nnmbers numbers with the feet. feet. They concluded apraxia of cortical innervatory paralysis': paralysis': the the disturbance distnrbance of gait gait initiation initiation being at a cortical cortical cortical level with the the motor mechanism for level with for movement movement of ofthe thelegs, legs, at at lower lower levels, remaining intact. From the above historical From historical descriptions, it is clear clear that frontal lobe lobe damage damage can can canse aa syndrome syndrome with with two two distinct components. The with movecause The first first is is difficnlty difficulty with initiation involving involving varying combinations of of gait gait ignition ignition failnre, failure, shnffling, shuffling, ment initiation freezing these freezing and and difficulty difficnltymaking makingturns. tnrns. The The second second component component common common to these descriptions virtually all the descriptions is disequilibrium diseqnilibrinm or poor balance. In virtnally the above above accounts, acconnts, descriptions of of disordered disordered gait gait patterns patterns include include these these two two basic clinical findings. the descriptions

parkinsonism and and cerebral cerebral multi-infarct multi-infarct states Vascular parkinsonism states (1929) gave gave wider wider currency cnrrency to to the idea Critchley (1929) idea of of 'arteriosclerotic 'arteriosclerotic (vascnlar) (vascular) parkinsonism' and and described described rigidity rigidity and and slowness slowness of movements in the syndrome syndrome along absence of the characteristics characteristics of of these these gaits gaits along with with the the absence of tremor. tremor. He stated that the were essentially essentially the the same same as as those those described described by by Marie Marie (1901) (1901) as as'marche 'marche àa petit petit pas', pas occurred in état état lacnnaire. lacunaire.Some Some of of the the patients patients he hedescribed, described, however, however, may may which occnrred have had mnltiple multiple system system atrophy, atrophy, progressive progressive supranuclear corticobasal have snprannclear palsy or corticobasal ganglionic interpret his his findings. findings. ganglionic degeneration, degeneration,which whichlimits limitsour onr ability ability to to interpret Nonetheless, the characteristic characteristic gait was steps, shnffling, shuffling, difficnlty difficulty Nonetheless, was one with short steps, initiating movements, difficnlty difficulty with with turning, tnrning, vertigo, giddiness giddiness and and poor balance. clearly similar frontal This description is clearly similar to to that that described described occurring occurring secondary secondary to to frontal lobe damage. damage. Critchley's Critchley's description gaits as weakness of lobe description of of the the gaits as being being due due to aa weakness walking out to the the relative relative strength of the individual movements walking out of of proportion to movements of of is also very similar of gait gait apraxia. apraxia. the limbs is similar to Meyer Meyer and Barron's description of Fitzgerald and and Jankovic Jankovic (1989) (1989) subsequently subsequently studied studied 10 10 patients patients with with marked Fitzgerald gait involvement and 100 gait difficulty difficulty and and minimal minimal upper upper limb limb involvement and compared compared them to 100 patients with with typical typical PD. PD. They They used used the phrase phrase 'lower 'lower body body parkinsonism' parkinsonism' to to patients describe lower describe patients patients in in the the former former group who, compared compared to PD patients, had a lower of symptoms, symptoms, more more initial initial complaints complaints of of disordered disordered gait gait and and were were mean duration of to levodopa levodopa comcommore likely to to have have hypertension. hypertension. Of this group 20% responded to 96% of the patients patients with with PD. PD. They They also stated and pared to 96% stated that gait disturbance and postural instability instability were the dominant dominant motor motor features, features, similar similar totoCritchley's Critchley's postural were the Clinically the gait gait disturbances disturbances manifested manifested as as slowness, slowness, shuffling, shuffling, interinterpatients. Clinically freezing, initiation initiation failure, failure, turning turningdifficulties difficulties and and response response to tovisual visual cues. cues. mittent freezing, They speculated speculated that the the ability ability to to overcome overcome freezing freezing by by visual visual cues cues (discussed (discussed They later) suggests suggests that lower body parkinsonism parkinsonism and PD, PD, the proprolater) that in patients with lower gramme learned movement movement (gait) (gait) is preserved preserved but there is an inability inability to gramme for for the learned access accessit. it,They Theyproposed proposedthat that chronic chronic subcortical subcortical ischaemia ischaemia secondary secondary to to hypertenhyperten-

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vascular disease disease might might be be responsible responsible for for the the disorder disorder resulting resulting in in disconnecsive vascular (BG) and and the supplementary motor tion between basal ganglia ganglia (BG) motorarea. area.We We will will elaborate further on these neuro-anatomical and and neuro-physiological neuro-physiological correlations correlations later later in this chapter. chapter. Chang et al. (1992) investigated 250 250 patients patients with with parkinsonism parkinsonism and performed (1992) investigated performed imaging (MRI) (MRI) on on 13 patients who who did not respond or showed magnetic resonance imaging 11/13 (85%) (85%) had ischaemic ischaemic lesions that a poor response to levodopa. Of this group 11/13 for their parkinsonism. parkinsonism. Bradykinesia, Bradykinesia, rigidity rigidity and gait gait diswere thought to account for turbance was prominent resting tremor was absent. They prominent but resting They did did not not comment on the presence presence or absence absence of disequilibrium. Three types of lesions lesions were common: frontal infarcts, basal ganglia lacunar deep frontal lobe infarcts, basal ganglia lacunar infarcts infarcts and periventricular and deep subcortical lesions. They who subcortical white white matter lesions. They concluded concluded that that parkinsonian patients who show no response response to to levodopa levodopa therapy therapy should shouldbe beinvestigated investigated for for aavascuvascushow poor or no aetiology. lar aetiology. Thompson Thompson and and Marsden Marsden(1987) (1987)described described 12 12patients patientswith with Binswanger's Binswanger's disease disease whom had or subcortical vascular vascular states, states, all allofofwhom had longstanding longstanding hypertension hypertension and who presented walking difficulty. difficulty. They presented with walking They described described the the gait gait as as combining combining 'brady'ataxia'. Gait kinesia' and 'ataxia'. Gait abnormality was was described described as asbeing beingout out of of proportion proportion to the observed clinical findings findings on bedside testing and gait gait ignition failure, failure, shuffling shuffling described. With either seated seated or or lying, lying, and disequilibrium were again described. With the patient either upper limb limb mobility mobility and andfacial facial expressions expressions were were relatively relatively well preserved. Patients presented with insidious insidious decline decline in in mobility, mobility, difficulty difficulty walking and and falls. falls. Diagnosis was made made based based on CT CT scan scan evidence evidence of bilateral bilateral low was low attenuation attenuation white white matter lesions frontal and parietal parietal lobes. lobes. Four patients also also had lacunar lesions throughout throughout the frontal infarcts. suggested that the disorder was similar frontal infarcts. The The authors suggested similar to that seen in frontal lobe lobe lesions, lesions, hydrocephalus hydrocephalus and and 'senile 'senile disorders disorders of of gait'. gait'. They They proposed proposed that that the syndrome is due to damage to afferent afferent and and efferent efferent interconnections interconnections of the leg areas areas cortex, the supplementary motor motor cortex, cortex, the the basal basal ganglia, and between the motor cortex, the cerebellum. As already already suggested, suggested, there obvious and and significant significant overlap overlap the cerebellum. there is obvious gait disorders associated associated with frontal frontal lobe lobe damage damage from from either either vascuvascubetween the gait or nonvascular nonvascular causes, causes, vascular vascular parkinsonism parkinsonism and and Binswanger's Binswanger's disease. disease. In In lar or addition, different different disease disease processes, processes, including frontal lesions, lesions, diffuse diffuse cerebral cerebral addition, including frontal disease, multiple ischaemia, disease, multiple lacunar infarcts, basal ganglia infarcts infarcts and subcortical ischaemia, may lead all these raises lead to to very very similar similar gait gait disorders. disorders. The The similarity similarity of all these disorders disorders raises the question as to whether they can be understood in terms of of aa unifying theory.

Current classifications Nutt et et al. al. (1993) have aimed aimed to to do do just just that. that. In an attempt (1993) have attempt at at clarification, clarification, they group these gait disorders together as 'higher-level gait disorders' on the basis that

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the abnormal abnormal gaits are due dne to disorders of the highest gaits are highest sensorimotor sensorimotor systems systems and

cannot be accounted for for by by the neurological neurological signs, signs, if if any, that are elicited by stanbedside examination. Basic Basicmotor motor functions functions such such as as power power and and coordination dard bedside function with no visual, labyrinthine should be intact, as should sensory function labyrinthine impairNutt et et al. al. (1993) (1993) divided divided these these gait gait disorders disorders into into five five groups: cautious cautious gait, gait, ment. Nutt isolated failure, subcortical subcortical disequilibrium, disequilibrium, frontal frontal disequilibrium, disequilibrium, isolated gait gait ignition ignition failure, and frontal gait gait disorder, disorder. However, However, this classification isis confusing some and frontal this classification confusing in in that some (frontal gait disorder), types of gaits are classified classified according accordingtotopresumed presumed location location (frontal others according to clinical clinical phenomenology phenomenology (isolated gait ignition failure, cautious gait) subcorgait) and and others according to a mixture of the two two (frontal (frontal disequilibrium, subcortical disequilibrium). disequilibrium). In the next next section section we weshall shalldevelop develop this thisclassification, classification, tical In the attempting to relate the clinical phenomena to to presumed presumed sites sites of of lesions. lesions.

ignition — the interaction interaction between the basal ganglia ganglia and and the the frontal frontal lobes Gait ignition - the lobes these probably represent represent types types of of motor programming these gait disorders disorders probably programming failure failure similar to those seen in in PD, PD, we shall propose possible locations of infarcts infarcts within the pathways controlling the the motor programming of we need need pathways controlling of normal normal gait. gait. But But first we understand how how this this system system works works normally, normally, how it can malfunction in in PD PD and and to understand can give give us insights into into what what isishappening happeningininvascular vascular}-ILGDs. HLGDs. how this can The motor cortex distinct areas in the frontal The cortex contains contains several several distinct areas in frontal lobes, lobes, which which receive modulatory receiveinputs inputs from from sensory sensory pathways, pathways,motor motor control control structures structures and modulatory pathways, including and BG. BG. This cluster cluster of of distinct distinct frontal frontal fields fields is pathways, including the thalamus and fundamentally involved involved in fundamentally in movement movement planning planning and and performance performance (Donoghue and Sanes recognized fields fields are: are: Sanes 1994). The most widely recognized 11 the the primary primary motor motor cortex cortex(Ml), (M1),which whichprobably probablycontrols controlsmuscle muscle force force and and the the direction of movement; 2 the thepremotor premotorarea area(PMA), (PMA),which which isisprobably probably involved involved in coupling environmenenvironmental cues to to motor acts and may be responsible responsible for for the the motor motor response response to to external maybe stimuli (Chen (Chen etet al, al. 1995 1995 and and see see Fig. Fig. 6,1) 6.1) involved in in the the preparprepar3 the the supplementary supplementary motor motorarea area(SMA), (SMA),which which isis possibly possibly involved ation and execution execution of of complex complex voluntary voluntary movements, movements,especially especially ifif these movements are and may ments are learned, learned, and may thus be responsible responsible for for the internal internal cueing cueing and and guidance guidance of of acquired, acquired, skilled skilled motor motor acts of the limbs (Roland et al. 1980, Chen et al. 1995, Thaler et al. 1995 1995 and see see Fig. Fig. 6.1). internally cued, cued, well well learned learned motor motor act act - it In the case case of normal walking - an internally suggested that SMA fires probably has been suggested that the SMA fires just just prior prior to gait ignition. This probably reflects preparatory subcomponent of aa movement movement sequence sequence reflects preparatory activity activity for for each each subcomponent pro(Georgiou et al. 1993). 1993). This This preparatory preparatory activity may may represent represent submovement submovement programme selection, i.e. i,e, aa complex complex set setof ofinstructions instructions for for each each submovement, submovement, which which As As

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Gait apraxia and and multi-infarct multi-infarct states Gait apraxia

Externally cued movement

Sensory Stimuli Internally cued movement movement

Fig. Fig. 6.1 6.1

Mechanism of gait ignition. BG, basal ganglia; ganglia;M1, Ml, primary motor cortex; Mechanism BG, basal cortex; PMA, PMA, premotor SMA,supplementary supplementary motor motor area; Thal, Thai, ventro-lateral ventro-lateral nucleus nucleus of of the thalamus. area; SMA, *Visual vestibular *Visual, vestibular and proprioceptive proprioceptive inputs. inputs.

is subsequently sent sent to to the theMl. M1.Activity Activityininthe theSMA SMAisisswitched switchedoff offby by phasic phasicactivactivgenerated by by the BG. This probably probably provides provides aa nonspecific nonspecific cue cue both both to trigger ity generated BG. This submovement (i.e. SMA SMAsends sendsthe theinstructions instructionstotothe theM1) Ml) and and to instruct the the submovement SMA SMA to to prepare prepare for for the the next (Phillips et al. 1994). 1994). This This allows allowsthe thesubmovement submovement to to executed normally time (Georgiou (Georgiou et et al. al. 1993). 1993). It It isis this this interaction, interaction, be executed normally and and on time activity from activity in in the the SMA, SMA, which is between phasic activity from the BG and premotor activity responsible of predictable, learned, automatic movement movement responsible for for the the smooth smooth running of sequences internal cues. cues. The The sequence sequence of activation activation is different different sequences that that depend depend on internal occur in in response response to to external external cues. cues.In In this this situation situationthe theBG!SMA BG/SMA when movements occur pathways theory, be be bypassed: bypassed: sensory sensory information information pathways could, could, according according to to this theory, from feeds directly directly into PMA through visual, auditory from the environment feeds into the PMA through visual, auditory and

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proprioceptive pathways and the PMA activates the the M1 Ml (Marsden pathways and PMA subsequently subsequently activates (Marsden 1989). 1989).

Motor programming failure in in PD PD and the response response to external external cues cues

pathological situations situations such such as as PD PD it has been argued that there In pathological there isis disordered disordered cueing in the the BG BG means means cueing from from the the BG: BG: aa disturbance disturbance in in internal internal rhythm formation in the SMA SMA is not switched switched off on time time (Georgiou (Georgiou et et al. al. 1993, 1993, Freeman Freeman et al. al. that the 1993). Submovements preparatory activity activity 1993). Submovements are are therefore therefore not triggered triggered and no new preparatory occurs in the SMA. This This causes causes some some of of the the classical classical clinical clinicalfeatures featuresof ofPD PD includincludfreezing. Furthermore, PD patients patients seem seem ing gait ignition failure, bradykinesia bradykinesia and freezing. Furthermore, PD disadvantaged by the absence absence of external cues from environment to be greatly disadvantaged from the environment and it is likely likely that in the presence presence of of damage damage to to the theBG!SMA BG/SMA pathways, pathways, patients (Georgiou rely heavily heavily on on intact sensory/PMA sensory!PMA pathways pathways to to initiate submovements (Georgiou et al. al. 1993). 1993). This This view viewisissupported supported by by the the existing existing PD PD literature, literature, which which shows shows that a variety variety of of tasks, tasks, including including finger finger tapping, tapping, drawing movements, learning strategies, gies, set set shifting, shifting, and and walking, walking, improve improve in in response response to external cues (Georgiou et al. 1993, Buytenhuijs Buytenhuijs et et al. al. 1994, 1994, Fimm Fimm et al. 1994, 1994, Martin Martin et al. 1994, Morris et al. 1994). 1994, Phillips et al. 1994). New concepts of of vascular vascular HLGDs HLGDs

It is probable that an analogous situation situation may may exist exist in other higher-level higher-level gait disorespeciallyininthose thosepatients patientswith with 'parkinsonian' 'parkinsonian' type gait gait disorders disorders in cerebral ders, especially multi-infarct states states (CMIS). (CMIS). It is possible caused by multi-infarct possible that that this this is caused by infarction infarction in in the SMA BG or indeed in in SMA or or its its connections connections via via the the periventricular periventricular white matter to the BG BG itself. itself. This Meyer and Barron's Barron's (1960) (1960) original original concept concept of gait gait the BG This supports supports Meyer apraxia apraxia being being caused caused by by any any mesial mesial frontal frontal lesion, lesion,which whichisisthe the anatomical anatomical site siteof of SMA. Further is gained from current evidence evidence suggesting that damage the SMA. Further support is (periventricular white leucoaraiosis detected detected on magnetic magnetic resoreso(periventricular white matter lesions or leucoaraiosis critical pathways linking the BG to nance scanning) to critical to the the ventrolateral ventrolateral nucleus of and to to the the SMA SMA leads leads to to abnormal abnormal gait gait in in early early vascular vascular dementia dementia the thalamus and (Hennerici et al. 1994). Chang et al. (1992) (1992) also also found found an association between vascular pseudoparkinsonism and and frontal frontal or orBG BG infarcts infarcts or or leucoaraiosis. leucoaraiosis. These critically timing cues cues from from the the BG BG as happens in in cally placed placed infarcts infarcts presumably presumably disrupt disrupt the timing and may may therefore therefore be be expected expected to to cause cause similar similar gait gait abnormalities. abnormalities. Some Some PD and vascular HLGDs HLGDs find find walking walking easier external patients with vascular easier when when responding responding to external cues, (Wright 1979, 1979, cues, e.g. e.g. stepping stepping over over objects objectsor orcoloured coloured patterns patterns on on the ground (Wright et al. al. 1992), 1992), as as is is commonly commonly seen seen in in PD PD(Bagley (Bagley etet al. al.1991, 1991,Weissenborn Weissenborn Jantra et 1993). ItIt may context of CMIS, that virtual reality reality 1993). may be be relevant, relevant, therefore, therefore, in in the the context

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images simulating 'obstacles' in the patients' patients' physical physical world have been shown to be an effective form of of rehabilitation rehabilitation in PD (Weghorst Marsden (1989) effective form (Weghorst et al. 1995). Marsden (1989) has hypothesized that the the success success of these treatments involving involving external cues may to input input feeding feeding directly directly into into the the PMA PMA from from the thesensory sensory cortex, cortex,bypassing bypassing be due to the damaged or deafferented deafferented SMA, SMA, as already already stated above. above. However, significant number of do not have However, aa significant of patients patients with withvascular vascular }-ILGDs HLGDs do have movement movement ignition and timing problems, but but present present with with disequilibrium disequilibrium as as their their primary complaint. complaint. This This is is clearly consistent primary consistent with with both the original descriptions of gait apraxia and isis reflected reflected in in the the classification classification of gait apraxia and of vascular parkinsonism and et al. al. (1993), where feature in in three three of of the the five five Nutt et where disequilibrium disequilibrium is a prominent feature of disordered disordered gait gait described: described: frontal frontal gait disorder, frontal frontal disequilibrium patterns of disequilibrium. ItIt is is possible that these patients have a primary and subcortical disequilibrium. primary dissensory/PMA pathways and while they have order in their sensory!PMA have no no difficulties difficultieswith with autoautomatic internally cued movements, movements, since since the the BG!SMA BG/SMA pathways pathways are are intact, they are unable fully to including proprounable fully to utilize utilize sensory sensory information information from from the environment, including prioceptive, auditory, vestibular vestibular and visual visual information information to help initiate and control submovements. extent 'walking 'walking around the dark', dark', having having submovements. They They are are to to some some extent around in the difficulty integrating movement sequences; sequences; hence difficulty integrating external external information information into their movement hence their disequilibrium. disequilibrium. The The dependency dependency of of equilibrium equilibrium on visual visual cues cues isis supported supported by studies in normal individuals that show that vision influences influences the the amplitude amplitude and body sway sway during during quiet quiet stance stance (Day (Day et et al. al. 1993). In contrast with patients patients velocity of ofbody whose predominant feature is gait gait ignition ignition failure, failure, these whose predominant feature these patients patients would would not not be expected to benefit cues. expected benefit from from environmental cues,

Classification based based on the above hypotheses We which would would link We would suggest the following classification classification of vascular HLGDs, which

the clinical clinical features features with with the the putative putative mechanisms mechanisms of of normal normal gait gait and and the the location location and meaningful meaningful way way (see (see Table Table 6.2). of pathological pathological damage in a transparent and Ignition apraxia with movement Patients with vascular vascular HLGDs HLGDs with movement ignition ignition difficulties, difficulties, such as as gait gait ignition failure, shuffling, shuffling, and difficulty difficulty with turns and and freezing freezing have infarcts in the BG/thalamus/SMA BG!thalamus/SMA pathways pathwaysand/or and/or ischaemic ischaemic lesions lesionsinintheir their connections connections in in the matter (Fitzgerald (Fitzgerald and and Jankovic Jankovic 1989). 1989). These patients have have periventricular white matter difficulties with movements. We We further hypothesize difficulties with internally internally driven driven automatic automatic movements, further hypothesize that these patients' gait gait characteristics characteristics should improve improve when when externally externally cued. We We suggest suggest this this gait gait disorder disorder be be named named ignition apraxia. This group will include include isolated impairment of gait gait ignition ignition and and possibly possibly some cases of'cautious impairment of of 'cautious gait' from from the classification of (1993). However, However, the latter is a very wide classification of Nutt Nutt et al. (1993), wide and probably

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Table 6.2 Classification Classification of of vascular vascular HLGDs HLGDs

Ability to alter Response cadence in to visual response to

Clinical features

cues

auditory cues

Site of lesion

Gait ignition failure, failure, shuffling, freezing shuffling, freezing

Yes Yes

Yes Yes

SMA, BG or connections

Equilibrium apraxia apraxia Poor balance and and falls falls

No

No

PMA or connections

Gait ignition failure, failure, shufffing, freezing. shuffling, Poor balance and and falls falls

Yes Yes

Yes Yes

PMA or connections and SMA, BG BG or or and SMA, connections

Ignition apraxia apraxia

Mixed gait apraxia

unhelpful unhelpful category category and encompasses encompasses patients whose gaits are hesitant for for aa wide wide variety of reasons including visual impairment, impairment, falls falls and and loss loss of of confidence. confidence. Equilibrium apraxia We hypothesize hypothesize that patients with with vascular vascular HLGDs HLGDs causing causing disequilibrium disequilibrium have have

infarcts in the the sensory/PMA sensory/PMA pathways pathways and/or and/or ischaemic ischaemic lesions lesionsin intheir their connections connections infarcts in the periventricular white white matter. matter. These These patients patients will will have have difficulties difficulties primarily with the automatic with externally externally cued cued movements; movements; the automatic internal internal cueing cueing mechanism mechanism isis cued. We suggest normal. The patients' improve when externally cued. patients' gait should not improve suggest that that apraxia. This group includes subcortical this gait disorder be named equilibrium apraxia. disequilibrium and frontal frontal disequilibrium disequilibrium in inthe theclassification classification of of Nutt Nutt et et al. al. (1993). Mixed gait apraxia lesions may may affect affectthe the connections connections of of both the SMA Clearly lesions SMA and PMA and it is is to be expected that there there will will be be patients patients with with both both disequilibrium disequilibrium and and gait gait ignition ignition expected that difficulties.We Wesuggest suggestthat thatthis this gait gait disorder disorder be be named mixed difficulties. mixed gait gait apraxia. apraxia. The The clinical features featuresseem seemtotocorrespond correspond to to frontal frontal gait gait disorder disorder in in the the classification classification of of clinical Nuttetal. Nutt etal.(1993). (1993).

Reduction to two two types types (plus (plus aa mixed mixed type) Reduction of of the the classification classification of of vascular vascular }-ILGDs HLGDs to makes it possible possible to to relate relate the the problem problem to putative underlying physiological physiological mechanisms, in turn related related to to damage damage at at anatomically anatomically distinct distinct sites. sites. The The validity of this anatomically classification will anatomically underpinned underpinned classification will need need to to be be tested tested with with blind blind interpreof patients with with vascular vascular HLGDs, HLGDs, with tation of neuroimaging ofpatients with particular particular attention SMA, PMA PMA and periventricular periventricular white matter. Further Further correlations correlations may may be to SMA, white matter. the responsiveness responsiveness (or lack lack of responsiveness) responsiveness) of external sought with the of the gait to external

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Gait apraxia apraxia and and multi-infarct multi-infarct states Gait cues. The outcomes of uot only be of these studies will not

of theoretical iuterest: interest: a clearer understanding of of the underlying underlying pathophysiology pathophysiology of the different different types of vascuvascular HLGDs will create create aa better better framework for rational rational therapeutic interventions HLGDs will framework for interventions et al. al. 1997). 1997). (Mickelborough et Towards aa rational therapy The standard treatment treatment for for these these gait gait disorders disorders centres centres on physiotherapist adminadministered gait gait retraining. retraining. The The elements have not not been istered elements of such such treatment, however, however, have clearly documented, have their clearly documented, nor have their relationship to the underlying pathophysiological clearly defined. ical mechanisms mechanisms been clearly defined. Edwards Edwards et et al. al. (1990) (1990) have haveemphasized emphasized that that such process is an essential essential precursor to to developing developing and and evaluating evaluating therapies. therapies. such a process Using that used used above above in in describing describing our our classification classification of Using similar similar thinking thinking to to that of HLGDs, HLGDs, we we have have designed designed aa comprehensive comprehensive treatment treatment schedule for these gait disorders, with of 31 31 possible possible interventions interventions (Mickelborough (Mickelborough et 1997). In orders, with a total of et al. al. 1997). In developing the developing the schedule schedule we we have have drawn drawn on on recognized recognized interventions interventions for for the the gait initiation failure failure seen seen in in PD PD and and the the disequilibrium disequilibrium seen seen in in stroke stroke and and also also on on the the by Edwards Edwards et al. (1990) approaches recommended by (1990) in in standardizing standardizing physiotherapy schedules for research. Essentially, Essentially, we that vascular vascular HLGDs HLGDs will will be we have have postulated postulated that amenable to amenable to physiotherapeutic physiotherapeutic interventions interventions under under three broad headings and we call these call these the the 'modules 'modules of of intervention'. intervention'. The The treatment treatment schedule modules are: 11 Physiotherapeutic Physiotherapeutic interventions interventions to to treat treat gait gait initiation initiation and and turning turningdifficulties difficulties (5 (5 exercises) to improve 2 Physiotherapeutic Physiotherapeutic interventions interventions to improve postural postural alignment alignment and and enhance enhance balance reactions (16 (16 exercises) exercises) 3 Physiotherapeutic interventions aimed aimed atat the the other components Physiotherapeutic interventions components of of vascular vascular HLGDs (10 (10 exercises) exercises) Using the the schedule schedule interventions an individual patient requires will depend on the pattern The specific specific interventions pattern

be tailored tailored to to it. it. ItIt isis envisaged envisaged that all patients of their disordered gait and should be should initially be be assessed assessed and and treated treated within within each module. The choice of subseexercises will evaluation and continuing assessment. assessment. The quent exercises willthen then depend depend on on evaluation The reason for this is that most most cases cases of vascular HLGDs are mixed to some extent: even have elements elements of sub'pure' ignition ignition or or equilibrium equilibriumapraxias apraxiasmaywell may wellhave patients with 'pure' clinical balance problemsrespectively. respectively. clinical balance or ignition problems The efficacy of this this schedule schedule will will require require objective objective evaluation, evaluation, using simple clinefficacy of outcome measures as more complex measures, which might include ical outcome as well as more include gait gait analysis using footprint footprint data from an three dimensional video analanalysis using an inked inked walkway, walkway, three ysis, electrogoniometry accelerometry (Holden et et al. al. 1984, 1984, Waagfjord Waagfjord et al. al. ysis, electrogoniometry and accelerometry

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Wilkinson Wilkinson et al.1995). al.1995). These latter are are not not yet yetwidely widely available available (Bell (Bell et et al. al. 1996). important to to determine whether whether improvements seen seen on on more sophisti1996). ItIt isisimportant cated tests tests translate translate into functional cated functional improvement. improvement. We are currently currently using using such such methods to to evaluate evaluate this schedule, specifically specifically comparing methods comparing itit to to treadmill treadmill retraining. 1990, 1990,

Conclusion There is an increasing increasing awareness awareness that higher-level higher-level gait gait disorders, disorders, typically typically seen seen in in

PD, may with other conditions, may also also be associated associated with conditions, in in particular particular cerebral cerebral multimultiinfarct states. we set set out out the the evolution evolution of of understanding understanding of In this chapter, we of these gait disorders. We have suggested suggested that that the observed We have observed clinical clinical features features may be related related to to putative putative underlying anatomical with what is currently understood anatomical damage damage in accordance accordance with understood about the initiation and and maintenance maintenance of of gait. gait. We We have proposed a classification classification of of vascular HLGDs the main types of vascular HLGDs into into ignition ignition apraxia, apraxia, equilibrium equilibrium apraxia, apraxia, and mixed apraxia. This is a simplification simplification of the classification classification of Nutt et al. (1993) and explanations of of the gait gait disorder seen in PD. It builds on current explanations It is is postulated postulated that that ignition apraxia apraxia is is due due predominantly predominantly to to damage damage to to the the supplementary supplementary motor motor area area or to its connections connections with with the basal basal ganglia ganglia and and that that equilibrium equilibrium apraxia apraxia is is due due to to damage to the sensory/premotor area pathways. The correlative of this is that the damage to the area pathways. The correlative of this is that the former former will will respond respond well well to to external external cues cues (as (as the the major major problem is the difficulty difficulty in in internal cueing), and that the latter will not respond to external cues as the pathinternal cueing), and will not respond external cues as the pathways that ways that utilize such cues are damaged. We suggest suggest that this classification classification of the elements elements of of apraxia apraxia may may have have implicaimplications for the more precise targeting and tailoring of physiotherapy physiotherapy and other interventions for for patients with vascular vascular HLGDs. HLGDs. The therapeutic ventions The hypothesis hypothesis and and the therapeutic implications implications arising arising from from itit remains remains speculative speculativeatat present present and and needs needs to to be be supby future future research, research. ported by

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Marie PP (1901) lacunaires de désintégration desintégration etetdededifférentes (1901) Des Des foyers foyers lacunaires différentesautres autresétats étatscavitaires cavitairesdudu cerveau. RevueNeurologique, Neurologique, 281-98. cerveau. Revue 21,21, 281—98. Marsden Disorders,Vol. Vol. Marsden CD CD (1989) (1989)Slowness Slowness of of movement movementininParkinson's Parkinson'sdisease. disease.In: In:Movement MovementDisorders, 4, Suppl. 1, 1, eds. eds. Fahn Fahn SSand andMarsden MarsdenCD, CD,pp. pp.26—37. 26-37.New NewYork: York: Raven Raven Press. Press. Martin KE, KE, Phillips Phillips JG, JG, lansek R, R, Bradshaw Bradshaw JL (1994) Inaccuracy and instability instability of of sequential sequential movements in Research, 102,102, 131—40. movements in Parkinson's Parkinson'sdisease. disease.Experimental ExperimentalBrain Brain Research, 131-40.

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Mickelborough J,J, Liston V, Tallis Tallis RC (1997) Conventional physiotherapy physiotherapy Liston R, R, Harris Harris B, B,Pomeroy Pomeroy V,

of higher-level gait disorders higher-level gait disorders in patients patients with with cerebral cerebral multi-infarct multi-infarct states. states.Physiotherapy Physiotherapy Theory and 13,13, 127—38. Practice, 127-38. Theory andPractice, Morris ME, (1994) The pathogenesis of ME, lansek lansek R, R, Matyas Matyas TA, TA, Summers OJJ 0JJ (1994) of gait gait hypokinesia hypokinesia in in Parkinson's Parkinson'sdisease. disease.Brain, Brain,117, 117,1169—81. 1169-81. Nutt JG, JG, Marsden CD, CD, Thompson PD PD (1993) (1993) Human Humanwalking walkingand andhigher-level higher-level gait gait disorders, disorders, particularly ininthe 268—79. Neurology,43,43, 268-79. particularly theelderly. elderly.Neurology, KE, Bradshaw JL, disease Phillips JG, Martin KE, JL, lansek lansek R (1994) (1994) Could bradykinesia in Parkinson's disease simply of of Neurology, 241, 439—47. Journal Neurology, 241, 439-47. simply be be overcompensation. overcompensation.Journal Roland PE, Larsen B, B, Larsen LarsenNA, NA,Skinhoj SkinhojEF(1980) (1980)Supplementary Supplementarymotor motor area area and other cortical areas in organization of of Clinical Neurophysiology, 43,43, organization of of voluntary voluntarymovement movementininman. man.Journal Journal Clinical Neurophysiology, 118—36. 118-36. Thaler D, Chen YC, Nixon PD, Stern Stern CE, CE, Passingham Passingham RE RE(1995) (1995)The Thefunction function of of the the medial medialpreYC, Nixon premotor Brain Research, 102,102, 445—6. Experimental Brain Research, 445-6. motor cortex. cortex.1.1.Simple Simplelearned learnedmovements. movements.Experimental Thompson PD, PD, Marsden Marsden CD CD (1987) (1987) Gait Gait disorders disorders of of subcortical subcortical arteriosclerotic arteriosclerotic encephalopaencephalopa2, 2, 1—8. Disorders, 1-8. thy: Binswangers Binswangersdisease. disease.Movement MovementDisorders, Van Bogaert L, L, Martin Martin P (1929) Van Bogaert (1929) Sur deux deux signes signes due syndrome syndrome de de desquiibration desquilibration frontale: frontale: l'apraxie dela statique. Encep hale, 24,24, 11—18. l'apraxie de lamarche marcheetetl'atonie l'atonie statique. Encephale, 11-18. Waagfjord LevangiePD, PD,Certo CertoCME CME(1990) (1990)Effects Effectsofoftreadmill treadmilltraining trainingon on gait gait in in a hemiWaagfjörd J,J,Levangie paretic patient. 70,70, 549—58. PhysicalTherapy, Therapy, 549-58. paretic patient.Physical Weghorst SJ, SJ, Prothero Prothero J, Furness Furness T, T,Anson Anson D, D, Riess ifiessTT (1995) (1995)Virtual Virtual images imagesin inthe the treatment treatment of Parkinson's Virtual Reality Conference 11, 30, MedicineMeets Meets Virtual Reality Conference 11, 242—3. 30, 242-3. Parkinson'sdisease diseaseakinesia. akinesia.Medicine Weissenborn 5S (1993) The The effect effect of using aa two-step verbal verbal cue cue to to aavisual visual target target above above eye eye level on the 79,79, 26—31. the Parkinsonian Parkinsoniangait: gait:AAcase casestudy. study.Physiotherapy, Physiotherapy, 26-31. Wilkinson Menz HB, HB, Raspovic RaspovicAA (1995) (1995) The The measurement measurement of of gait gait parameters parameters from footWilkinson MJ, MJ, Menz footprints. TheFoot, Foot,5, 5, 84-90. prints. The 84—90. Wright WB WB (1979) (1979)Stammering Stammeringgait. gait.Age Ageand andAgeing, Ageing,8, 8, 8-12. Wright 8—12.

The epidemiology epidemiology of Parkinson's The Parkinson's disease disease and parkinsonism in elderly subjects subjects Jolyon Meara Meara and Peter Jolyon Peter Hobson Hobson

Introduction Parkinson's disease disease (PD) (PD) in in populations - the epidemiology of PD The study of Parkinson's is an arduous and and difficult difficult undertaking. undertaking.Descriptive Descriptive epidemiology epidemiology of of aa disease disease provides a picture of of the the prevalence prevalence — the theamount amountofofdisease diseasepresent presentinina agiven givenpopulapopulation; the incidence incidence - how frequently cases develop frequently new cases develop in in aa given given population population over time; the mortality mortality - the risk of death associated associated with the disease; and the natural natural history of disease is best measured by by incidence incidence as as prevaprevahistory of the disease. The risk of disease lence lence figures figures can can be be distorted distorted by by differential differential survival survival between between study study populations. populations. Analytical epidemiology investigates the factors, and Analytical epidemiology investigates the associations, associations, exposures, risk factors, comorbidities of a disease disease in an effort effort to to determine determine the theaetiology. aetiology. There There are are also also comorbidities increasing disability, handicap, increasing attempts attempts to describe the disability, handicap, quality quality of of life life and and health health specific diseases and social service service provision provision resulting from specific diseases (see (see Chapter Chapter 8). The major major causes causes of of neurodegenerative neurodegenerative disease, disease, Alzheimer's Alzheimer's disease disease (AD), (AD), PD PD neurone disease disease (MND) (MND) all all share share aa strong strong age age associated associated risk and motor neurone risk and are likely likely to to be be an an issue issueof ofincreasing increasingpublic publichealth health concern concern as as aa result result of of the the worldwide worldwide populations.Certified Certified deaths deathsfrom from AD, AD, PD PD and and MND MND have have been projected projected aging of ofpopulations. to overtake cancer as the second cause of death in in the the USA USA by the year second commonest cause year 2040 (Lilienfield (Lilienfieldetal. 1990). et al. 1990). major difficulty in interpreting interpreting the many and varied epidemiological A major difficulty in epidemiological studies studies PD is is aa relatively relatively rare disorder, the diagnosis diagnosis of which, for practical practical of PD is that PD purposes, relies relies solely solely on the clinical clinical skills of history taking and bedside bedside examinaexaminapurposes, tion. Diagnostic accuracy for for PD, PD, particularly particularly in in older older subjects, subjects, isispoor poor (Hughes (Hughes et et PD is is strictly a single disease or is is better al. 1992). There There is also also debate debate on whether PD termed aa syndrome caused by several several diseases diseases(Duvoisin (Duvoisin 1989). 1989). The The studies studies of of autoautosomal familial parkinsonism indicate that differing differing neuropathology can somal dominant familial cause identical identical clinical clinical pictures pictures between between pedigrees pedigreesand and that that identical identical pathology pathology can can within aasingle single family. family. Disagreement even even exists exists cause varied clinical clinical presentations within as to to what what constitutes constitutes the typical typical pathology pathology of PD PD and there is a pressing pressing need need for for more detailed and extensive extensive prospective clinicopathological clinicopathological cohort studies. studies. These These 111 111

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Table 7.1 Key Keyareas areastotoaddress addressininthe theevaluation evaluationof of epidemiological epidemiological studies in

parkinsonism for the diagnosis diagnosis of of parkinsonism parkinsonism and the method of •• The criteria used for of establishing establishing diagnosis (case note note review, review,screening screeningquestionnaire, questionnaire, or or bedside bedside examination) (case of the the population population studied • The characteristics characteristics of • The methods of of case case ascertainment used used (medical (medical records, records, drug drug prescription, prescription, recall recall by PD will will be be medically medically physicians, total census approach). approach). Many Many prevalent prevalent cases cases of of PD at the time of of the study study and and some some medically medically known cases cases will undiagnosed at willnot not be be on on drug drug treatment or or be be under under medical medical review review of vascular vascularparkinsonism, parkinsonism, and and drug-induced • The inclusion or exclusion exclusion of cases of parkinsonism elderly subjects subjectsininresidential residentialhomes, homes,nursing nursinghomes homesor or other other • The inclusion or exclusion exclusion of elderly long-term care institutions subjects with dementia • The inclusion or or exclusion exclusion of of elderly subjects

factors complicate the the interpretation of factors can considerably considerably complicate of epidemiological epidemiological studies in PD. Certain aspects aspects of of such studies studies need need to to be be critically critically evaluated evaluated (see (see Table Table 7.1). Prevalence PD affects affects all allracial racialgroups groupsand andhas hasaafairly fairlyuniform uniformworldwide worldwidedistribution, distribution, though

prevalence in Africa, Africa, China reduced (Zhang (Zhang and and Roman Roman 1993). 1993). the prevalence China and and Japan is reduced Differences Differences in in prevalence prevalencebetween between racial racial groups groups may may reflect reflectdiffering differing environmenenvironmenrather than than genetic genetic factors factors (Schoenberg (Schoenberg et et al. al. 1988, 1988, Jendroska Jendroska et et al. al. 1994). 1994). tal rather Prevalence Prevalence differences differencesbetween betweenstudies, studies,even evenafter afteradjusting adjustingtoto aastandard standard populamay be be artefacts artefacts of of diagnosis diagnosis and andsurvival. survival. Despite Despite differences differences in in absolute absolute tion, may prevalence between also prevalence between countries, countries, disease disease risk risk may may be be similar similar ifif mortality rates also vary. Prevalence rates for PD in subjects over the age of 65 years years appear appear to be similar across (de Rijk Rijk et al, al. 1997), 1997). This study, based on 14636 14636 across several several European European countries (de individuals over the age age of of 65 65 years yearsininfour four countries countries using using aa total total census census approach or stratified stratified random samples, reported aa prevalence of 2.3% for for parkinsonism and 1.6% for PD, age age adjusted 1991 European European standard standard population. population. Studies Studies 1.6% for PD, adjusted to to the 1991 around world based based on all all ages ages suggest suggest aa prevalence prevalence for around around the the world for PD PD of of around 120/100000 120/100 000population. population. PD PD shows shows aa strong strong exponential exponential age age associated associated risk risk that that appears maintained even even in in extreme extreme old old age age (Ben-Shlomo (Ben-Shlomo 1996). 1996). PD is is appears to to be maintained slightly more men than than in inwomen, women, which which may may reflect reflect differences differences in slightly more common common in men occupational exposure to environmental agents agents causing causing PD.

prevalence studies studies using using aa total total census censusapproach, approach, up up to to aathird third or or more more of of subsubIn prevalence jects ascertained ascertained as having PD were were medically medically undiagnosed undiagnosed before before the study, even even

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countrieswith withwell welldeveloped developed health services (Schoenberg et al.Morgante 1985, Morgante in countries health services (Schoenberg et al. 1985, et al. 1992, de Rijk Rijk et al. 1997). A A study study of all residents over the age age of 65 65 years in a US retirement retirement town revealed prevalence of of known known PD PD of of 2% 2% and and medically US revealed a prevalence medically undiagnosed PD (Khatter et et al. al. 1996). 1996). Undiagnosed, Undiagnosed, but clinically clinically symptosymptodiagnosed PD of 5% (Khatter matic, parkinsonism 1% of older older subjects subjects in general general practice in the the parkinsonism was found found in 1% UK (Meara (Meara et al. 1997). 1997). Drug Drug prescription prescription for PD appears appears to be an an unreliable unreliable UK for PD method of of case case ascertainment used alone, alone, as many subjects subjects in receipt of medication will will not have have PD PD or or even even parkinsonism parkinsonism (Meara (Meara et et al. al. 1999) 1999) and and aa proportion proportion of known cases of PD, PD, likely likelytotobe beelderly elderlyand andfrail frailor orwith withminimal minimal symptoms, symptoms, will not be on active drug treatment (Morgante (Morgante et et al. al. 1992). 1992). Incidence Changes in disease disease incidence incidence provide aa powerful powerful tool tool to to investigate investigate disease disease risk, risk,

disease associations and disease aetiology. Unfortunately, Unfortunately, incidence studies of disdisease associations disease aetiology. eases that are are relatively relativelyinfrequent infrequentrequire requirelong-term long-term follow-up follow-up of ofpopulations populations and and eases that must achieve high levels levelsof ofdisease diseaseascertainment. ascertainment. In In PD PDthe thebest bestlong-term long-term data on incidence come from from the the longitudinal longitudinal study study from from Rochester, Rochester, Minnesota Minnesota incidence has has come (Rajput et al. al. 1984). 1984). These studies indicate aa relatively relatively unchanging age adjusted adjusted (Rajput unchanging age incidence for PD of of around around 20/100000 20/100000population populationbetween betweenthe theyears years1967—1979. 1967-1979. However, overall incidence, an increasing incidence for disease disease However, despite despite aa constant constant overall in older subjects and a falling falling incidence in younger subjects may have have occurred occurred over this time (Ben-Shlomo 1996). 1996). Mortality

Several studies from around the the world world seem seem to indicate that mortality mortality isis still still at least doubled in PD PD despite despite drug drug treatment treatment(Hoehn (Hoehnand andYahr Yahr1967, 1967,Ebmeier Ebmeieretetal. al.1990, 1990, and that age specific specific mortality mortality rates rates appear appear to to be be increasing increasing in in Bennett et al. 1996) 1996) and those over 75 years (Lilienfeld et al. 1990, Clarke years old old and and falling falling in in younger younger patients (Lilienfeld Clarke 1993, 1993, for for detailed detailed review review see seeBen-Shlomo Ben-Shlomo 1996). 1996).One One reason reason for for this this finding, finding, and there are several several competing competing explanations, explanations, is isthat that the the incidence incidence of of PD/parkinsonism PD/parkinsonism in older subjects may maybe be increasing.

Long-term care is likely likely to to be be particularly particularly prevalent prevalent in in long-term care Parkinsonism is care institutions institutions such as hospitals, hospitals, nursing homes and residential/retirement such residential/retirement homes homes (Moghal (Moghal et et al. al. 1995). group. A study 1995). Few Fewstudies studies have havebeen beenspecifically specificallytargeted targetedatatthis thispopulation population group. in United States States of over over 5000 5000 nursing nursing home home residents residents over age of 55 in the United over the the age prevalence for for medically medically diagnosed PD PD of of nearly nearly 7% 7% (Mitchell (Mitchell et et years reported aa prevalence al. 1996), 1996). In study nursing nursing home residents with more al. In this this study home residents with PD PD tended tended to to be be more

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disorientated, more depressed, more functionally disabled disabled and to deteriorate deteriorate faster faster over an an 18 18 month month period period than residents without PD. Hallucinations and dementia over dementia appear to to be be two two major major factors factors increasing increasing the the risk risk of of admission admission to to nursing nursing homes homes appear of elderly people (Goetz and Stebbins Stebbins 1993, 1993, 1995). 1995). Nursing home home resiresiof elderly people with with PD (Goetz dents who develop after admission to to the the nursing nursing home homeare areless less likely develop parkinsonism parkinsonism after assessed and cases to be diagnosed, assessed and treated. treated, One study in France found that 42% of cases of PD elderly subjects PD in elderly subjects living living in in institutions institutions were were medically medically undiagnosed undiagnosed (Tison institutions et al. 1994). 1994). In In this this study, study, out out of a sample sample group of 357 people living in institutions 63 63 subjects subjects (18%) (18%) were were found found to to have have parkinsonism parkinsonism with with 36 36 of of this this group group (57%) as PD. We have problem by by asking asking nursing nursing officers officers being diagnosed as have approached approached the problem residents with known PD and also also residents residents with in nursing homes to report residents with both and shuffling shuffling gait. Out of of aa study study group group of of 2005 2005 nursing nursing home home residents, residents, tremor and was reported possible PD known PD was reported to be present in 6.8% and possible PD (tremor plus gait disturbance) was was reported reported in 2% of of residents residents (unpublished (unpublished observations). Further data define the parkinsonism in nursing nursing data are are required required to define the extent extent of of the the problem problem of parkinsonism homes and the extent extent to to which which diagnosis, diagnosis, assessment assessment and reduce homes and treatment treatment can reduce improve quality quality of of life life in this frail frail and vulnerable group (Larsen (Larsen et handicap and improve al. 1991).

Risk factors factors for PD Risk PD The contribution of of epidemiology epidemiology to to our our understanding understandingofofrisk riskfactors factors for for PD PD has has

been disappointing. The existence existence of autosomal dominant familial familial parkinsonism parkinsonism clearly indicates indicates that that Lewy-body Lewy-body type type pathology pathology can have a completely completely genetic genetic basis basis clearly (Golbe et al. 1993). 1993). However, However, the balance of environmental environmental and and genetic genetic factors factors (Golbe the balance determining the risk risk of of sporadic sporadic PD PD isis unclear unclear (see (see Fig. Fig. 7.1). 7.1). Molecular biology is likely to the study study of of regularegulalikely to make make an an important important contribution to this area through the (Bandmann et et al. al. 1998). 1998). Exploring Exploringthe of environenvirontory gene products (Bandmann the contribution contribution of and genetic genetic inheritance may may be helped by the study of migrant populations populations ment and et al. al. 1988). 1988). (Schoenberg et Aging and the risk risk of of PD PD

have clearly clearly shown shown that the risk Prevalence studies have risk of of PD PD is is powerfully powerfully associated associated this risk risk appears appears to to fall fall in advanced age. age. This may maybe with age, though this be explained by difficulty detecting and diagnosing diagnosing PD PD in in very very elderly elderly subjects subjects and, and, given given the the the difficulty small absolute absolute numbers, confidence intervals this extreme extreme of age. age. small numbers, confidence intervals are are wide wide at at this Studies have included elderly subjects Studies using using population population methodology that have subjects in in instiinsticare have have shown that the the risk risk of of PD PD increases increases continually continually with age age (Tison (Tison tutional care et al. 1994). The aging and PD may may be explained explained by age age related cell The link link between between aging death, intrinsic aging processes processes in in the the brain brain increasing increasing the the susceptibility susceptibility to to PD, PD, or or

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Age +++ +++

Family history in first

Head injury (?)

degree relatives ++ Rural residence (?)

Protective effect of smoking ++

Protective effect of dietary antioxidant

Pesticide exposure ++

vitamins (?) (?)

Age related cell cell death death (?)

Fig. Fig. 7.1 7.1

Risk factors factors for Parkinson's Parkinson's disease. disease.

by increasing the length of exposure to exogenous or endogenous causative causative agents.

Most PD to to be be slightly slightly Most prevalence prevalence and and case-control case-control studies studies indicate indicate the the risk risk of PD increased for the male male sex. sex.

Environmental, lifestyle and life event risk factors for for PD PD Environmental factors, associated comorbidities and and lifestyle lifestyle have been implicated in the aetiology of PD and can can be examined examined by by epidemiological epidemiological techniques. Large Large scale cohort cohort population studies scale studies can can investigate investigate risk factors with the greatest greatest degree degree are time time consuming consuming and andexpensive expensive to to execute, execute, particularly particularly given given of certainty, but are the relative relative rarity rarity of of PD PD in in most age age groups. groups. Case-control Case-control studies, where where individuals with disease are cases without the the als with the index disease are matched matched with with one one or two control cases can more more quickly quickly investigate investigate hypotheses of of disease disease disease, are are easier easier to to mount mount and can causation. Case-control subject to many sources of bias and conconcausation. Case-control studies studies can can be be subject founding can make make interpretation of of results results quite quite difficult. difficult. Problems include founding that can the definition definition of cases, cases, the the selection selection of of controls, controls, the the degree degree of of matching matching that that takes takes importantly, recall recall bias bias of of patients patients with with aa disease disease compared to to subsubplace and most importantly, disease. The diagnosis of PD will jects without aa disease. will make make an an individual peer back anxiously into the past to to try try to to find find some some explanation explanation and will will increase increasethe the recollection recollection of exposures exposures to to chemicals chemicals and and other other events. events. The The manner manner in which which interviews interviews with with cases and information can can also also bias bias the the cases and controls controls are are conducted conducted in in order to obtain information results if the interviewer is aware of the aetiological hypotheses being tested by the aware of the aetiological hypotheses being tested by the

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study. aetiology such such as as infection, infection, head head injury, injury, and toxin expostudy. Theories of disease aetiology

sure can be examined in matched groupings of Cell death death in in the of cases and controls. Cell substantia substantia nigra in in PD PD appears appears to to develop develop rapidly rapidly over over the six six years years or so before before clinical be clinical signs signs of of disease, disease,indicating indicatingthat that exposures exposures at at or before before this time might be particularly relevant factors. However, latent period period particularly relevant as as risk risk factors. However,the the length length of of the latent disease development between a 'permissive' exposure exposure and disease development is unknown. The most robust epidemiological The epidemiological finding, replicated replicated in several several population and and develop PD case-control studies, has been that people people who who smoke smoke are are less less likely likely to develop (Doll et al. al. 1994). Whether this this is is due due to to a protective protective effect effect of of smoking, smoking, or or to to the the (Doll et 1994). Whether existence PD personality personalitythat thatmakes makessmoking smokingless lesslikely, likely, isis unclear. unclear. existence of of a premorbid premorbid PD al. 1983), 1983), induced MPTP MPTP parkinsonism parkinsonism (Langston (Langston etet al. The description of neurotoxin induced coupled free radical theory of cell cell death in PD, resulted in several studies coupled with the free examining PD. The The best best case-control case-control studies studies examining environmental environmentalrisk risk factors factors for for PD. (Semchuk et al. 1991, 1991, 1992) 1992) suggest and aapositive positive (Semchuk suggest aa link link between between the the risk of PD and family occupational pesticide pesticide use. use. family history history of of PD, past past significant significant head head injury and occupational sporadic cases cases of PD. Apart However, such factors factors may explain explain only about about a third of sporadic studies have have reported reported variable variable from a general link to rural living, other case-control studies have not defined defined risk risk factors factors any any more more precisely. precisely. findings and have Cohort studies avoid studies, though though the relaavoid much of the bias of case-control case-control studies, tive rarity of PD and the tive rarity the fact fact that that ititdevelops develops late late in in life life requires requires the the long-term long-term follow-up of large groups of subjects. subjects. The The incidence incidence rate of of PD PD can can be be measured measured in groups some exposure exposure felt felt to be be relevant relevant to to the the risk risk of of developdevelopgroups with with and without some ing increased risk of ing PD. PD, One One small small cohort cohort study study of of head head injury injury did did not detect an increased (Williams et al. 1991) 1991) and a further large study of dietary antioxisubsequent PD (Williams dant vitamins indicated a protective protective effect effect of of vitamin vitamin C, C, though though not of vitamin E 1994). Further hypotheses hypotheses generated generated by basic medical science (Cerhan et al. 1994). science need need developed concerning the aetiology aetiology of appropriate to be developed of PD PD that can be tested in appropriate cohort and case-control epidemiological studies. At At present present the the analytical analytical epidemepidemiology iology of of PD awaits awaits new theories to test. Genetic risk of PD PD

have recentlybeen recently been made made in in genetic genetic studies studies of of parkinsonism, parkinsonism, Considerable advances have

particularly in relation particularly in relation to to defining defining the the genetic genetic basis basis of of familial familial parkinsonism parkinsonism monozygotic (Golbe et al. 1993) 1993) and and the re-analysis re-analysis of of concordance rates for PD in monozygotic and dizygotic twin studies (Johnson et et al. al. 1990). 1990). The balance between genetic predisposing causative factors environmental exposure sporadic PD PD disposing and and causative factors and and environmental exposure in in sporadic remains determined (Hawkes (Hawkes 1997, 1997, Bandmann Bandmann et et al. al. 1998). 1998). Considerable Considerable remains to be determined interest exists exists in the genetic genetic basis basis of of neurodegenerative neurodegenerative mechanisms mechanisms that could could interest account PD (Fahn et al. al. 1998). 1998). Studies Studies of familial parkinsonism large account for for PD of familial parkinsonism in in the the large Contursi kindred led to the detection detection of of aa gene gene defect defect segregating segregating to to chromosome

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Epidemiology 4q21—22 that encodes for a cytoplasmic protein called 4q21-22 (Polymeroponlos (Polymeropoulos et et al. al. 1996). 1996).

alpha-synnclein alpha-synuclein

The epidemiology of parkinsonism parkinsonism not not due due to to PD PD

prevalence of of parkinsonism parkinsonism in elderly subjects snbjects may may be be much mnch higher than preThe prevalence previously thought although overall overall PD PD acconnts accounts for for aronnd around 70% 70% of of all all cases cases of vionsly thonght and althongh medically figure will medically diagnosed diagnosed parkinsonism, parkinsonism, this fignre willbe belower lowerininolder olderpopulations popnlations in in whom other other canses causes of parkinsonism parkinsonism are are more more common. common. How How mnch much lower lower has has whom never never been been determined. determined. One retrospective retrospective case casenote notestudy stndyhas hasreported reported that that up np to to of all all snbjects subjects over the age age of 65 years may have a third of have evidence evidence of of parkinsonism, though definite diagnosis be made in only around 10% of this gronp group thongh a definite diagnosis of of PD PD could conldbe aronnd 10% (Bennett et al. 1996). The The diagnosis diagnosis of of parkinsonism parkinsonism was was based based on on the the finding finding of of two following: bradykinesia, tremor. two or more of the following: bradykinesia, gait gait disturbance, distnrbance, rigidity and tremor. in this stndy study may may have A possible reason for such snch a high high prevalence prevalence of of parkinsonism parkinsonism in because of the freqnency frequency of postural in elderly elderly snbjects. subjects. Although been becanse postnral tremor in Althongh the likeliest essential tremor, methodology likeliest cause canse of of such snch tremor tremor would wonld be be essential tremor, the the study stndy methodology would have counted such tremor tremor as as a parkinsonian sign. A community total censns census wonld connted snch parkinsonian sign. commnnity total 'door-to-door' stndy study of elderly elderly subjects 'door-to-door' snbjects reported reported finding finding tremor tremor in in 43% 43% of of the study stndy group gronp and half of these individuals individnals were diagnosed as having having essential essential tremor tremor (Khatter et al. al. 1996). 1996). We have have studied prevalence of medically medically nndiagnosed undiagnosed (Khatter stndied the prevalence tremor in in general general practice in the UK based based on the use nse of a screening screening questionnaire qnestionnaire 12% of to subjects snbjects over over the the age age of of 65 65 years yearsand and found fonnd that that tremor was reported by 12% the study (Meara et et al. al. 1997). 1997). Essential stndy group (Meara Essential tremor tremor was was diagnosed diagnosed in in half half of of this this group. Very little little isis known known of of the the epidemiology of parkinsonism parkinsonismnot not due due to to PD in Very epidemiology of elderly people. Multisystem Multisystem degenerative diseases, such such as as multiple multiple system system atrophy (MSA) and parkinson(MSA) and progressive progressive supranuclear supranuclear palsy palsy (PSP), (PSP), that that can can present as parkinsonism, are the onset onset of of MSA MSA is uncomare much much rarer disorders than PD. Furthermore, the mon after the age of of 70 70 years, years,though though this this may may reflect reflectthe thepoor poor prognosis prognosis of of patients late onset onset disease disease (Wenning (Wenning et et al. al. 1994). 1994). The The epidemiological epidemiological study study of of PSP PSP is is with late made difficult by the fact that possibly half the course course of of the the disease disease may may pass before it becomes clinically from rapidly progressive progressive becomes possible possible to to distinguish this disorder clinically PD. The and has has The epidemiology epidemiology of of vascular vascular parkinsonism parkinsonism is also also poorly poorly understood understood and not been studied studied systematically systematically in elderly elderly populations (Critchley (Critchley 1981, 1981, Fitzgerald Fitzgerald and Jankovic 1989, Fenelon Fenelon et al. al, 1995). Elderly Elderly people people appear appear to be more susceptible to drug-induced parkinsonism parkinsonism (see (see Chapter Chapter 4) 4) and and are are also also much much more morelikely likely than younger people to be prescribed prescribed neuroleptic neuroleptic drugs. The The relationship relationship between between AD and extrapyramidal extrapyramidal signs signs is intriguing intriguing and some some cases cases of parkinsonism parkinsonism in in AD elderly subjects (Hamill et al. al. 1988, 1988, Hulette Hulette et et al. al. 1995). 1995). elderly subjects may may result result from from AD AD (Hamill

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elderly subjects subjectswith with extrapyramidal extrapyramidal signs signs appear appear to to have have higher higher mormorInterestingly, elderly tality and to be be at at greater greater risk risk of of developing developing cognitive cognitive impairment than than subjects subjects without such such signs signs (Richards (Richards etet al. al. 1993, 1993, Bennett Bennett et et al. al. 1996). 1996). Conclusion The increasing increasing number number of descriptive studies of of PD PD in various descriptive epidemiological epidemiological studies various

populations indicate indicate that that the the prevalence prevalence of of PD PD isisremarkably remarkably similar similar worldwide worldwide with with the exception exception of certain certain racial racial groups in in whom whom the the risk risk of ofdeveloping developing PD PD appears differences in appears reduced. reduced. Apparent Apparent differences in reported reported prevalence prevalence rates rates tend tend to disaponce allowance allowance for for differing differing methodologies methodologies and population structure structure are are pear once and population made. The risk of developing PD is is strongly related to to age. age. ItIt is is unlikely that age age related related cell death death in in the the brain is sufficient sufficient alone alone to to result result in PD and the effect age more cell effect of age likely reflects reflects the the impact impact of of disease, disease, genetic genetic factors, factors, and and exogenous exogenous and and endogenous endogenous environmental agents. agents. The The most most powerful powerful risk risk factor factor for for PD PD after after age age itself is aa pospositive genetic studies studies itive family family history history of of the disease. Exciting Exciting recent recent developments in genetic of familial familial parkinsonism parkinsonism may in time help elucidate elucidate the the pathophysiological pathophysiological genetic mechanisms cases of PD. A mechanisms behind behind some some cases of sporadic sporadic PD. A multifactorial multifactorial basis basis for for most most cases cases of of sporadic sporadic PD seems seems likely, likely,based basedon onthe the findings findings of of analytical analytical epidemiologcase-control designs. designs. ical studies studies using cohort and case-control Parkinsonism as a syndrome syndrome appears appears to to be be far far more more common in elderly people people Parkinsonism than previously previously thought and and results results in in considerable considerable misdiagnosis, misdiagnosis, inappropriate treatment and and missed missed therapeutic therapeutic opportunities. opportunities.In Inelderly elderly subjects subjects there there appears appears PD, AD, MND and dementia dementia with with Lewy Lewy bodies to be considerable overlap between PD, suggests common genetic genetic and environmental environmental factors. factors. that suggests

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Health and social needs needs of of people people with Parkinson's disease and and the the worldwide worldwide Parkinson's disease organization of their care care Peter Peter Hobson Hobson

Introduction Trying to unravel the complex complex health health needs needs of of elderly elderly people people is is aa major major task task faced faced by health health care care systems systemsthroughout throughout the the world, world, made made all all the the more more important important by the fact fact that that this this group is aa disproportionately disproportionately high consumer of health care resources. resources. societies, but also also developing countries, are are facing a demoNot only industrialized societies, graphic challenge as graphic challenge as life life expectancy expectancyincreases. increases.The Thehealth health needs needs of of this this group group are complex variable, reflecting reflecting the extreme extreme heterogeneity heterogeneity of of elderly elderly people. people. complex and and variable, However, reflecting and However, all alltoo too often often care care tends tends to to be be fragmented fragmented and haphazard, reflecting ageist values. values. reinforcing ageist The health and social needs of elderly patients with Parkinson's disease (PD) can can be analysed in terms terms of specific needs related related to to PD PD and and also also in in terms terms of general analysed in specific needs needs arising arising from from any any chronic chronic illness in an elderly person. This This chapter chapter will will not needs illness in elderly person. attempt to give give definitive social definitive answers answers to to what what isis needed needed to to meet meet the health and social needs needs of of patients with PD. It will will instead instead try try to to give give aa brief brief description description of of the the way way in which which services services have havedeveloped developedfor forpatients patients with with PD PD and and how how research research might might best address of such such services. services. address the future development of

Demographic changes The proportion proportion of of populations populations throughout throughoutthe theworld worldaged aged65 65 and and over over is estimated

to increase substantially substantially into the next century. century. This change results from from a slowing down rates, aa decline decline in fertility fertility rates and from from advances advances in medical medical down of mortality rates, care and public health. By the year 2025 countries are are likely likely to have 2025 around around 59 countries have more (Kinsella and Taeuber 1993). The than 22 million elderly people in their population (Kinsella The States population will will have elderly in United States have the the greatest greatest proportion proportion of elderly in the the world world phenomenonexclusive exclusive to to by the year 2000. These These demographic changes are not aa phenomenon and North North American American nations. nations.Developing Developing nations nations are are the industrialized European and also also experiencing experiencing similar similar changes. changes. China's China'selderly elderlypopulation population in in the the next next quarter quarter of 122

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Health and social social needs needs a a

century is is expected expected to double and and Japan Japan has has already already experienced experienced an increase of

7—14% theirelderly elderlypopulation population between (Kinsella 1996). 1996). The The impact 7-14% inintheir between 1970—1996 1970-1996 (Kinsella of demographic changes be especially especiallyimportant important for diseases diseases that that demonstrate changes will be demonstrate a strong age associated associated risk such as PD PD and and Alzheimer's Alzheimer's disease. disease. The The burden burden of such neurodegenerative century neurodegenerativediseases diseaseswill willsignificantly significantlyincrease increaseinto intothe the next next century (Lilienfeld et al. 1990, Zhang 1992). (Lilienfeld al, 1990, Zhang and Roman 1992), Health systems throughout throughout the the world world are are presented presented with with the the problems problems of of Health care care systems how how to to deliver deliver services services and finance finance healthcare healthcare for for elderly elderly people people with with finite finite Developing countries often have to meet this challenge, handicapped by resources. Developing (Gibson 1992). 1992). Within developing nations there there political and economic instability (Gibson is often privately financed financed health care and as as a often a lack lack of of adequate adequate state state support support or privately result services, if exist, are likely likely to difficult to to access. access. result health health services, if they they exist, to be basic and difficult Because Because of of the the differing differing health health care care systems systems in in place place throughout throughout the world itit isis difficult, ifif not difficult, not impossible, impossible, to to draw draw international international comparisons comparisons of of optimal optimal care care for for patients with with PD. PD. However, However, itit is is still possible to draw together guidelines guidelines of of what is is patients considered to be best practice in the provision of services for for PD PD patients and their carers.

organization and provision of services for PD viewview The organization and provision of services for— PDa -global a global A review of of the the organization of services for for PD and how these services services are are financed A review in a selected is presented in in Table Table 8.1. The differences selected number of countries is The major differences seen the organization organization and and delivery delivery of of care carereflect reflect socio-economic socio-economic policies policies seen in the by the various countries. The information for for this this chapter chapter has has been drawn drawn pursued by from of sources. sources. The vast majority of of published work work was sourced from from from a number of electronic databases such as Medline. Medline. The The Internet Internet also provided a useful useful source of information for contact contact addresses addresses of various PD PD societies societies throughout the information for of the various throughout the discussion groups Internet were were also also contacted contacted for their their assisassisworld. PD discussion groups on the Internet tance.

The organization and provision provision of of services services for for PD PD The main medical contact contact for for patients patients with withPD PDisis the the family family doctor, doctor, or or general general pracpractitioner (GP). (GP). A A typical GP practice in the UK UK will have five patients have no no more than five PD. It It is is unlikely therefore that aa GP GP will will be able able to develop develop sufficient sufficient experwith PD. tise psychosocial problems encountered by by tise to deal with the complex medical and psychosocial PD patients throughout throughoutthe thecourse courseofoftheir theirillness illness(Mutch (Mutch1992). 1992).The TheGP GPacts acts as as the the gatekeeper for access access to gatekeeper to more specialist services services for for PD where they exist. The complex needs needs of of PD PD patients patients may may be bebest bestmet metthrough through aa multidisciplinary multidisciplinary team (MDT) of health and and social social work professionals. professionals. In countries team (MDT) In aa number number of countries

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Table 8.1 8.1 Organization of services services for Parkinson's Parkinson's disease disease patients in selected countries countries

United States of America

In the United States private physicians physicians manage the the majority of patients with PD. PD. specialist neurological neurologicalmanagement management in in the the form form of of movement movement disorder disorder More specialist clinics isis restricted clinics restrictedto to university university departments departments and large large urban urban hospitals. reimbursed for for the thecare care of of elderly elderly patients patients with with PD. PD. Geriatricians are not reimbursed

Patients through direct direct payments or private health insurance finance most 65 years the medical services. For patients or over over the the age age of of 65 patients who are disabled or federal federal government Medicare meets healthcare costs. The Medicaid Medicaid programme covers some of the poor. covers Canada

for Parkinson's disease patients can be organized differently The services services for differently from from province to province and sometimes region to region depending on local funding arrangements. arrangements. Some Some of of the the large large cities cities have multidisciplinary movement disorder disorder clinics. clinics. Some elderly patients patients are managed by geriatricians, managed by neurologists. Those living with younger patients managed living in in remote remote or rural support. areas depend upon the family doctor for support.

The Canadian national national health health care care system system (Medicare) (Medicare) isis based on on aa universal universal coverage for all all of ofthe the population. population. The Medicare system system isis funded funded from from the coverage for federal income tax. The cost for for drug treatment is federal is usually usually restricted to those those over the age age of of 65 65 years. years. United Kingdom

Routine care for patients with PD GP can can refer refer the the PD is is managed by the GP. The GP by a geriatrician geriatrician or neurologist. Specialist patient for for specialist assessment assessment by Specialist clinics are clinics are increasingly increasinglybeing being developed developednationwide. nationwide. A Anumber number of PD nurse specialists, UK Parkinson's Disease Disease Society and specialists, initially initially supported supported by the UK pharmaceutical industry, have been been appointed. Health care is provided provided through free and covers covers the the entire the National Health Service Service (NHS), which is free population. Funding Funding for for the the NHS comes from sources: national population. from a number of sources: national health insurance levied on both employers and employees; employees; aasmall smallproportion proportion of funding funding also comes comes through through prescription charges (4%); the vast majority of funding for the NHS comes from from general general taxation. taxation. Private Private health health care care insurance insurance is available to offer offer limited limited coverage. coverage. available in in the the UK although this tends to

Denmark

In Denmark there there are are an an estimated estimated 6000 6000 patients patients with with PD. PD.Services Services are provided mainly from neurologists either in in university university departments, departments,specialist specialist clinics clinics or or their general general in private practice. In more remote areas patients are managed by their practitioners. The Thehealth healthcare caresystem system in in Denmark Denmarkisisvery very comprehensive comprehensive and and isis practitioners. financed through through taxation (85%) and patient payments (15%). Partial payment payment for drugs is met by the health service, however however some some groups groups such as the the elderly elderly are exempt from payments.

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Health and social social needs needs

Table 8.1 (cont.) (cont.)

Finland

Services for patients patients with with PD are generally generally provided provided through through general Services for practitioners and and neurology neurology clinics. clinics. There is one movement disorder clinic funded by by The The (Helsinki University University Hospital) Hospital) and a rehabilitation centre funded Finnish Finnish Parkinson's Disease Disease Society. Society.The TheSocial SocialInsurance InsuranceInstitution Institution of of Finland Finland (KELA), which (KELA), which has has been been in existence for 60 years, is financed financed by employers other means means (15%). (15%).The TheKELA KELA (20%), employees employees (18%), the the state (48%) and other partial funding funding of offees fees charged charged by by physicians, physicians, clinics, clinics, medication medication costs costs provides partial and transportation. This has has to to be be applied applied for for annually annually by patients. Some costs transportation. This costs are financed financed by by state state sponsored sponsored gambling gambling (the Slot Machine Association). The laws and statutes of social laws social welfare welfare are are another another source source of of funding funding for patients.

Estonia

Neurologists at local general hospitals hospitals manage patients with PD. There is also also aa University clinic clinic atat Tartu Tartu (south (south Estonia) Estonia) and a multidisciplinary clinic at University Tallinn has helped helped to Tallinn (capital of Estonia). The Estonian Parkinson's Society has develop more access to specialists specialists and and specialist specialist physiotherapy physiotherapy groups. The develop access to care system in Estonia is is funded wholly wholly by the state. state. health care

Japan

Access to specialist specialisthelp help for for PD PD patients patients is mostly through neurology Access to neurology clinics. clinics.

The Japanese healthcare system system is is financed financed through through private health insurance insurance approximately 20% of medical costs. In common with with with patients paying for approximately other chronic illnesses illnesses when when patients patients with PD reach a certain level of of disability disability all medical financed. medical charges are ftnanced.

South Africa Africa

In urban areas there are a number of hospital-based movement disorder disorder clinics. Neurologists also treat patients privately at a number number of of clinics. clinics. Those living in the more remote or any form form of of or rural rural areas areas of of South South Africa Africa often often do not receive receive any medical assistance. The state state finances finances health health services servicesin inthe the provincial provincial areas areasof of Africa. However, South Africa. However,the theamount amount the the state state contributes contributes to an individual's health health care is means tested. Those patients with private medical health insurance insurance tend tend to tohave haveaccess access to to better better services. services.

New Zealand

Patients in urban areas by neurologists neurologists or Patients areas are managed in specialist clinics clinics by geriatricians with a specialist specialist interest in movement disorders. disorders. The The Parkinsonism Parkinsonism of field field officers officers (usually Society of New Zealand also provides provides a number of assist in of patients. patients. New New registered nurses) who assist in the the support support and management of world, in in 1938, 1938, to offer offer free public Zealand was the first country in the world, hospitals. The revenues revenues for for health health care care are are raised raisedthrough through taxation taxation in a similar similar Interestingly, in as a way to to that of the UK. Interestingly, in terms terms of total health expenditure as GDP, New percentage of GDP, New Zealand Zealand appears appears to to be be aa modest modest spender spender compared to other countries.

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specialist movement disorder disorder clinics clinics have have been established, established, usually usually in in large large cities cities

settings, and can provide a focus for the activity and academic settings, activity of a MDT (Mutch (Mutch 1992). Medical input input to specialist clinics is is usually usually from from trained trained neurologists 1992). Medical specialist clinics neurologists or geriatricians. geriatricians. Clinics Clinics can can provide provide accurate accurate diagnosis, diagnosis, assessment, assessment, referral referral to to other other disciplines, review (Meara (Meara and Hobson 1998a, 1998a, disciplines, therapy therapy interventions interventions and regular review plays an in the the treatment treatmentof ofall all Meara et al. al, 1999a). 1999a). Physical Physicaltherapy therapyplays an important important part in formal evidence evidence exists exists for its effectiveness effectiveness (Gibberd et stages in PD, though though limited formal al. 1984, Formisano al. 1981, 1981, Scott Scott and and Caird Caird 1983, 1983, Robertson Robertson and and Thompson Thompson 1984, Formisano et al. al. 1992, Comella 1994). Psychological Psychological and coupled 1992, Comella et et al. 1994). and educational educational programmes coupled and intensive intensive group group therapy therapy are are increasingly increasingly being prowith MDT interventions and useful additions to medical medical therapy therapy (Gauthier (Gauthier etet al. al.1987, 1987, Ellgring Ellgring et al. posed as useful 1990, Montgomery et et al. al. 1994). 1994). The The clinical clinical effectiveness effectiveness of specialist specialist clinics has 1990, received long-term receivedlittle littleattention, attention, though though one one study study in the UK has shown some long-term benefits (Meara 1998b). A vital secondary benefits (Meara and and Hobson 1998b). vital link link between between the the GP GP and and secondary care specialist PD care could could be be provided provided by by the the introduction introduction of specialist PD nurses nurses to to promote of shared shared care, care. This This type of of specialist service better organization of service isis currently currently being being evaluated skills in comprehensive comprehensive geriatric assessment assessment is evaluated in in the UK. A nurse with skills is demands of of the the PD PD specialist specialist nurse nurserole role(see (seeTable Table 8.2). 8.2). likely to to most ably meet the demands In the UK several several studies studies have have shown shown aa high high level levelofofunmet unmet need need in in patients patients with carers in in terms terms of of the theprovision provision and anduptake uptakeofofservices services(Oxtoby (Oxtoby 1982, 1982, PD and carers et al. 1986, 1986, Peto 1997, Meara 1997). Less Less than one-third Mutch et Peto et al. 1997, Meara and and Hobson 1997). than one-third of assessment by a MDT (see (see Table Table 8.3). of patients with PD undergo assessment 8.3). The The improveimprovements uptake of of physiotherapy physiotherapy and and speech speech and and language language therapy therapy services services ments in the uptake reported in later studies (Peto et al. 1997, Meara and Hobson 1997) may be be reported in later studies (Peto et al. 1997, Meara and Hobson 1997) may accounted for by the recent increase in movement movement disorder disorder clinics clinics (see (see Table Table 8.4). Peto et al. 1997 1997 found that service use increased with longer duration of disease, disease, found service increased duration of even when a local specialist are still but even specialist service service exists existsaahigh highproportion proportion of patients are not referred for specialist assessment (Meara and Hobson 1997). More research not referred for specialist assessment (Meara and Hobson 1997). More research isis determine ififnew new or orexisting existingservices services for for PD PD are are effective effective and efficient. needed to determine and efficient. Social networks and support in in PD PD

been found Social relationships and support networks networks have have in a number of studies been and service service utilization (Walliston (Walliston et et al. al. to be factors predictive of health outcomes and 1983, House 1988, Sugisawa Sugisawa et 1994, Bosworth Schaie 1997). 1997). Social 1983, House et al. 1988, et al. 1994, Bosworth and Schaie networks share common characteristics through their size, source source and and proximity proximity of of family confamily or or friends. friends. Social Social networks networks can can act act as asaameans meansof ofemotional emotional support, support, or conversely, as versely, asaabarrier barrier to to adequate adequate help. help. The The ability ability of of an an elderly elderly person person with with PD PD to to independent will will be be clearly clearly influenced influenced by their access access to help remain independent to support support and help al. 1992, 1992, Wenger 1991). A of studies studies have have investigated investigated (Berkman et al, Wenger 1991), A small small number of

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Health and social social needs needs Table 8.2 The potential roles roles of of aaPD PD specialist specialist nurse

Community outreach support from from aa movement disorder disorder clinic • Liaison Liaison with GPs and primary primary health health care care teams; teams; PD PD specialists; specialists; therapy services; other community/hospital-based services; services; private private sector sector and and voluntary voluntary groups community/hospital-based services; social services; • Rapid hospital admissions admissions Rapid assessment assessment and follow-up for both acute and elective elective hospital • Drug Drug therapy monitoring/advice monitoring/advice • Identifying Identifying the the unmet needs of patients, carers and health professionals professionals • Education, Education, support support and andadvice advice for for patients, patients, carers carers and and other otherhealth health professionals professionals • Palliative care • Assessment of elderly elderly patients patients in in residential/nursing residential/nursing home care •

Table therapy service service inputs inputs for for PD patients patients in the UK Table 8.3 Recent therapy

Service

Occupational therapy Occupational Physiotherapy Speech and language language therapy therapy Speech

Oxtoby (1982) n=261 « = 261

Mutch et al. (1986) «n=267 = 267

Peto et al. (1997) n=178 «=178

Meara and Hobson (1997) n=172 w= 172

13%

25% 7% 7% 4%

24%

26% 29% 30%

17% 3%

39% 25%

Table 8.4 Service receipt (%) (%) in the previous year year for for patients attending a movement Service receipt disorder disorder clinic compared to those managed by their general practitioner

Service

Specialist clinic clinic managed managed Specialist n=102 «=102 (%)

Occupational therapy Occupational Physiotherapy Speech therapy Speech GP contact Social worker Physiotherapist District nurse

37 43 46 98 98 17 17 43 15 15

Note: Note: * p<0.05 *P<0.05

GP managed n=70 n = 70 (%) (%) 99* * 88* * 66* *

60 60** 55** 55* *

55**

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Table 8.5 8.5 Service provision utilization utilization frequencies frequencies(%(%) Service provision (%) in 12 12 month period period by by support support network type

Service Service

Family Family dependent (n=16) («=16)

GP

16 (100%) 16(100%)

Consultant/Specialist Consultant/Specialist

15 15

Occupational Occupationaltherapist therapist Physiotherapist Physiotherapist

Socialworker Social worker Speechtherapist Speech therapist Care Care assistant assistant District nurse District nurse

8 6 1 1 7 1 1 6 8

6

(94%) (50%) (50%) (37%) (37%) (6%) (6%) (44%) (44%) (6%) (6%) (37%) (37%)

Locally Locally

Local selfself-

Wider

integrated integrated

contained contained

(n=35) (n = 35)

(n=33) (n = 33)

community (n == 11) (n=11)

Private Private (n=15) («=15)

(94%) 33 (94%) 19 (54%) 19 (54%) 8 (23%) 8 (23%) 11(31%) 11 (31%) 33 (9%) (9%) 11(31%) 11 (31%) 9 (26%) 9 (26%) 1 1 (3%) (3%)

30 30 (91%)

11(100%) 11 (100%)

11(73%) 11 (73%)

23 23 (70%)

7 (64%) (64%)

6 (40%) 6 (40%)

9 (27%) 9 (27%) 10(30%) 10 (30%) 6(18%) 13(39%) 13 (39%) 33 (9%) (9%) (12%) 4 (12%)

3 3 (27%) (27%) 22 (18%) (18%)

44(27%) (27%) 44(27%) (27%) 1 1 (7%) (7%) 44(27%) (27%) (27%) 4 (27%) (33%) 5 (33%)

11

(9%)

3 3

(27%) (27%)

3 3

(27%) (27%)

22 (18%) (18%)

the social with PD (Mccarthy social support of people with (McCarthy and and Brown Brown 1989, 1989, Ehmann et al. 1990, Miller et et al. al. 1996), All these these have have shown shown that that low self esteem, poor coping 1990, Miller 1996). All self esteem, coping strategies and depressive are associated with reduced strategies and depressive symptomatology symptomatology are associated with reduced social social support. We We have examined and help help provided provided by by social social networks and support. examined the support and of PD PD patients living living in the commucommuthe influence they have have upon upon the management of nity using a network typology typology devised devised by Wenger (Wenger 1990, 1990,1991). 1991). The results (see Table (see Table8.5) 8.5)indicate indicatethat that the the social social network network influences influences the the delivery deliveryof of appropriappropriate (unpublished observations). observations). ate services services and and interventions interventions for for patients patients with with PD (unpublished service providers This has implications for health professionals, service providers and planners, who need to recognize recognize and aware of the importance importance and and influence influence of need and be made more aware and networks networks in in chronic chronic disease. disease. social support and

Meeting the needs of caregivers caregivers carers of patients with PD, aged and may Carers PD, usually a spouse, spouse, are are likely to be similarly aged also also suffer suffer from from chronic chronic illness. illness. Caring caring for an elderly spouse with PD can result in deterioration of marital relationship relationship to nurse and patient patient (Pinder (Pinder the deterioration of a marital to that that of of nurse 1990). Estimates Estimates of of the the financial financial costs costs for for informal informal care care in in the the UK UKsuggest suggestan an annual annual figure (Adams 1991), 1991). Stress, frustration, exhaustion, figure of of around around 24 billion pounds (Adams frustration, exhaustion, depression physical illness illness are commonly reported reported by by caregivers caregivers (Mui (Mui 1995). 1995). depression and physical of care care in in PD PD is is reflected reflected in the high levels levels of The burden of of carer carer distress distress reported reported in et al. 1991, 1991, Miller et al. 1996, O'Reilly several studies (Calder et O'Reilly etet al. al. 1996, 1996,Herrmann Herrmann et al. 1997, Meara et al. 1999b). 1 999b).The TheUK UKParkinson's Parkinson's Disease Disease Society Society discussion discussion docMeeting aa need? need? (1994) (1994) addresses addresses the needs needs of of patients patients and andcarers carersand andrecrecument Meeting ommends provision of of appropriate appropriate respite respite care, care, relevant relevant and and intelligible intelligible ommends the the provision

129 129

Health and social social needs needs

information on PD and how to obtain et al. information obtain help help and and gnidance. guidance.Arksey Arkseyet al. (1998) evalnated the needs of carers of patients with physical disabilities who who had had been recently uated discharged from hospital hospital and found found that what they required was clear, clear, unambiguunambigudischarged from helpful information. Although Although the the carer carer isis likely likely to be the most most ous, practical practical and helpful important person in in the the provision provision of of care care for for the the patient, patient, this this vital vital role role is is often often undervalued. More More research research needs to be undertaken undertaken poorly recognized, ignored or undervalued, in this area in PD in order to to find find ways ways of reducing reducing the distress distress that can be assoassociated with caring.

Economic appraisals appraisals of of the delivery delivery of care for elderly patients with PD care for PD resources is is aareality realitythroughout throughout all all healthcare healthcare systems systemsno no matter matter how how Rationing of resources well funded. well funded. In the last 20 20 years years there there has hasbeen been aa rapid rapid growth growth in in the the introduction effective, but for PD. PD. New and expenexpenof more effective, but also also more more expensive expensive drug drug treatments treatments for sive sive drug drug therapy therapy that that improves improves motor motor impairment impairment in terms of reducing reducing tremor tremor or rigidity may have have only only marginal marginal effects effectson onhandicap handicap or or disadvantage disadvantage to to the the patient. patient. More data are needed needed relating to the cost benefits benefits of of existing existing drugs drugs compared compared with with new improvements in disadvantage disadvantage and new drug drug therapies therapies for for PD PD in terms terms of improvements and health health related on related quality quality of life. life. Appropriate Appropriate evaluation evaluation of of the the impact impact of new treatments on related quality quality of of life life would give give patients, specialists specialists and health care care planplanhealth related ners information to make make therapeutic and and funding funding decisions. decisions. Some Some counners better better information tries with state financed health health care care systems, systems, such such as as the the UK, UK, place place financial financial limits limits upon the introduction introduction of of new new or orexisting existing treatments treatments and andevidence evidence of of the the clinical clinical effectiveness of funding will will be be given. given. effectiveness of treatment treatment is increasingly being sought before funding This approach should be adopted in in developing developing countries countries where where high high levels levels of ecoeconomic and social deprivation already limit health care provision. In the absence of adequate state state support or private insurance, many patients with PD are faced faced with with difficult choices. difficult choices. In In self-funding self-funding their their treatment treatment patients may elect to to reduce medication expensive drugs ication or substitute expensive drugs for for cheaper cheaper ones. ones. Many Many drugs drugs used used in in the treatment of of PD PD such such as as combinations combinations of of levodopa levodopa and and decarboxylase decarboxylase inhibitors, dopamine agonists agonists and enzyme enzyme inhibitors, such as as selegiline selegiline and tolcapone, are dopamine and tolcapone, expensive and unaffordable in many areas of the world. Neurosurgical procedures, expensive and unaffordable though not widely available, available, may maybe beaacheaper cheaperlong-term long-termoption optionthan than drug drug therapy therapy in many countries.

Putting research research into into practice difficulty of of collecting collectinginformation information that that will will inform inform not only health care proThe difficulty fessionals,but but also alsopatients patients and and their their carers carers cannot cannot be be underestimated. underestimated. In In the the UK UK fessionals, assess the clinical audit is employed as a means to assess the quality of services services and and to develop

130

P. Hobson Hobson P.

effective interventions that effective that will will result in improvements improvements in in patient patientcare. care.However, However, by failing to to take take into into account its nature clinical audit tends to focus on clinical needs, failing expectations of the expectations of carers carers and and patients. patients. There There is is aa need need to to work work with with the the patients patients service. Patients with PD who attend attend aa spespewho actually make use of the particular service. cialist cialist clinic clinic may may all all appear appear to to have have the the same service service need, yet yet in reality individual needs are very dissimilar. Evidence of clinical clinical effectiveness effectiveness alone alone will will not not alter alter praceffects of cultural, political, social and economic economic factors. factors. tice given given the the confounding effects Epidemiological cost benefit benefit Epidemiological studies, studies, clinical clinical trials, trials, observational observational studies studies and cost studies are are methods representation of patient populations and and meameastudies methods of ensuring representation of interventions. interventions. Studies Studies such as as these should provide provide estimates estimates suring outcomes of of the the size size of ofspecific specificillnesses illnessesand andthereby therebyallow allowhealth healthcare careplanning planningto tomatch match need need resources. The with resources. The assessment assessment and and evaluation evaluation of of patients' patients' experiences experiences and and their their treatment isis acknowledged acknowledged as a vital Elderly people treatment vital component component of health care. Elderly people tend or excluded excluded from a significant significant proportion of of these studies. to be underrepresented or Furthermore, the methodologies methodologies used research may used in outcome research may also also be be inapprofor elderly people even when they have priate for have been been included in studies. studies, To illustrate the generic generic SF-36 SF-36 (Ware 1992), a health this point the (Ware and and Sherbourne 1992), health related related quality of demonof life life instrument, instrument, has has been been used used in in aa number number of countries and has been demonto have have excellent excellent psychometric qualities. qualities. However, However, the SF-36 may not be an strated to appropriate in elderly elderly people people with (Hill et al. al. 1996, 1996, Hobson and appropriate instrument in with PD (Hill 1997, Parker et al. 1998). All Meara 1997, Alltoo too often often such such generic measures are used inappropriately without without the the addition additionofofdisease disease specific specific or single domain measures from from propriately which more meaningful meaningful conclusions conclusions may drawn. Hunter (1996) (1996) argues argues that that which may be be drawn. there is not one one definitive definitive way to evaluate evaluate new or existing services and that a colservices and laboration of of varied varied research research disciplines disciplines and research research methodologies methodologies are are the the key key to laboration effective evaluation for the provision and organization of health services. effective evaluation for the provision of health services.

Palliative care in PD PD There is a lack lack of of any any formal formal guidance guidance on the management management of ofpalliative palliative care care in in PD. PD.

In the the UK UK patients patients with with advanced advanced PD PD who who require require nursing nursing care care are are usually usually managed in in nursing nursinghomes homeswith withroutine routinemedical medicalcare careprovided providedby bythe theGP. GP.Specialist Specialist involvement involvementisis unusual unusual at at this this stage, stage, often often because because of of the the inappropriateness inappropriateness of of attendance at an outpatient outpatient clinic. clinic. However, However, at this stage stage in in PD PD management there between is still still aa very verygreat greatneed need for forclose closecooperation cooperation and effective effectivecommunication communication between patient and andfamily family (Meara (Meara 1998). 1998). Most Most research research in in palpalhealth professionals and the patient liative care cancer, who liative care has has concentrated concentrated on on patients dying of cancer, who have have tended tended to be younger and cared for in hospitals or hospices. One study found found that elderly people illness do not receive receive the with terminal illness the same same professional professional medical medical interventions interventions as as (Seale and Cartwright 1994), 1994). Lindop et al, al. (1997) argue that the the younger patients (Seale

131 131

Health and social social needs needs existing models models of of palliative care for for cancer cancer patients patients need need to to be broadened palliative care broadened to to encompass the needs encompass the needs for for all patients with terminal terminal illnesses illnesses and should should include include their families families and carers.

Conclusion The WHO and and various various PD PD working working parties parties have have suggested suggested guidelines guidelines on on what what serserthe needs needs of of patients patientswith withPD. PD.However, However, the thechallenge challenge is is vices are needed to meet the to these proposals within existing existing healthcare healthcare systems systems throughout the to implement these throughout the world. Greater emphasis placed upon upon collaboration between health and emphasis should be placed social their carers. carers. social care care professionals, professionals,the the voluntary voluntarysector sector and and patients patients and and their Information technology technology can can facilitate facilitate the dissemination of of what is is considered to be PD. This Thistechnology technologyisisnot notavailable available exclusively exclusively managementof ofPD. best practice in the management to health care care professionals, professionals, and will will be increasingly increasingly employed by patients, their employed by carers and better service service and and more more effective effective intercarers and by pressure pressure groups to demand better ventions.

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Gibberd FB, Page PageNGR, NGR,Spencer SpencerKM KM(1981) (1981)Controlled Controlled trial trial of physiotherapy physiotherapy and occupational occupational

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CW (1997) (1997) Coping Coping with with chronic neurological Herrmann M, M, Freyholdt Freyholdt U, U, Fuchs Fuchs G, G, Wallesch Wallesch CW neurological impairment: aa contrastive of Parkinson's contrastive analysis analysis of Parkinson's disease disease and and stroke. stroke. Disability Disability and Rehabilitation, 1, 1, 6—12. Rehabilitation,19,19, 6-12. Hobson JP, Meara RJ RJ (1997) (1997) Is Is the the SF-36 SF-36 Health Health Survey SurveyQuestionnaire Questionnaire suitable suitable as as a self self report report JP, Meara measure of of the the health health status statusofofolder olderadults adultswith withParkinson's Parkinson'sdisease. disease.Quality QualityofofLife LifeResearch, Research, 3, 6, 213—16. 3,6,213-16. House health. Science, 241,241, 540—5. House JS, JS, Landis HR, HR, Umberson UmbersonDD(1988) (1988)Social Socialrelationships relationshipsand and health. Science, 540-5. Hunter DJ DJ (1996) (1996) Evaluation Evaluation of ofhealth healthservices. services.In: In:Epidemiology EpidemiologyininOld OldAge, Age,eds. eds.Ebrahim Ebrahim5,S, Kalache A, pp. 85—95. London: BMJ BMJ Publishing Publishing Group. 85-95. London: Group. Kinsella K (1996) Demographic S and Kalache Kinsella Demographicaspects. aspects.In: In:Epidemiology EpidemiologyininOld OldAge, Age,eds. eds.Ebrahim Ebrahim S and Kalache London:BMJ BMJPublishing Publishing Group. Group. A, pp. 32—40. 32-40. London: Kinsella K, Taeuber C (1993) An Office. World IL II.Washington: Washington:US USGovernment GovernmentPrinting Printing Office. An Ageing World Lilienfeld DE, Chan E, Ehland Ehland J,J, Goldbold GoldboldJ,J,Landrigan LandriganPJ, PJ,Marsh MarshG, G,Perl PerlDP DP(1990) (1990)Two Twodecades decades of increasing increasing mortality mortalityfrom fromParkinson's Parkinson'sdisease diseaseamong amongthe theUSUSelderly. elderly.Archives ArchivesofofNeurology, Neurology, 7, 7,731—4. 731-4. Lindop E, Beach R, R, Read ReadS S (1997) A composite model model of of palliative palliative care for the UK. International International Journal ofofPalliative 3, 5, Journal PalliativeNursing, Nursing, 3, 287—92. 5, 287-92. McCarthy B, Brown Brown R (1989) (1989) Psychosocial Psychosocial factors Parkinson's disease. disease. British Journal of of McCarthy factors in Parkinson's Clinical 28,28, 41—2. ClinicalPsychology, Psychology, 41-2. Meara RJ Journal, 38, 4, RJ (1998) (1998) Late Latestage stageParkinson's Parkinson'sdisease. disease.Prescribers' Prescribers' Journal, 38,233—42. 4, 233-42. Meara RJ, Bhowmick Bhowmick BK, BK, Hobson Hobson JP JP (1999a) (1999a) Accuracy Accuracy of of diagnosis diagnosis in in patients patients with presumed presumed Parkinson's 99—102. Parkinson's disease disease in inaacommunity-based community-baseddisease diseaseregister. register.Age Ageand andAgeing, Ageing,28,28, 99-102. Meara RJ, Hobson JP JP (1997) (1997) Levels Levels of service provision for people people with with Parkinson's Parkinson'sdisease: disease:AA survey of community community registered ofofthe Journal theBritish BritishAssociation Associationfor for registeredpatient's patient'sperceptions. perceptions.The TheJournal Service totothe 4, 4, 3—10. Service theElderly, Elderly, 3-10. Meara RJ, Hobson JP (1998a) Comprehensive assessment assessment of patients with with Parkinson's Parkinson's disease. disease. Mature MatureMedicine Medicine—- Canada, Canada, 1,1,3,3,15—18. 15-18. Meara RJ, Hobson Hobson JP (1998b) (1998b) A longitudinal follow-up follow-up of patients with Parkinson's Parkinson's syndrome syndrome who attend attend aaspecialist specialist movement movementdisorder disorderclinic. clinic.Age Ageand andAgeing, Ageing,27, 27,Suppl. Suppl.1,1,55. 55. 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Health and social social needs needs EB, Lieberman Lieberman A, A, Singh SinghG, G, Fries FriesJF JF(1994) (1994)Patient Patienteducation education and and health health promoMontgomery EB, tion in Parkinson's disease: A randomised controlled controlled trial. trial.American American Journal Journalofof tion can can be be effective effective in Medicioe, 97, Medicine, 97,429—35. 429-35. Mui AC (1995) (1995) Caring for the frail elderly parent. A comparison of adult adult sons sons and and daughters. daughters. Gerontologist, 35,35, 86—93. Gerontologist, 86-93. aodand Mutch WI WJ (1992) (1992) Specialist Specialist clinics: clinics: AAbetter betterway waytotocare? care?Journal JournalofofNeorology, Neurology,Neurosurgery, Neurosurgery, Psychiatry, 55, 41—4. Psychiatry, 55,Suppl. Suppl. 41^. Mutch Mutch WJ, WJ, Strudwick Strudwick A, A, Roy Roy SK, SK, Downie AW AW (1986) Parkinson's Parkinson's disease: disease: disability, disability, review review and and management. British 282, 534—6. BritishMedical MedicalJouroal, Journal, 282, 534-6. management. O'Mahony PC, PG, Rodgers Rodgers H, H, Thomson ThomsonRC, RG,Dobson DobsonR,R,James JamesOFW OFW(1998) (1998) IsIsthe theSF-36 SF-36 suitable suitable for for assessing health status Ageiog, 27,27, 19—22. statusofofolder olderstroke strokepatients? patients?Age Ageand and Ageing, 19-22. O'Reilly F, F, Finnan Finnan F, F,Allwright AllwrightS,5,Smith Smith GD, CD, Ben-Shlomo Ben-Shlomo Y Y (1996) (1996) The The effects effects of of caring caring for for aa O'Reilly spouse with Parkinson's spouse Parkinson's disease disease on on social, social, psychological psychological and and physical physical well-being. well-being. British British Jouroal Practice, 46, 46, 507—12. JournalofofClioical Clinical Practice, 507-12. Oxtoby M M (1982) (1982) Parkinsoo's Parkinson'sDisease DiseasePatieots Patients their Social Needs. London: Parkinson's Disease aodand their Social Needs. 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SugisawaH, H, Liang LiangJ,J,Liu LiuXX(1994) (1994)Social Socialnetworks, networks,social socialsupport supportand andmortality mortality among among older older Sugisawa people of of Gerontology: Social Sciences, 49, S3—13. peopleininJapan. Japan.Journal Journal Gerontology: Social Sciences, 49, S3-13. BA, Alagna SW, De Walliston BA, Dc Villis Villis BM, BM, De DcVillis VillisRF RF(1983) (1983)Social Socialsupport support with with many aspects of health 2, 367—91. Psychology, 2, 367-91. health and andillness. illness.Health HealthPsychology, Ware JE, Sherbourne Sherbourne CD (1992) The MOS 36 item short short form form survey survey(SF-36): (SF-36):conceptual conceptualframeframework Care, 30, 30, 473—8 3. work and anditem itemselection. selection.Medical Medical Care, 473-83. Wenger CC (1990) Change and adaptation in informal Wenger GC (1990) Change informal support support networks networks of ofelderly elderly people people in in Wales Journal ofofAgeing 375—89. 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The drug treatment of Parkinson's disease in elderly people Theresa A Zesiewicz and Robert A Hauser

Introduction The goal of medical management of Parkinson's disease (PD) is to control signs and

symptoms for as long as possible while minimizing side effects. Medical therapy generally provides good control of symptoms for 4 to 6 years, though disability

continues to progress despite best medical management, and many patients develop long-term complications. Such complications include motor fluctuations and dyskinesia associated with long-term levodopa therapy (Chase et al. 1993). Other common causes of disability in late stage disease include postural instability and dementia. A key consideration in the treatment of elderly patients is that they are more susceptible to side effects from medication. Older people are more likely than younger individuals to be taking more prescribed and over the counter medication for a range ofdiseases. Medication prescribed for one condition can worsen another and side effects from medication can be mistaken as a new disease process and lead to further unnecessary prescribing (Williamson 1978). Cognitive impairment and delirium, both of which commonly develop as side effects of drug therapy, reduce compliance with drug treatment. As people live longer the prevalence of PD will increase. PD is a significant risk factor for admission to nursing homes. Over 50% of prevalent cases of PD in one epidemiological study in France were living in nursing homes (Tison et al. 1994). Hallucinations are a particular factor that increases the risk of admission to nursing homes in PD and in many cases this will be related to the drug therapy prescribed for PD (Goetz and Stebbins 1995). Residents with PD make up around 5% of the nursing home population and there is evidence to suggest that optimal drug treatment in this group may have delayed or even prevented some admissions to nursing homes (Larsen et al. 1991). The selection of drug therapy is often guided by information derived from clinical trials. Unfortunately, elderly people are under represented in drug trials and a post hoc analysis of elderly subgroups is rarely provided (Avorn 1990). Very few 134

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Drug treatment clinical trials have examined the best way to use available antiparkinsonian medi-

cation for different age groups, over either the short or long-term. Thus, until such information is available, a heavy reliance is placed on theoretical considerations, and anecdotal and personal experience. Not all drugs are available worldwide; in particular, apomorphine, domperidone and Madopartm' preparations are not currently licensed for use in the United States.

Drugs used in the treatment of PD in elderly patients Levodopa Levodopa combined with a peripheral decarboxylase inhibitor (PDI) is the gold

standard of drug treatment for PD. It usually provides the greatest symptomatic improvement with the fewest side effects. Ehringer and Hornykiewicz (1960) dem-

onstrated that P1) is associated with decreased striatal dopamine concentration, but dopamine is not useful as a therapeutic agent because it does not cross the blood—brain barrier. Carlsson et al. (1957) had already reported that the administration of the dopamine precursor, levodopa, reversed reserpine induced parkinsonism in rats. Levodopa was subsequently demonstrated to improve signs and symptoms of P1) (Cotzias et al. 1968). Levodopa is a large neutral amino acid, with a serum half life of approximately one hour (Nutt and Fellman 1984). It is primarily absorbed in the proximal small bowel by a saturable, carrier mediated transport system. The stomach is capable of absorbing levodopa only to a limited extent (Cedarbaum 1987). Meals and anticholinergic medications slow gastric emptying and delay levodopa absorption (Cedarbaum 1987). In older patients, gastric stasis results in slower gastric emptying and increased duodenal levodopa absorption (Evans et al. 1980). In addition, first pass metabolic decarboxylation of levodopa in the gastrointestinal tract is reduced iii older individuals. Because of these age associated changes, levodopa bioavailability is as much as 20% greater in the elderly (Evans et al. 1980). Levodopa bioavailability is approximately 40% in young volunteers. Levodopa

undergoes extensive peripheral metabolism, and less than 1% is excreted unchanged in the urine (Abrams et a!. 1971). Nausea and vomiting are common side effects of levodopa due to the peripheral metabolism of levodopa to dopamine, which stimulates the area postrema of the medulla. Levodopa is usually administered with a P1)1 that does not penetrate the blood—brain barrier to reduce circulating peripheral dopamine, thereby minimizing the incidence of nausea and vomiting, and to increase the central bioavailability. Co-careldopa (SinemetTM) contains the PDI carbidopa; co-beneldopa (Madopartm') contains benserazide. Both carbidopa and benserazide reduce the amount of levodopa needed to achieve a clinical response by approximately 75%. Around 75—100mg of carbidopa per day

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usually sufficient to saturate peripheral decarboxylase, but some patients may require up to 200mg (Rinne et al. 1973). The peripheral half life of levodopa when administered with a decarboxylase inhibitor is approximately two hours (Nutt et al. 1985). Levodopa absorption is facilitated by the administration of domperidone, a peripheral dopamine antagonist that promotes gastric emptying (Mearrick et al. 1974). are

In older patients we introduce levodopa/POl at a dose of 50mg once each morning and increase to 5omglevodopa three or four times daily, or 100mg levodopa three times daily over a three to four week period. Further titration is based on clinical response. Most patients remain on 400—600mg of levodopa/PDI for several years. Higher doses can and should be used if necessary to adequately control motor symptoms. Levodopa/PUI is usually administered one half hour before or one hour after meals to achieve the most consistent absorption. Acute side effects of levodopa/PDI administration include nausea, postural hypotension, confusion and hallucinations. Nausea is the most common side effect of levodopa/PDI therapy. If nausea occurs, it can often be reduced by administering the dose immediately following meals or with a carbohydrate snack. If nausea persists, increasing the carbidopa dosage is usually quite helpful. Some patients require up to 200mg of carbidopa per day. Domperidone can usually control this problem though other antiemetics such as metclopramide and prochlorperazine

should be avoided due to their central dopamine receptor blocking action. Uncommon side effects include anorexia, somnolence, increased serum transaminase levels and worsening of closed angle glaucoma. Levodopa/PDI can cause neuropsychiatric toxicity, ranging from delusions to delirium. Older patients are more vulnerable to these side effects. Postural hypotension is a potentially serious side effect of levodopa/PDI in older patients as it can lead to syncope, falls, and fractures. A review of all medications taken by the patient that may contribute to the problem should be undertaken. A reduction of the levodopa dosage may be necessary.

Levodopa/carbidopa controlled-release The controlled (sustained) release (CR) preparation of co-careldopa is more slowly

absorbed and provides more sustained serum levels than standard co-careldopa (Goetz et al. 1987). The bioavailability of levodopa in CR formulation is roughly 80% that of standard co-careldopa. Bioavailability is greater in the elderly, possibly owing to an age related decrease in gastric emptying. In addition, administering levodopa/carbidopa CR with food increases levodopa bioavailabiity by roughly 50%, and elevates peak plasma levels by 25% (Wilding et al. 1989). Co-careldopa controlled-release is available in a 200/50mg and 100/25mg strengths. We introduce co-careldopa CR at a dose of one 100/25 mg tablet per day and

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Drug treatment increase to three tablets per day over several weeks. Another co inmon dosing sched-

ule is co-careldopa CR 200/50 twice daily. To convert a patient from standard levodopa/carbidopa to CR) the daily levodopa dosage is increased roughly 20% while the number of daily doses is decreased by 30—50% (Rodnitzky 1992). Further titration is undertaken as clinically indicated.

Co-careldopa CR is as effective as standard co-careldopa when dopamine replacement therapy is first required and may be more convenient (Block et al. 1997). Later in the disease, patients with motor fluctuations and no dyskinesia often experience less 'off' time following conversion from standard to CR formulation (Feldman et al. 1989). However, CR formulations tend to worsen peak dose dyskinesia, particularly later in the day when levodopa levels accumulate. Because co-careldopa CR takes longer than standard formulation to 'kick in' and provide clinical benefit, many patients with motor fluctuations take a combination of standard and CR co-careldopa as their first morning dose. Intake of subsequent CR doses is scheduled so that one dose takes effect before the previous dose wears off. A bedtime levodopa/carbidopa CR dose maintains therapeutic plasma concentrations longer into the night, resulting in greater mobility for patients who awaken within a few hours. However, this may lead to nightmares or hallucinations in some patients. Co-beneldopa

HBS

Co-beneldopa HBS (hydrodynamically balanced system) is a slowly dissolving

preparation that floats on stomach contents for 5—12 hours, providing sustained release of levodopa proximal to its site of absorption. The bioavailability of cobeneldopa HBS is 50 to 60% that of standard formulation (Koller and Pahwa 1994). The pharmacokinetic profiles of co-careldopa CR and co-beneldopa HBS are similar.

Open label studies have found the HBS preparation to be effective in reducing motor fluctuations for patients on standard co-beneldopa. A randomised, doubleblind, cross-over trial comparing standard and HBS preparations demonstrated significantly better clinical function while patients were taking co-beneldopa HBS, but no difference in 'off' time as measured by patient diaries (de Michele et a!. 1989).

Dopamine agonists There are five orally active dopamine agonist drugs available to treat PD: the ergot agonists — bromocriptine, pergo!ide, and cabergoline, and the non ergot agonists — pramipexole and ropiniro!e. An account of the parenterally administered agonist apomorphine, which is only available on a named patient basis in the US, is given in Chapter 3.

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In contrast to levodopa, dopamine agonists do not undergo oxidative metabo-

lism, and the long-acting dopamine agonists provide relatively sustained dopaminergic stimulation. Animal studies and preliminary clinical information suggest that dopamine agonists are associated with a lower incidence of motor fluctuations and dyskinesia than levodopa/PDI (see below). Long-acting dopamine agonists are effective as monotherapy in early disease and as adjunctive therapy in late stage disease for patients who are experiencing motor fluctuations on levodopa/PDI. In early disease, dopamine agonist monotherapy provides symptomatic benefit comparable to levodopa/PDI. Dopamine agonists reduce 'off' time, improve motor function, and allow levodopa dose reductions. When a dopamine agonist is added to levodopa/PDI, it may be necessary to reduce the levodopa dose if dopaminergic effects such as peak dose dyskinesia or hallucinations emerge or worsen. However, as the disease progresses, dopamine agonists alone become insufficient to control symptoms and no longer provide as much symptomatic benefit as levodopa/PL)I. Dopamine agonists are generally less well tolerated than levodopa/PDI. Side effects, such as postural hypotension, nausea and vomiting, and confusion can be minimized by introducing dopamine agonists at a low dose and slowly escalating, in order to identify most of those that do occur when they are mild. Rarely, an elderly patient will experience a dramatic side effect, such as syncope, with the first intake despite starting with the lowest dose. Although it is commonly stated that

the elderly are more prone to side effects from dopamine agonists than levodopa/carbidopa, to our knowledge this has not been systematically evaluated. Patients with dementia are prone to hallucinations and increased confusion, and patients with low blood pressure or orthostasis may experience an exacerbation of hypotensive symptoms. Beyond these examples it is generally difficult to predict who will encounter side effects from a dopamine agonist. In our experience, many nondemented elderly patients tolerate dopamine agonists quite well and derive clinical benefit (Hindle et al. 1998). Bromocriptine Bromocriptine is an ergot alkaloid synthetic cyclic derivative of lysergic acid, with

both pre and postsynaptic effects. It was originally developed as a prolactin inhib-

itor, but was also found to have antiparkinsonian activity (CaIne et al. 1974). Bromocriptine is a strong D2 receptor agonist and a weak D2 receptor antagonist. It is rapidly absorbed from the gastrointestinal tract, strongly bound to plasma protein, and undergoes 90% first pass metabolism. Peak plasma levels are achieved in 30 to 210 minutes and its half life is approximately seven hours. Side effects include nausea, orthostatic hypotension, and psychiatric symptoms.

Bromocriptine monotherapy improves signs and symptoms of early PD (Bromocriptine Multicentre Trial Group 1990). However, it is less effective than

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Drug treatment levodopa over the long-term. Olanow (1988) found that bromocriptine monother-

apy provided symptom reduction comparable to levodopa/PDI for six months but less benefit than levodopa/PDI after six months. One retrospective study that has been criticized on methodological grounds found that oniy one third of patients retained an adequate response on bromocriptine alone after three years. However, patients treated with bromocriptine were reported to have a lower incidence of motor fluctuations and peak dose dyskinesia than those treated with levodopa (Rinne 1987). The most troublesome side effect of bromocriptine is postural hypotension. As many as 33% of patients will experience dizziness or lightheadedness when bromocriptine is first introduced. Nausea is also a common problem. Neuropsychiatric side effects include hallucinations, confusion, nightmares, agitation, and mood changes. Bromocriptine causes psychiatric disturbances more commonly than levodopa (Montastruc et al. 1993), which limits its use in elderly patients with preexisting cognitive impairment. Other side effects include burning dysaesthesiae and livedo reticularis, a rash of the lower extremities. Rarely, pleural effusion, erythromelalgia, pulmonary and retroperitoneal fibrosis, and peripheral vascular disease have been described (LeWitt and CaIne 1982). We start bromocriptine at a dose of 1.25mg per day and titrate up to 10mg per day over one month. Further titration is based on clinical response and side effects. The usual maximum dose is 30—40mg per day. Pergolide Pergolide is a semisynthetic clavine ergoline agonist that stimulates both Dl and

D2 receptors (Goldstein et al. 1980). It is roughly 10 times more potent than bromocriptine on amg formg basis and is a strong D2 receptor agonist and a weak Dl receptor agonist. As with bromocriptine, pergolide is highly bound to plasma proteins and undergoes extensive first pass metabolism. Peak plasma levels are achieved in one to two hours, and its half life is 20—27 hours. For unexplained reasons, the half life of pergolide, derived from the suppression of prolactin secretion, is far longer than the therapeutic action of the drug in PD, which is at most around 6 hours. Pergolide is effective as monotherapy in early disease and as an adjunct to levodopa/PDI in advanced disease (Mear et al. 1984, Olanow et al. 1994). Pergolide reduced 'off' time by 32% compared with 4% with placebo in a double-blind study comparing pergolide to placebo as an adjunct to levodopa in patients with motor

fluctuations (Olanow et al. 1994). Motor function improved 35% in pergolide treated patients compared with 17% in placebo treated patients (P<0.001). Pergolide permitted a significant mean levodopa dose reduction of 24.7% compared with 4.9% in the placebo group. A single blind, crossover study found that

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pergolide provided significantly greater benefit than bromocriptine in PD patients

experiencing a declining response to levodopa (Pezzoli eta!. 1994). Side effects of pergo!ide are similar to those of bromocriptine. We introduce pergo!ide at a dose of 0.25mg per day and s!ow!y increase to approximately 0.25mg three times daily over severa! weeks. Further titration is undertaken based on clinical response. The usual maximum dose is 3—4mg per day. Cabergoline Cabergoline is an ergot dopamine agonist with strong D2 and weak Dl receptor

affinity. Its plasma half life is approximately 65 hours, thereby allowing once a day dosing. Cabergoline is introduced at a dose of 0.5mg/day and slowly escalated to a maximum dose of 4—5 mg/day. A one year comparison of cabergoline to levodopa as early monotherapy found that over 80% of patients in each group experienced

>30% improvement in motor disability (Rinne et al. 1997). A total of 38% of cabergoline treated patients required levodopa supplementation by one year. Patients remaining on monotherapy exhibited clinical improvement comparable to those taking levodopa. As an adjunct to levodopa/carbidopa in suboptimally controlled patients, cabergoline provided significantly more 'on' time at six months in comparison to placebo and the cabergoline group was taking 18% less levodopa (Hutton et al. 1993). Pramipexole Pramipexole is a synthetic amino benzathiazol non-ergot agonist that binds with

high affinity to the D2 family of dopamine receptors, especially the D3 receptor (Mieraau and Schingnitz 1992). It has little affinity for Dl, 5HT, muscarinic, or adrenergic receptors. Pramipexole is rapidly absorbed, and reaches peak concentration in about two hours. It is 15% bound to plasma protein. The half life is approximately eight hours in young volunteers and 12 hours in elderly volunteers. Urinary excretion is the major route of elimination, and 90% of pramipexole is recovered in the urine unchanged. Renal insufficiency decreases elimination. Pramipexole clearance is about 75% lower in patients with severe renal impairment and 60% lower in patients with moderate renal insufficiency. Pramipexole is effective as early monotherapy and as an adjunct to levodopa in advanced disease. In a large, prospective, double-blind trial comparing pramipexole to levodopa in early disease, pramipexole significantly improved motor scores compared with pretreatment values (Shannon et al. 1997). A six month trial comparing pramipexole to placebo as add on therapy in levodopa treated patients with motor fluctuations found that pramipexole reduced 'off' time by 31% compared with 7% in placebo treated patients (Lieberman et al. 1997). Motor function was

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Drug treatment improved by 25% in the pramipexole group compared to 12% in the placebo

group. Pramipexole permitted a 27% reduction in levodopa dose compared to 5% in the placebo group. Pramipexole is introduced at a dose of 0.125mg/day and increased to 0.5mg three times daily over one month. The usual maximum dose is 3 to 4.5 mg/day. Side effects include hallucinations, somnolence, nausea, constipation, and insomnia (Shannon 1996). As a non ergot dopamine agonist, pramipexole should not cause

ergot related side effects such as erythromelalgia, and the rare but potentially serious complications of pleural and retroperitoneal fibrosis. Ropinirole

Ropinirole is a nonergoline doparnine agonist that binds selectively to D2 receptors

with little affinity for Dl, 5HT, muscarinic, or adrenergic receptors (Eden et al. 1991). It is rapidly absorbed and extensively metabolized by the liver. The elimination half life of ropinirole is approximately six hours. Maximal plasma concentration is reached after approximately one and a half hours in fasting patients and after approximately four hours when taken with meals. Ropinirole clearance is reduced by 30% in elderly patients. Ropinirole is effective as early monotherapy and as an adjunct to levodopa/PDI in advanced disease. In a six month study comparing ropinirole to placebo in early PD, motor function was significantly improved, by 24% at six months in ropinirole treated patients compared to a 3% worsening in placebo treated patients (Adler et al. 1997). Significantly fewer ropinirole treated patients required the introduction

of levodopa/PDI (11% vs. 29%). A six month study (Ropinirole Study Group 1996), comparing ropinirole with bromocriptine as early monotherapy, found ropinirole alone provided significantly greater improvement than bromocriptine alone (34% vs. 20%). Another six month study (Rascol 1996) comparing ropinirole to levodopa in early disease found that a similar percentage of patients experi-

enced >30% improvement (48% vs. 58%), although levodopa treated patients experienced significantly greater improvement overall (32% vs. 44%). Ropinirole reduces 'oft' time, improves clinical function and permits levodopa dose reductions in patients with fluctuating disease. In a comparison of ropinirole to placebo as adjunctive therapy to levodopa, 27.7% of ropinirole treated patients had at least a 20% reduction in levodopa dose and at least a 20% reduction in 'off' time at six months compared to 11% in the placebo arm (Kreider et al. 1996). Ropinirole is introduced at a dose of 0.25mg per day, with an initial target dose of 1 mg three times daily reached over one month. The usual maximum is 6—8mg three times daily. Side effects are similar to those of the other currently available non-ergoline dopamine agonist pramipexole.

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CH3O

COMT

HO

R

SAM Mg Fig. 9.1

SAH

Methylation of catechol substrate by COMT (catechol-O-methyltransferase). SAM, S-

adenosyl-i-methionine; SAH, S-methyl-L-homocysteine; R, side chain. L-dopa +

DDC inhibitor + COMT-inhibitor

Circulation

Wood—brain

barrier

— COMT inhibitor Fig.

9.2

DDC inhibitor

Mechanism of peripheral COMT inhibition. Reprinted with permission from Kaakkola et al. (1996).

COMT inhibitors Two inhibitors of catechol-O-methyltransferase (COMT), tolcapone and entaca-

pone, have been developed and are available in some countries for clinical and trial use. COMT is one of the primary enzymes responsible for the catabolism of levodopa. COMT catalyses the transfer of a methyl group from S-adenosyl-i-methio-

nine (SAM) to the hydroxyl group of a catecholamine (see Fig. 9.1). When levodopa/PDI is administered, COMT metabolism of levodopa to 3—0-methyldopa (3—OMD) is prominent. 3—OMD has no antiparkinsonian activity and may marginally decrease levodopa absorption and transport across the blood—brain barrier (Nutt et al. 1987). Peripheral COMT inhibition blocks the peripheral metabolism of levodopa to 3—OMD, thereby extending the area under the time concentration curve and making more levodopa available for transport across the blood brain—barrier (see Fig. 9.2). When a COMT inhibitor is added to levodopa/PDI therapy, striatal dopamine levels are increased for longer. Central COMT

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Drug treatment

inhibition might further maintain striatal dopamine levels by blocking the central metabolism of dopamine to homovanillic acid. COMT inhibitors reduce 'off time, improve motor function, and allow levodopa dose reductions in advanced patients with motor fluctuations on levodopa. Tolca pone

Tolcapone is a reversible COMT inhibitor, with both peripheral and central activ-

ity (Zurcheretal. 1990, Mannisto and Kaakkola 1990, Spencer and Benfield 1996). It is rapidly absorbed and has a half life of approximately two hours. Tolcapone has been demonstrated to improve motor function and allow levodopa dose reductions in patients on levodopa with either a stable or fluctuating response. A six month double-blind, placebo-controlled trial evaluating tolcapone in PD patients experiencing a stable response to levodopa/carbidopa found that tolcapone significantly

improved motor function and decreased mean total daily levodopa doses compared to placebo (Waters et al. 1997). In patients with motor fluctuations on levodopa/PDI, a comparison of tolcapone (200 mg three times daily) to placebo found a significant mean reduction in 'off' time of 48% in the tolcapone group compared to 20% in the placebo group at three months (Rajput et al. 1997). Tolcapone allowed a 24% reduction in levodopa dose compared with a 1.6% increase in placebo treated patients. Another double-blind placebo-controlled study evaluating tolcapone in patients experiencing motor fluctuations on levodopa found tolcapone reduced 'off time by 19% (Kurth et al. 1997). The central effects of tolcapone do not seem relevant to its actions in PD, as drug naïve patients do not improve on monotherapy with tolcapone (Hauser et al. 1998). The suggested possible antidepressant action of tolcapone needs to be confirmed. Tolcapone is usually introduced at a dose of 100mg three times daily. Side effects

of tolcapone are mostly those related to increased dopaminergic stimulation and include dyskinesia, nausea, hallucinations and hypotension. Dopaminergic side effects can usually be improved by decreasing the levodopa dose. Patients with peak

dose dyskinesia often experience a rapid and substantial increase in dyskinesia requiring a 25—50% reduction in levodopa dose. Other side effects include dizziness and urine discolouration. Of note, approximately 10% of patients experience diarrhoea and 3% discontinue tolcapone because of this side effect. Onset of diarrhoea usually occurs four to 12 weeks after initiation of therapy, but is uncommon after six months (Waters et al. 1997). There has been recent concern over the development of abnormal liver function tests in patients taking tolcapone and rare cases of fatal hepatic failure. In the US the recommendation has been made to monitor liver function tests regularly in patients on this drug and to use the drug only after other treatments have failed. In the European Union the product licence for tolcapone has been suspended and the drug withdrawn.

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Entacapone

Entacapone is a reversible, peripheral COMT inhibitor that is less potent than tolcapone (McNeely and Davis 1997). Entacapone is rapidly absorbed and has an

elimination half life of two to three hours (Mannisto et al. 1992, Myllyla et al. 1992, Nissinen et al. 1992). It is usually administered at a dose of 200mg with each levo-

dopa/PDI intake. Entacapone reduces 'off' time, improves clinical function, and allows levodopa dose reductions in patients with motor fluctuations on levodopa. In a six month study, entacapone significantly increased 'on' time. At the end of the six months, entacapone treated patients experienced a 1% improvement in motor function while taking 13% less levodopa, compared to placebo treated patients whose motor function had worsened by 9%, while taking 3% more levodopa (Kieburtz et al. 1996). The shorter duration of action of entacapone means that it must be administered with each dose of levodopa. This may be an advantage if fine adjustment of levodopa plasma levels is needed over the day. Side effects are very similar to those of tolcapone, though diarrhoea may be less of a problem. Studies thus far have not suggested that entacapone causes abnormal liver function (see tol-

capone) and monitoring of liver function does not appear to be necessary in patients on this drug. Monoamine oxidase-B inhibitors Selegiline (deprenyl, EldeprylTM), in oral doses up to 10mg/day, is a selective and irreversible inhibitor of monoamine oxidase type B (MAO-B). It is generally not associated with sympathomimetic crises ('cheese effect') caused by nonselective monoamine oxidase inhibitors and the concomitant ingestion of tyramine or other monoamines. MAO- B inhibition partially blocks dopamine metabolism, thereby increasing striatal dopamine. Selegiline may also stimulate dopamine synthesis and inhibit dopamine reuptake. Selegiline's plasma half life is approximately 40 hours, but loss of activity is dependent on the generation of new MAO-B enzyme in the brain. Selegiline may therefore provide clinical effects for several months after discontinuation, though rapid clinical deterioration in disease control has been seen when selegiline is rapidly withdrawn. Selegiline is usually administered at a dose of 5 mg with breakfast and lunch. It is given early in the day to minimize the likelihood of insomnia, which could be caused by its amphetamine metabolites. As monotherapy in early disease, selegiline provides modest symptomatic benefit and delays the need for levodopa (Parkinson Study Group 1993). In patients with motor fluctuations on levodopa/PDI, selegiline reduces 'off' time and improves clinical response (Presthus and Hajbe 1983). When used as an adjunct to levodopa/PDI, selegiline can exacerbate dopaminergic side effects including dyskinesia, thereby necessitating a reduction of the levodopa dose. Other possible side effects include gastrointestinal distress, insomnia,

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Drug treatment confusion or hallucinations, increased liver enzymes, and rarely, peptic ulcer

disease (Golbe 1989). Controversy exists as to whether selegiline confers a neuroprotective effect and whether in older patients selegiline is associated with increased mortality. In animal models selegiline promotes protein synthesis that inhibits apoptosis, a type of programmed cell death. However, a neuroprotective effect has yet to be clearly demonstrated in PD. An open label, prospective study from the P1) Research Group of

the United Kingdom found that selegiline, in combination with levodopa, was associated with more deaths than levodopa alone (Lees et al. 1995). However, the conclusions of this study are limited by a variety of methodological shortcomings and this finding has not been observed in other studies. Further analysis of mortality in this study has shown that the increase in mortality in the levodopa/selegiline arm is still increased, but was no longer significantly greater than in the levodopa

monotherapy arm (Ben-Shlomo et al. 1998). Elderly patients in the combined treatment arm with a history of falls and dementia were more likely to die than similar patients on levodopa alone. Analysis of mortality in the DATATOP study did not show any increased mortality in patients assigned to selegiine combined with levodopa (Parkinson Study Group 1998). It would seem prudent to avoid the combination of levodopa with selegiline in frail elderly patients and to withdraw selegiline slowly in similar patients already taking this combination. Selective serotonin reuptake inhibitors (SSRIs), either alone or in combination with monoamine oxidase inhibitors such as selegiline, can theoretically cause a serotonin syndrome

characterized by myoclonus, tremor, diaphoresis, incoordination, mental status changes and possibly death. However, the occurrence of the serotonin syndrome in patients receiving both selegiline and a SSRI has not been reported and does not appear to be a clinically relevant issue (Waters 1994, Richard et al. 1997).

Amantadine Amantadine, or 1-amino-adamantine, is an antiviral medication found to provide

antiparkinsonian benefit (Schwab et al. 1969). Its exact mechanism of action is unknown, but it may increase dopamine release, block dopamine reuptake, stimulate dopamine receptors, block n-methyl-d-aspartate receptors and have anticholinergic activity. Amantadine is excreted unchanged in the urine. It is well absorbed, and has a half-life of approximately 24 hours in young volunteers. The elimination half life correlates with creatinine clearance, and is approximately twice as long in the elderly. Amantadine provides modest benefit for bradykinesia and rigidity (Butzer et al. 1975), and has less effect on tremor than anticholinergic drugs. Amantadine can be used as monotherapy in early PD or as an adjunct to levodopa/PDI in moderate and advanced disease.

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Amantadine is administered at a maximum dose of 100mg three times daily.

Several case reports suggest that the withdrawal of amantadine may be associated with dramatic worsening of parkinsonian features. The side effects of amantadine, especially confusion and hallucinations, limit the usefulness of this drug in older patients (Schwab et al. 1969). Anticholinergics Anticholinergic (antimuscarinic) medications can be useful to treat tremor in young physiologically fit patients with PD, but their use requires very careful consideration. They have little or no place in the treatment of late stage or late onset PD and should be avoided in frail elderly patients. Those currently in use are structural analogues of atropine and are muscarinic antagonists. Anticholinergics were initially found to have beneficial effects in PD when they were used by Charcot to control sialorrhea. Today they are mostly used to treat isolated or treatment-resis-

tant tremor. Trihexyphenidyl, cogentin, and benztropine are currently the most common anticholinergic medications used for the treatment of PD. They can delay gastric emptying, thereby slowing the delivery of levodopa to the small intestine. Anticholinergics have a greater incidence of side effects than levodopa. Elderly patients are especially susceptible to these side effects, which include confusion

(Broe and Caird 1973), memory impairment, hallucinations and sedation. Cognitive impairment has been demonstrated in health young volunteers given this class of drugs. The risk of neuropsychiatric side effects increases with increasing dose, age, and coexistent dementia (Robertson and George 1990). Anticholinergics may also cause constipation, visual blurring, dry mouth and urinary difficulties (particularly in men with prostatism). They should be used with caution in patients with narrow angle glaucoma. The anticholinergics are best introduced at a low dose and slowly escalated. Trihexyphenidyl is started at a dose of 1 mg/day and increased gradually to as high as 2mg four times daily. Benztropine is introduced at a dose of 0.5 mg/day and escalated to as high as 4—6 mg/day in divided doses. Anticholinergic treatment should never be withdrawn suddenly.

Theoretical considerations in the treatment of PD in elderly patients Is selegiline neuroprotective? That selegiline might slow the progression of PD was initially attributed to its

ability to inhibit MAO-B. MPTP (1 -methyl-4-phenyl- 1,2,3 ,6-tetrahydropyridine) is a protoxin that causes dopamine cell death and induces parkinsonism in animals and man, because it is oxidized to the neurotoxin MPP' (1-methyl-4-phenylpyridinium ion) by MAO-B (see Fig. 9.3). When selegiline is administered prior to

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Drug treatment CH3

fl N

MPTP

Fig. 9.3

..

Selegilune y

MAO-B

CH3

ifl MPP'

Oxidation of MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) to MPP I (1-methyl-4-

phenylpyridinium ion) is inhibited by selegiline, a selective inhibitor of MAO-B.

and parkinsonian symptoms are not elicited (Heikkila eta!. 1984). lfthere is an environmental agent similar to MPTP that causes PD in man, selegiline might prevent its oxidation and protect against dopamine cell damage. Similarly, if free radical formation from the oxidative metabolism of dopamine by MAO-B contributes to disease progression, inhibition of MAO-B by selegiline may reduce free radical formation and slow dopamine cell degeneration. An early retrospective study found that patients taking selegiline lived longer than those not taking selegiline (Birkmeyer et al. 1985). Based on these early observations, the Parkinson Study Group examined the ability of selegiline and tocopherol (vitamin E), alone or together, to slow the progression of PD (Parkinson Study Group 1993). The study conclusively demonstrated that selegiline in early PD delays the need for levodopa therapy and is MPTP, MPTP is not converted to

consistent with the hypothesis that selegiline may slow disease progression. However, the study also found that selegiline alone provided a small symptomatic

benefit and it cannot be excluded that the delay in need for levodopa was due entirely or in part to this small symptomatic effect. Another study designed to minimize symptomatic effects also suggested that selegiline slows progression of disability in PD (Olanow et a!. 1995). However, it is clear that selegiline does not stop disease progression and several studies have found that after several years of treatment, selegiline treated patients experience comparable disability to patients not treated with selegiline. Although several clinical studies have yielded results that are consistent with a neuroprotective action, this has yet to be conclusively demonstrated in PD patients. When selegiline monotherapy is used in early PD it is in the hope that dopamine neuronal degeneration may be slowed and that a delay in the introduction of levodopa may be associated with long-term benefit.

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Medication strategies in the treatment of PD in elderly patients General principles The signs and symptoms of P1) and their effect on function and handicap must be

evaluated in each patient to determine the need for treatment changes at any point in time. For any treatment under consideration, the relative likelihood of benefit versus the risk of side effects must be considered. Symptoms of the disease will

advance despite best medical management, and drug dosages will need to be adjusted as the disease progresses. Several guidelines for the drug treatment of PD have been developed (Koller et al. 1994, Bhatia et al. 1998). We make only one medication change at a time so that the effects of that change are clear. Symptomatic medications are initiated at a low dose and escalated slowly so that most side effects that do occur will be mild and appropriate action can then be taken. The optimal medication dose is the lowest one that will maintain adequate function for the patient. Treatment strategies in early PD For older patients, we place less emphasis on long-term theoretical considerations and focus on providing adequate symptomatic benefit in the near term with the fewest possible side effects. If a medication provides neuroprotective effects in PD, it is likely to do so throughout the course of the disease and it should be adminis-

tered from the time of diagnosis onward. We undertake a discussion with our patients to review current information regarding selegiline and interest in its potential neuroprotective effects. It is clear that selegiline administration in early disease will delay the need for symptomatic therapy. The younger the patient, the more critical is the need for therapy that will slow disease progression, and the more likely we are to initiate selegiline from the time of diagnosis. Symptomatic therapy is introduced when a patient experiences functional disability. If disability is due to bradykinesia, rigidity, decreased fine coordinated movements, soft speech, or shuffling gait, a dopaminergic medication should be introduced. Most patients require symptomatic therapy within one to two years after diagnosis. Tremor is variably responsive to drug treatment (Koller et al. 1994). If bradykinesia or rigidity are present in addition to tremor, we introduce a dopaminergic medication and monitor the clinical response. If troublesome tremor occurs in isolation or does not respond to dopaminergic medication, an anticholinergic can be prescribed cautiously, though is best avoided in elderly patients. Disabling tremor not responsive to other medications warrants a trial of clozapine (Friedman and Lan non 1990, Friedman et al. 1997). A surgical procedure such as thalamotomy or thalamic stimulation may be considered for medically refractory disabling tremor.

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Drug treatment In younger patients requiring more treatment to control akinesia and rigidity we

prefer to initiate therapy with a dopamine agonist. For patients with dementia and older individuals who may be prone to side effects from dopamine agonists, we generally initiate symptomatic therapy with levodopa/PDJ and rely more heavily on it throughout the disease. As a guideline we use early dopamine agonist monotherapy and a levodopa sparing strategy in patients under 65 years of age and rely on levodopa in patients over 70 years of age. For patients between 65 and 70 we make a judgment based on their general health and cognitive status. The more

robust and cognitively intact, the more likely we are to use dopamine agonist monotherapy followed by combination therapy (dopamine agonist plus levodopa/PDI) when necessary. We usually introduce levodopa therapy using the controlled-release formula-

tion. Fewer daily dosings may be required than with standard levodopa/PDI, thereby affording greater convenience. In addition, co-careldopa CR has been demonstrated to provide significantly better improvement in activities of daily living than standard formulation in a long-term study (Block et al. 1997). Most patients with bradykinesia and rigidity will experience an obvious reduction in symptoms once levodopa/PDI therapy has been underway for several weeks.

If no improvement is apparent, the reason why should be carefully considered. Focus may be placed on the wrong symptom, the diagnosis may be incorrect, or the levodopa dosage may be too low. Tremor may or may not respond to levodopa. If symptoms are minimal, improvement may be difficult to detect. For patients with moderate disability, the levodopa dose should be slowly escalated until symptoms are reduced or side effects are encountered. Failure to respond to levodopa raises serious doubts about the diagnosis of PD.

COMT inhibitors smooth levodopa serum fluctuations, and augment and extend the clinical response to levodopa/PDI. They are not known to provide efficacy except as adjuncts to levodopa/PDI therapy. We favour introducing a COXIT inhibitor at the time levodopa/PDI therapy is begun. This may allow lower levodopa dosages and fewer levodopa administrations through the day. In addition,

there is interest as to whether providing smoother levodopa-derived dopamine stimulation will afford a better long-term outcome and forestall motor fluctuations and dyskinesia. This strategy is usually well tolerated by both younger and older individuals. Treatment strategies for motor fluctuations in PD PD will continue to progress despite treatment with symptomatic medications. As clinical signs and symptoms increase, they may be controlled by increasing the levodopa/PDI dose, or by adding adjunctive medications including dopamine ago-

nists, COMT inhibitors, selegiline, or amantadine. We attempt to maintain the

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1. A. Zesiewicz and R. A. Hauser PROGRESSION OF CLINICAL RESPONSE

Early disease

M?derate disease

A

A

A

A

A

Advanced disease Fig. 9.4

Progression of clinical response. Despite the short half-life of levodopa/PDI, patients with early disease experience a sustained response through the day. As the disease progresses,

patients begin to notice 'wearing off' fluctuations, with the benefit of levodopa/PDI wearing off after a few hours. Ultimately, dinical response fluctuates more and more closely in association with peripheral levodopa and patients develop choreiform dyskinesias when dopamine peaks. Arrows indicate time of levodopa/PDI administration. Reprinted with permission from Hauser and Zesiewicz (1997b).

levodopa/PDI dose at or below 600mg per day for as long as reasonable ftmction can be maintained. Ifa patient experiences progression of symptoms on a low dose of levodopa/PDI, we prefer to add adjunctive medication rather than increase the levodopa dose.

End of dose / 'wearing off' motor fluctuations

Over time, nearly all patients regardless of age of onset of PD develop 'wearing off / end of dose' motor fluctuations (see Fig. 9.4) in response to levodopa/PDI (Chase et al. 1993). Patients will notice that levodopa provides benefit for a few hours and then wears off as bradykinesia, rigidity and tremor return. Wearing off motor fluctuations alone are relatively easy to treat. They can be alleviated by increasing the levodopa/PUI dose, administering levodopa/PDI doses more frequently, switching from standard levodopa/PDI to a controlled-release formulation, or by adding a dopamine agonist, COMT inhibitor, or selegiline (see Fig. 9.5). Adjustment of the antiparkinsonian medication regimen should eliminate 'off' time unless increasing doparninergic stimulation causes an intolerable side effect such as peak dose dyskinesia, hallucinations, orthostatic hypotension or somnolence.

Patients with prominent motor fluctuations and those who find they turn 'off' after a meal may benefit from a low protein or protein redistributed diet (Carter et al. 1989). Minimizing serum protein fluctuations reduces the variability of levodopa transport and helps stabilize the clinical response. Patients with both motor fluctuations and troublesome peak dose dyskinesia

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Drug treatment

(A)

(B)

(C)

Fig. 9.5

Management of a patient with motor fluctuations and no dyskinesia (A). Off time can be reduced by using a higher levodopa dose (B), switching to a long-acting preparation (B), adding a dopamine agonist, COMT inhibitor or selegiline (B), or by shortening the interdose interval (C). Arrows indicate time of levodopa administration. Reprinted with permission from Hauser and Zesiewicz (1997b).

present a challenging management dilemma. Peak dose dyskinesias occur at the

peak of the dosing cycle, coincident with high levels of dopamine, and consist of choreiform, twisting turning movements. They are exacerbated by increasing dopami nergic stimulation and diminished by reducing dopaminergic stimulation. Most patients prefer to be 'on' with mild peak dose dyskinesia than to be 'off'. Dyskinesia that does not cause functional difficulty or distress may not require a reduction of dopaminergic medication. However, dyskinesia can be severe in amplitude and as disabling as 'off' time (Hauser et al. 1997b). Increasing dopaminergic medication in these patients will increase disability from dyskinesia and decreasing dopaminergic medication will increase 'off' time. For patients with both

motor fluctuations and troublesome peak dose dyskinesia, it is important to attempt to maximize good functional time (i.e. time 'on' without dyskinesia or with nontroublesome dyskinesia). We find it very helpful to have patients complete a 24 hour home 'diary' (see Fig. 9.6) indicating their parkinsonian status at half hour intervals to understand their response to medication and the proportion of the day that they are under or over treated (Hauser et al.1997a). For patients with motor

fluctuations and dyskinesia we attempt to provide the most stable as possible dopamingeric stimulation within the target zone (see Fig. 9.7). Other motor fluctuations Other types of dyskinesia in PD such as wearing off dystonia and diphasic dysto-

nia/dyskinesia are less commonly seen in elderly patients. Wearing off dystonia

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PARKINSON'S DISEASE DIARY NAME Instructions: For each half-hour time period place one check mark to indicate your predominant status during most of that period. ON =Time when medication is providing benefit with regard to mobility, slowness, and stiffness. OFF =Time when medication has worn off and is no longer providing benefit with regard to mobility, slowness, and stiffness. Dyskinesia = Involuntary twisting, turning movements.These movements are an effect of medication and occur during ON time. Non-troublesome dyskinesia does not interfere with function or cause meaningful discomfort.Troublesome dyskinesia interferes with function or causes meaningful discomfort. Tremor is shaking back and forth and is not considered dyskinesia.

ON with non-troublesome dyskinesia

ON

Time

Asleep

OFF

without dyskinesia

6:00AM

ON with troublesome

:30

7:00 PM

ON with non-troublesome

ON with troublesome

dyskinesia

dyskinesia

dyskinesia

:30

8:00AM

8:00 PM

.

:30 9:00 AM :30

:30

9:00 PM

10:00AM

:30 10:00 PM•

.

:30

:30 11:00 PM

11:00AM •

:30

.

12:00AM

•

:30 1:00 PM :30 2:00 :30 3:00 :30 4:00 :30 5:00 :30

OFF

6:00 PM

:30

:30

Asleep

dyskinesia

:

7:00 AM :30

12:00PM

without

ON

Time

:30

1:00AM :30 !

PM

2:ObAM

PM

3:00 AM

:30 :30

•

4:00 AM

PM

:30 PM

Fig. 9.6

•

5:00AM

.

!

:30

Parkinson's disease diary. Used with permission, copyright

R. A.

Hauser (1997).

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Drug treatment

(A)

DYSKINESIA (B)

ON

(C)

Fig. 9.7

Management of a patient with motor fluctuations and dyskinesia. The treatment of patients with motor fluctuations and peak dose dyskinesia (A) generally involves providing less levodopa more frequently. The levodopa dose should be lowered until it brings on only mild dyskinesia (B). The time to wearing off then determines the interdose interval (C). Arrows indicate times of levodopa administration. Reprinted with permission from Hauser and Zesiewicz (1997b).

occurs in association with low or falling dopamine levels (McHale et a!. 1990). It

can occur early in the course of the disease and consists of involuntary, sustained, painful muscle contractions, often manifest as foot inversion or plantar flexion in the early morning hours or at times when dopaminergic medication has worn off. Wearing off dystonia may be improved by providing more sustained dopaminergic stimulation. This can be accomplished with controlled-release levodopa/PDI preparations, adding a COMT inhibitor to levodopa/PDI therapy, or by introducing a dopamine agonist. Diphasic dystonia/dyskinesia (D-I-D dystonia) is uncommon, and occurs at the beginning and end of the levodopa cycle. It may appear as a combination of dystonia and chorea, and commonly affects the lower extremities. D-I-D dystonia can be difficult to treat, but attempts should be made to increase and smooth dopamninergic stimulation so as to avoid 'turning on' and 'wearing off' fluctuations.

Freezing is the momentary inability to walk. It can cause troublesome gait difficulty and may affect up to one-third of patients with longstanding disease (Giladi et a!. 1992). Turning, walking through a doorway or anxiety often triggers freezing. If freezing occurs during 'off' periods, it can be improved by eliminating or minimizing 'off' time by increasing dopaminergic stimulation. However, the management of'on' period freezing is usually disappointing. Motor 'tricks' such as

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marching in place or walking over masked tape placed across a hallway may be helpful.

Treatment of the secondary symptoms of PD Sleep abnormalities

problems are common in PD, affecting 75—98% of patients. Such problems usually take the form of fragmented sleep with frequent awakenings and result in excessive daytime sleepiness. Difficulty turning over in bed, leg cramps, nightmares, dystonia, dyskinesia and leg jerks are also frequently reported. Nocturia is a common cause of poor sleep maintenance, but motor problems, anxiety and Sleeping

depression may also play a role. Sleep abnormalities, if bothersome, should be evaluated with an overnight polysomnography (PSG).

In older patients, chronic use of sedative hypnotics is generally not recommended because of possible cognitive side effects, next day residual effects, physi-

cal dependence and tolerance. When a hypnotic is required, the short acting zolpidem, an imidazopyridine that acts at the benzodiazepine receptor, has the advantage of causing relatively little hangover effect. Patients are asked to try to sleep each night without hypnotic medication, but if they are unable to sleep to take the medication. Some patients require regular hypnotic medication. If a patient complains of fragmented sleep due to nocturnal leg cramps or dystonia, a bedtime dose of a sustained release levodopa formulation or a long-acting dopamine agonist such as cabergoline may be useful. If vivid dreams and nightmares or dyskinesias are disturbing sleep, bedtime antiparkinsonian drugs may need to be stopped or reduced. It is important to recall that selegiine, which is metabolized to amphetamines, given too late in the day may lead to nightmares and insomnia. Depression, which is contributing to a sleep disorder, should be treated with an antidepressant, and tricyclics such as amitriptyline may reduce depression while promoting sleep. Urinary incontinence

Symptoms of urinary dysfunction in PD include urgency, frequency and nocturia.

Patients complaining of urinary symptoms should be evaluated by a urologist for prostatism and other structural problems, and undergo cystometric studies. The

most common type of urinary dysfunction in PD is detrusor hyperreflexia (Andersen et al. 1976, Pavlakis et al. 1983). The extrapyramidal system generally exerts an inhibitory effect on micturition, and the basal ganglia abnormalities of PD may cause detrusor dysfunction. Anticholinergic drugs are the mainstay of treatment for urge incontinence. Unfortunately, in elderly subjects these drugs often lead to significant side effects

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including cognitive impairment, constipation that can itself worsen bladder flinc-

tion, and postural hypotension. Anticholinergic drugs such as oxybutynin and propantheline may be used cautiously to improve incontinence. Newer drugs such as tolterodine and propiverin, both recently launched in the UK, may be associated with fewer central anticholinergic side effects. Patients with nocturia should be advised to maintain a good fluid intake of at least two litres per day, but should not take liquids late in the evening. Desmopressin, a synthetic analogue of vasopressin,

in the form of a nasal spray is useful for troublesome, refractory nocturia (Suchowersky et al. 1995). Urea and electrolytes need to be monitored in these patients as hyponatraemia and fluid overload can develop. Drooling

Drooling (sialorrhoea) is a late manifestation of PD that may occur in up to 70% of patients (Edwards et al. 1991). Drooling can potentially lead to serious problems

including aspiration. In PD drooling results from saliva pooling in the mouth owing to swallowing difficulty, rather than increased production of saliva. Treatment is commonly undertaken with anticholinergic medications that can reduce saliva production. However, these medications should be used with caution in the elderly, as they cause cognitive impairment, constipation, and dry mouth. The peripheral anticholinergic glycopyrrolate is useful to reduce saliva production with minimal cognitive side effects. Alternatively, increased dopaminergic therapy can improve swallowing and if tolerated may improve drooling. Seborrhoea

oiliness of the skin is a common problem in PD. Oiliness is most common in the forehead and central parts of the face (Flint 1977). Secretion of sebum, which is produced by sebaceous glands, is increased in patients with PD. Topical steroids and tar shampoo can be used daily for three days to treat seborrhea. Chronic use of topical steroids can lead to skin atrophy and should generally be avoided. A maintenance regimen consisting of frequent washing and the use of detergent shampoo will usually provide long-term benefit. Troublesome seborrhoea may warrant referral to a dermatologist. Excessive

Sexual dysfunction Impotence is a common problem in the elderly. Kinsey et al. (1948) reported that

by the age of 55 years, 7% of men are impotent, and by the age of 70 years, 27% are impotent. Verwoerdt et al. (1969) found that 35% of women aged 60—65 had no

interest in sex. Difficulty with sexual function is particularly common in PD; roughly 60% of male patients may experience erectile dysfunction (Singer et al. 1992).

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dysfunction usually has several causes, including decreased mobility, autonomic nervous system abnormalities, depression and side effects from drug treatment. Identification of the cause helps direct treatment. Antihypertensive medications including propranolol, clonidine, and methyldopa, as well as thiazide diuretics, digoxin and cimetidine, can cause sexual dysfunction. Antidepressants, especially the serotonin reuptake inhibitors, can also lead to impotence. If no cause is apparent, evaluation by a specialist should be undertaken. Vascular disease is a common cause of erectile dysfunction. The use of an externally applied device can help men achieve and maintain an erection. Sexual

Postural hypotension

of postural (orthostatic) hypotension are commonly encountered in PD (Senard et al. 1997). One definition of orthostatic hypotension is a drop of

Signs

30 mmHg in systolic blood pressure or a drop of 20 mmHg in mean blood pressure (diastolic pressure plus one third of the pulse pressure) when going from the supine

to standing position. Its mechanism in PD remains controversial. Recent data suggest that alpha-adrenergic supersensitivity may occur in response to low levels of plasma iioradrenaline (Senard et al. 1990), but the pathophysiology may involve a decreased ability to secrete renin (Barbeau et al. 1970), and impairment of baroreceptor reflexes. Neuropathological evidence of involvement of the autonomic nervous system in P1) has been described (Rajput and Rozdilsky 1976). Symptomatic postural hypotension usually causes feelings of dizziness or light headedness that occur during positional changes, but may cause unsteadiness, cognitive slowing, or other vague symptoms (H illen et al. 1996). Postural hypotension can cause significant morbidity and mortality by contributing to falls, limiting mobility, and interfering with rehabilitation efforts. Treatment of orthostatic hypotension includes discontinuing unnecessary medications, encouraging the patient to drink five or more glasses of fluid each day and adding salt liberally to the diet. Medical management includes the use of mineralocorticoids to increase intravascular volume (Hickler et al. 1959). Fludrocortisone is introduced at a dose of 0.1 mg once or twice a day, and can be increased to as high as 0.4 to 0.6mg daily. Supine hypertension and dependent oedema are common

side effects, and patients should be monitored for congestive heart failure. Midodrine is a peripherally acting alpha-I-agonist that causes vasoconstriction of both arterioles and venous capacitance vessels (McTavish and Goa 1989, Jankovic et al. 1993). The initial recommended dosage is 2.5mg twice or three times daily. The maintenance dose is as high as 40mg daily in divided doses. Side effects include scalp pruritus and tingling, pilomotor reactions, gastrointestinal complaints, headaches, and dizziness. It does not cross the blood—brain barrier, and is less likely

to produce central side effects than ephedrine. Midodrine is a selective alpha-

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Drug treatment adrenergic agonist, and is relatively free of beta adrenergic side effects, including

increased pulse rate. Several open label trials have found midodrine to control symptoms of orthostatic hypotension effectively in most patients (Kaufman et al. 1988).

Hallucinations Drug-induced changes in mental state, including hallucinations (mostly visual),

delusions, confusional states, and paranoid psychosis are common in elderly patients with PD (Goetz et al. 1982, Sage and Mark 1994, Sanchez-Ramos et al. 1996). The incidence of hallucinations is in the range of 20—33%. Elderly subjects may be particularly prone to hallucinations, especially in association with amantadine, anticholinergic, or dopamine agonist therapy. These medications appear to induce hallucinations and other psychiatric disturbances more readily than levodopa. Other possible risk factors for the development of hallucinations remain controversial, including disease duration, duration of levodopa exposure, and disease severity (Sanchez-Ramos et al. 1996). Hallucinations should be treated if they are bothersome to the patient or interfere with function, by gradual withdrawal of drugs most likely to cause or worsen this problem, such as amantadine, selegiline, and anticholinergics. Following this, dopamine agonist drugs and lastly levodopa itself may need to be reduced in dose or withdrawn. If hallucinations occur at night, bedtime doses of antiparkinsonian medications may be reduced or discontinued. An atypical neuroleptic drug, such as clozapine, may be useful in controlling hallucinations when antiparkinsonian medication cannot be reduced. Clozapine predominantly antagonizes mesolimbic pathways rather than nigrostriatal pathways and causes fewer extrapyramidal side effects than traditional neuroleptics (Doraiswamy et al. 1995). Potential side effects include somnolence, orthostatic hypotension and hypersalivation. It also carries a risk for agranulocytosis, necessitating weekly complete blood counts. Patients with PD require much smaller doses of clozapine than that used to treat schizophrenia. We start patients on a small chip of a clozapine tablet (— 3 mg, or one eighth of a 25mg tablet) and slowly escalate. Most patients are controlled on 12.5—50mg clozapine per day. For patients who cannot or will not comply with weekly blood monitoring, instead of clozapine we use the atypical neuroleptic risperidone, although it can worsen the motor signs of PD. Depression Depression (see Chapter 3) is the most common mood disturbance in PD, affecting

40—50% of patients (Cummings 1992). Tricyclic antidepressants, selective serotonm reuptake inhibitors (SSRIs), and atypical antidepressants have all been found

to improve depression in PD (Laitenen 1969, Andersen et al. 1980, Goetz et al.

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Consistent with their popularity in the general population, SSRIs are probably the most commonly used antidepressants in PD in the US. However, several case reports have noted worsening of parkinsonian symptoms during treatment with some SSRIs (Jansen-Steur 1993, Jiminez-Jiminez et al. 1994). This has not 1984).

been confirmed in population studies or open label studies of sertraline in depressed patients with PD (Hauser and Zesiewicz 1997a). Constipation Constipation is a common complaint in PD and transit time is prolonged in all seg-

ments of the colon (Jost and Schimrigk 1991). Both peripheral and central neurologic abnormalities may contribute to this problem. Lewy bodies have been found in the myenteric plexus of the colon and in the neurons of the dorsal group of the spinal cord (Oyanagi et al. 1990). Anismus, a dystonia causing paradoxical contraction of the striated sphincter muscles during defecation, may also contribute to constipation. As with other dystonias in PD, it may respond to dopamine agonist therapy (Jost and Schimrigk 1994). Treatment of constipation involves increasing stool bulk by adding fibre to the diet and by increasing daily liquid intake. Patients should be counselled to eat more fruit and vegetables, as well as bran products. Drugs that inhibit gastric motility, such as anticholinergics, should be discontinued. Cisapride enhances gastric motility, may improve colonic transit times (Jost and Schimrigk 1994) and can increase peak plasma levodopa levels by improving gastric emptying (Neira et al. 1995).

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Rehabilitation in in Parkinson's Parkinson's disease disease and parkinsonism Christopher D D Ward Ward

Introduction of rehabilitation rehabilitation in Parkinson's disease (PD) and This chapter presents an overview of disease (PD) types in parkinsonism not not dne dueto to PD, PD,prior prior to toaaseries series of of chapters reviewing reviewing specific specific types of The first of nonmedical nonmedical intervention. intervention. The first part of of the the chapter chapter discnsses discusses conceptnal conceptual issues parkinsonissnes relevant relevant to to progressive progressive neurological nenrological conditions conditions such snch as as PD PD and parkinsonism. counts as rehabilitation? rehabilitation? How all) can rehabilitation rehabilitation be distindistinism. What What connts How (if (if at at all) guished from care and snpport? support? IsIs rehabilitation rehabilitationeffective effective and and cost costeffective? effective? What gnished general service parkinsonism? The are the general service requirements reqnirements for for people people with with PD PD and parkinsonism? second part of of the chapter chapter considers considers how how services services can be designed to meet the needs needs specific are needs? What of people with these syndromes. How specific are the needs? What resources resonrces are are required? relevant reqnired? The The chapter chapter ends ends with with an an outline ontline of rehabilitation interventions relevant to different different stages stages of of PD PD and and to to other syndromes resembling PD.

Rehabilitation concepts in PD/parkinson ism PD/parkinsonism What counts as rehabilitation? If people people with with PD/parkinsonism PD!parkinsonism (the two terms are If are nsed used interchangeably interchangeably in this this section) are to benefit their individual individnal needs mnst section) benefit from health and and social social services services their must always occnpycentre centrestage: stage:each eachmedical medicaland andeach eachnonmedical nonmedicalintervention intervention must mnst always occupy in one one way way or or another, another, to toeveryday everyday life. life. All too often physicians physicians forget forget be relevant, in this example in prescribing is nnimunimthis truism, trnism, for example prescribing aa drug drng to to suppress snppress a tremor that is to the thepatient patientororininmechanically mechanicallycontinning continuingsix-monthly six-monthly ontpatient outpatient portant to appointments when doctor nor the the patient patient has has clear clear objectives objectives in appointments when neither neither the the doctor mind. Unfocnsed, Unfocused, essentially essentially aimless aimless activities activitiesofofthis thissort sort maintain maintain a distinction that need hardly hardly exist exist between between medical medical management management and and rehabilitation. that need Rehabilitation all worthRehabilitation in its broadest sense is is aa pragmatic pragmatic approach approach common to all desired ontcomes outcomes are are relevant relevant in in the the daily daily lives lives of while interventions; the desired of patients families. and families.

Onr Our broad definition definition of of rehabilitation rehabilitation rnles rules ont out any any distinction between between 'treat165

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ment' and rehabilitation: all treatments (including (including cures) cures) are are potentially rehabilirehabilitative. Rehabilitation Rehabilitation is is sometimes sometimes contrasted contrasted with with 'active' 'active' or 'medical' treatment treatment (one junior doctor wrote in the hospital hospital notes notes that 'nothing further could could be done done patient was was referred referred for for physiotherapy'), physiotherapy'), but the the distinction distinction isis spurious. spurious. so the patient are rarely rarely curative - all interventions in PD, for Medical and surgical interventions are example, essentially palliative. example, are are essentially palliative. Moreover, Moreover, nonmedical nonmedical modalities modalities such such as physiotherapy can in some circumstances be used as direct direct alternatives alternatives to to interveninterven12). Rehabilitation includes includes all all technoltechnoltions such as drugs or surgery (see Chapter 12). ogies ogies that that can can reduce reduce handicap.

should rehabilitation be distinguished from care? The crucial crucial point point is that How should care? The rehabilitation is is active and goal to meet the rehabilitation active and goal directed. directed. Designing Designing aa care care package package to the need for continuing care care or or support support isis aa rehabilitation rehabilitation activity; activity; routine routine delivery delivery of of care However, care care is is not not rehabilitation. However, care staff staffcan can and and should should participate participate in rehagoals, for example by detecting changing needs and by by collaborating collaborating in in bilitation goals, activities designed designed to prevent complications such routine activities such as contractures contractures and skin sores. Finally, isis there there aa boundary boundary between Finally, between rehabilitation and and palliation? palliation? Does Does there there come a point point where where rehabilitation rehabilitation has outlived usefulness in in the the course course of of proprocome outlived its usefulness gressive (palliation) must musttake takeits itsplace? place? gressive illness, illness,and andwhere wheresymptomatic symptomatic treatment treatment (palliation) In and similar similar disorders disorders a balance balance must often often be be struck struck between between symptom symptom In PD and control achieving functional functional goals goals that risk risk exacerbating exacerbating symptoms symptoms such such as as control and achieving or anxiety. anxiety. Rehabilitation Rehabilitation assessment assessment in progressive progressive disease constant pain or disease isis a constant intervention. Even Even process of discovering discovering what what isis (and (and what what is is not) not) an appropriate intervention, in terminal illness there are ways in which abilities can can be be facilfacilwhich a person's functional abilities impeded. Rehabilitation Rehabilitation concepts concepts are are therefore just as as relevant relevant to a good good itated or impeded. death as to a good life. life. Implementing rehabilitation

can be be more precisely understood within the framework Rehabilitation can precisely understood framework endorsed endorsed by the World of Impairments, World Health Health Organization Organization International International Classification Classification of Disabilities and 1980). Interventions Interventions are are directed directed in in various various Disabilities and Handicaps Handicaps (WHO 1980). combinations towards pathologies pathologies (disease (disease processes), (deficits in combinations towards processes), impairments impairments (deficits anatomy physiology), and disabilities (failure specific tasks anatomy or physiology), and disabilities (failure to to perform perform specific tasks to to an agreed However, in each case agreed standard). However, case all all interventions interventions must finally finally be be evaluated evaluated of their their separate separate or or combined combined impact impact on on handicaps handicaps (disadvantages). (disadvantages). A in terms of A current revision of the ICIDH framework framework (WHO (WHO1997) 1997) isis likely likely to redesignate disabilities no fundamental fundamental abilities as as 'activities' 'activities' and and handicap handicap as 'participation', but with no change change in in concepts. A Afurther further proposed proposed level levelwill willinclude includethe theperson's person's environment environment - social social and physical factors factors that that often often largely largely determine determine the extent of disadvantage.

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Rehabilitation When the scope for for reducing reducing impairment impairment or disability is is limited, limited, rehabilitation rehabilitation can can often be directed directed towards towards modifying modifying the the environment. often be Disadvantage should be understood to Disadvantage should to include includesubjective subjective as aswell well as as objective objective states. fact of carrying the diagnosis diagnosis of of PD, PD, irrespective irrespective of states. For For example, example, the the mere fact any disabilities, disabilities, could could weigh weigh aa person person down with a sense sense of of doom doom or worthlessness. reason for for not not equating equating handicaps handicapswith withphysical physical disabilities disabilities is that handihandiAnother reason caps include caps include potential potential as as well well as as actual actual states. states.Thus, Thus, in in comparison comparison with with others of the same age a person with PD PD is is severely disadvantaged by an increased increased risk risk of of falls falls fractures and will be still still further disadvantaged by aware of and fractures further disadvantaged by not being made aware of risks could be reduced. reduced. how such risks

Disadvantage provides aa philosophical philosophical focus focus for for the the rehabilitation rehabilitation approach approach but Disadvantage provides difficult concept concept to to handle in routine practice is a difficult practice because because disadvantages disadvantages are subjective and variable and notoriously notoriously difficult difficult to toevaluate evaluate objectively. objectively. Moreover, Moreover, in jective progressively accommodate progressively disabling disablingdisorders disorders such such as as parkinsonism parkinsonism we we need need to accommodate potential as as well well as as actual actual disadvantages. disadvantages. We experimenting with potential We have have been been experimenting with a scheme scheme called called PILS, PILS,which whichbreaks breaksdown downhandicap handicapor or disadvantage disadvantageinto into four four broad broad areas that are targets targets for for rehabilitation: rehabilitation: Prevention, Prevention, Independence, Independence, Lifestyle Lifestyle and areas Social Professor Paul Paul Social Resources Resources (the (the PILS PILS scheme scheme resulted resulted from from discussions with Professor Dieppe, Dr Ted Ted Cantrell Cantrell and and Dr John Burn). In the PILS encompasses assessment assessment and and reduction of risks PILS scheme, Prevention Prevention encompasses risks of physical, and social physical, psychological psychological and social complications. complications. Prevention Prevention receives receives little little emphasis in conventional models rehabilitation since emphasis in conventional models of of rehabilitation since these, these, along along with with the ICIDH, have largely largely been been designed designed to to accommodate accommodate acquired, nonprogressive disabilities such PD, by contrast, contrast, rehabilitation rehabilitation must process abilities such as as stroke. stroke. In In PD, must be aa process extended across problems as as well as to those extended across time time and and orientated towards future problems which have already already occurred. occurred. The The anticipatory anticipatory stance, so so fundamental fundamental to the practice geriatric medicine, medicine, is crucial Physicians have in tice of of geriatric crucial in PD. Physicians have an an important important role in disability, in anticipating disease behaviour behaviour and future disability, in contributing contributing to the planning and in in helping helping patients patients and and families families to of future future patterns of care and rehabilitation and take take avoiding avoiding action. action, Rehabilitation Rehabilitation in in this this context context can therefore therefore be seen seen as as aa spespecialized form empowering individuals individuals to future cialized form of of health health promotion, empowering to promote future and well-being well-being (Anderson (Anderson 1996). 1996). autonomy and Independence, the second second target target domain in Independence, the in the the PILS PILS scheme, includes activities activities of daily daily living living and and mobility. mobility.Note Notethat that we weplace placeindependence independence in in aabroader broader context context than is is customary. As implied earlier, dependency always the implied earlier, dependency is not always the crucial crucial deterdeterminant of of handicap. handicap. Whilst dependency dependency can be a major disadvantage disadvantage the person full independence independence may maystill still be beseverely severely disadvantaged. disadvantaged. However, However, individuals individuals with full similar levels levels of dependency vary in their their degree degree of of disadvantage. disadvantage. Note Note also also with similar that dependency and and physical physical disability are by no nomeans meanssynonymous. synonymous. that dependency are by

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Impairments in communication and and in in cognition cognition often often require require others others to to take take over over practical tasks (for (for example example control control of ofpersonal personalfinances). finances).Similarly, Similarly, depression, depression, anxiety,often oftenadds addsto todependency dependency in in parkinsonism. parkinsonism. Studies of carers and especially especially anxiety, of of people people with with severe severe head head injuries injuries have havedemonstrated demonstrated that behavioural behavioural problems outweigh physical disabilities disabilities as as sources sources of of carer carer stress stress (Florian (Florian et al. al, 1989) 1989) and and the of PD PD carers carers suggests that for them, them, too, too, physical physical difficulties difficulties are easier to testimony of cope psychological impairments (McCarthy and and Brown Brown 1989). 1989). cope with with than psychological impairments (McCarthy Lifestylerefers referstotothe theperson's person's roles roles and and aspirations aspirations and for reasons already Lifestyle already given must not not be be identified identified with with independence. independence. Thus, Thus,for forsomeone someonewhose whoselife life revolves revolves around grandchildren the role role of of grandparent grandparent can can be beconserved conserved despite despite severe severe around grandchildren physical disabilities. physical disabilities.Physicians Physiciansare arenot notinin aa good good position position to to understand what is meaningful to their patients patients and and what what they theymost mostvalue valuein intheir theirlives. lives. The The artificial artificial meaningful environment clinic, day produces a limited limited and and environment of of a clinic, day hospital hospital or or inpatient inpatient unit produces of the the individual. individual. More More can can be gleaned from unrepresentative picture of from a home visit, visit, although even there there the the doctor-patient doctor—patientrelationship relationshipoften ofteninhibits inhibits communication. communication. Information from from other othersources, sources,for forexample exampleprofessional professionalcolleagues, colleagues,isis essential essential if Information 'lifestyle' goals negotiated. 'lifestyle' isisto tobe beunderstood understood and valid rehabilitation goals The fourth PILS PILS target target area, area, Social Social resources, resources, includes includes the theenvironmental environmentallevel level identified is to review aa identified in in the the revised revised ICIDH. ICIDH. An An important important goal in rehabilitation is person's assets narrow the gap gap (the (the disadvantage) disadvantage) caused caused by person's assets and and to to narrow by lack lack of of are three three important importantresource resourcecategories. categories. resources. Money, Money, housing housing and and transport are category of of prevention prevention we we need need to to consider consider how howphysical physical assets assets can can be be Under the category conserved financial impliconserved in in the the future. future, For example, example, exploring exploring the the insurance and financial cations of diagnosis diagnosis can be considered considered a rehabilitation activity. activity. cations of of PD PD at the time of However, as family family members, members, However, aa person's person's key key assets assets (so (so to to speak) speak) are people such as other informal informal carers and professional professional carers. carers. Catering Catering for fortheir their current current needs needs and and anticipating future difficulties difficulties is always always aacentral central goal goalininrehabilitation. rehabilitation. Two Two imporimporissues arise. Firstly, Firstly, the spouses and others others extend extend beyond beyond any any role role tant issues the needs needs of spouses have in physical physical care must be be used used carefully. carefully. they may have care and and the designation 'carer' must Secondly, Secondly,no no one one isis required required to to care care for for people people with with PD PD when when they they are are fully fully indeindependent but but one oneor ormore morerelatives relatives or or friends friends are are likely likely to care pendent care aboutthem about them and therefore may require require information, information, education education and and psychological psychological support. third fore may support. A A third general diverge radically general issue issue regarding regarding carers carers is is that that when when their needs diverge radically from from those of the focus of rehabilitation must must be be carefully carefully reviewed. reviewed. This dilemma dilemma the patient the focus cognitive or behavioural is often often encountered when cognitive behavioural factors factors prevent prevent someone someone with fully acknowledging the needs of of aa spouse. This justifies regarding the two two PD from fully individuals individuals as as separate separate 'clients' 'clients' (Ward (Ward and and McIntosh 1993).

Rehabilitation in PD generates complex pattern of linkages between between personal Rehabilitation generates a complex between pathologies, pathologies, impairment, disabilities, and professional professional perspectives, perspectives, between disabilities, and disadvantages, disadvantages, and and between between intervention intervention targets such such as as prevention, prevention, independence,

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Rehabilitation lifestyle and social resources. Such Such complexity complexity has two two implications, which are the

cornerstones cornerstones for rehabilitation. rehabilitation. Firstly, Firstly, complexity complexity often often requires requires aamulti multi profesprofessional approach. One One reason reason for for this is that the sional approach. the pre-eminence pre-eminence of of personal personal values values calls for assessment, rather than one one that that isis calls for aa subtle subtle and and multifaceted multifaceted approach approach to assessment, dominated by by doctors doctors or or by other professional groups. groups. Not all all rehabilitation is is team dominated based, but aa single individual can rarely meet meet any any but but the the simplest simplest functional functional needs the advice advice and and support support of ofothers. others.AAvery very wide wide range range of of health health and andsocial social without the technologies and interventions interventionshas hasto tobe beconsidered. considered.Secondly, Secondly, complexity complexity calls calls for for feasible goals a structured response: feasible goals must must be be identified identified and agreed. In summary, progressive disorders such such as as PD PD require require the the term term rehabilitation to progressive disorders be extended beyond its conventional usage to to describe describe aa process processthat that isis continuous continuous (even (even though punctuated by by time time limited limited goals). goals). Rehabilitation Rehabilitation describes describes the full full range of activities activitieswhich whichare aredirected directedtowards towardsreducing reducingdisadvantages, disadvantages,including includingnot not also risks risks of of future future complications. complications. Rehabilitation Rehabilitation also also only actual actual handicaps, but also includes includes interventions interventions to increase increase aa person's person's ability abilitytoto 'participate' 'participate' in in their their chosen chosen activities (to activities (to use use the the terminology terminology of of the the revision revision of ofthe the ICIDH), ICIDH), or or to to sustain sustain their their Rehabilitation chosen 'lifestyle' 'lifestyle' (in (in PILS PILSterminology) terminology) both both now now and and in the future. Rehabilitation is aa person person centred, active, active, structured structured and usually usually multidisciplinary process. effectiveness of of rehabilitation rehabilitation The effectiveness This question makes little sense sense when when addressed addressed to the Does rehabilitation work? work? This totality of potential rehabilitation approaches approaches and and technologies technologies (Soderback (Soderback 1995). 1995). Rehabilitation as a whole whole cannot be evaluated comprehensively comprehensively because because itit is is insepinseparably bound multiplicity of of medical medical and and nonmedical nonmedical processes. processes. Individual Individual arably bound to aa multiplicity rehabilitation 'technologies', especially especiallyphysiotherapy, physiotherapy,have havebeen beenthe the subject subject of of formal evaluation (Ward (Ward 1992) 1992) as the next next chapters. chapters. formal evaluation as described described in in more detail in the Many studies, however, impairments Many such such studies, however, demonstrate demonstrate effects effectsofof therapies therapies on on impairments (Comella et 1994, Johnson Johnson and and Pring Pring 1990) 1990) and and can cantherefore therefore scarcely scarcely be be (Comella et al. 1994, counted as evidence of the effectiveness effectivenessof ofrehabilitation rehabilitation in in reducing reducing disability disability and should assess assess the benefits disadvantage. Evaluations of rehabilitation should benefits of the two key key processes effective goal goal processes identified identified earlier, earlier, notably notably multidisciplinary multidisciplinary working working and effective setting. evidence, for example example on on the the effectiveness effectiveness of stroke units units setting. There There is some evidence, (Kalra 1996), that that can can be be interpreted interpreted to suggest suggest that that structured structured specialist teams have effect on outcomes of of people people with with neurological neurological disabilities. disabilities. Evidence Evidence is is a positive effect emerge on on the the effectiveness effectiveness of structured rehabilitation rehabilitation processes processes in in beginning to emerge multiple sclerosis, sclerosis, aa progressive progressiveneurological neurologicaldisorder disorderwith with some some similarity similarity to to PD PD al. 1997). 1997). There is is still, however, a severe (Freeman et et al. (Freeman severe paucity paucity of of published published evidence on the benefits benefits of of multidisciplinary multidisciplinary interventions for people with PD or other progressive disabling gressive disabling neurological neurological disorders.

The PILS framework provides provides aa starting starting point for evaluating evidence on PD and PILS framework

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where possible, possible, from from studies for extrapolating, extrapolating, where studies on other other disabling disabling conditions. conditions. Regarding the first PILS PILS dimension, prevention, prevention, there is is aa remarkable remarkable lack lack of of evieviinterventions in dence on the effectiveness effectiveness of interventions inPD. PD.ItIt isis likely likely that some some at at least least of of the the multifactorial interventions reduce falling falling in in at-risk at-risk elderly elderly people people multifactorial interventions shown shown to to reduce (Tinetti et al. 1994, Campbell Campbell et al. al, 1997) 1997) should should be be applicable applicable to to people people with PD. Demonstration of of reduction reduction in inrates rates of of other other adverse adverse events events (for (for example episodes of aspiration pneumonia unwanted nursing nursing home home admissions), admissions), or even of of aspiration pneumonia or or unwanted or even level of risks in PD. PD. reduced level risks of such events, has has not yet been attempted in Demonstrating improvements the second second PILS PILS domain, domain, is is Demonstrating improvements in independence, independence, the difficult difficult in in a progressive progressive disorder disorder such such as as PD. PD. No No studies studies have havedemonstrated demonstrated a sustained improvement improvement in independence independence as as aa result result of of rehabilitation rehabilitation interventions interventions tained although mobility has has benefited benefited from from impairment impairment focused focused physiotherapy. physiotherapy. The The although mobility important and andyet yet the themost mostelusive elusive outcomes outcomes concern concern the the effect effect of rehabilirehabilimost important lifestyle. A A controlled controlled evaluation evaluation of of interventions on onthe thethird thirdPILS PILSdomain, domain,lifestyle. tation interventions a specialist Hampshire town town showed, showed,by by comcomspecialist PD PD community community team based in a small Hampshire parison with controls, controls, aa significant significant improvement improvement in mood mood as as measured measured by by the the parison General Hillier 1979) General Health Health Questionnaire Questionnaire (Goldberg and Hillier 1979) in in people peoplewith with PD PD and and their carers. carers. This This suggested suggested that the team team had had an anoverall overall effect effect on on improving improving in their quality observations). Anecdotes Anecdotes abound, abound, quality of of life life (C. D. Ward Ward et al. unpublished observations). although without quantitative quantitative confirmation, confirmation, to tosuggest suggest that that providing providingspecialized specialized supportto topeople peoplewith withPD PDfacilitates facilitates the the achievement achievement of of individindividmultidisciplinary support goals such ual goals such as as resuming resuming aa social social activity, activity,ororundertaking undertaking aa short short trip trip independently. social resources, resources, specialist specialist advice advice undoubtedly undoubtedlyincreases increasesthe the dently. In In the realm of social to benefits benefits although, although, again, again, formal formal evidence evidence is is lacking. lacking. uptake of entitlements to and parkinsonism: parkinsonism: matching Rehabilitation in PD PD and matching services services to needs needs The aim of this and and subsequent subsequent chapters chapters is is to develop develop an overall overall description description of

for PD PD and and parkinsonism parkinsonism not due to PD, taking into account rehabilitation services for firstly the the epidemiology epidemiology and and clinical clinical characteristics characteristics of of PD PD and and parkinsonism, and firstly in this this chapter. chapter. secondly the concepts of rehabilitation proposed in Must services be diagnosis diagnosis specific? specific? The needs needs of people people with with PD/parkinsonism, PD/parkinsonism, and and therefore therefore the the services services and and

resources they they require, require, can can be be divided divided into into those those common to all resources all people with disdisabilities, those those shared shared by by people people with with progressive progressiveneurological neurologicaldisorders disorders and and those those abilities, to individual individual syndromes. syndromes.By By grouping grouping needs needs in in this this way way we we should be be able able unique to determine the extent extent to to which which services services must be specialized specialized and extent to to determine and the the extent populations of of different different sizes. sizes. which they can be delivered in populations

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Generic social and and healthcare needs Generic social

The fascination of parkinsonism parkinsonism as asaa specialist specialist field field may sometimes tempt tempt us us to to lose lose of an an obvious obvious fact. fact,Many Manyneeds needsofofpeople withPD/ PD!parkinsonism parkinsonism are are common common sight of people with to other causes causes of of long-term long-term disabilities disabilities whether whether neurological neurological or otherwise. otherwise. to other Disabled people rightly insist insist that that most of their needs are better described in Disabled in social social than in medical terms. Although the clinical clinical profile profileof ofeach eachparkinsonian parkinsonian syndrome syndrome is highly distinctive distinctive the experience of disability isis dominated, dominated, for many, by is highly the experience of disability for many, nonspecific mobility, increased increased nonspecific burdens burdens such such as reduced reduced independence, impaired mobility, reliance informal carers carers and andassociated associatedpsychological psychological stresses. stresses. Professional Professional reliance on informal service specialist contributions whilst whilst service providers providers sometimes sometimes over over value value their own specialist underestimating the importance importance of, of, for for example, example, daily home care. Even from the medical point of Even from of view view there is a generic aspect aspect to PD!parkinsonPD/parkinsonism, which gives gives rise to a range range of of problems problems experienced by older people in general. pathologies, which which tend Thus people with PD!parkinsonism PD/parkinsonism are are liable liable to to multiple multiple pathologies, to interact with the parkinsonism or or to to be be masked masked by it. Symptoms Symptoms such such as as pain, pain, can sometimes be linked linked to to dyspnoea, constipation, weight loss loss and incontinence can the neurological disorder disorder but may, may, alternatively, alternatively, have the neurological have independent independent causes causes that that the rehabilitation team team isis likely likely to overlook overlook unless unless expert expert medical medical surveillance surveillance is available. Needs common to to progressive progressive neurological neurological disorders disorders A somewhat more specific group of needs are shared A somewhat specific group shared by those those with with progressive progressive neurological disorders (excluding (excluding Alzheimer's disease/dementia), disease/dementia), with with aa total total prevprevalence of perhaps perhaps 400 400 per per 100000. PD and and parkinsonism alence of 100000. PD parkinsonism together constitute constitute the the largest proportion these conditions conditions in the the community community (Mutch (Mutch et et al. al. 1986). 1986). largest proportion of these Multiple sclerosis sclerosis (prevalence (prevalence around 100—200 per100000) 100000)accounts accounts for for most most of Multiple 100-200 per the (Rodriguez et 1994). Motor the remainder (Rodriguez et al. 1994), Motor neurone neurone disease disease and and Huntington's Huntington's disease affect disease affectaamuch muchsmaller smallernumber number of ofpeople peoplealthough although with with aa disproportionately disproportionately impact on on services. services. high impact The most obvious so that that obvious feature feature shared by these conditions is is progressiveness, progressiveness, so rehabilitative forward looking and preventive preventive in approach. approach. rehabilitative management management must be forward The progression progression of PD gives gives rise rise to to phases phases in in which which different different needs needs predominate, from diagnosis, when support are are especially especially imporfrom the time of diagnosis, when information information and support tant, through through the the phases phases of of increasing disability to the late stage stagewhen whenpalliation palliation and and family emphasized, although the rehabilitation approach approach remains remains relrelfamily support support are emphasized, evant. The The British British Multiple Multiple Sclerosis Sclerosis Society needs can can be be evant. Society has has suggested suggested that that needs divided into those associated associated with (1) the time of diagnosis, (2) mild mild disability disability (3) (3) divided severe disease (Multiple (Multiple Sclerosis Sclerosis Society 1997). A severe disability disability and and (4) terminal disease A very very similar PD and and parkinsonism parkinsonism although although age age related related similar division division could could apply apply to to PD issues issues would wouldbe be different. different.Thus, Thus,the theneed needfor forinformation information and and support support at the time

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diagnosisisisstrongly strongly expressed by individuals and by organizations representaof diagnosis expressed by individuals and by organizations representative of all the progressive progressive neurological disorders (The (The Neurological Neurological Alliance Alliance 1996). 1996). Nonparkinsonian share many many of of the the opportunities opportunities for for health health promoNonparkinsonian syndromes syndromes share for example example prevention prevention of of falling. falling. Similarly, Similarly, there is a shared tion, for shared need need for for forward issues such as as finances, finances. However, However, in survey of planning on issues in aa recent recent community community survey of progressive neurological Derbyshire, we few people with progressive neurological disorders disorders in Derbyshire, we found found that few to enable enable them them to to plan plan ahead ahead in in view view of their had been provided with information to progressive disabilities, progressive disabilities, or or knew knew where whereto to obtain obtain such such information. parallels have have been been reported reported between consecutive service service users users with with PD PD Striking parallels and with MS MS (McKinney (McKinney et 1998). The The range and and frequency frequency of of symptoms symptoms and with et al. 1998). reported by the two groups were surprisingly similar, similar, as were the profile profile of of restricin activities activities of daily daily living living and the degree degree of psychological psychological stress tions in stress in in patients and carers, as 28. In conclusion, as measured measured by by the the General General Health Health Questionnaire Questionnaire - 28. it appears likely that that there are large overlaps overlaps in in the service service requirements requirements for people that the the specificity specificity of of needs needs associated associated with progressive neurological disorders and that could be be overstated, overstated. with PD and with parkinsonism could Needs specific to PD/parkinsonism PD/parkinsonism

rehabilitation Whilst it is important to to appreciate appreciate the generic generic context of disability, disability, rehabilitation unless there there isis full full recognition recognition of of the the pattern of cannot be fully effective effective unless of needs needs assoassociated with with PD One dominant dominant need ciated PD and and specific specific parkinsonian parkinsonian syndromes. syndromes. One need in in PD/parkinsonism is is for medical PD/parkinsonism medical and and nursing nursing support support to institute an appropriate drug especially to monitor monitor ititininrelation relationtotofunctional functionalobjectives. objectives.The Theimporimporregime and especially drug regime regime with with pragmatic pragmatic goals goals can can hardly hardly be be overstated. overstated. As As tance of linking the drug next chapter chapter describes, describes, a specialist specialist nurse is likely likely to improve the relevance relevance of the next to improve of everyday life. medical treatment to everyday life.Provision Provisionof ofinformation information and and training training to to patients has been shown to improve the quality of of medical medical management, management, with gains in in mobility, subjective sense sense of of self-efficacy self-efficacy (Montgomery et et al. al. 1994). 1994). ity, independence independence and subjective reported for her husband was nearly intolerable. He reported that the the burden burden of caring for requested her help several several times times nightly nightly to get him him to the toilet. toilet. The use of of controlledcontrolledrelease levodopa at night helped the the situation. situation.

A A woman

A A woman woman with with inadequate inadequateresponse responsetotolevodopa levodopawas wasreceiving receivingthree threedifferent differentformulations formulations

of levodopa with inter-dose intervals intervals as short as as 30 30 minutes. The sheer complexity complexity of the drug regime regime inhibited inhibited her her from from going going out out and and was a source of distress drug distress for for her somewhat obsessional obsessional husband. husband.AA simpler simpler regime regimehad hadno noadverse adverseeffects effectson onmobility mobility but butconsiderably considerably reduced the requirement requirement for for care.

with severe 'on-off with 'on-off’ fluctuations fluctuations was taught to administer administer subcutaneous subcutaneous apomorapomorphine, initially initiallytotoenable enable him him to to give give aa speech speech at his wedding without fear of unpredictable akinesia. akinesia. A A man man

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second important A second important distinguishing distinguishing characteristic characteristic of of PD!parkinsonism PD/parkinsonism has has already been been mentioned; in in these these syndromes syndromes physical physical and psychological deficits deficits are characteristically characteristically combined. Psychiatric disorders are sometimes the critical critical deterdeterminants of overall disability disability and PD. These These include only anxiety anxiety and handicap in PD. include not only (Gotham et et al, al. 1986, 1986, Routh et al. 1987), but and depression (Gotham but also also hallucinations hallucinations and related psychotic psychotic symptoms drug related symptoms (Saint (Saint Cyr Cyr et et al. al. 1993). 1993). Dementia, Dementia, that that is is progressive multimodality cognitive failure, failure, affects affects 10—30% 10-30% ofofpatients PD gressive multimodality cognitive patients with with PD (Brown and Marsden Marsden 1984). 1984). Even Even at the the level level of of physical physical disability disability one might might (Brown describe PD as a behavioural as much as as aa physical physical disorder. The everyday everyday obserdescribe environmental stimuli stimuli such such as as obstacles obstacles or thresholds thresholds cause cause akinetic akinetic vation that environmental freezing evidence that even even motor motor tasks tasks such such as aswalking walking have have aa cognitive cognitive freezing provides provides evidence aspect. The aspect. The communicative communicative impairments impairments in PD are a complex complex blend blend of of motor motor and cognitive deficits, expressive fluency, cognitive deficits, including including not only reduced expressive fluency, impaired impaired articuand phonation phonation (Critchley (Critchley 1981), 1981), but but also also deficits deficits in perception of prosodic prosodic lation and speakers (Pell (Pell 1996). 1996). Psychometric evidence evidence of of deficits deficits in selective selective cues from from other speakers (Cooper etet al, al. 1991, 1991, Brown and Marsden 1991) 1991) help to explain the familfamilattention (Cooper iar observation that distraction, for for example through anxiety, anxiety, impedes motor perperformance increases disability. state termed termed formance and and increases disability.Dramatic Dramaticchanges changesinin aa mood mood state 'affect-arousal' occur 'affect-arousal' occurinin parallel parallelwith with fluctuations fluctuationsininmobility mobilityasasaa result result of of response levodopa treatment (Brown et 1984). There least one one response to to levodopa treatment (Brown et al. 1984). There is is thus thus at least disability, cognitive potential link between motor disability, cognitive impairment impairment and disturbances in mood in in PD. PD. A A

man with recently recently diagnosed, diagnosed, relatively relatively mild mild PD PD had had become become incapacitated incapacitated by by an an anxiety anxiety state that had compounded compounded his hisphysical physical disability. disability. AA series series of therapy therapy sessions sessions with aa cliniclinical psychologist psychologist helped helped him him and his wife to resume activities. resume some of their previous previous social activities. which One root cause of of the the anxiety anxiety was was that that he had had been traumatized traumatized by the abrupt way in which the diagnosis had had been been communicated communicated and by the the lack lackof ofsubsequent subsequentinformation information about his outlook. future outlook.

An important distinguishing distinguishing characteristic characteristic of PD (as opposed to parkinsonism) is the variability variability of disability. disability. Health professionals, as Health and social care professionals, as well well as as the the general public, have disabilities in PD are are general have difficulty difficulty in in acknowledging acknowledging that that motor disabilities highly variable. erratic response response to to levolevovariable, Variability Variability is is most most dramatic dramatic in people with erratic dopa but was was observable observable before before the the levodopa levodopa era era (Granger (Granger 1961). 1961). Predictable Predictable dopa fluctuations place place additional restrictions restrictions on on people's people's lives lives whilst diurnal fluctuations whilst unpredictcause intense intense psychological psychological distress distress as as well as practical inconvenable fluctuations cause ience. maximum ience. Rehabilitation Rehabilitationmust musttake takeaccount accountofofboth both the the minimum minimum and maximum abilities experienced hour. abilities experienced from from day day to to day day and and from from hour to hour. A A woman aged aged 65 65 was was reluctant reluctant to to use use her her disabled disabled parking parking sticker sticker in in the thelocal localsupermarsupermar-

ket. Other often made made critical critical comments commentswhen when they they saw saw her her able able to walk ket. Other shoppers shoppers often walk

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C. D. Ward apparently normally from despite the fact that an hour from the thecar car — - despite hour later later she she was was barely barely able able to walk walk 10 10 metres metres unaided. A woman woman with 'wilful' and with severe severe motor motor fluctuations fluctuations was was described described by by nursing home staff as 'wilful' and 'malingering' 'malingering'because because she she asked asked for assistance assistance in dressing, having having been been observed observed to to be beable able

to knit knit skilfully skilfully at other times.

Rehabilitation resources resources in PD/parkinsonism The three groups of needs, those associated with disability in general, with with progres-

sive neurological disorders, disorders, and specifically with PD PD and parkinsonism, sive neurological specifically with parkinsonism, provide provide some indications indications of range of of services services required required for for the the rehabilitation rehabilitation and and some of the range ongoing of people people with with these these diseases. diseases. Existing Existing resources, ongoing support of resources, whether whether adeadequate underprovided, must be be coordinated, coordinated, preferably preferably by a nominated nominated key key quate or underprovided, worker, and essential between social serworker, and some degree of teamwork is essential between health health and social vices. Rehabilitation process and must therevices. Rehabilitation in in PD/parkinsonism PD!parkinsonism is is aa continuous continuous process fore largely community general framework framework (see (see Table Table 10.1) 10.1) can be be fore be be largely community based. A general adapted largely determine taken adapted to local conditions, which will largely determine the possible form taken disorder clinic, clinic, in in by such a team. The team can be based in a specialist movement disorder a day day hospital, hospital, or within a service service developed developed for for people people with with progressive progressive neurologneurological ical disorders. Sensitivity to to the the specific specific characteristics characteristics of of PD/parkinsonism PD/parkinsonism requires all health Sensitivity to the advice of at least least one one clinician with and social service providers to have access to experience disabilities which can be encounencounexperience of of the the full full range range of of impairments and disabilities Because of tered. Because of the the rarity rarity of of PD/parkinsonism PD!parkinsonism such experience cannot be gained within aa social services services area area or or primary primary care group, which typically typically serve serve aa populapopulaof around around100 100000. 000. A A hospital-based hospital-based specialist specialistisisessential essential to to enable enable the theteam teamtoto tion of full account of key principles such as medical management, variability variability of of disdistake full ability and psychological fulfilled in various ways psychological factors. factors. The medical roles can be fulfilled ways (see Table as assessment of (see Table 10.1). 10.1).AAneurologist neurologist isis appropriate appropriate for some roles such as diagnosis and review of complex treatment regimes. regimes. However, However, as a recent recent survey survey of of British demonstrated (C. (C.D. D.Ward Ward and and C. C. Young, Young, unpubunpubBritish neurologists and trainees demonstrated observations),neurologists neurologistsare arelikely likelyto tobe berelatively relatively inexperienced inexperiencedininassessassesslished observations), ing disability family, and disability in in the the context context of of home home or family, and nor are they well well positioned positioned to to medical complications complications or to to liaise liaise with with community community services, services. A handle emergent medical geriatrician is often often better better placed placed to acquire the necessary necessary specialist specialist experience experience and and work effectively effectively within multidisciplinary team. age psychiatry psychiatry has an an to work within a multidisciplinary team. Old age important role (Wilkinson (Wilkinson1992), 1992),as ashas hasclinical clinical psychology. psychology. important role

The roles The roles of other health health professionals professionals are described described more fully fully in succeeding succeeding chapters. The The contribution of aa specialist PD nurse nurse (see Chapter 11) can can often often be chapters. specialist PD (see Chapter to provide professionals less experienced experienced in in PD PD (including (including doctors) with advice and as well well as contributing directly directly to the the management management of of individuals individuals with with support, as

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Table 10.1 Key functions functions of a rehabilitation service 10.1 Key service for PD/parkinsonism PD/parkinsonism

multiprofessional team goals with with the the patient/client patient/client and The multiprofessional - jointly assess assess and negotiate goals and family family worker (health or social professional as appropriate) (health and social social services) -— identify key worker social services professional appropriate) inform and educatepatient/client patient/client family - inform and educate andand family -— establish strategies strategies to prevent future complications complications -— jointly monitor progress progress training programme -—joint joint training - establish establish diagnosis diagnosis and interpret interpret and andcontrol controlemerging emerging symptoms symptoms assess prognosis — monitor treatment treatment in in liaison liaison with with nursing nursing colleagues colleagues — provide psychiatric assessment assessment

Medicine

— —

Nursing

—liaise with medical medical colleagues colleagues (consultant (consultant and GP) - liaise with — inform inform and advise on symptoms — negotiate and monitor drug drug regimes regimes and and associated associated functional goals

- assess assess functional functional abilities abilities and and provide provide therapy therapy programmes programmes

Therapy

—

—

provide and coordinate coordinate information information on onavailable available resources resources assess social needs provide or organize for care care and and support organize resources for

—

provide information information

—

give support

Social services

Voluntary organizations organizations (local and national) (local

Specialistinformation information and Specialist advisory advisory services services

accessto tolocal localand andnational national information information databases databases on on statutory and allow access voluntary voluntary services services — provide coordination of of information and and education education strategy strategy for local service users —

complex medical or or nursing nursing needs. needs. With With expert expert advice advice aa community community nurse nurse can complex medical develop some of the roles of the specialist nurse at a local level. This can include develop some roles specialist at a local level. include supervision of interventions such as apomorphine. The supervision The community community nurse nurse isis often often in a better position than than the thespecialist specialist nurse nurse to toact actas asaakey key worker worker who who isis locally locally accessible and activities of rehabilitation accessible and in in touch touch with with the activities of other other members of the rehabilitation team. Depending on individual individual needs a therapist may prove team. Depending prove to to be be appropriate as the key worker, member currently currentlymost mostactively actively involved. involved. worker, through through being the team member Such team working working and and key key working working is is only only possible possible when a Such a fluid fluid approach to team group of professionals professionals have have established establishedaapattern pattern of oftrust trust and and collaboration, collaboration, usually usually facilitated facilitated by by effective effectiveleadership. leadership.Quality Qualityofofservice servicethen thendepends dependsnot notso somuch much on on the particular professional qualifications qualifications of of individuals individuals as as on the competence competence of of the the team as aa whole. whole. Some Somedegree degreeofofmanagement management and and leadership leadership isisrequired required to to control control case loads, case loads, to to prevent prevent over over dependency dependency of of patients/clients patients/clients on professionals professionals and and to

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continuing follow-up and support as ensure continuing follow-up and as needs needs change change over over time. There There are opportunities to rationalize rationalize the the roles roles of of health health and and social social services services so as to ensure ensure that people people with PD PD and and their theirfamilies families have have continuous continuous access access to to available available inforinforand services. services. In the the UK, UK, voluntary voluntary organizations organizations such such as as the the Parkinson's Parkinson's mation and Disease Disease Society Society (PDS) (PDS)and and the the private private sector sector can can play play aa complementary complementary role, especially welfare workers. cially ifif they they employ employ voluntary voluntary or paid welfare workers, People People with with PD will will also also for disabled disabled people, such benefit from from organizations providing general information for as details details of of locally locallyavailable availablespecialized specializedtransport transport facilities facilitiesand and from from organizations as the Citizens Citizens Advice Advice Bureau Bureau (see (see Table Table 10.1). 10.1). such as Is there a role for intensive Is intensive inpatient inpatient rehabilitation? rehabilitation?

Faced with complex complex disabilities disabilities and and distressed distressed relatives, relatives, physicians have often been

offer hospital admission for 'a 'a good good sort sort out' out' and and'to 'togive givethe thefamily family aa tempted to offer firstly, to break'. There is a need, firstly, to disentangle disentangle rehabilitation rehabilitation from from respite care, and secondly, to hospital as aa context context for for assessment. assessment. secondly, to recognize recognize the the limitations limitations of of hospital Hospital admission admission can precipitate precipitate mental mental confusion, confusion, with with disastrous disastrous results, results. A A Hospital argument against against hospital hospital admission admission and and other other forms forms of of intensive intensive rehabilrehabilfurther argument not all all problems problems in PD are long standing, itation is that most if not standing, and and permanent than days days solutions require behavioural changes that take weeks weeks or or months rather than Short term term functional functional gains gains have have often often not been sustained for long to bring about. Short after intensive intensive therapy therapy courses courses for for PD. PD. periods after Rehabilitation strategies in in PD PD Rehabilitation is is relevant relevant at at all all stages stagesin in PD PDbut but different different strategies strategies are are required required at at Rehabilitation different stages. stages. People People with with PD PD typically typically experience experience two two or or three obvious lifetime different lifetime milestones. The first first of of these, these, always, always, is the time of diagnosis. diagnosis. A second milestone milestone maybe perceived perceived at at the time when disabilities disabilities begin to impact significantly significantly on daily maybe life, leading need for for home adaptations. adaptations. A life, leading perhaps perhaps to to early early retirement retirement or or to to the the need stage is initiated initiated by recognition recognition of of dependency, dependency, often often an insidious insidious process process third stage marked by a progressive progressive shift shift in in family family relationships relationships and and responsibilities, responsibilities, such such that a wife is acknowledged as as a carer. Finally there is a terminal stage stage calling calling wife or or husband husband is for palliative care. for intensified intensified support and palliative Each milestone milestone marks a shift Each shift in a person's subjective subjective experiences experiences and perceived needs and there is is a parallel shift shift in requirements for for information. information. Accessible Accessible and appropriate information has has aa large large part to play in ensuring that high quality rehaservices are available available at bilitation services at critical critical junctures. We We are are experimenting with a signpost leaflet signpost leaflet designed designedtoto enable enable people people toto select selecttopics topicsof of interest interesttoto them them at different different phases phases of of their their disease, disease, from from diagnosis diagnosis onwards. onwards. Piloting Piloting the the leaflet leaflet in in an an

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outpatient clinic has has shown shown that people are selective in their their requests requests for for informaselective in tion, although the majority request more information about about their their disease. disease. PD from diagnosis diagnosis to to disability disability PD — - from

The rehabilitation priorities priorities in in this this phase phase are are for prevention. The best outcomes are likely to result result from from setting setting up productive long-term relationships likely to relationships (partnerships) (partnerships) between patients and and professionals professionals so so as as to to allow allow access access to to timely timely advice advice while while between avoiding excessive reduce avoiding excessivedependency. dependency.Professional Professionalsupport support can can probably help to reduce the likelihood of depression, anxiety, and and social social dysfunction; dysfunction; expert expert advice advice prevents prevents impulsive life decisions, impulsive and and inappropriate inappropriate life decisions, for for example example premature premature retirement retirement or premature discontinuance of of driving. driving. One determinant determinant of of the the quality quality of such relationships, beyond the control of professionals, family to fessionals, isisthe the approach approach initially initially adopted adopted by by the the individual individual and the family to the fact disease. For some distance distance from from professional professional fact of of chronic disease. For some, some, maintaining maintaining some facilitates adjustment (Bury 1991). 1991). Opportunities preventive help facilitates adjustment (Bury Opportunities for setting up preventive strategies limited. The The likeliest likeliest source source of useful useful practical advice advice for this this strategies are are then limited, such as as the PDS. All group will be be through through voluntary organizations such All patients patients should aware of the PDS at the time of diagnosis so be made aware so that they can make their own choices about involvement at local or national national level. level. choices For many people, resistance to professional professional help can be traced partly to the way which they they were were informed informed about the diagnosis and prognosis. Lack of commuin which Lack of nication, lack of information and and lack lack of of family support at the the time time of of diagnosis diagnosis are are support at common experiences experiences that subsequently to resistance resistance to professional professional common that contribute subsequently to dysfunctional dysfunctional responses responses to illness. Inaccurate inforhelp, and to Inaccurate or misconstrued misconstrued information can can also also be damaging: man was excessivelypreoccupied with quite mild was excessively preoccupied with mild symptoms symptoms of of PD, PD,which which persisted persisted despite partial response to levodopa. levodopa. He claimed that a junior junior doctor doctorhad hadassured assured him him that that drugs would would 'cure' him.

A A

Another elderly elderly man, man, whose whose significant significant disabilities disabilities eventually eventually responded responded well well to tolevodopa, levodopa, delayed accepting treatment treatment because because he he had had been been told told that the drug produced distressing delayed accepting distressing

involuntary movements movements and and other other adverse adverse effects. effects.

pitfalls can can be be avoided avoidedor or minimized minimizedby byensuring ensuringthat thatthe the person person communicommuniSuch pitfalls cating the diagnosis is adequately adequately trained. A hospital specialist who who has no contact patients with with late late stage stage PD PD isis likely likely to communicate aa misleadingly misleadingly positive with patients prognosis little experience experience of correspondingly over over prognosis while while those those with with little of PD PD may be correspondingly pessimistic. At diagnosis there should be multiple multiple opportunities opportunities to pessimistic. At the the time time of diagnosis there should receive Ideally, as Romford receive and and absorb absorb information. information. Ideally, as in in the the system system pioneered pioneered in in Romford (Oxtoby 1988), 1988), the the physician physician charged charged with with communicating communicating the the diagnosis diagnosis isis (Oxtoby

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C. D. D. Ward C.

Table 10.2 10.2 Preventive Preventive advice in in early early PD PD

Advice

Rationale

Dispel unjustified unjustified anxieties Psychological issues Be well informed about about PD PD Dispel

Realistic Realistic expectations expectations

Social issues

Physical fitness

Promote communication

Discourage social social isolation

Maintain social social roles

Discourage social social withdrawal

Financial advice advice

Optimize resources resources for future future

advice Employment advice

Prevent Prevent premature premature (unwanted) (unwanted) retirement

advice Housing advice

Discourage Discourage move move to to unsuitable house

Review of Review of transport transport needs

Anticipate eventually being unable to drive

Promote general general health

Reduce risks risks (cardiovascular (cardiovascular etc) Reduce

Monitor body weight

Useful long-term long-term index of well-bethg Useful well-being in PD

care Dental care

Reduce future future speech/swallowing speech/swallowing problems Reduce

Anti-osteoporosis strategy

risk of falls, falls, reduced reduced bone bone density density High risk and fractures in late PD (Taggart (Taggart and 1995) Crawford 1995)

Specific exercises

Reduce future future dystonic dystonicpostural postural deformity deformity Reduce

supported supported by byaa professional professional colleague colleague such such as as a social worker or nurse who can concontinue to answer questions and and to to provide provide support support both both at at the the initial initial appointment appointment answer questions and subsequently. subsequently. A A follow-up follow-up phone phone call call by byaanurse nurse or or doctor doctor from from the clinic, the day day after after an an initial initial appointment, appointment, is an option when resources are limited. In contrast to to those those who who keep keep professionals professionals at arm's arm's length, length, some people will actively engaged can II do?'. do?'. wish to be actively engaged in in 'fighting' the disease - they will ask 'What can preventive strategies strategies early early in PD, Whilst there is little scientific scientific evidence evidence to to support preventive it is reasonable offer a menu of rational rational advice, advice, which could could include include the the issues issues reasonable to to offer listed in Table 10.2. Physiotherapists routinely recommend programmes of exerlisted Table 10.2. Physiotherapists routinely recommend programmes exercises gait. This cises to to discourage discouragepostural postural deformity deformity and and to to maintain maintain the quality of gait. advice although as asyet yet there there isis only only weak evidence for the efficacy efficacy advice can can be be supported, supported, although of reducing the severity severity of disability in PD (Doshay (Doshay of physiotherapy physiotherapy in in reducing of long-term long-term disability 1962). 1962). A subjective sense feeling that that one's one's actions actions can can influence influence at sense of of self-efficacy self-efficacy - the feeling least some some aspects aspects of of the the present present and future - contributes positively to well-being well-being and in other contexts contexts appears appears to to influence influence physical physical health (Mendes (Mendes de de Leon Leon et et al. al. 1996). Some scientific foundation nevertheless be 1996). Some activities activities without without solid solid scientific foundation can nevertheless

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Rehabilitation health promoting promoting ifif they A range range of ofso socalled calledcomplementary complementary they enhance enhance self-efficacy. self-efficacy. A

therapies can can be be justified justified on on these these grounds grounds and also because because they they are are a means means of of therapies reducing anxiety and increasing increasing social social confidence. confidence. PD earlydisability disability PD — - early

when drug treatment Even when treatment isis effective effective most most people people will will begin begin to to experience experience significant disabilities within aa few years of diagnosis and treatment. significant Acknowledgement of even of of the word disability - is psychologiof disabilities disabilities - or even cally challenging their sitsitcally challenging for for many many people people and may trigger a radical reappraisal of their uation, requiring requiring psychological psychological support. Those Those who were were previously reluctant to to accept advice rehearsal of educational process process begun accept advice may may now now do do so, so, and and rehearsal of the educational begun at diagnosis maybe more more productive productive at at this this stage. stage. People People should be given the oppordiagnosis tunity to be better informed across across aa range of topics that previously previously seemed irrelethem. Many Many will be maybe vant to them. be unaware unaware of of their their full full entitlement entitlement to benefits benefits and maybe interested other social social topics such as interested in in information information on other such as as housing housing and and transport as well as on on medical aspects of PD. Those Those with with several several years' years' experience experience of of PD PD cannot cannot to be well informed and and aa signpost signpost leaflet, leaflet, as as described above, provides be assumed to to meet meet the the individual's individual's perceived perceived needs for information as as disabilan opportunity opportunity to ity progresses. Many interventions can can be be helpful helpful in response Many interventions response to the onset of specific specific impairments or disabilities disabilities (Caird (Caird 1991), 1991), as as shown in in Table Table 10.3. PD late stage PD PD — - late

Whilst the division of disease progression into into phases phases is is artificial, there is a qualitative distinction distinction between between the stage when disabilities disabilities are are moderate moderate and compatible tive stage when compatible with full independence, independence, and and aastage stage when dependency dependency isis unavoidable. unavoidable. There There isis then then open acknowledgement of a spouse spouse as a carer carer - with momentous effects acknowledgement of effects on relarelationships. Disability and dependency result result from from increasing increasing physical physical deficits, deficits, tionships. Disability and dependency declining communicative Response to declining communicative ability ability and and cognitive cognitive impairment. impairment. Response to drugs is less less satisfactory satisfactory and and less less predictable. predictable. There There are escalating escalating risks risks of of physical physical complicomplications such such as as falls, falls,fractures, fractures,pressure pressuresores soresand anddysphagia dysphagiaand andnutritional nutritional probproblems. There are also increasing risks of adverse psychological psychological and social events: for for example psychiatric related psychosis; psychosis; and physical or example psychiatric symptoms symptoms including including drug related and physical or carers. psychological ill health of carers. The rehabilitation agenda agenda remains remains relevant relevant in in late late stage stage PD, although the the goals goals of the multidisciplinary multidisciplinary team team take take account account of a sharper decline decline in physical physical (and sometimes cognitive) function (see (see Table Table 10.4).

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Table 10.3 10.3 Interventions in in early early PD PD

Problem

Interventions

Disturbed sleep

Physiotherapy advice and equipment to facilitate turning in bed advice and facilitate turning Drug treatment, treatment, including changes changes to to antiparkinsonism antiparkinsonism drugs drugs Drug

Dysarthria/dysphonia

Speech therapy advice advice to to maintain maintain communication Speech therapy Dental care

Dysphagia

Speech and language therapy therapy advice advice to reduce reduce risks risks Dental care

dexterity Impaired dexterity

Occupational therapy (OT) of (OT) advice advice and provision of small items of equipment (crockery, (crockery, adapted pen, etc)

Self-care difficulties difficulties

OT advice, adapted clothing clothing (eg (eg Velcro Velcro fastenings), equipment equipment (e.g. (e.g. electric electric toothbrush)

Reduced mobility, Truncal instability

OT home assessment assessment (reduce risks, risks, improve function) function) Physiotherapyadvice advice: remedial remedial treatment programme and and Physiotherapy maintenance exercises exercises OT advice on car transfers, car adaptations (Dubinsky (Dubinsky etet al. al. 1991) 1991) Assessment Assessment of driving competence (mobility centre)

Sexual dysfunction dysfunction et al. al. 1990, 1990, (Brown et al. 1990) 1990) Koller et al.

Skilled assessment of physical, physical, pharmacological pharmacological and psychological Skilled assessment factors Counselling Alteration in drug drug regimes regimes to reduce reduce adverse adverse factors such as impaired mobility

Rehabilitation strategies strategies in parkinsonism not not due due to to PD PD Rehabilitation strategies in degenerative degenerative parkinsonian syndromes syndromes are are qualitatively qualitatively similar to those for PD. One important importantdifference difference isisthat thatdrug drugtreatment treatmentisisless lesslikely likely play aa critical critical role role in in controlling controlling disability disability (although (although significant significant response response to to to play dopaminomimetic system atrophy). dopaminomimetic agents agents may may sometimes sometimes be be seen seen in in multiple system Another point of of contrast contrast is is the the more more rapid rapid rates rates of of progression progression typically typically seen seen in in multiple system system atrophy (MSA) (MSA) and in progressive supranuclear palsy (PSP). both multiple Rehabilitation of many people with these syndromes is complicated by delayed delayed recrecognition of the true nature of of the disorder disorder (initially, (initially, PD maybe mimicked). mimicked). There There is often often aa consequent consequent failure to keep pace with disabilities as they develop. The rehabilitation programme may may fail fail to take full full account of disabilities disabilities not commonly bilitation not commonly observed observed in PD. For For example, example, visual visual deficits deficits resulting resulting from from reduced reduced range of conincontinence isis an an early early feature in in MSA. MSA. For the most most jugate gaze occur in PSP and incontinence

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Table 10.4 10.4 Interventions Interventions in inlate latestage stage PD PD

Problem

Interventions

Erratic response to drugs

Intensive monitoring monitoring and revision of drug regime, with specific specific functional functional Intensive aims

Prolonged akinetic freezing freezing

al. 1990) Use of 'tricks', including inverted walking stick (Dietz et al. Apomorphine therapy

Imp aired mobility Impaired

Walking aids; e.g e.g. wheeled wheeled walking frame frame Wheelchair (with good postural support support to todiscourage discourage scoliosis) scoliosis) Equipment to assist assist transfers transfers carer burden burden and to reduce risk risk Home adaptations or rehousing to reduce carer of falling

Pain (Quinn et et al. al. 1984) 1984)

Improved postural postural support (seating to control Improved (seating and sleeping) sleeping) to musculoskeletal pain. Use Useof ofapomorphine apomorphine to to treat treat 'off 'off period pain musculoskeletal pain.

Feeding and and nutrition nutrition Feeding (Kempster and Wahlqvist 1994)

Neater Monitor nutritional status status (dietician); (dietician); equipment for for feeding feeding (e.g. Neater eater); monitor monitor dysphagia, dysphagia, remedial treatment (speech (speech and and language language therapist); therapist); use use of of thickeners for for fluids fluids and and modified modified diets diet; rarely alternative feeding route (e.g. (e.g. PEG tube or night time time fine fine bore bore nasogastric nasogastric feeding) feeding)

Bladder symptoms (Gray et al. 1995) (Grayetal. 1995)

Review medication (anticholinergics) Review medication (anticholinergics) for improved mobility and undressing Urinary urgency: OT advice for undressing Nocturnal frequency: frequency: optimize sleeping and night-time mobility mobility (see (see below); night-time night-time antidiuretic treatment (desmopressin use of of (desmopressin spray); use for 'off 'off period periodvoiding voiding difficulty difficulty pergolide; apomorphine for Consider unrelated urological problem

Constipation (Jost (Jost 1997) 1997)

Review medication medication (anticholinergics) Review (anticholinergics)

Disturbed nights

Optimize mobility in bed Optimize antiparkinsonism medication at night Review home home environment for Review for safety safety Review needs of carer Hypnotic or antidepressant drugs drugs if appropriate appropriate

Skin care (risk of sores)

pressure areas, areas,transfers, transfers,continence, continence,nutrition nutrition Monitor and manage pressure

Psychiatric drug reactions Psychiatric

Review of of drug regime Review Antipsychotic medication Counselling and support for patient and Counselling and family family

Dementia

Monitor cognitive function; acknowledge acknowledge implications for family; family; appropriate appropriate support supportservices services (including (includingAlzheimer's Alzheimer's Disease Disease Society Society for severe dementia) dementia)

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C. D. D. Ward C.

part, however, impairments and disabilities disabilities encountered encountered in in parkinsonism parkinsonism are qualhowever, impairments

itatively similar similar to to those seen in PD, even though often itatively often of ofgreater greaterseverity. severity. In In MSA MSA the severity of of dysphagia dysphagia frequently frequently requires requirespercutaneous percutaneous endoscopic endoscopic gastrostomy may be necessary due due to to severe severe posposearly use of a wheelchair may (PEG) feeding and the early hypotension. The The ethics ethics of of life life sustaining sustaining measures measures in in degenerative degenerative parkinparkintural hypotension. particularly in inPSP, PSP, which which isis often often rapidly rapidly progressive, progressive, need need to tobe becarefully carefully sonism, particularly considered on procedures procedures such such as asPEG PEG feeding. feeding. considered before before embarking on Conclusion The quality of life life of of people people with with PD PD and and parkinsonism, parkinsonism, and of their families, families, can

be improved through rehabilitation. directed interventions rehabilitation. Multidisciplinary, Multidisciplinary, goal directed focused on handicaps - the disadvantages disadvantages incurred by by disease disease and disdisshould be focused ability. of the the ability. Many Many of of these these are aregeneric genericbut but some some can can only only be be understood understood in terms of of neurological neurological impairments impairments characteristic characteristic of of specific specific parkinsonian parkinsonian synsynpattern of dromes. repeatedly stressed dromes, One One point repeatedly stressed in in this this chapter chapter isisthe the importance importance of of adopting aa preventive risk: preventive approach. approach. One of the dimensions of handicap/disadvantage handicap/disadvantage isisrisk: progressive disorders bring about about physical, physical, psychological and social complications progressive can be be avoided avoided if if they they are are anticipated. anticipated. A A second second recurring theme has has been been that can information: people with PD and parkinsonism need need continuing continuing access access to sources sources of information and and education education throughout throughout the course course of their their disease disease to enable enable of information promote their their own own independence independence and andwell-being. well-being. them to promote

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Dietz Dietz MA, MA, Goetz Goetz CG, CG, Stebbins Stebbins GT GT (1990) (1990) Evaluation Evaluation of of aa modified modified inverted inverted walking walking stick stick as asaa treatment 5, 5, 243—7. Disorders, 243-7. treatmentfor forparkinsonian parkinsonianfreezing freezingepisodes. episodes.Movement MovementDisorders, Doshay LJ U (1962) Doshay (1962) Method Method and and value value ofofphysiotherapy physiotherapy ininParkinson's Parkinson's disease. disease. New New England Journal 878—80. JournalofofMedicine, Medicine,266, 266, 878-80. Vetere-Overfield B, B,Wiltfong Wiltfong D D (1991) (1991) Driving Dubinsky RM, Gray C, Husted D, D, Busenback K, K, Vetere-Overfield 41,41, 5 17—20. in Parkinson's Parkinson'sdisease. disease.Neurology, Neurology, 517-20. Florian family dynamics and Florian V, V, Katz KatzS,5,Laman LamanVV(1989) (1989)Impact Impact of oftraumatic traumatic brain damage on family functioning: aareview. functioning: review.Brain BrainInjury, Injury,3,3,2 19—33. 219-33. JA, Langdon Langdon DW, DW,Hobart HobartJC, JC,Thompson Thompson AJ AJ(1997) (1997)The Theimpact impactof ofinpatient inpatient rehabilitarehabilitaFreeman JA, tion 42,42, 236—44. Neurology, 236-44. tion on onprogressive progressivemultiple multiplesclerosis. sclerosis.Annals AnnalsofofNeurology, Goldberg DP, DP, Hillier Hillier VF VF (1979) (1979) A A scaled Goldberg scaled version version of the the General General Health Health Questionnaire. Questionnaire. Psychologi cal Medicine, 9, 139—45 Psychological Medicine, 9, 139^5 Gotham A-M, Brown RG, RG, Marsden Marsden CD CD (1986) (1986) Depression Depression in in Parkinson's Parkinson's disease: disease: aa quantitative quantitative and of of Neurology, Neurosurgery and Psychiatry, 49, 3849, 1—9. Journal Neurology, Neurosurgery, and Psychiatry, 381-9. and qualitative qualitativeanalysis. analysis.Journal 11,11, 538—43. Granger ME ME (1961) (1961) Exacerbations Exacerbationsininparkinsonism. parkinsonism.Neurology, Neurology, 538-43. Gray R, R, Stern Stern G, G, Malone-Lee Malone-Lee JJ (1995) (1995) Lower Lowerurinary urinary tract tract dysfunction dysfunction in Parkinson's disease: Gray disease: 499—504. changes relate relate to to age ageand andnot notdisease. disease.Age Ageand andAgeing, Ageing,24,24, 499-504. Pring TR TR (1990) (1990) Speech Speech therapy therapy in in Parkinson's Parkinson's disease: review and and further further data. Johnson JA, JA, Pring disease: aa review British Journal of of Communication, 25, 25, 183—94. British JournalofofDisorders Disorders Communication, 183-94. Jost WH WH (1997) Gastrointestinal motility problems in patients with Jost (1997) Gastrointestinal with Parkinson's Parkinson's disease. disease. 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in Parkinson's Medicine,97, 97, Parkinson's disease: disease: A A randomised randomised controlled controlled trial. trial.American AmericanJournal JournalofofMedicine, 429—35. 429-35. Multiple with MS. London. Multiple Sclerosis Sclerosis Society (1997) (1997) Standards StandardsofofHealthcare Healthcarefor forPeople People with MS. London. Mutch WJ, WJ, Strudwick Strudwick A, A, Roy Roy SK, Downie AW (1986) Parkinson's Parkinson's disease: disease: disability, disability, review review and and management. Journal, 293, 675—7. BritishMedical Medical Journal, 293, 675-7. management.British Oxtoby M andand forfor thethe Long-term M (1988) (1988) AA Strategy Strategyfor forthe theManagement ManagementofofParkinson's Parkinson'sDisease Disease Long-term Patients and and their their Carers. Carers.London: London:Parkinson's Parkinson'sDisease DiseaseSociety. Society. Support of Patients Pd MD 693—704. Pell MD(1996) (1996)On Onthe thereceptive receptiveprosodic prosodicloss lossininParkinson's Parkinson'sdisease. disease.Cortex, Cortex,32,32, 693-704. Quinn NP, NP, Koller Koller WC, Lang Lang AE, Marsden Marsden CD (1984) (1984) Painful Painful Parkinson's Parkinson's disease. disease. Lancet, Lancet, 2, 1366—9. 1366-9. Rodriguez M, Siva A, Ward Ward J, J, Stolp-Smith Stolp-Smith K, K, O'Brien O'Brien P, Kurland Kurland L (1994) (1994) Impairment, Impairment, disabildisability and handicap in multiple sclerosis: A population-based study in Olmsted County, and handicap in multiple sclerosis: A population-based study in Olmsted County, Minnesota. 44,44, 28—33. Neurology, 28-33. Minnesota.Neurology, Routh Ahlskog JE JE (1987) (1987) Parkinson's disease complicated Routh LC, LC, Black Black JL, Ahlskog complicated by byanxiety. anxiety.Mayo MayoClinic Clinic Proceedings, 733—5. Proceedings,62,62, 733-5. Saint Cyr JA, Taylor Taylor AE, AE, Lang LangAE AE(1993) (1993)Neuropsychological Neuropsychological and and psychiatric psychiatric side effects effects in in the treatment ofofParkinson's 547—552 Neurology,43,43, S47-S52 treatment Parkinson'sdisease. disease.Neurology, Soderback 1(1995) rehabilitation. Critical I (1995) Effectiveness Effectiveness of rehabilitation. CriticalReviews ReviewsininPhysical Physicaland andRehabilitation Rehabilitation Medicine, Medicine,7,7,275—86. 275-86. Taggart H, Crawford Crawford VV (1995) (1995) Reduced Reducedbone bone density densityofof the the hip hip in Taggart H, in elderly elderly patients patients with with Parkinson's 326—8. Parkinson'sdisease. disease.Age Ageand andAgeing, Ageing,24,24, 326-8. Neurological Alliance Alliance (1996) (1996) Living Living with with aaNeurological NeurologicalCondition: Condition:Standards StandardsofofCare. Care. The Neurological London. London. Tinetti ME, G, Claus Claus EB, Garrett P, Gottschalk ME, Baker Baker DI, DI, McAvay McAvay G, EB, Garrett Gottschalk M, Koch Koch ML, Trainor K, K, Horwitz RI (1994) multifactorial intervention intervention to reduce the risk (1994) A multifactorial risk of of falling falling among elderly elderly people living Journal of Medicine, 331,331, 82 1—7. England Journal of Medicine, 821-7. people living in in the thecommunity. community.New NewEngland Ward CD Reviews in in Clinical Gerontology, 2, 254—68. CD (1992) (1992) Rehabilitation RehabilitationininParkinson's Parkinson'sdisease. disease. Reviews Clinical Gerontology, 2,254-68. Ward CD, McIntosh S (1993) The rehabilitation process: a neurological perspective. In: Ward CD, McIntosh S (1993) The rehabilitation process: a neurological perspective. In: Neurological Rehabilitation,eds. eds.Greenwood GreenwoodR, R, Barnes Barnes MP, MP, McMillan Neurological Rehabilitation, McMillan TM, Ward Ward CD, CD, pp.i3—27. Edinburgh: Edinburgh: Churchill Livingstone. pp.13-27. Livingstone. Wilkinson D (1992) The psychogeriatrician's view: management management of chronic neurological neurological disabildisability in the community. Journal of Neurology, Neurosurgery, and Psychiatry, 55, Suppl. the community. Journal of Neurology, Neurosurgery and Psychiatry, 55, Suppl. 41-4. World Health Organization ClassificationofofImpairments, Impairments,Disabilities, Disabilities, Organization (1980) (1980)The TheInternational InternationalClassification Relating to to thethe Consequences of Disease. Geneva: and Handicaps Handicaps - aaManual ManualofofClassification Classification Relating Consequences of Disease. Geneva: WHO. World Health Organization Organization (1997) (1997)The TheInternational InternationalClassification ClassificationofofImpairments, Impairments,Disabilities, Disabilities, Handicaps. Draft and Handicaps. Draft Revision, Revision,1-beta, 1-beta,Geneva: Geneva:WHO. WHO.

Rehabilitation, nursing and elderly patients with with Parkinson's Parkinson's disease disease Sally Sally Roberts Roberts

Introduction nursing assessment assessment and and management of Parkinson's disease (PD) in The successful successful nursing elderly patients requires requires a sound sound knowledge knowledge not not only of the natural history elderly patients history of of PD PD people, but but also alsothe the important important principles principles that that underpin geriatric medicine in older people, nurse will will be be considered considered specifically specifically in in and social aspects of aging. The role of the nurse relation to PD though much much of of the the discussion discussion isis equally equally applicable applicable to other causes causes Parkinsonism due due to to multisystem multisystem degenerative degenerative disease disease is is usually of parkinsonism. Parkinsonism more rapidly disabling disabling than PD and and also also has has aa shorter shorter natural natural history. history. more rapidly than PD Rehabilitation successful management involves a joint Rehabilitation is at the core of successful management in PD and involves effort between effort between the the patient, patient, carer carer and multidisciplinary team.

Nursing care provision in in PD PD depends upon an understanding Best nursing care depends understanding of of the the impairments, impairments,disabilities disabilities and handicaps that result result from from PD. PD. This This isis not notalways always easy easy to to achieve achieve as as PD PD is, is, except relativelyrare rare disorder. disorder. Many Many nurses nurses in in primary primary health except in extreme old age, aa relatively care and in hospital service may have little little experience experience or knowledge of this disease. disease. However, PD are are However,the the principles principlesunderlying underlyingthe theplanning planningofof nursing nursing care care in in PD to chronic chronic progressive progressive neurological neurological disease disease in general, general. This is an imporimporcommon to tant area area for for nurse nurse education, education, as as the theprevalence prevalence of of Alzheimer's Alzheimer's disease, disease, PD and and motor neurone neurone disease disease will will substantially increase in the twenty-first twenty-first century. century. The The (and other other coexisting coexisting illness) illness) is continuity of care for the elderly patient with PD (and is also aa nursing nursing priority - whether the care care provision provision setting setting is is the the person's person's own own also residential or nursing nursing home, home, the the GP GP surgery, surgery, the the hospital hospital outpatient outpatient home, aa residential department or during during aa period period of of inpatient inpatient care. care. Continuity Continuity of of nursing nursing care care is is department increasingly increasingly difficult difficulttoto achieve achieveininall allof ofthese these care caresettings settingsand and 'team' 'team' nursing nursing is is often a poor substitute. often The nature of of PD PD and and the the special special aspects of this condition must must always always be taken into account in planning planning nursing nursing activity, activity, even if the episode may not be directly directly 185

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related to PD (e.g. an an acute acnte or or planned planned surgical snrgical admission to hospital). An example of this this is is seen seen with with 'routine' 'rontine' drug drng administration when of antiparof when the the availability availability of kinsonian medication is restricted to the times times of of formal formal drug drng rounds ronnds on the restricted only to ward. Role of nursing assessment assessment in PD PD care A balanced will assist the rehabilitation balanced and and holistic holistic nnrsing nursing assessment assessment will assist the rehabilitation team members in making appropriate choices in care care and and treatment treatment regimes. The nurse nnrse members choices in regimes. The is often often ideally ideallyplaced placedto torecognize recognizethe thelinks linksbetween betweenmedication medication change and altered behaviour (or symptoms) symptoms) and can can 'alert 'alert the the prescriber' prescriber’ to snch such changes changes (Smith (Smith behavionr allow 1998). Re-assessment Re-assessment will willprovide providemeasurable measnrable outcomes ontcomes of of intervention and allow evaluation of the patient's cnrrent current health statns status before fnrther further nnrsing nursing diagnodiagnothe evalnation sis and and care care planning planning takes takes place. place. Demographic Demographic detail, detail, functional fnnctional abilities abilities and and drug dmg are all a part history are part of the general nursing nnrsing assessment. Vital signs signs (lying (lying and and standstanding in the the baseline baseline assessment assessment of ing blood blood pressure pressnre and and weight) weight) are are important in of the elderly elderly patient patient with with PD. PD. Standardized Standardized assessment assessmenttools, tools, covering coveringthe thedomains domains of of the nnrsing nursing assessment, assessment, can be used measure impairments, disimportance in the nsed to measnre abilities Such tools can abilities and and handicap handicap related to PD and any existing comorbidities. Snch measures (snch (such as as the Barthel measnre measure of of ADL) ADL) or specific specific to aspects of be generic measnres Scale). A more account (such as as the Unified Parkinson's Disease Disease Rating Scale). PD (snch more detailed acconnt of assessment assessment tools tools is is given given in in Chapter Chapter 3. Comprehensive Comprehensive assessment assessment by by the the whole whole rehabilitation team shonld should avoid avoid overlap and dnplication duplication in in the the assessment assessment process. effort can be achieved achieved by prior agreement between between health health professionprofessionEconomy of effort als assessment tools should assess assess what and when. when. als about abont the assessment tools to to be used nsed and who shonld nurse is is ideally placed The nnrse placed to to coordinate the comprehensive assessment process at baseline and reassessment after therapeutic interinterbaseline and at times of reassessment after drug drng and and non-drug non-drng therapentic ventions. Of all health professionals professionals the nnrse nurse is is most most likely likely to spend the the longest longest period of of time time with with an an elderly elderly person person with with PD PD and and their theirfamily. family. This This contact contact isis likely period to take take place place over over an extended extended period period of of time. time. The The psychological psychological support snpport and encouragement during care care planning improves comenconragement of patient and carer together dnring munication and gains mutual confidences. The nurse can actively actively promote promote the the mnnication and gains mntnal confidences. The nnrse can direct involvement in the the process process of of care care planning. planning. This This will will assist in involvement of the patient in identifying, prioritizing and focusing on the real problems faced at that time by by that that identifying, prioritizing and focnsing on the real problems faced at that time rather than thanthose thoseperceived perceived by by health health professionals. professionals. patient, rather

The goals of nursing nnrsing care care for for aa person person with with PD PD will will include inclnde improvement improvement and and The goals of maintenance of of the the individnal's individual'soptimnm optimumlevels levelsofofqnality qualityofoflife lifefor foras aslong longas as posposmaintenance sible by preventing or redncing reducing nnwanted unwanted or ornegative negative infinences. influences. A A conceptnal conceptual sible by preventing nursing can can be nsed used as a gnide guide for nurse towards towards action, prodncing producing model of nnrsing for the nnrse gains for for the the patient patientwith withPD, PD,asasdiscnssed discussedby byKelly Kelly (1995) (1995) in in aa case case positive health gains

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study using Orem's Model of nursing care (Orem 1980). 1980). The nurse acts acts as a contact and resource, able able to to recognize recognize needs needs then then initiate initiate appropriate referral in order to to optimize care provision.

Core nursing components in in PD PD care key components components to to the the nursing nursing care care plan plan and and management management for are shown shown The key for PD are in Fig. 11.1. Medication

Treatment regimes become more complicated Treatment regimes become complicated with with disease disease progression. progression. Elderly Elderly patients are also likelytotobe beon on concurrent concurrent medication medication for for other diseases. Timing patients also likely diseases. Timing of oral oral medication medication becomes becomes more more crucial crucial in in maintaining maintaining optimum optimum 'on' time in the food intake. The nurse patient's life, as as does does the the relationship relationship of of oral oral medication medication to food can provide careful explanation can provide a careful explanation of of drug drug actions actions to to help help improve improve and and maintain compliance and can also explain explain the potential potential side side effects effects of antiparkinsonian antiparkinsonian compliance and can drugs. In order to assist assist the patient patient with with tablet tablet administration administration the nurse nurse may may drugs. In order suggest suitable portable suggest the the introduction introduction of a suitable portable container container with with labelled labelled partitions partitions (often called called aa 'dosette'), patient or carer carer can can fill fill with the the scheduled scheduled (often 'dosette'), which which the the patient tablets allows the patient to to see see at at aa glance glance when tablets tablets tablets for for the day. This This method allows regime. This approach can can be particuparticuare due and helps maintain aa complex drug regime. larly useful useful when when significant significant cognitive cognitive impairment impairment is present. The nurse can also provide a written list of medication that that may may have have an adverse adverse effect on PD PD (such effect on (such as neuroleptic drugs and cinnarazine). cinnarazine). The The nurse nurse may may play play aa assessment of elderly elderly patients treatparticularly vital role in the assessment patients for for apomorphine treatment, organizing and performing an an apomorphine apomorphine challenge challenge test, test, assessing assessing treattreatment response, monitoring side effects, effects,and andinitiating initiatingand and maintaining maintaining patients patients on on treatment (see (see Chapter Chapter 3). continuous apomorphine pump treatment

Communication Communication is directly directly impaired impaired by by PD PD and and this this is is aa major major source source of of handicap handicap for patients with PD. Difficulties with communication communication between patient and for Difficulties with and carer, carer, patient and therapist, and patient and and doctor doctor can can lead lead to misunderstanding misunderstanding and increased The nurse nurse can can make make an an early early referreferincreased anxiety anxiety and and handicap handicap in the patient. The ral to a speech and language language therapist for for assessment assessment and advice advice on how how best to to maintain functional functional communication. communication. AAphysiotherapist physiotherapist will will be be able able to give give advice advice exercise to improve speech. speech. on exercise to make make the the person person more aware of of breathing breathing control to improve The The nurse nurse can can motivate motivate and and encourage encourage the the patient patient to to maintain maintain the the programme programme of of therapy interventions. Family Family and carers can be made aware aware that as communicacommunicaand carers tion becomes more difficult difficult it is for patients to to be be allowed allowed time to talk, talk, is important important for

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Core nursing nursing components components in in the the management managementof patients Core of PD patients

Communication -- verbal, verbal,written writtenand andeducational educational packages packages

Medication (administration, (administration, side side effect effect profiles)

maintaining mobility) Mobility (transfers, (transfers, initiating and maintaining

Special senses senses (hearing and vision)

Nutrition (weight (weight, diet diet,protein protein metabolism) metabolism)

bladder/bowel function) Elimination (control of ofbladder/bowel function)

Personal hygiene (washing, (washing, dressing dressing etc.) etc.)

Sleeping (insomnia, daytime drowsiness, vivid dreams)

Sexuality (expressing (expressing sexuality, age and sexuality)

care (planning (planning care care in in community, community, symptom relief) Palliative care relief)

Psychological needs (dementia, stress, stigma, depression)

/ t

Multidisciplinary Multidisciplinary referral referral Speech and language language therapists therapists Speech and Physiotherapists Occupational therapists Psychology services services Dietetics Social workers Fig. 11.1 Fig. 11.1

Nursing plan and management for PD PD patients.

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sentences finished finished for for them, and to not to have sentences to speak speak for for themselves. themselves. Recognition Recognition that, despite communication difficulties, difficulties, the person retains retains the the same same personality, personality, humour and feelings, feelings, needs needs to be tactfully reinforced by the nurse, especially especially when humour and when the burden of caring brings about family family frustrations, frustrations. Handwriting also deteriorates in in PD PD though usually not to the extent of losing also deteriorates usually not financial financial control by being being unable unable to to sign sign aa document document or cheque. If this does occur, the patient should should seek seek legal legal advice so so that that a new new signature signature or the signature of a designated person can be accepted as financial financial authority. large amount amount of often complex and conA second problem is is that in PD there is a large confusing information information that that may fusing may need need to to be be discussed discussed with with patients patients and and families. families. Critical Critical communication needs needs to to take take place place on on at at least least three three stages stages in in the the disease: disease: to PD PD first first develops, develops, and and at at the the palliative palliative stage stage at diagnosis, when dependency due to ofPD. of PD. Most individuals fail fail to retain much of of even even the most basic information given given to to them at the time time the the diagnosis diagnosis of of PD PD isis first first given. given. It It is is aa shattering shattering diagnosis diagnosis to many people fuelled fuelled by fears of aa progressive neurological disease disease and and knowledge knowledge of other people, or elderly relatives relatives with with PD PD recollected recollected from from childhood. childhood, It is also also aa time of considerable fear and anxiety anxiety about the the future. future. Feelings Feelings of of anger, anger, frustrafrustration, denial and despair despair may be very very evident can provide provide evident at this time. The nurse can timely and accurate information information concerning concerningPD PDand andthe thelikely likelyimpact impactthis thiswill will have have on the patient's future, future. Such information can can be reinforced reinforced in writing and also by the provision of appropriate leaflets leaflets and videotapes videotapes from from the Parkinson's Disease Disease Society. The knowledge of an individual and their family and lifestyle gained over Society. The knowledge of an their family and lifestyle gained over the time of the the nurse's nurse's involvement involvementwith withthe thepatient patient can canhelp helpdetermine determine the the timing, timing, the amount and and the the detail detail of information that that needs needs to to be be given given about PD. Mobility

The nurse, who often often has more frequent frequent contact with the patient and and their their family, family, can motivate motivate and encourage encourage the patient to to maintain maintain the theprogramme programmeofofexercises exercises recommended physiotherapist. One most useful useful exercises exercises can recommended by by the the physiotherapist, One of of the most can be be walking with with good stride length, putting the heel down first, toes upwards upwards and feet well apart (to (to assist assist balance). balance). However, However, sitting or or standing standing exercises exercises may be also also well useful, abilities. useful, depending on the patient's abilities. When rising rising from from aa chair, chair, the the nurse nurse can can instruct instruct the the person person to to put their feet When feet together, lean lean forwards forwards and and using using the the arms of the chair for stability push upwards together, with the legs. The The nurse nurse can can encourage encourage the the use use of of the the arms arms of of the the chair chair to to help help the sit down down safely. safely. The of these these strategies strategies regupatient sit The patient patient may need reminding of larly, foot care care may may become become difficult; difficult; larly, encouraged encouraged by family family and and carers. carers. Independent Independent foot the position to monitor the the need need for for referral referral to to aa chiropodist chiropodist the nurse will will be be in in a position Neglected foot care may (podiatrist). Neglected may result result in in pain pain and and mobility problems.

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can finctnate widely in in PD PD and and this needs Mobility can fluctuate widely needs to be be explained explained to to carers carers and family and also particnlarly ifif the the patient patient is is and family and also other health care care professionals, professionals, particularly admitted fluctuations have admitted to hospital. Motor finctnations have provided provided the the basis basis for for the the erroneous occasions throughout day and ons conclusion conclnsion that that the the patient patient 'isn't trying' on occasions thronghont the day and night. (and other other care care settings) settings) the the knowledge knowledge of of finctnations fluctuations night. When When in hospital (and in mobility can influence not possible bed in mobility can infinence not only possible bed positioning positioning in in relation relation to toilets and facilities on but also also the the expectations expectations of of staff staff placed placed on the the and other facilities on the ward, bnt patient. Assessments Assessments for for state state disability disability allowances, allowances, snch such as as Attendance Attendance Allowance Allowance patient. in in the UK, usually nsnally have have set set eligibility eligibilitycriteria criteriathat thatneed needtotobe be met met in in terms terms of of disdisability. state, when when maximally maximally mobile, mobile, may may fail fail to to ability. Patients Patients assessed assessedinin the the 'on' state, reach day mobility mobility would very reach eligibility eligibilitycriteria criteriaeven eventhough thonghmost most of of the the day wonld be be very much mnch worse. often closely linked with with impaired balance and falls. canse aa dradraclosely linked falls. Falls Falls cause Mobility is often matic loss of confidence confidence in in elderly elderly patients patients with with PD, PD,which whichin inturn tnrn leads leads to to reduced rednced mobility mobility and increased increased risk risk of falls. falls. The The nurse nnrse can help overcome overcome this this spiral spiral of of immobility that develops develops after snch such a life life threatening threatening event by promoting safe exercise activities. cise and and confidence confidence boosting activities. for risk risk of of developing developing The elderly person with PD needs to be regnlarly regularly assessed assessed for pressnre dne to possible periods of immobility, weight weight loss, loss, incontinence incontinence and pressure sores due associated with with the aging process. process. Appropriate Appropriate aids to pressure pressnre relief general decline associated can be curative be discussed discnssed with with the the patient and family as a preventative preventative rather rather than cnrative intervention. of safe safe driving driving in in PD PD may may need need to to be be discussed discnssed with with patients patients and famThe issne issue of ilies by the the nurse. nnrse. It is important ilies by important that thataafnll full assessment assessment of of driving driving ability ability isis made, made, otherwise possibly as otherwise the elderly elderly person person with with PD may prematurely prematnrely stop driving, possibly as aa result family pressure. absence of sensory loss loss and cognitive cognitive problems resnlt of family pressnre. In In the absence problems a of driving driving skills skills is is often often the the best best way way to to assess assess fitness to drive. drive. practical test of hearing and Special senses — - hearing and vision

Althongh hearing and visnal Although hearing visual impairment do do not not arise arise directly directly as as aa resnlt result of of PD, PD, impairment of very common in elderly of special special senses senses is very elderly people and magnifies magnifies the impairments impairments dne due to to PD. PD. The The nnrse nurse shonld shouldassess assess vision vision and and hearing hearing ability ability so so that that remediable impairment impairment in in these these areas areas can be treated appropriately. Visual Visnal problems due dne to ocular ocnlar causes canses can can increase increasethe the mobility mobility and and balance balanceproblems problems of of PD PDand and can can Hallucinosis is also increase difficulties difficnlties with with communication. commnnication. Hallncinosis also more more common common in the cause. Impaired hearing is presence of impaired vision and hearing from whatever canse. extremely adults and and isis often often helped helped by by the the removal removal of of wax wax in the the extremely common in older adnlts external the provision provision of of aa hearing aid. aid. external auditory anditory meatus meatns and/or the

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Nutrition Oral hygiene hygiene can can be be aa problem problem due due to the patient clearing their their patient having having difficulty difficulty clearing to infection infection and ulceration. mouth of of food food properly. properly. The stale food residue can lead to ulceration. The nurse can remind the patient to check oral cleaning, cleaning, promote promote oral hygiene hygiene and and also fitting. The The altered altered saliva saliva in in PD PD and and the the tendency to a also ensure dentures are well fitting. despite drooling drooling can can result result in in poorly poorly fitting fitting dentures. dentures. dry mouth despite Eating is, for Eating is, for most people, people, aa pleasurable pleasurable and often often social social event. event. The nurse nurse needs to to support the patient in needs in maintaining maintaining their their confidence confidence and ability ability in independent feeding. The family may already be aware of difficulties the patient patient is pendent feeding. The family may already be aware of difficulties the experiencing and encouraged to participate in in levels levels of social social function experiencing and can can be encouraged function at meal times times with with which which the the patient patient can cope. Presentation and and the preference of meal cope. Presentation preference of taste need need to to be be taken taken into into account account when when preparing preparingmeals. meals. A A person person patients for taste PD may may have impaired impaired sense sense of appearance of food food with PD of smell smell and and taste, so so the appearance is and meal meal enjoyment. enjoyment. It may may take great is very very important important in appetite stimulation and ingenuity offer a liquidized liquidized meal that looks looks interesting, interesting, appealing appealing and and tasty. tasty. ingenuity to to offer Smaller, acceptable, as large meals Smaller, more more frequent frequent meals and snacks may be more acceptable, exhausting and food getting getting cold cold long before before being can be exhausting and time consuming with food finished. Excessive anduncontrollable uncontrollable drooling drooling will willcause causeembarrassment embarrassment and and discomfort Excessive and if clothing patients eventually resort to to the use of if clothing is penetrated. penetrated. Many Many patients eventually resort of padding padding under the clothes. clothes. The nurse nurse needs needs to to be be aware aware of this and and advise advise on on skin skin care care to to prevent soreness soreness developing developing under under the pad. Maintenance of body body weight is important. Calorie Maintenance of weight is Calorie intake intake normally normally needs needs to match energy expenditure. The person with PD may require many more calories to and dyskinesia dyskinesia (Davies (Davies et et al. al. 1994). 1994). Regular compensate for increased muscle tone and weight weight monitoring needs to be aa part part of of the the ongoing ongoing nursing nursingassessment assessment in in conconwith expert expert advice advice from the dietician, dietician. junction with

Elimination

Problems with urinary continence continence are are common common in in elderly elderly people people and and are are also also frefrePD. The extent to which incontinence in in elderly elderly patients with PD is due quent in PD. to aging or directly to PD is still still unclear. unclear. Incontinence Incontinence is rarely rarely a part part of day to to day as a result maybe accepted accepted as as inevdiscussion or debate with family and friends and as itable elderly subjects. whatever means available available to itable by by elderly subjects. This This is is coped coped with with by whatever to the patient, sometimes sometimes for for aa long long period period until until containment containment becomes becomes impossible, impossible, patient, distress, before before medical help is sought. causing social social embarrassment embarrassment and distress, Following careful careful assessment the nurse will be able able to to establish establish the the nature of any any problem and bladder bladder function problem with bowel bowel and function and refer refer the patient patient on on to to specialist specialist medical or nursing advice. advice. Urinary difficulties difficulties are rarely simply urge incontinence incontinence

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due to detrusor hyperactivity or pure stress stress incontinence due due to to pelvic pelvic floor muscle weakness. Bladder Bladder dysfunction dysfunction in elderly patients with PD is usually usually multifactorial in nature and not not all all causes causes are likely likely to to be be responsive responsive to to treatment. Constipation is is a common problem problem encountered encountered by by elderly elderly subjects and patients with PD. In elderly patients with with PD PD constipation can result from a reduced intake elderly patients of oral dysfunction, drug drugside sideeffects, effects, oral fluids fluids or or dietary dietary bulk, bulk, immobility, autonomic autonomic dysfunction, or focal focal dystonia dystonia of the pubococcygeus pubococcygeus causing 'anisnus' - aa failure failure of of muscular attempting to to defecate. defecate. The nurse can provide relevant and approapprorelaxation when attempting nutritional advice advice and and encouragement encouragement in in participating participating ininregular regularexercise, exercise, priate nutritional stimulant which will will promote bowel function. Many elderly patients need to take stimulant laxatives regularly laxatives regularly to to avoid avoid constipation. Sexuality Nurses, along with with other health care care professionals, professionals,tend tendto toskirt skirtaround around the the impact impact

of disability on sexual sexual function. function. In relation to aging Ware (1998) (1998) found found of disability on aging Catesby Catesby Ware 25% of the 80 years sexual relations, relations. The feeling feeling that 25% years plus plus group group reported normal sexual of insufficient insufficient skill skill in in dealing dealing with with needs needs of of the the chronically chronically disabled in terms of sexuality, as described described by by Conine Conine and Evans (1982), suggests suggests aa more more proactive proactive nursing approach is often often provided. provided. Burgener Burgener and Logan Logan (1989) (1989) consider consider that the the approach than is nurse, before the role role of of sexual sexual counsellor, needs to be comfortable comfortable before embarking on the with their their own own sexuality. sexuality. Other Other considerations considerations should should include include knowledge knowledge of with sexual response in in older older adults adults and and the the possible possible limitations limitations set setby by physical physical normal sexual impairment. A nurse can more easily easily inquire effect of inquire about the effect of PD PD on on the the patient's patient's for more more sensitive sensitive concerns. concerns. sexual relations, and this will provide provide a starting point for will give to ask' ask' (Burgener (Burgener and and Logan Logan 1989), 1989), as as itit isis This will give the the patient patient 'permission to unlikely that the elderly elderly patient will broach the subject subject - possibly feeling unlikely feeling itit to be inappropriate. inappropriate. Patient Patient ownership ownership of of the the problem problem may may pose pose certain certain barriers barriers to to the the assessment. Encouragement nurse in her assessment. Encouragement to to the patient to see the problem as not will assist in providing just theirs, but shared with their partner, will providing a more complete and mutually beneficial beneficial picture. Sexual problems may not be Sexual be directly directly associated associated with with PD. PD.Gillie Gillie (1990) (1990) suggests suggests that one of impotence one in in ten ten men men suffer suffer from from impotence impotence and andreports reportsthat that50—80% 50-80% of is is due to a physical cause. In aa study of of very very elderly elderly subjects subjects Catesby Catesby Ware (1989) (1989) reports that that loss loss of erectile capability seems to be be due due to to multiple causes. In women, reduced vaginismus may play a role role in in decreased decreased sexual sexual desire. desire. reduced lubrication lubrication and vaginismus discussion, the nurse nurse may may establish establish whether the the problem problem isis of of aa physical physical Through discussion, nature or due to other influences. influences. Conine and Evans (1982) endorse the feeling feeling that that sexual sexual intimacy intimacy isis dependent dependent (1982) endorse on effective effective communications communications between between aa couple, couple, especially especially if there is is limited movemovement or spontaneity - as as occurs in PD. The decline in verbal verbal and nonverbal sexual

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can be be mutual. Reduced of touch and responsiveness can Reduced frequency frequency of and caressing caressing may may become evident (with or without the the intention intentionof ofhaving having sex). sex). Timetabling of sexual around best medication Timetabling of sexual activity activity around medication responses, responses, continence continence problems and privacy will will require require open open discussion discussion by by the the couple. couple. The The nurse's nurse's role role will be be to to facilitate facilitate and and encourage encourage such such enhanced enhanced communication. Practical advice can be offered offered regarding regarding alternative alternative positioning, positioning,devices devices availavailduring sex. sex. Literature sources sources can be able, and and minimizing the risk of incontinence during and many many helpful helpful leaflets leaflets are are available available in in the the UK UK from from The The Association Association provided, and to Aid the Sexual Sexual and Personal Relationships Relationships of People with a Disability (SPOD) Parkinson's Disease Disease Society (PDS). and the Parkinson's Admission to hospital when admitIndividuals with PD are are sometimes placed placed in considerable considerable difficulty difficulty when procedures, a period period of planned respite admission, ted to hospital either for elective elective procedures, admission, or as an an emergency emergency admission to medical, surgical or orthopaedic orthopaedic wards. wards.Busy Busy ward ward staff always appreciate medication in in PD. PD. staff do do not always appreciate the the critical critical nature nature and timing of medication The spontaneous motor motor fluctuations fluctuations of PD PD and and the thesometimes sometimes unpredictable unpredictable The spontaneous response medication are are often often poorly poorly appreciated. appreciated. Standard Standard procedure response to to medication procedure on on medadmission to hospital is often often the removal of of medication from patients, with medication situation is ication only only being being dispensed dispensed at at the the fixed fixed times times of of drug drug rounds. This situation clearly undesirable clearly undesirable for for patients patients with with PD PD on complex complex drug regimes. After After admission admission to hospital patients with PD should be be allowed allowed to self-medicate self-medicate whenever whenever practipractiadmission. cal, given given the the circumstances circumstances of their admission. There has been been very little scientific scientificstudy studyofofthe the impact impact of of PD PD on on common There has very little common medical and surgical emergencies. Considerably more more work work needs needs to to be done done in in this this area to inform clinical clinical practice. Clinical Clinical anecdote suggests suggests that PD area that patients with PD less good after both both elective elective and and emergency emergency orthopaedic orthopaedic surgery. surgery. have less good outcome after This impression is supported by by some (Coughlin (Coughlin and and Templeton Templeton 1980, 1980, Eventov Eventov et al. 1983, 1983, Gialanella Gialanella et et al. al. 1991), 1991),but butnot not all allstudies studies of offemur femur fracture fracture in patients with PD (Turcotte et al, al. 1988), 1988). These were were all all retrospective retrospective case case note note studies. studies, Anaesthesia presents presents particular particular problems problems for Anaesthesia for patients with PD PD (Severn (Severn 1988). 1988). Intubation can Intubation can be be difficult difficult due to axial deformity and laryngospasm (Backus (Backus 1991). 1991). Swallowingproblems problems in in PD PD increase increasethe the risk risk of of perioperative perioperative aspiration aspiration and may Swallowing lead to to increased increased risk risk of of postoperative postoperative chest chest infections infections (Vincken (Vincken et et al. al. 1984). 1984). lead Muscular Muscular rigidity rigidity can can make make adequate adequate ventilation ventilationthroughout throughout the the operative operative period difficult to difficult to achieve achieve (Stoelting (Stoeltingetet al. al. 1988). 1988).Tremor Tremorcan canbe bemistaken mistakenon on the the heart heart monitor for ventricular ventricular fibrillation fibrillation (Reed (Reed and and Han Han 1992). 1992).Several Several anaesthetic anaesthetic monitor for electrolyte imbalagents may may worsen worsen PD PD (halothane, neuroleptics, opioids), cause electrolyte imbalance (succinylcholine), or result result in in unstable unstable blood blood pressure pressure control due to the additional underlying autonomic dysfunction dysfunction of of PD, PD.

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Guidelinesfor for the the management management of the patient Table 11.1 11.1 Guidelines patient with withPD PDover over aasurgical surgical admission On admission

Accuratelydetermine determine existing existing drug drug treatment treatment for P1) (family physician physician • Accurately PD (family to be contacted contacted to confirm confirm this) and the usual timthg may need to timing of of drug drug treatment • Carefully pattern and fluctuation Carefully note the descriptions of disease pattern fluctuation reported by the patient and/or caret carer.The Thetiming timing of of fluctuations fluctuations are are important for for optimal optimal patient care care • Inform admission Inform team team normally normally looking looking after the patient's PD of their admission

Pre-operative care

Continue antiparkinsonian drug treatment as to the the point point of of • Continue as closely closely to anaesthesia as possible possible (in (in consultation with senior anaesthetist) anaesthetist) •• Restart medication if surgery cancelled • Subcutaneous Subcutaneous apomorphine apomorphine or or parenteral parenteral anticholinergic anticholinergic drug drug treatment should be considered • Maintain Maintain adequate adequate hydration

Operative care

The surgical surgical and and anaesthetic anaesthetic team team need to be aware of potential • The problems and hazards of anaesthesia anaesthesia in PD

Post-operative care

Recommence antiparkinsonian antiparkinsonian medication as soon as possible possible after after • Recommence recovery • Avoid that worsen worsen parkinsonism Avoid anti-emetics, sedatives sedatives and analgesics analgesics that

There is is still stilluncertainty uncertainty about about how how best best to to handle handle the the drug drug treatment treatment for PD in the patient patient undergoing undergoing surgery. surgery. Generally, Generally, it is is advised advised that levodopa levodopa should should be be given given right right up up to to the the operative operative period period to to minimise minimise complications complications and and improve improve the postoperative recovery recovery (Hyman (Hymanetetal. al.1988, 1988,Katz Katz et et al. al. 1990, 1990, Reed and Han speed of postoperative 1992). However, 1992). However, itithas has also alsobeen beenclaimed claimedthat that levodopa levodopa can can be be discontinued discontinued up to one day preoperatively adversely effecting Harrison preoperatively without without adversely effectingoutcome outcome (Pollard (Pollard and and Harrison 1989). ItIt would for as as 1989), would seem seemprudent prudent to to maintain maintain antiparkinsonian drug treatment for preoperatively as as soon soon as as possible possible after after long preoperatively as possible possible and and to restart medication as surgery. Apomorphine by subcutaneous infusion can control parkinsonian sympsurgery. Apomorphine infusion symptoms before, before, during and and after after surgery. surgery. Domperidone Domperidone can can be be given given rectally rectally to toms control vomiting. Apomorphine needs to be seriously seriously considered considered to control nausea nausea and and vomiting. Apomorphine needs to control symptoms in in patients patients with with moderate moderateto tosevere severe PD PD undergoing undergoingelective elective sursurgical gical procedures. Guidelines can be be developed developed for for managing managing PD PD on general medical, surgical surgical and and Guidelines can 11.1) to to reflect reflect local localprocedures procedures and and needs. needs. The The PD PD orthopaedic wards (see (see Table Table 11.1) specialist nurse can play an important important role role in indeveloping developing good good practice practiceby byinvolvinvolvspecialist ing developing guidelines medical, ing relevant relevant stakeholders stakeholders in in developing guidelines for for managing managing PD PD on medical, orthopaedic wards. wards. surgical and orthopaedic

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Palliative care care people express express wishes wishestotobe beininfamiliar familiar surroundings, surroundings, Towards the end of life life many people

and with the people closest closest to them, them. ItIt is is the nurse's nurse's role role to to facilitate facilitate these requests requests coordinate the the best best care care in this setting. and then coordinate Lindop that current Lindop et al. al. (1997) (1997) suggest suggest that current trends trends for for palliative palliative care care are are now now moving from from hospital requiring multidisciplinary moving hospital to community community - again requiring multidisciplinary and interagency greater degree than has has been previously previously been interagency working, working, but but to a much greater thought. Responsibility Responsibility lies with the nurse to to liaise liaise with relatives relatives about all all aspects aspects care in in these these final final stages. stages. of nursing care

PD specialist specialist nurse -—the thefuture future of of nursing care in PD The PD PD that primary health care teams can indepenDue to the rarity of of PD PD it is is unlikely unlikely that dently develop develop the necessary expertise to to optimally manage PD. In the UK, where necessary expertise primary health health care care groups groups are are becoming becoming increasingly increasingly responsible responsible for for purchasing purchasing health care, a new trend isis emerging emerging with with commissioners commissioners 'buying in' in' specialist specialist health care, nursing services services from established established specialist specialist sites sites for for PD PD care. care. In the UK the PDS, supported by the pharmaceutical industry, industry, has has actively actively promoted the development development of of the the PD PD specialist specialist nurse/nurse nurse/nurse advisor. advisor. The The PD PD specialspecialfulfil the the entire entire major major nursing roles in the care of patients patients with PD and ist nurse can fulfil al. 1988) 1988) their families. The success (Oxtoby et et al. success of of the the initial initial PDS PDS funded funded pilot study (Oxtoby has led significant expansion of these posts around around the theUK. UK. Specialist Specialist nurses nurses led to a significant have have been been based based in in primary primary care care and and have havebeen beenlinked linkedto toestablished establishedmovement movement disdisclinics run by geriatricians geriatricians and neurologists. Publication of a detailed study order clinics progress in in the the UK UK evaluating evaluating the theeffectiveness effectiveness and and cost cost effectiveness effectiveness currently in progress eagerly awaited. of the PD specialist nurse isis eagerly The qualification requirements for for employment nurse are conqualification requirements employment as a specialist specialist nurse stantly improve academic academic and clinical clinical standards standards of stantly being being reviewed reviewed in in order order to improve nursing nursing care. care. The The necessary necessary clinical clinical supervision supervision may may be be difficult difficult for for specialist specialist nurses to find, and guidance guidance relating relating to to clinical clinical practice can can be be difficult difficult to achieve achieve of many many PD PD nurses. nurses. Guidelines referring referring to clinical clinical and due to the autonomy of and professional specialist nurses widely debated by by fessional practice practice for for PD specialist nurses are presently being widely professional Royal College professional nursing groups in the UK, including the Royal College of of Nursing. Nursing. In In the future the specialist specialist nurse maybe may be responsible responsible for for direct direct prescribing prescribing in in PD PD and and already nurse diagnosis of parkinsonism already nurse specialists specialists are are competent competent in in the accurate diagnosis in primary care. care. The The specialist specialist nurse can can increase increase the awareness awareness of the possibility possibility elderly people by involving nursing of parkinsonism in elderly involving primary primary health health teams and nursing staff in screening procedures to detect detect early early signs of this condition. home staff

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Summary The The

central role role in in the the long-term long-term management management of of PD and and parkinsonnurse has a central management of these condiism in elderly subjects. The successful successful and effective effective management dependent upon uponthe theskill, skill, knowledge knowledge and and organizational organizational abilities abilities of of nursing nursing tions is dependent staff. The nurse's nurse's role role covers coversnot not only onlytraditional traditional nursing care, but also an extended aspects of the medical role supporting supporting the drug treatment of PD. Other important aspects specific nursing rehabilitation interinterspecific nursing role role in in PD include include education, education, promotion of rehabilitation ventions, support counselling of patient, patient, and and enhancing enhancing communication communication ventions, support and counselling professionals. The nurse can provide aa between the patient, carer and other health professionals. care in the traditional traditional multidisciplimultidiscipliplanning of of care pivotal role in the integration and planning nary team. team,

REFERENCES

WW, Ward Ward RR, Vitkun SA, Fitzgerald D, D, Askanazi Askanazi JJ (1991) (1991) Postextubation Postextubation laryngeal Backus WW, RR, Vitkun SA, Fitzgerald spasm in an unanesthetized patient with Parkinson's disease. Journal of Clinical Anesthesia, 3, 3, an unanesthetized patient with Parkinson's disease. Journal of Clinical Anesthesia, 314—16. 314-16. Burgener S, S, Logan Logan G (1989) Concerns of the Burgener (1989) Sexuality: Sexuality: Concerns the post-stroke post-stroke patient. patient.Rehabilitation Rehabilitation Nursing, Nursing,14, 14,4,4,178—81. 178-81. Catesby Ware J (1989) Impotence Geriatric Medicine, 5, 2,5,301—14. Impotenceand andageing. ageing.Clinics Clinicsinin Geriatric Medicine, 2, 301-14. Conine TA, Evans JH JH (1982) (1982) Sexuality Sexuality reactivation reactivation of of chronically chronically and disabled adults. Journal TA, Evans Journalofof Allied Health, 261—70. Allied Health,11, 11,261-70. Coughlin Clinical Coughlin L, Templeton Templeton J (1980) (1980) Hip fractures fractures in patients patients with with Parkinson's Parkinson's disease. disease. Clinical Orthopaedics Related Research, 148,148, 192—S. Orthopaedicsand and Related Research, 192-5. Davies KN, KN, King KingD, D,Davies DaviesHH(1994) (1994)AAstudy studyofofthe thenutritional nutritional status status of elderly patients with Davies elderly patients Parkinson's 142—S. Ageing,23,23, 142-5. Parkinson'sdisease. disease.Age Ageand andAgeing, Eventov fracturesinin patients patients with Eventov I, Moreno M, Geller Geller E, Tardiman R, R, Salama Salama R R (1983) (1983) I-Tip Hip fractures Parkinson's syndrome. Journal of Trauma, 23, 2, 98—lOl. Parkinson's syndrome. Journal of Trauma, 23, 2, 98-101. Gialanella Mattioli F, F, D'Alessandro D'Alessandro G, G, Bonomelli Bonomelli M, M, Zancan Zancan A, Luisa Luisa A A (1991) (1991) Prognosis Prognosis of of Gialanella B, Mattioli femur fractures in and Extrapyramidal Disorders. in parkinsonian parkinsonianpatients. patients.In: In:Parkinson's Parkinson'sDisease Disease and Extrapyramidal Disorders. Pathophysiology eds. Agnoli A, A, Fabbrini G, G, Stocchi F, pp. 591—4, John Pathophysiologyand andTreatment, Treatment, eds. Agnoli Fabbrini Stocchi F, pp. 591—4,London: London: John Libbey. Libbey. Gillie O 0 (1990) Calvert's Impotence:One OneininTen TenMen. Men.Channel Channel4 4Television TelevisionPublication. Publication.London: London: Calvert's (1990)Impotence: Press. Press. Hyman SA, WD, Maciunas Maciunas RJ, RJ, Allen Allen GS, GS,Berman Berman ML ML (1988) (1988) Perioperative Perioperative management management SA, Rogers WD, for transplant transplant of ofautologous autologousadrenal adrenalmedulla medullatotothe thebrain brainfor forparkinsonism. parkinsonism.Anesthesiology, Anesthesiology,69,69, 618—22. 618-22. Katz J, J, Benumof Benumof JL, Katz JL, Kadis Kadis LB LB (1990) (1990) Anesthesia Anesthesia and Uncommon Uncommon Diseases. Diseases. Philadelphia: Philadelphia:WB WB Saunders Company. Company. Kelly G G (1995) A Self-care approach. 40—1. Times,91,2, 91, 2, 40-1. approach.Nursing NursingTimes,

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Nursing Beach R, R, Read ReadSS(1997) (1997)AAcomposite composite model model of palliative palliative care care for for the the UK. UK. International International Lindop E, Beach Journal 3, 5, JournalofofPalliative PalliativeNursing, Nursing, 3,287—92. 5, 287-92. DG, Schreibman Schreibman DL, DL, Matjasko Matjasko MJ MJ (1990) (1990) Neurological Neurological diseases. diseases. In: In: Anesthesia Anesthesia and Martz DG, and Uncommon Diseases, Diseases, eds. Katz Katz J,J, Benumof Benumof JL, JL,Kadis KadisLB, LB,pp. pp.560—89. 560-89. Philadelphia: Philadelphia: W W BB Uncommon Saunders Company. ofof Practice. New York: McGraw-Hill. Orem DE DE (1980) (1980) Nursing: Nursing:Concepts Concepts Practice. New York: McGraw-Hill. P, Porteous Porteous A, Thurood 5, Oxtoby M, Fthdley Findley L, Kelson N, Pearce P, S, Wood Wood A A (1988) (1988) A A Strategy Strategyfor for the Management of Long-term and their Parkinson's Disease Diseaseand andfor forthethe Long-termSupport SupportofofPatients Patients and their of Parkinson's Carers. Carers.London: London:Parkinson's Parkinson'sDisease DiseaseSociety. Society. Pollard BJ, Harrison MJ MJ eds eds (1989) (1989) Parkinson's Parkinson'sdisease. disease.In: In:Anaesthesia Anaesthesiafor forUncommon UncommonDiseases, Diseases, p. 194. Scientific Publications. 194. Oxford: Blackwell Blackwell Scientific Reed AP, AP, Han Han DG DG (1992) Intraoperative Reed Intraoperative exacerbation exacerbation of of Parkinson's Parkinson's disease. disease. Anesthesia Anesthesia and and Analgesia, 850—3. Analgesia,75,75, 850-3. Journal of Anaesthesia, 61,61, 761—70. Severn AM (1988) Parkinsonism Parkinsonismand andthe theanaesthetist. anaesthetist.British British Journal ofAnaesthesia, 761-70. Smith 5S (1998) (1998) AAnew newlook lookatatelderly elderlycare. care.Nursing NursingTimes, Times,94,94, 42-3. 14,14, 42—3. Stoelting system. In: Stoelting RK, RK, Dierdorf Dierdorf SF, SF,McCammon McCammon MD MD eds eds (1988) (1988) Diseases Diseases of of the the nervous nervous system. Anesthesia and Co-existing Disorders, 2nd 2nd edition, pp. New York: Anesthesia and Co-existing Disorders, pp.263—354. 263-354. New York: Churchill Churchill Livingstone. Livingstone. and Parkinson's Parkinson's disease. disease. Clinical Clinical Turcotte R, Godin C, Duchesne R, Jodoin A (1988) Hip fractures and Orthopaedics Related Research, 256,256, 132—6. Orthopaedicsand and Related Research, 132-6. Vincken WG, SB, Doilfuss Dollfuss RE, RE, Darauay Darauay CM, CM, Cosio Cosio MG MG (1984) (1984) Vincken WG, Gauthier Gauthier SB, RE, Hanson Hanson RE, Involvement of upper airway Involvement airway muscles in extrapyraniidal extrapyramidal disorders. disorders. A A cause cause of of airflow airflow limitalimitation. of of Medicine, 311, 438—42. EnglandJournal Journal Medicine, 311, 438-42. tion.New NewEngland

Rehabilitation, physiotherapy and elderly patients with with Parkinson's Parkinson's disease disease Hilary Chatterton and Brenda Brenda Lovgreen Lövgreen Hilary

Introduction A primary motor motor disorder, disorder, such such as as Parkinson's Parkinson's disease disease (PD), appears to be an ideal target for physiotherapy intervention. Referral Referral to physiotherapy physiotherapy is recommended in the early stages of the the disease disease (Dobbsetal. (Dobbs et al. 1992) 1992) and there is evidence of the clinical ical effectiveness effectivenessofofphysiotherapy physiotherapyatatthis thistime time (Comella (Comella et et al. al. 1994). 1994). Unfortunately Unfortunately itit is still still more more common common for for referral referral to be be delayed delayed until the the disease disease is advanced advanced 1982), when initiate aa preventative preventative treatment strategy strategy (Oxtoby 1982), when the the opportunity to initiate passed. has passed. significant role role to to play play in in the the short short and long-term managePhysiotherapy has a significant management of of PD. PD. Physiotherapy must be integrated with with other other therapies. therapies.This This will will maxmaximize carer by by ensuring ensuring consistency consistency of imize the the benefits benefits of of therapy therapy for for the patient and carer approach, reinforcement of treatment aims, aims, and and by by developing developing appropriate comcomapproach, reinforcement strategies (Kauser (Kauser and and Powell Powell 1996). 1996). pensatory strategies

The pathophysiology of the motor motor disorder disorder in in PD PD deficiency of of dopamine dopamine in in the the basal basal ganglia gangliamotor motor control control is is impaired impaired in in Due to a deficiency PD. Patients this in terms of Patients experience experience this of difficulties difficulties with initiating, initiating, maintaining, maintaining, from one sequence sequence of ofvoluntary and changing from voluntary movement movement to to another, another.Excess Excess abnormovement, such such as as tremor, tremor, may may also also be be present. present. Balance Balance is often often mal involuntary movement, in elderly elderly patients patients with with PD. PD. Clinical examination examination defines defines these these difficulties difficulties impaired in in terms of akinesia, bradykinesia, postural reflexes. reflexes. bradykinesia, tremor, rigidity and impaired postural Impaired to these these problems problems and and aa Impaired integration integration of of normal control may contribute to sense of increased increased effort effort of movement movement may may result result (Lovgreen (Lövgreen and Cody 1997). 1997). sense and Cody During active voluntary movements sensory sensory feedback feedback is utilized utilized by the central central During active voluntary movements nervous system to interpret the the ongoing ongoing action actionin interms termsof offorces forces that that are are being being gengenerated Impulses, known as as corollary diserated and trajectories trajectories of movements produced. Impulses, charges, charges, are are generated generated in in the the higher levels levelsof ofthe thenervous nervous system systemand and are are integrated integrated with afferent afferent feedback movement. In there with feedback to to give give an an internal internal sense sense of of the the movement. In PD there 198

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Table 12.1 Aims of physiotherapy treatment in 12.1 Aims in PD

Ensure each eachpatient patient isisfunctioning functioningto tomaximum maximum potential potential • Ensure • Maintain possible Maintain the the patient patient atatthe thehighest highest level level of functional independence for for as as long as possible • Slow decline and onset onset of movement movement problems within the constraints imposed by Slow the rate of decline the disease disease process • Prevent Prevent the secondary complications of immobility • Educate Educate both the the patient patient and andcarers carers to to enable enable them them to to take take the the lead lead role role in in the the physical physical management of of this disease disease •

appears appears to be some failure failure in this model with kinaesthetic information generated generated during during a movement movement being being incorrectly incorrectly compared compared to original original motor motor commands commands (Moore 1987, 1987, Sanes and Shadmer Shadmer 1995, 1995,Dietz Dietz et et al. al. 1993). 1993). Movement Movement problems may not only only be be of of aa physical physical origin, origin, but may may also also result result from deficits deficits of of communication communication and/or cognition. from cognition. Cognitive Cognitive problems may present in many forms and may may be initially recognized recognized when when advice advice isis not not followed followed due due to to lack of understanding.

Clinical guidelines for clinical clinical practice practice in inthe the management management of of In the United Kingdom (UK), guidelines for neurological published by Association of neurological and and elderly elderly patients patients have have been been published by the Association Chartered Physiotherapists Physiotherapists Interested Neurology (ACPIN the Chartered Interested inin Neurology (ACPIN 1995) 1995) and and the Physiotherapists with with aaSpecial Special Interest Interest in in Elderly Elderly People People Association of Chartered Physiotherapists (ACSIEP 1991). holistic (ACSIEP 1991). Physiotherapy Physiotherapy in PD should be assessment based, taking aa holistic involve joint and individualized approach to meet the needs of patients, and should involve joint patient and andcarer carer(Baker (Baker etet al. al. 1997, 1997, Greenfield et al. goal setting setting between between therapist, patient 1995). The overall overall aims of physiotherapy treatment treatment in in PD PD are areshown shownininTable Table 12,1, 12.1. To To achieve the aims aims of of treatment treatment it is necessary necessary for for patients patients with with PD PD to to be be referred referred to to achieve the the physiotherapist at or even before diagnosis is confirmed. An open referral the physiotherapist at or even before diagnosis is confirmed. open referral system course system is is advocated, advocated, where where initial initial referral referral should be for a lifetime, not just aa course of treatment.

Assessment Frequent reassessment is necessary necessary to to evaluate evaluate efficacy efficacyofoftreatment, treatment, to to identify identify the for modification, and to ensure pertinent need for pertinent issues issues are are addressed. There is a tendency within within the profession profession for for physiotherapists physiotherapists to to use use assessment assessmentprocedures procedures that that are individual to their own One small small own practice practice or or institution institution(LOvgreen (Lövgreen et al. 1996). One

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survey reported reported that 74% 74% of of therapists therapists do do uot not use useexistiug existing standardized standardized assessassess-

ments (Robb and Lennon Lennon 1997). 1997). A A standardized standardized assessment assessment is is available available from from the the Parkinson's Society in in the UK (Franklyn Parkinson's Disease Disease Society (Franklyn 1986). 1986). Other Other scales scales commonly commonly physiotherapists include the internationally internationally recognized recognized Webster Webster Rating Rating used by physiotherapists the }-Ioehn Hoehn and Yahr Yahr scale (LOvgreen (Lövgreen etet al. scale and the al. 1996). 1996). Assessment procedures should should give give an an overview of the the general Assessment procedures overview of general status of of the the as well well as addressing all relevant physical patient, as patient, physical aspects. aspects. This This should should include include funcreference to initiation, sequencing, timing and and quality quality of of movemovetional ability with reference ment, posture, posture, balance, balance, and and underlying underlying muscle muscle tone. Soft tissue tissue length length and and ment, tone. Soft extensibility area to to assess assess (Williams (Williams 1990), 1990), as as is respiratory respiratory extensibility isis also also an an important area and exercise exercise tolerance (Hovestadt (Hovestadt etet al. al. 1989, 1989, Sabate Sabate et al. 1996). The relfunction and evance affecting activity evance of of autonomic autonomic disturbances affecting activity should should also also be be considered considered (e.g. (e.g. dizziness hypotension).Assessment Assessment should should take take place place dizziness on on standing standing due to postural hypotension). at defined time relation to antiparkinsonian antiparkinsonian drug-induced drug-induced motor motor improveimproveat a defined time in relation (Morris et et al. al. 1996). 1996). ment (Morris A model for for assessment assessment has has been been developed developed that that acknowledges acknowledges the the composite composite effects of of physical, physical, psychological psychologicaland and social social problems problems (Schenkman (Schenkman and and Butler effects Butler 1989). Standardized scales 1989). Standardized scales such as the Webster Webster scale, Parkinson's Disease Disease Society Scale Profile may also be utilized (Wade 1992). Scale and and the the Functional Limitations Profile Communication, which is necessary for the the patient's patient's understanding, understanding, education necessary for and compliance with the management programme, and compliance with programme, can can initially initially be be assessed assessed in a subjective way assessment in collabosubjective way followed, followed,when when indicated, indicated, by by more more detailed assessment ration with a speech speech and language language therapist. disorders, such therapist. Mood Mood disorders, such as as depression depression and anxiety, can also influence influence a patient's patient's performance. performance. Disease Disease specific specific health and anxiety, related quality quality of life life measures PDQ-39 (Jenkinson (Jenkinson et al. 1995) 1995) and related measures such such as as the the PDQ-39 PDQL (DeBoer (DeBoer et al. 1996) 1996) have also also recently PDQL recently been been developed. developed. A A further further area of therapeutic liaison liaison between between disciplines disciplines is in assessing cognitive function. function. therapeutic Assessment of cognitive cognitive function function is is essential essentialin intreatment treatment planning planning and in the interof outcome outcome measures measures of of treatment. treatment. The The Mini-Mental Mini-Mental State State Examination Examination pretation of (MMSE) is (MMSE) is an an easily easilyapplied appliedscreening screening test test for for cognitive cognitiveimpairment impairment (Folstein et al. 1975), though allowance needs for age age and prior education education achieveachieve1975), though allowance needs to to be be made made for score can be correctly interpreted. interpreted. ment, before the score A problem solving approach to treatment treatment isis preferred, preferred, which which should should facilitate facilitate the the clinical (Higgs 1992). Assessment only the the clinical reasoning reasoning process process (Higgs Assessment needs needs to to address not only experienced by the patients, but also their underlying causes. functional problems experienced For soft For example, example, difficulty difficulty experienced experienced in in getting getting up up from from sitting could be due to soft weakness in tissue shortening, weakness in the extensors extensors of of the the hip hip and and knee, knee, inability inability to to transtransfer weight weight over over the support, difficulty difficulty initiating the movement, movement, or or fer the new base of support, problems in timing and and sequencing sequencing the movement. movement. Therapists Therapists must must be able able to problems differentiate differentiate between between possible possible causes causessosothat that treatment treatment can can be be directed directed appropri-

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Mental planning & preparation

Fig. Fig. 12.1 12.1

The eclectic approach approach to physiotherapy treatment treatment in in PD. PD.

ately to resolve underlying problems, problems, prior prior to to patient patient re-education within aa funcresolve underlying func-

tional movement context. context. Assessment should lead to joint goal setting and an individualized treatment plan. plan. vidualized Treatment approaches

treatment of There are a variety of approaches to the physical treatment of patients patients with with PD PD(see (see Fig. 12.1). 12.1). The The development development of of the the physiotherapy physiotherapy management management of of PD PD lends lends itself itself Fig. which is is mirrored mirrored in the evolution to a problem solving solving approach, which evolution of of approaches approaches to the physical physical management management of of other other neurological neurological conditions, conditions, e.g. stroke. to the Approaches identified as as being being in in current use, singly Approaches identified singly or in combination, combination, include include Bobath, Conductive Education Education and andFlewitt—Handford Flewitt-Handford (Lovgreen (Lövgreen et et al. al. 1996). 1996). The The traditional approach approach in inthe theUK UKisisthe theFlewitt—Handford. Flewitt-Handford. This is an exercise exercise based based approach aiming aiming to correct correct gait. gait. It places places a particular emphasis on rotatory approach particular emphasis on the rotatory movement (Handford 1986). 1986). Patients Patients are series of components of movement are instructed instructed in a series of exercises, which These exercises exercises appear to exercises, which they they are are expected expected to to continue continue at home. These standard to to all all patients. patients. The The literature literature suggests suggests that treatment aims aims of of be standard that the treatment approaches are are similar similar to to those thoselisted listed in inTable Table 12.1 12.1 (Schenkman (Schenkman et et al. al. 1989, 1989, various approaches Cutson et al. al. 1995, 1995, Brown Brown and and Read Read 1996). 1996). There There are are also also other other recognized recognized Cutson approaches, such as the the Motor Relearning Relearning Programme, which which could could be be used used in in the treatment of of PD PD (Carr (Carr and and Shepherd Shepherd 1987), 1987).

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Early referral referral

Assessment

1

Home

Problem Home

Outpatient or inpatient do m ici Ii ary domiciliary

treatment Fig. 12.2 12.2

The management cycle for physiotherapy interventions interventions in in PD. PD.

Ideally regular regular contact contactwith with the the patient patient should should be be maintained maintained and and once initial Ideally referral has has been been received received the the patient patient should should have have open open access access to to the the physiotheraphysiotherareferral an urgent urgent review review can can take take place. pist. This will will ensure ensure that if the the patient deteriorates an The physiotherapist should should also also initiate initiate regular regular reviews reviews with the patient so so that the the patient's condition condition can can be be monitored monitored and and management management programmes programmes adapted adapted patient's accordingly. accordingly.This Thismay maytake takethe theform formof of alterations alterationstoto the the patient's patient's home home maintenance programme and/or aa course of physiotherapy on either an inpatient programme and/or inpatient or domiciliary ciliary basis, basis, as asappropriate appropriate (see (see Fig. Fig. 12.2). 12.2).ItItisisthe theauthors' authors'experience experiencethat that patients patients patients with with degenerative degenerative neurological neurological conditions such as as with PD, unlike other patients multiple sclerosis, are the are poor poor at at initiating initiating contact with the therapist, and therefore the contact with the patient. patient. responsibility should be on the therapist to maintain contact in early PD Physiotherapy in

In the early stage stage of of PD PD key key goals goalsare: are:coping copingwith withthe the impact impact of of diagnosis, patient education, the promotion promotion of of aa healthy healthy lifestyle, lifestyle, and and good good habits habits relating relating to to posture and regular activity. activity. The The main main emphasis at this this stage stage isisattempting attempting to to slow slowdown down the the rate and maintain maintain normal normalfunction. function.Early Earlyreferral referral isis necessary necessary so rate of deterioration and significant that these aspects of of management management can can be be established establishedprior prior to tothe theonset onsetof ofsignificant movement deficits. deficits. Patients Patients may maybe be reassured reassured that thatthey theymay mayexercise exercisesafely, safely, as it has movement

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Fig. 12.3 12.3

The relationship between between social, social,physical physical and and psychological psychological factors factors and mobility mobilityininPD. PD.

been established that cardiovascular and metabolic responses do not established that cardiovascular and responses to exercise exercise do not

differ between early early stage stage PD PD and and age age matched matched subjects subjects without without PD (Protas et differ between et al. al. 1996). Where Where available, available,patients patientsand and carers carers can can contact contact societies specifictoto the the 1996). societies specific disease and disease and gain gain access accesstotothe theregional regionaland andlocal localsupport supportgroups. groups.Information Information in in the the form of advice, literature literature and videos relating to many aspects of the disease disease are are curcurrently available available from from specific specificsocieties. societies.Physiotherapy Physiotherapydepartments departments often often produce produce their own information and and advice advice sheets. Physiotherapy and disease disease progression progression With disease progression motor impairments become more pronounced. At disease progression motor At this this stage itit is necessary necessary to to begin begin correcting correcting the the movement stage movement disorders. The secondary secondary problems that arise loss of of problems that arise from from these these disorders disorders (e.g. (e.g. soft soft tissue tissue shortening, shortening, loss confidence, weakness also be addressed. Functional rereconfidence, weakness through through disuse, etc.) etc.) must also movement should should include include all all aspects aspects of of the the patient's patient'slifestyle. lifestyle. Social Social education of movement isolation being socially socially accepted isolation leading leading to to fears fears of of not being accepted has has aa large large impact impact on on patients with movement movement disorders disorders (see (seeFig.12.3). Fig. 12.3).As As the the disease disease progresses there is reduction in the the proportion proportion of ofpatients patients participating participating in in activities activities outside outside the the a reduction in comparison comparison to to activities activities that that are are home homebased based (Oxtoby (Oxtoby1982). 1982).This This was was home in most apparent where where group group activities activities were concerned. Ongoing education of patient and carers carers is important to deal deal with the impact of

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this gradual decliue. uecessary to to maintain maiutain their involvement decline. It is necessary involvement with a manage-

disease process, process, whilst whilst not not ment programme programme to to minimize minimize or ordelay delay the the effects effects of the disease raising unrealistic unrealistic expectations expectations of of treatment treatment outcomes outcomes (Nolan and Grant 1989). raising 1989). in late stage PD Physiotherapy in

With further disease towards a corrective corrective and and compendisease progression progression the emphasis emphasis towards satory approach approach increases. increases. The more assistance assistance to perform perform satory The patient will require more exercise exerciseprogrammes programmesatathome homeor or in inthe thephysiotherapy physiotherapydepartment. department. Compensatory strategies may be taught taught to toovercome overcome irreversible irreversible or or severe severe movement movement strategies may need need to be deficits. deficits. Close Closeliaison liaisonmust must exist existwith withother other members members of of the the multidisciplinary multidisciplinary team of aids and adaptations to promote independent independent to ensure appropriate prescription of function. This may involve involvehome homevisits visitsby bythe thecommunity communitytherapists. therapists. Inappropriate Inappropriate introduction of ofaids aidsmay mayincrease increase the the rate rate of of decline decline as as patients patients rely rely timing of the introduction on the aid, rather than using using the movement movement still available available to tothem. them. The The introducintroducof walking aids is a particularly difficult area to deal with as common tion of particularly difficult common aids, such as lateral as sticks sticks and and frames, frames, are are designed designed to to cope cope with with instability instability in in anterior anterior and lateral directions, not posteriorly. Assessment for walking walking aids aids must must include the reason for such as as aa balance balance tool. As the patient moves moves into the the palliative palliative their prescription, such disease aa more is required. Treatment Treatment moves moves stage of the disease more compensatory approach is from active assisted patient from active assisted activities activitiestowards towardsmore more passive passivetreatment treatmentasas the the patient becomes less less able able to to participate participate and and goals goals become become more more limited. limited. The The management of the and psychological psychological effects effects of severe the impact of the emotional and severe disability disability requires requires careful careful handling handling by by the the therapist. therapist. Over Over time time an an extension extension from from the the treatment treatment of aa movement disorder disorder to aa more more holistic holistic approach approach is is required required (Kauser (Kauser and and pure movement Powell Powell 1996). 1996).The Thecarer's carer'ssubjective subjectiveburden burdenalso alsohas hasimportant important implications implications for for the the therapist (Zarit (Zarit and and Zarit Zarit 1982). 1982). Hospital, community and domiciliary physiotherapy physiotherapy The environment environment for for treatment treatment is is important. important. If If the the main main aim aim of of treatment treatment is is for for

to function function effectively effectively in the patient to in their their own own home, then it may be most approthere. Treatment Treatment at at home home can can be adapted to specific specific conditions, priate to treat them there. such as selection selection of of aa chair chair from from which which to to practice practice sit sit to to stand stand movement movement in order quadriceps. Domestic stairs to strengthen the quadriceps. stairs are are often often narrower narrower and and steeper steeper than than hospital settings settings and therefore therefore practice practice at home is is likely likely to more those in hospital to be be of more benefit. However, However, space insufficient for activities and specialized specialized benefit. space may may be be insufficient for certain activities equipment lacking, lacking, in which which case case hospital-based hospital-based therapy therapy may may be be more more appropriate. The The difference difference in in effectiveness effectivenessbetween betweenhospital-based hospital-based and and domiciliary domiciliary physiostroke (Gladman (Gladman and and therapy has been the subject subject of of attention in the literature on stroke 1994, Gladman et al, al. 1994). 1994). Similar Similar research available to Lincoln 1994, research data needs to be available

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inform purchasers pnrchasersofofcare care Banks (1989) in a small inform forfor PD.PD. Banks and and CairdCaird (1989) in a small study, stndy, found fonnd domiciliary physiotherapytoto have more effect hospital-based therapy. domiciliary physiotherapy have more effect thanthan hospital-based therapy. Hospital physiotherapymay may occur an individual or group as a comHospital physiotherapy occur on on an individual or group basis,basis, or as aorcomof both. both. To To some extent the selection selection of bination of of individual individual or or group group treatment is reliant the philosophy philosophy underlying underlying the treatment treatment approach. approach. A A group group allows allows reliant on the patients recognize common common problems problems and and share share solutions, solutions, giving giving mutual mutual patients to to recognize support and and encouragement. encouragement.An Anexample example of ofthis thisformat formatwas wasaasix six week week interdisciinterdisciplinary group group intervention interventionthat thatone oneofofthe theauthors authorshas hasbeen beeninvolved involvedwith. with.This Thisfeafeaplinary tured therapy, occupational occupational therapy and physiotherapy physiotherapy sessions. sessions. During tured speech therapy, each of the half half day day sessions sessions aavariety varietyof oftherapeutic therapeutic assessment assessment and and treatment treatment was offered. Educational from aa pharmacist, pharmacist, social social worker, worker, PD Society Society local local offered. Educational input input from branch officer, officer, dietician and and medical medicalstaff staff was was also included. A significant improveimprovebranch ment in patients' patients' psychological well-being well-being has has been been suggested suggestedafter afterparticipation participation in in (Gauthier et et al. al. 1987). 1987). a group programme (Gauthier

Hydrotherapy Hydrotherapy have aa role role in in the the management management of PD. The The warmth warmth of the water Hydro therapy may have can assist assist in inthe thereduction reduction of of rigidity rigidityprior prior to to undertaking undertaking aa stretching stretching programme programme in the water. Buoyancy Buoyancy can can be be used used to to assist assist movement movement to increase increase range and may with also be be used used as as aaresistance resistancetotomovement movement when when strengthening strengthening muscles. Patients with poor balance on land land often often find find walking walking in in the the water water easier, easier, as the hydrostatic prespressure of the water increases increases stability. stability. The Thewarmth warmth of of the the water water also also promotes promotes general relaxation eases the pain associated associated with stiff stiff muscles. muscles. Hydrotherapy Hydrotherapy has has relaxation and and eases the pain known psychological psychological benefits benefits and and isis often often aatreatment treatment that that patients enjoy and may increase patients' confidence. Hydrotherapy Hydrotherapy can can also also be used as a recreational recreational activactivity increasing social contacts. Patients fearful of the the water, water,with with unstable unstable cardiac cardiac or or pulmonary pulmonary conditions, or fearful of with continence problems, are unsuitable for this therapy. therapy. Patients Patients with with autonomic dysfunction be at risk from dysfunction may be from syncope syncope due to vasodilatation induced by warm warm water.

movement components/deficits Treating specific movement Rotational components of movement movement All normal rotatory components. normal movements movements possess possess rotatory components. These These are are essential essential for for normal function. function. Axial Axial mobility in particular particular has has been shown to correlate positively overall physical 1996). In with overall physical performance performance (Schenkman (Schenkman et al. 1996). In PD PD rotation is one is reflected reflected in the difficulties difficulties patients of the first movements to be lost and is patients experience in in an an enclosed enclosed space and during gait. gait. Loss Loss of ence in turning over in bed, turning in of

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trunk and pelvic rotation results results in in aa 'legs 'legs only gait' with inability to turn turn (Yekutiel (Yekutiel al. 1991). 1991).Whole Whole body body rotation rotation using a swivel swivel chair chair in in which which the the patient is rotated et al. to stimulate the as a way way of of improving improving the vestibular vestibular system system has also been suggested suggested as increasing stride length, and facilitating facilitating movement movement initiation (McNiven (McNiven posture, increasing 1986). 1986). Range of movement movement

to maintain maintain soft soft tissue tissue length length and range Stretching exercises exercises to range of of movement movement frefreform part of the physiotherapy physiotherapy session session and and the the patient's patient's home home maintenance quently form Muscle that is immobilized demonprogramme. Muscle immobilized in a shortened position has been demonchanges. These changes strated to undergo structural changes. changes include: include:reduction reduction in in number number of sarcomeres, an increase in proportion of ofconnective connective tissue, tissue, muscle muscle fibre fibre of sarcomeres, an increase in the proportion atrophy, abnormal cross-bridge cross-bridge formation formation (Williams (Williams atrophy, changes changes in in fibre fibre type, type, and and abnormal 1990, Carey 1993). Working 1990, Carey and and Burghardt Burghardt 1993). Working in in aa shortened shortened range has been shown increase the speed speed at which which these these changes changes occur occur (Williams (Williams 1990). to increase 1990). In In PD PD an increase percentage of Type I muscle muscle fibres fibres with decreased decreased percentage percentage and increase in in percentage of Type atrophy of Type Type IIII fibres fibreshas hasbeen beenreported reported (Edstrom 1970). Placing muscle muscle on on a stretch, stretch, and and advice regarding posture posture at at rest and during Placing advice regarding during specific daily assospecific daily activities, activities, has has been been shown shown to be beneficial beneficial in preventing length associated ciated changes. changes. A A stretch stretch of of 30 30 minutes minutes every every two two days daysisisrecommended recommended to to prevent prevent changes immobilized muscle muscle (Williams (Williams 1990). 1990). Exercises Exercises should encourage changes in in immobilized should encourage movement through full full range. range. The The use use of of sensory sensory targets targets to to give give feedback feedback as to movement full range has been achieved achieved may be beneficial, whether full beneficial, as as there there is is aa tendency tendency for for as well well as repeated movepatients to lose amplitude, as as speed speed of of movement, movement, during repeated ments. For For example, example, knee knee rolling rolling from from side side to to side side in incrook crooklying lyingisisaa suitable suitable exerexerments. cise, surface of cise,but but the the patient patient should should be aware aware of of touching touching the the bed bed with with the outer surface as the lower lower limbs are are lowered. lowered. the knees as Flexor musculature musculature is is at at particular particular risk of shortening due to the Flexor the flexed flexed posture adopted by the patient, particularly when more time is is spent in the sitting position. Therefore these muscles need to be the focus of treatment, treatment, even even in in the the early early stages, stages, order to to maintain maintainlength. length.Flexor Flexormuscle muscle shortening shorteningcauses causes extensor extensor muscle muscle in order lengthening with resulting muscle muscle imbalance. imbalance. Lengthening lengthening Lengthening also also puts puts muscle muscle at at aa mechanical disadvantage. Correcting the flexed flexed posture posture of of the the trunk and limbs will have a beneficial beneficial effect re-education and strengthening strengthening of of the the extensors, extensors, have effect on on the the re-education allowing physiological range allowing them them to to work work in their physiological range of of movement. Gait disturbances

Physiotherapy may be targeted targeted towards towards one one or more aspects may be aspects of gait gait re-education

Achillestendon tendon stretches, stretches, re-education re-education of and to improving confidence in walking. Achilles

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Physiotherapy active dorsiflexion, and of of dorsiflexion in the ankle strategy with its dorsiflexion, and dorsiflexion in strategy of balance, balance, with

strike and and retropulsion. incorporation into into gait gait pattern, pattern, are are used used for for decreased heel strike Muscle maybe Muscle strengthening strengthening may be required. Visual Visual and and auditory cues have have been been used used to to aid initiation initiation of movement movement and and to to increase increase step step size size (McIntosh (McIntosh et al. al. 1997). aid Counting and singing singing may may aid aid dysrhythmia. dysrhythmia. Distraction Distraction techniques, techniques, rocking, rocking, Counting use of of external external cues cues may maybe freezgetting heels to floor, and the use be ways of coping with freezing episodes. episodes. Trunk Trunk mobilization mobilization techniques techniques may may improve improve the the rotational rotational component of of gait gait and and therefore therefore arm arm swing. swing. Objective Objective evaluation evaluation of gait may include include simple measures measures such such as as 3600 360°turn, turn,six sixminute minuteororten tenminute minutewalk walktests. tests.These Thesehave have reliable measures of gait in early early and PD (Schenkman (Schenkman been shown to be reliable and moderate PD etal. et al. 1997). The patient should practise practise manoeuvring manoeuvring in insmall small spaces, spaces, negotiating doorways doorways and obstacles. ItIt isisimportant important that re-education should should include include walking walking on different different surfaces, such such as aswooden wooden floors, floors, carpets, carpets, grass, grass,and andrough rough ground ground including slopes, surfaces, kerbs and steps and should include include functional functional activities activities as as well well as distance walking (Schenkman et al. 1989). Stairs rail, and and (Schenkman Stairs need need to to be be practised practised with with and without aa rail, differing size size of step rise. with differing Compensatory strategies A characteristic of PD is the the difficulty difficulty aapatient patient experiences experiences in ininitiating initiating movement of movements. movements. This This involves involves the and changing the direction of the complex complex integration functions (Schneider (Schneider et et al. al. 1987). 1987). The physiotherapist physiotherapist can can of sensory sensory and and motor functions to overcome overcome this this deficit deficit (Ype (Ype et et al. al. 1995). 1995). have a valuable role in teaching strategies to Simple strategies strategies such such as as visual visual cues, cues, for for example example upturned upturned walking sticks, walking through over sticks, sticks, and comthrough ladder rungs or over and the use of 'flip flap' frames, have commonly used in gait gait re-education re-education (Dunne (Dunne etet al. al.1987). 1987). Significant Significant improvemonly been been used ments in some temporal aspects aspects of gait have have been cues been demonstrated using visual cues (Bagley et et al. al. 1991), 1991),though though there was no carry over when when cues were were removed. removed. There is a need investigate how need to to investigate how long long training training needs needs to to continue continue before before the the long-term effects suggested effects suggested by byWorm Worm (1988) (1988) may may occur. occur. Visual Visual cues, cues,in inthe the form form of of tapes tapes used on the the floor, floor, were were investigated by Martin (1967) (1967) in the as markers markers to form patterns on setting to facilitate facilitate ongoing movement for patients with freezing freezing problaboratory setting However, using lems. However, using floor floor or low level level visual visual cues cues may may increase increasethe the patient's patient's tendency towards flexed posture, further impairing impairing balance balance (Weissenborn (Weissenborn 1993). 1993). dency towards a flexed The chosen stationary objects objects used used by by Bagley Bagley et al. (1991) (1991) may may have implications implications for patient's environment, environment, such such as as carers. carers. Mobile Mobile cues cues such as those for others in the patient's used (1987) and Worm (1988) (1988) may may be more more acceptable. acceptable. The sucused by Dunne et al. (1987) cessful use cessful use of of visual visual targets targets above above eye eye level leveldemonstrates demonstrates how visual targets can be carried over environmental conditions, doors carried over into into normal normal environmental conditions,both bothinin and and out out of doors

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(Weissenborn 1993). 1993). In In this this single single case case report report it is is difficult difficult to to determine determine if the gait

visual targets, targets, or or to to postural postural improvement improvements noted were due to use of visual caused by use of targets targets above above eye eye level. level. Falls and balance problems Patients with PD PD frequently frequently fall, fall, particularly backwards, although falls falls are rarely

reported. One One study study suggests suggests that over over a third of of patients with PD PD give give a history of fallswith with around around a tenth of falls of patients patients falling falling at at least least once once aa week week (Koller (Koller et al. 1989). instability, bradykinebradykineFalling has has been been shown to correlate positively with postural instability, sia and rigidity rigidity (Koller (Koller et al. 1989). 1989). The cause of fall may be related related to the the sia The cause of the fall changes changes in in posture posture altering altering the the centre centre of of gravity gravity in in relation relation to to the the base base of of support. support. flexion and trunk trunk flexion flexion move move the the centre centre of of gravity gravity backwards so that the the Plantar flexion Decreased hip line of gravity is displaced displaced towards towards the the back back of of the base of support. Decreased and ankle movements trunk have have been been shown shown to to be be movements and an inability to extend the trunk associated 1990). The associated with with falls falls (Gehlsen (Gehlsen and and Whaley Whaley 1990). The increase increase in in onset latency of dorsiflexor stretch reflex reflex contributes difficulties in the ankle dorsiflexor contributes to the difficulties in coping coping with with posposperturbation(Woolacott (Woolacottand andShumway-Cook Shumway-Cook 1990). 1990). Primary Primary and and for /or seconseconterior perturbation dary muscle weakness, ankle dorsiflexors, dorsiflexors, may also be associated associated weakness, in in particular the ankle with falls falls (Gehlsen (Gehlsen and Whaley Whaley 1990). 1990). Foot Foot and and nail naildeformities, deformities, increasingly increasingly with common in older subjects, have also also been been identified identified as as independent independent risk factors factors for falls (Speechley falls (Speechleyand andTinetti Tinetti 1990). 1990).ItItisisimportant important that that foot foot problems are identified and early referral (chiropodist).Falls Falls lead lead to to loss loss of confidence confidence referral made to a podiatrist podiatrist (chiropodist). and fear of further further falling. falling. Patients Patients tend reduce activity activity to lessen the and fear tend to reduce to lessen the risk of falling, though leads to secondary secondary muscle falling, though this leads muscle weakness, weakness,further further deterioration deterioration of of increased risk risk of further falls. falls. balance mechanisms, and increased The therapeutic of the the fall The therapeutic management management of fall should should address address all components components of normal movement and should include normal movement and include soft soft tissue tissue length, length, range range of of movement, movement, muscle tone and strength, strength, and and the theinitiation initiationofofappropriate appropriatelevels levelsofofactivity. activity. muscle tone and Environmental fall should be identified identified and and Environmental factors factorsthat that may may contribute contribute to to the fall adaptations made as required. Balance Balance must must be be re-educated re-educated in static and dynamic activities in aa variety of settings. Patient confidence will improve as the functional activities safely again. also be patient learns to function safely again. The The patient patient and and carer carer must must also be taught floor in in the the event event of of aa fall fall occurring. The provision of of perperhow to get up from the floor systems that can be activated activated by the event event of of aa fall fall and and sonal alarm systems by the the patient in the help will will also also increase the confidence confidence of both patient patient and and carer. carer. rapidly summon help Evaluation of balance

A number of of methods methods of of clinical clinical evaluation evaluation of of balance balance in in the the elderly elderly exist, exist, such as the Berg Scale Scale (Berg (Bergetetal. al,1989) 1989)and andthe the 'Get 'Get up up and and Go' test (Mathias et al. 1986). Although these Although these may may test test balance balance in in relation relation to to function, function, they they do do not not predict predict those

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Physiotherapy at risk risk of falling. Tinetti et et al. (1986) describe a balance balance and and gait assessment falling. Tinetti (1986) describe assessment that

fulfils both both of these roles fulfils roles or, or, alternatively, alternatively, the 'Falls' 'Falls' questionnaire questionnaire (Isaacs (Isaacs 1986) 1986) could measure of of dynamic dynamic balance, balance, the the functional functional reach could be used. A new clinical clinical measure measure, has been found in in elderly elderly subjects subjects to to correlate correlate significantly significantly with physical physical frailty et al. al. 1990). 1990). The Balance Balance Performance frailty (Duncan et Performance Monitor Monitor is of frequent frequent use in the physiotherapy physiotherapy departments as as aa means means of of objectively objectively measuring postural postural sway (Sackley et al. 1992). Walking aids A walking aid is is rarely rarely aa solution solution to to falls, falls, and and the the patient, having been been told an aid walking aid aid will be supplied, arrives in the Physiotherapy Department Department with with false false expectations. expectations. does not take take aa great great deal deal of ofimagination imaginationto towork workout outthat, that, in in aa patient patient at at risk risk of of It does falling frames serve serve no purpose purpose falling backwards, backwards, conventional conventional aids aids such such as as sticks sticks and and frames land on on top top of of the the patient patient when when they they fall. fall. A more appropriate referreferother than to land ral would be for the management management of falls. falls. In PD walking frames serve to to increase increase trunk flexion frames may only serve flexion by further comcompensating for the inability the ankle, pensating for inability to dorsiflex dorsiflex the ankle, and inhibit inhibit aa reciprocal reciprocal gait gait pattern. pattern. ItIt isis often useful to supply an aid that is higher than would normally be the case. Most 'correctly' sized sized and to case. Most frames frames supplied supplied to to patients patients with with PD are 'correctly' and tend to trunk flexion. flexion. A A reciprocal gait pattern is is easier easier to achieve using a rollator, worsen trunk though these can 'run away' away' with patients who have a festinant festinant gait, and braking systems easy to operate. operate. The The 'Arrow 'Arrow Walker' Walker' overcomes overcomes the systems are are not not easy the problem problem of due to to size, size, is only practical for use in rehabilitation and and backwards instability but, due institutional settings (Farley (Farley et et al. al. 1996). 1996). Sticks may institutional settings may serve serve more more as as aa useful useful warning warning to others that aa person person isis unsteady unsteady on on their their feet feet than thanas asaa way way of of improving improving balance. balance. give an aid is a complicated issue. The unnecessary provision of aids may When to give render the patient unnecessarily dependent, with secondary secondary weakness and increasingly ingly impaired impaired balance. balance. If If an an aid aid is given given and and secondary secondary complications complications are are anticipated, these may be exercise be minimized minimized by by including including in in the rehabilitation or home exercise activities to counteract counteract this this effect, effect. For For example, example, if if aa frame frame increases increases programme activities trunk flexion flexion additional additional exercises exercises to extension and strengthen to encourage encourage trunk trunk extension and strengthen neck extensors extensors would be prescribed. prescribed. back and neck

Dystonia

Dystonia in PD can can significantly significantly impair functional functional ability. ability. Several Several types of dystonia occur in PD (Kidron and Melamed 1987). 1987). Therapists need to be aware that even though the patient does not present in the physiotherapy physiotherapy department with dystonia, it may be present at other times. times. Physiotherapists Physiotherapists can assist assist the the patient with dystonia to develop develop strategies strategies to enhance function. function. For For example, example, compensatory compensatory dystonia to enhance fixing and lower lower limbs limbs can can allow allow upper limb function function and and weight weight fixing of of the the trunk and

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bearing to reduce dystouia dystonia prior prior to to fuuctioual functional activities. activities. In In all all aspects aspects of of the the treattreat-

ment programme therapists can can learn learn from from listening listening to how how patients already already deal with their disabilities. Dystonia may may be be painful painful (Kidron disabilities. Dystonia (Kidron and Melamed 1987) 1987) and physiotherapeutic modalities helpful in this this situation. situation. physiotherapeutic modalitiesfor forpain pain relief relief can can be be helpful of muscle, muscle, which in Dystonia may also cause cause changes changes in in the the mechanical mechanical properties of may lead lead to to contracture. contracture. Clinically Clinically decreased decreased range movement has been turn may range of of movement observed affected muscle groups (Edwards (Edwards 1996). 1996). Massage Massage and specific specific soft observed in the affected techniques may maybe beneficial in in preventing preventing these these changes. changes. tissue mobilization techniques be beneficial Exercise tolerance As progresses some some patients patients report As PD progresses

an increasing sense of of fatigue fatigue and and reduction tolerance that that can can undermine home based programmes. This in exercise exercise tolerance based exercise exercise programmes. can result from from many causes causes including including an increase increase in in the the perception of the sense of effort control. A further cause cause may be effort of of moving moving due due to to abnormal abnormal central motor control. increased force increased force generation generation during during the the production production of a movement. This This phenomenon has been studied in newly diagnosed PD. The affected affected diagnosed patients with unilateral PD. was found force during weight weight matching limb was found to produce more force matching experiments experiments than unaffected limb, limb, which which could could lead lead to to earlier earlier fatiguability fatiguability (LOvgreen (Lövgreen and the unaffected and Cody Cody 1997). 1997). Respiratory function

have been been shown shown to to have have an an impact impact on activities of Restrictive respiratory deficits deficits have activities of daily living living in in PD (Sabate et al. 1996). Restrictive deficits may may be be caused caused by rigiddaily Restrictive deficits ity, contracture contracture or or tremor ity, tremor in in trunk trunk muscles muscles (Estenne (Estenne et et al. al. 1984). 1984). Alternating Alternating abduction and adduction of of the vocal chords chords and and tremor of the glottal and and supraglottal areas laryngoscopy in in patients patients with with PD. PD.Flow Flow volume volume areas has has been been noted noted during laryngoscopy abnormalities show show regular regular acceleration acceleration and deceleration deceleration with with aafrequency frequency loop abnormalities similar to that of of tremor tremor recorded recorded in in the theextremities extremities (Vincken (Vincken et et al. al. 1984). 1984). The The similar above on aa pre-existing obstructive obstructive deficit deficit in elderly elderly above changes changes maybe maybe superimposed on patients, coupled coupled with with age age related changes in lung function (Renwick (Renwick and Connolly Connolly patients, 1996). This 1996). This may may explain explain the the obstructive obstructive deficits deficits noted noted in some patients by Sabate etal. et al. (1996). early stages stagesof ofthe thedisease diseaseititisisimportant importanttotoencourage encouragethe thepatient patienttotounderunderIn the early activity, which respiratory muscle muscle take regular aerobic activity, which will will assist assist in in maintaining respiratory thoracic mobility. mobility. Raising postural strength and thoracic postural awareness awareness and and postural postural correction are essential part emphasizing strengthening strengthening of the extensor extensor are an essential part of management, emphasizing musculature. Patients Patients may may also also receive receive instructions in in specific specific exercises musculature. exercises to to maintain maintain mobility. In the middle middle stages stages of disease the patient may may need need to be be trunk mobility. of the disease the patient in breathing breathingexercises. exercises. Respiratory Respiratory muscle muscle training training devices devices to to increase increase instructed in respiratory strength and endurance endurance may may be useful, but have not been evaluated evaluated in

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stages, attention to postnre, PD (Chatham et al. 1996). In the later stages, posture, seating seating advice, and breathing exercises to maintain a clear airway airway are are the the aspects aspects of of treatment treatment on exercises to which for the which to focus. focus. In the middle middle and and late late stages stages of PD PD itit may may be be necessary necessary for physiotherapist speech and language language therapist physiotherapist to to work work in conjunction conjunction with the speech therapist to enable the achieve adequate for speech. speech. This may enable the patient to achieve adequate respiratory respiratory support for involve involve assistance assistancewith withpositioning positioning and and improvement improvement of of thoracic mobility prior to speech therapy. Other considerations considerations all aspects aspects of ofthe thepatient's patient's problems. problems. Orthostatic Orthostatic hypoTherapists need to consider all tension has been associated due to tension associated with subjective subjective reports of dizziness, dizziness, possibly possibly due increased blood supply to muscles increased muscles on exercise exercise (Hillen et al. 1996). Pain and limb limb in PD PD (Hillen (Hillen and andSage Sage 1996). 1996). These These are are areas areas to to oedema have also also been been reported in which physiotherapy physiotherapy can Transcutaneous nerve nerve stimulation (TENS) (TENS) which can contribute. Transcutaneous may be used in the management management of of pain in in neurological neurological condicondiand acupuncture may tions. improved by by advice and exercises, exercises, prescription tions, Limb oedema maybe improved prescription of of antiembolic stockings, stockings, and and the use of massage massage or or the the Flowtron. Sensory dyspnoea, anxiety and panic attacks are frequent frequent in PD and require sensitive (Hillen and and Sage Sage 1996). 1996). Relaxation techniques may may be be sitive and and careful careful handling (Hillen appropriate for some of these patients.

Evidence base base for for physiotherapy physiotherapy treatment has often been criticized for the lack of formal justification of of its its Physiotherapy has often been criticized for lack of formal justification efficacy. Evaluation efficacy. Evaluationhas hasbeen beenhampered hampered by a lack lack of of sensitive sensitiveand and reliable reliable measures measures that reflect reflect treatment aims aims in conditions conditions where where the the signal signal may be small small compared to the large relalarge volume volume of of noise. noise. Therapy Therapy interventions interventions rely on on the the interaction and relationship between patient as well well as administered. In In tionship between patient and therapist, as as the the treatment administered. this context context the medical medical model controlled trial is is rarely a suitmodel of the randomized controlled able methodology. methodology. Medication effects effects can also confound assessments assessments of of physical physical able therapy interventions around the time time of of acute acute admissions admissions when drug drug therapy therapy is is therapy interventions altered. commonly altered. There is little published evidence concerning the effectiveness of physiotherapy effectiveness of in PD. Most Most published published studies studies display display the the problems problems that that are are frequent frequent in rehabilitation research: poor descriptions of of treatment treatment schedules, schedules, small small numbers numbers of of subjects, subjects, poor classification classification of of subjects, subjects, flawed flawed or or incomparable incomparable methodologies, bias, and insufficient clinically meaningful present. insufficient power power to detect clinically meaningful improvements even if present. Comella et al. (1994) crossover trial of modComella (1994) in a small, small, single single blind, blind, randomized, crossover erately disabled significant improvement United erately disabled patients patients found found aa significant improvement inin the the total United Parkinson's motor and and ADL ADL Parkinson's Disease DiseaseRating RatingScale Scale(UPDRS) (UPDRS)score scoreand and inin the the motor

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H. Chatterton and and B. B. Lovgreen Lövgreen subsections. subsections. The trial consisted consisted of of aa four four week week intensive intensive programme programme of ofexercise exercise

therapy with timed motor motor tasks tasks measured measured before before and and after after intervention. intervention.Chan Chanetetal. al. (1993) in aa multiphase multiphase single singlecase casestudy studydemonstrated demonstrated improvement improvement in in gait gait paramparam(1993) in eters treatment phase. phase. A A significant significant eters (step (step and and stride length, cadence), during the treatment improvement in a 10 10 metre walking test was was found group receiving receiving a combifound in a group nation of physiotherapy and drug therapy as as opposed opposed to to the the group group receiving receiving drug drug alone (Formisano (Formisanoetetal. al.1992). 1992).Yekutiel Yekutiel et al. (1991) identified after after a treattreattherapy alone programme improvements improvements in in posture posture and and gait gait and a reduction in the number ment programme of falls. Palmer (1986) reported improvements in grip grip strength, strength, gait, gait, fine fine of falls. Palmer et et al. al. (1986) reported improvements motor reduction in in tremor tremor after after aa12 12 week week treatment period. period. motor co-ordination, and reduction (1989) found found beneficial beneficial effects effects after aa domiciliary domiciliary exercise exercise programme. Hurwitz (1989) and Stelmach Stelmach (1990) (1990) studied the the effects effects of practice Worringham and practice on reaction time and suggested suggested that that patients patients with with PD PD can can use use advanced advanced information information to to programme activities However, in Pedersen activities reducing reducing reaction time. However, in a small small uncontrolled uncontrolled study Pedersen effect from et al. al. (1990) (1990) found found no long-term effect from a physical physicaltherapy therapy programme. programme. Weiner and Singer Singer (1989), while while reviewing reviewing evaluative evaluative studies studies of of physiotherapy physiotherapy intervenfor Parkinson's Parkinson's disease, disease, suggest suggest that that the the psychological psychological effects effects of exercise exercise may tion for They suggest suggest that physiotherapy physiotherapy is is the the primary primary motivating motivating factor. factor. be important. They Kuroda al. (1992) (1992) found that physical physical exercise exercise reduced reduced mortality mortality in in PD. PD. Kuroda et al. found that Participation in group occupational occupational therapy therapy programme found to Participation in a group programme has has been been found significantly improve Barthel Index scores (Gauthier et et al. al. 1987). 1987). significantly

The way forward

A conference by the conference organized organized by the European European Parkinson's Parkinson's Disease Disease Society Society in 1996 1996 confirmed the the poor co-ordination, confirmed co-ordination, funding funding and and prescription prescription of ofphysiotherapy physiotherapy across Europe. Europe. Arising Arising from from this this a research research group group of physiotherapists from the across the UK, UK, close contact with the the UK UK Parkinson's Parkinson's Disease Disease Society, Society, have working in close have formulated for seeking European agreement on on the the most most effective effective methods methods of of physiophysioproposals for (service models modalities) to to facilitate facilitate evaluation evaluation of of best best therapy (service models and treatment modalities) practice. The phase of this project project is is now now underway. underway. Additionally, Additionally, the practice. The last phase the Open Learning Learning Project Project (Baker (Baker etet al. al. 1997) 1997)has hasidentified identifiedan an educational educational need need to to improve improve communication skills skills of of health health and and social social care care professionals professionals to better deal deal with with the communication situations faced faced by severely severely handicapped and chronically chronically ill ill patients. patients. the complex situations handicapped and

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Schenkman Schenkman M, Shipp Shipp KM, KM, Chandler Chandler J,J, Studenski Studenski SA, SA, Kuchibhatla Kuchibhatla M (1996) (1996) Relationships Relationships between mobility between mobility of of axial axial structures structures and andphysical physicalperformance. performance. Physical PhysicalTherapy, Therapy, 76, 76, 3, 3, 276—85. 276-85. Schneider JS, JS, Diamond SG, Markham CH (1987) Parkinson's disease; sensory and motor probprob37,37, 951—6. lems in in arms armsand andhands. hands.Neurology, Neurology, 951-6. Speechley M, TinettiM Tinetti M (1990) Assessment of risk and prevention of of falls falls among among elderly elderly persons: persons: role of the Canada, 42, 42, 4, 75—9. the physiotherapist. physiotherapist.Physiotherapy Physiotherapy Canada, 4, 75-9. Tinetti MayewskiRR (1986) (1986) Fall Fallrisk riskindex indexfor forelderly elderlypatients patientsbased basedon on number number Tinetti ME, ME, Williams TF, Mayewski of chronic of of Medicine, 80,80, 429—34. chronicdisabilities. disabilities.American AmericanJournal Journal Medicine, 429-34. Vincken WG, Gauthier Gauthier SG, Vincken WG, SG, Doilfuss Dollfuss RE, RE, Hanson Hanson RE, RE, Darauay Darauay CM, CM, Cosio Cosio MG MG (1984) (1984) Involvement EnglandJournal Journalofof Involvement of upper airway airway muscles muscles in in extrapyramidal extrapyramidal disorders. disorders.New New England Medicine, Medicine,311, 311,438—42. 438-42. Wade DT (1992) Measurement University Press. Measurementin in Neurological NeurologicalRehabilitation. Rehabilitation.Oxford: Oxford:Oxford Oxford University Press. Weiner S, 5, Singer C C (1989) (1989) Parkinson's Parkinson's disease diseaseand andnon nonpharmacological pharmacologicaltreatment treatment programmes. Journal oftheAmerican Geriatrics Society, 37, 359—63. Journal of the American Geriatrics Society, 37, 359-63. Weissenborn 5S (1993) (1993) The The effect effect of using using aa two two step step verbal verbal cue cue to to aavisual visual target targetabove aboveeye eyelevel level on 79,79, 1, 26—31. Physiotherapy, 1, 26-31. on the the Parkinsonian Parkinsoniangait: gait:AAcase casestudy. study.Physiotherapy, Williams PE (1990) (1990) Use Use of of intermittent intermittent stretch stretch in in the prevention loss in in Williams PE prevention of of serial serial sarcomere sarcomere loss immobilized immobilized muscle. muscle.Annals Annalsofofthe theRheumatic RheumaticDiseases, Diseases,49, 49,316. 316. Woolacott MH, Shumway-Cook Shumway-Cook A A (1990) (1990) Changes Changes in in postural posturalcontrol controlacross acrossthe thelife lifespan span—- A systems approach. Therapy, 70,70, 12, 12, 799—807. approach.Physical Physical Therapy, 799-807. Worm GM ArchivesofofPhysical PhysicalMedicine Medicineand and GM (1988) (1988) Recovery Recovery of of motion motionininParkinson's Parkinson'sdisease. disease.Archives Rehabilitation, 463—4. Rehabilitation,69,69, 463-4. Worringham (1990) Practice Practice effects effectson onthe theprogramming programming of discrete discrete movement movement Worringham CJ, CJ, Stelmach GE (1990) in Parkinson's of of Neurology, Neurosurgery, and Psychiatry, 53, 702—4. Parkinson'sdisease. disease.Journal Journal Neurology, Neurosurgery, and Psychiatry, 53, 702-4. Yekutiel MP,Pinasov PinasovA, Shahar G, G, Sroka Sroka H H (1991) A clinical clinical trial trial of of the the re-education of Yekutiel MP, A, Shahar of movemovement Rehabilitation, 5, 207—14. ment in in patients patientswith withParkinson's Parkinson'sdisease. disease.Clinical Clinical Rehabilitation, 5, 207-14. Ype PT, PT, Wiebo Wiebo H, H, Bruwoer Bruwoer H, Johannes Johannes PWFL PWFL (1995) (1995) Training Training of of compensational compensational strategies for impaired gross gross motor motor skills skills ininParkinson's Parkinson'sdisease. disease.Physiotherapy PhysiotherapyTheory Theoryand andPractice, Practice,11,11, 209—29. 209-29. Zarit SH, Zarit JM JM (1982) (1982) Families Families under stress: stress: Interventions Interventions for for caregivers caregivers of of senile senile dementia dementia patients. Psychological patients. Psychological Therapy Therapy and andResearch ResearchPractice, Practice,19, 19,461—71. 461-71.

Rehabilitation, occupational therapy and and elderly patients with with Parkinson's Parkinson's disease disease Jackie Hughes Jackie

Introduction Occupational therapy (OT) (OT) has been defined defined as the prescription prescription of occupations, occupations,

interactions and and environmental adaptations to to enable enable the the individual to regain, interactions environmental adaptations individual to regain, develop or retain occupational skills and roles roles required required to to promote personal welldevelop or skills and achieve meanbeing. OT should also be concerned with helping an individual to achieve ingful, relevant social social and ingful, purposeful purposeful goals goals and and relationships relationships appropriate appropriate to to the relevant cultural setting setting (Hagedorn 1992). 1992). This definition definition emphasizes emphasizes the uniqueness of cultural the uniqueness each individual of establishing establishing goals in rehabilitation that are are each individual and the importance of relevant to the patient. OT OT intervention interventionininPD PDisislikely likely to take take place place over the whole history of of the the disease, disease, which may last for for many years. of the natural history years. This This chapter chapter describes some describes some of of the the areas areasinin rehabilitation rehabilitation in in PD PD that that are are the the particular particular concern concern frame of of reference reference for OTis OT is suggested. suggested. of the occupational therapist. A model and frame The multidisciplinary team for The value value of of multidisciplinary team working working isis emphasized, emphasized, as as isis the the need need for assessment in OT and greater commitment to to evidence evidence based based practice. practice. assessment Aims of intervention intervention of of 01 OT in in PD PD For people with PD PD daily daily life can become consumed with coping coping with with social social isolaisolation, estranged relationships, relationships, loss loss of of roles, roles, changing changing roles roles and and changes changes in in physical physical appearance. Not surprisingly, PD is is associated associated with with significant significant levels levels of of stress, stress, appearance. Not surprisingly, and carer. carer. Older patients with PD PD will also anxiety and depression in both patients and also disease states be limited in daily roles by by other other concurrent disease states that that will will summate summate with the effects effects of of PD. PD.The Thetherapist therapist needs needs to to be be aware awareof ofthe thepotential potential for for problems problems in in elderly patients anticipate these these whenever whenever possible possible by by emphasizing emphasizing active active elderly patients and to anticipate prevention prevention of complications complications and and general general health health promotion. promotion. The therapist is is concerned with maximizing maximizing abilities and compensating approappropriately for for limitations limitations that that PD produce. The The aims priately PD will will produce. aims of intervention intervention are are to prevent disability and maintain maximum prevent disability and maximum physical, physical, emotional, spiritual, social, social, and psychological psychological function function for for as as long long as as possible possible (see (see Table Table 13.1). vocational and 211 217

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Table 13.1 Aims of of intervention intervention of 01 13.1 Aims OT in PD

To provide provide an understanding understanding of of the the disease disease • To provide provide education education and support • To assist assist the the individual individual and and carer cater to achieve achieve aapositive positiveattitude attitudetowards towardskeeping keepingindependent independent • To To prevent or or delay delay disability • To restore function, function, roles and relationships • To plan interventions that are are appropriate appropriate to to the the needs needs of of the the patient, patient, carer carer and and family family • To settings To maintain quality quality of of life near to the the end end of of life life in palliative care settings •

A frame of reference and aa model model of of practice practice for for OT 01 reference and

A humane humane frame frame of of reference reference emphasizes emphasizes holistic holistic practice practice and andisisflexible flexible enough enough to to allow aa rehabilitative rehabilitativeapproach approachtotothose thosewith withdeteriorating deteriorating conditions. conditions. AAframe frameof of allow reference knowledge, principles research reference provides provides an an organization organization for for the knowledge, principles and and research findings practice. A A model is is the system or process in which findings that that underpin underpin practice. which theories or ideas framework for pracideas are are presented presented in in an an organized organized format, and provides a framework model, the the Reed Reed and Sanderson Sanderson Model, Model,works works well well in practice in in PD PD tice. One such model, (see Fig.13.1). (see Fig.13,1).Occupation Occupation isisseen seenasasbeing beingfundamental fundamentaltotohuman human health health and this model emphasizes autonomy, independence, actualization actualization and selfselfmodel emphasizes autonomy, functional functional independence, actualization.

Assessment in 01 OT Standardized methods methods of assessment Standardized assessment are required required to to objectively objectively measure measure the the complex disabilities disabilitiesand andhandicaps handicapsthat that result result from from PD. PD. The The domains domains of of imporimporcomplex of activities of daily living, handicap, tance in assessment by the OT consist of handicap, quality of life life and and cognitive cognitivefunction function (see (see Table Table13.2). 13.2).Unfortunately, Unfortunately,the themost mostpopular popular and simple indexing systems systems are are not not standardized standardized due due to a long long standing standing reluctance reluctance of of adopt such such schemes. schemes. Standardized Standardized and and validated validated assessment assessment tools tools are are therapists to adopt establish the the clinical clinical effectiveness effectiveness of OT in chronic diseases diseases such as as PD. needed to establish of these these two two Assessment tools can be generic or disease specific specific and and a combination of assessments is often practical way way forward forward (Wade (Wade 1992 1992 and see types of assessments often the most practical Chapter 3).

01 Specific assessments in OT The Canadian Canadian Occupational Occupational Performance Performance Measures Measures (COPM) (COPM) measures measures perforperfor-

mance mance that is is both important important and andspecific specific to to the theindividual individual (Law (Law et et al. al. 1994). 1994). It It includes self-care self-care assessment, assessment, domestic domestic activity, activity,work work and and leisure. leisure.The The emphasis emphasis on on occupational of this this assessment assessment less less relevant relevant to to elderly elderly people. people. occupational work makes parts of

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Table 13.2 areas for for the the therapist in PD 13.2 Assessment Assessment areas

Range of of movement movement - grip and pinch strength Range • Fine Fine motor motorcontrol control— - coordination, handwriting • Gross Gross motor motorcontrol control— - rolling, turning, positioning, balance and transfers • Tolerance and endurance • Sensory Sensory loss •

• Cognitive Cognitive function • Self-concept Self-concept • Mood • Coping Coping skills skills • Social situation situation and support network • Role Role performance • Daily Daily living skills - kitchen work, aids • Personal Personal care • Productivity—work Productivity - work skills skills • Leisure Leisure interests interests and and hobbies • Home Home environment environment— - risk assessment • Environmental Environmentalbarriers barriers—- adaptations

The Barthel Index (Collin et al. 1988) has been been widely widelyused usedin inoccupational occupational therapy 1988) has and is the 'gold 'gold standard' standard' assessment assessment of of physical physical ADL. ADL. The The Functional Functional Independence Measure (FIM (FIM 1993), 1993), and the Assessment Assessment of Motor Processing Processing and Skills Skills (Fisher (Fisher 1995) 1995) can can also alsobe beused usedto to determine determine dependency in PD. Several factors factors need need to tobe beconsidered consideredbefore before interpreting interpreting the results of functional Several assessment assessment in in PD. PD. These Theseinclude includethe thenatural natural variability variabilityand and fluctuating fluctuatingnature nature of of and anxiety, anxiety, the interinterPD, the presence of comorbidity, the influence of depression and action medication and and communication communication problems. problems. Assessment Assessment should lead to to action of medication focused focused and and planned planned interventions that deal with with problems problems relevant relevant to to the the patient, patient, carer crucial to carer and and family. family.Motivation Motivationon on the the part part of of the the patient patient and carer is crucial to the success of success of any any intervention intervention and and will will depend depend upon upon the intervention being linked to meaningful meaningful goals.

for 01 Specific areas areas for OT intervention intervention in in PD PD and grooming Dressing and The importance importance of of an an individuaPs individual's style style must must be be recognized recognized when when giving giving advice advice on

clothing. change lifelong lifelong habits to to clothing. Whereas Whereas some some individuals individuals will will be be happy happy to change increase of dressing dressing advice advice is increase independence, independence, others others may may not. not. The The main main points of is to to clothes that that are are easily easily taken off, off, with fastenings fastenings that are easy to use and promote clothes

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Occupational therapy Occupational accessible. Most Most clothing can can be be adapted adapted easily. easily. Dressing Dressing encompasses encompasses cognitive cognitive

skills such such as asthought thought processing, processing, planning planning and sequencing and is also also a treatment skills activity that can can be be used used to to promote balance balance and and coordination (Turner activity that (Turner 1992). 1992). Washing and bathing Bathing and washing may need to be more frequent in in PD PD due due to to the the oiliness oiliness of of the the skin. The is a common site for accidents in elderly The bathroom bathroom is elderly patients patients with with balance balance as a priority the therapist therapist must must ensure ensure the thesafety safety of of the the and mobility problems and as individual individual whilst whilst carrying carrying out out these these activities. activities. Good Good lighting, lighting, non-slip non-slip bath bath mats mats and the provision of shower units can help reduce accidents. Toilet Toilet and and bathroom bathroom to open open outwards outwards with withaalocking locking mechanism mechanism accessible accessible from from doors should be hung to sides. It may may be necessary necessary to such as as aa perching perching both sides. to introduce some equipment such stool to reduce the fatigue of standing and and when when balance balance is is impaired. impaired. In In later later stages stages it maybe necessary necessary to suggest hoists or strip washes in the home involving personal carers or or family family members. home carers Toileting

PD constipation constipation is common as is frequency frequency and and urgency urgency of of micturition micturition due to In PD unstable bladder. bladder. Physical Physical disability disability compounds these problems problems by making making an unstable compounds these getting to more difficult. difficult. Grab rails and a seat raise can help getting to and using the toilet more and off off the toilet. Clothing needs needs to be manageable and all all requirewith getting on and ments for hygiene should be close close to easy to use. to hand and easy Eating and and drinking drinking be Due to problems with tremor, tremor, akinesia akinesia and and swallowing swallowing difficulties, difficulties, meal times can be a nightmare for patients with the patients and and their their families. families. The The OT OT needs needs to to work work closely closely with physiotherapist to establish appropriate positioning positioning at at meal times and with physiotherapist to establish appropriate meal times with the the speech speech therapist therapist and dietician dietician to ensure ensure adequate adequate and safely safelyadministered administered nutrition. nutrition. and on on occaoccaGeneral advice can be given given such such as as taking taking smaller smaller more more frequent meals and sions adapted cutlery and non-slip non-slip table table mats matscan canbe beusefully usefully supplied. supplied. Eating Eating meals meals response to to medication medication isis maximal maximal may may also also be worth trying. trying. when the response

Promoting a safe environment Helping to maintain maintain home home safety, safety, particularly in in the the kitchen kitchen when preparing preparing meals meals and on outside outside paths leading leading around the the house, house, is is aa very important aspect aspect to to the the work of the therapist. progression in PD therapist. The The risk risk of of falling falling increases increases with disease disease progression need to to be be advised advised on what what best best to to do do after after having having fallen. fallen. The proviproviand patients need sion maintain confidence. confidence. Advice Advice can given on sion of personal alarms can help to maintain can be be given on the positioning of of kitchen appliances and how how to safely safely move move and and handle handle items items (e.g. (e.g. by sliding a kettle kettle rather than lifting it).

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Table 13.3 equipment in 13.3 Provision Provision of equipment in PD PD

Timing Appropriateness

Environment Safety Choice

Is it appropriate; is there still scope scope for for further treatment? Is it practical; does it improve quality of of life or encourage dependency? dependency? Is the home physically suitable and practical (e.g. (e.g. space, floor floor surfaces)? surfaces)? Is it safe safe for for the the individual individual in in his/her his/her environment? Is it what what the patient wants and and will will the carer support support its its use? use?

Providing equipment equipment Providing The use use of of equipment needs Table 13.3). 13.3). IfIf introduced introduced needs careful careful consideration (see (see Table

too soon itit can rather than than maintain can promote promote an an environment environment of of dependency, dependency, rather maintain independence. Inappropriate Inappropriate provision provision of of equipment equipment can can worsen worsen impairment impairment in in independence. trolley may may exacerbate exacerbate spinal spinal flexion flexion because because PD. For example, the use of a wheeled trolley it is pushed far ahead ahead and and increases increases the risk risk of of falls. falls. Walking Walking equipment pushed too far equipment that works well as aa wheeled zimmer frame, frame, may may be be well in the clinic or Day Hospital, such as at home due due to to thick pile carpet or other environmental hazards! In inappropriate at In late stage PD, particularly if falls falls are a major problem, it may be necessary to conare major it may be necessary to consider a wheelchair for outside use and then even perhaps in the home if internal distances are long. A powered may also also powered wheelchair, wheelchair, either either self self or or attendant attendant operated, may significantly improve life in late stage stage disease. be seen seen significantly improve quality of life disease. Equipment Equipment must be to be useful to the patient patient and and carer carerand andalso alsoas asaaresult result be be actually actually used. used. A A considconsiderable prescribed aids and appliances appliances remain unused unused erable number number of inappropriately prescribed and often further clutter up the home. and often further Transfers and and mobility mobility The patient patient should should be encouraged encouraged to assist assist in transfers transfers for as as long long as as possible. possible. Carers may need back care education. The OT should work work closely closely with the physiophysiocarer. Specialist Specialist programmes in concontherapist and carer. programmes of of dance dance and and movement movement run in junction with the the physiotherapist physiotherapist may may help help to maintain maintain mobility mobility and and balance. balance. junction Conductive programmes may may benefit benefit some some patients. patients. The The most most effective effective Conductive education programmes elements of this group therapy approach can be incorporated in generic rehabilitaelements of this group therapy approach be incorporated by therapists. therapists. tion programmes by Driving

The ability to drive is aa major major factor factor determining the quality elderly quality of life of many elderly people. people. Although Although in inthe theUK, UK,for for example, example,there thereisisaalegal legal responsibility responsibilityfor for the thedriver driver the Driver Driver and andVehicle Vehicle Licensing Licensing Agency to inform the Agency of of the the onset onset of PD, this diagno1997). Problems with sis alone does not necessarily preclude preclude safe safe driving driving (Anthony 1997). akinesia, tremor and dyskinesia may make driving less safe in akinesia, tremor dyskinesia may make driving less safe in PD. PD.In In PD PD reaction reaction times and and movement movement times times may may be beslowed slowedand andcognitive cognitivevisuospatial visuospatial skills skills times

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cognitive impairment impairment and dementia in impaired. The development development of significant significant cognitive in late stage PD will also also preclude preclnde safe assess aa person's person's safety safety safe driving. The best way to assess to drive is by by aa practical practical test. test. Several Several Driving Driving Assessment AssessmentCentres Centres are are located located in in the the assessments are are carried carried ont outand andadvice adviceisisalso also UK. Physical assessment assessment and driving assessments offered to passengers. offered Handwriting skills vitalcommunication commnnicationskill. skill.It Itis is nsed to convey onr thonghts, Handwriting is aavital used to convey our thoughts, ideas ideas and to record information. In PD handwriting can deteriorate to the extent of preadequate written commnnication communicationand andexpression. expression.In Inmany manycases cases the inabilinabilventing adeqnate ity to sign a cheqne vital part of A ity cheque can can take take away away a vital of the the individnal's individual's antonomy. autonomy. A specimen new signature given to financial institutions authorize specimen of of aa new signatnre can can be be given to financial institntions to to anthorize financial individual therapy, therapy, can can be financial transactions. transactions. Writing, Writing, as as group gronp therapy therapy or or individnal included inclnded as as part part of of the the programme programme of rehabilitation. Counselling in PD health professionals find themselves themselvescounselling connsellingatatsome somepoint point during dnring the Most health professionals find helping process. process. Each Each will have varying levels levels of helping of skill. skill. Counselling Connselling can can be be described being, rather than than aa set set of skills (Egan 1990). A as away a wayof ofbeing, A coming coming together together of experience, awareness awarenessand andcommunication commnnication isisthe themost mostimportant important part part of of the the relationrience, relationship between patient. The The nse use of of listening listening skills, allowing between therapist and patient. allowing the the patient to be heard, is an aspect of this work. It is your liman important aspect is important important to know yonr itations and to know when to seek seek fnrther further advice advice from aa psychologist. The onset of dependency in formal dependency in PD PD is a time when many patients may benefit benefit from from more formal counselling. Many patients will display fear, anger and shame at this time. connselling. Many patients will display fear, anger and shame at this time. Emotional adaptation appears appears to to be be an an important importantpart partofofaasuccessful successful coping coping stratstrategy in PD, yet is neglected by therapy interventions. Turnbull (1992) promotes egy in PD, yet is neglected by therapy interventions. Tnrnbnll (1992) promotes aa 'progressive paradigm' prob'progressive paradigm' for referral in PD, with patients being referred before problems have developed, to promote emotional adaptation. More research is needed to developed, to promote adaptation. More research is explore which effective in explore which type type of counselling connselling approach is most effective in meeting meeting the the needs needs of patients and carers. carers. Stress, anxiety and depression depression

Anxiety is a common and and disabling disabling condition condition arising arisingininPD. PD.Social Social anxiety anxiety is is parparticularly common common in PD as patients patients are afraid ticularly afraid of the reactions reactions of others in social social situations withdraw from from social social life. life. Social Social isolation isolation may also also represent represent the situations and withdraw results in in isolation and and lack lack onset of significant significant cognitive cognitive impairment. impairment. This, in turn, turn, results of opportunity to to practice practice coping coping strategies strategies and and increases increases vulnerability to depresdepression. Relaxation therapy effectively used explored sion. Relaxation therapy can can be be effectively usedinin PD PD and and needs needs to to be explored more fully fully in in group group and individual treatments.

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also common common in PD and often with anxiety. is imporDepression is also often coexists coexists with anxiety. ItIt is tant for for the the therapist therapist to to recognize recognize and and detect detect depression depression as as this can often often be successfullytreated treatedby by explanation, explanation, support, support, cognitive cognitive therapy therapy and and drug treatment. cessfully treatment. Patients described of prompting' prompting' Patients described as as having having 'poor 'poor motivation' motivation' or or 'needing 'needing a lot of usually are expressing expressing cognitive cognitiveproblems, problems, depression depression or or aa combination combination of the two. Cognitive deficits

problems are are common common in PD and result Cognitive problems result in reduction reduction of of activities activities and increased disadvantage. disadvantage. The The assessment cognitive function function should increasingly increased assessment of cognitive increasingly OT. Training in techniques of of cognitive cognitive therapy, therapy, under the the become a role for the OT. direction neuropsychologists, will direction of neuropsychologists, will bring bring aa much much needed needed expertise expertise to to rehabilitarehabilitateams. Tiredness Tiredness and and fatigue fatigue exacerbate exacerbate difficulties difficulties with and tion teams. with concentration and rehabilitation programme programme must must account account for for fatigue fatigue with with aa balance balance memory and aa rehabilitation of activity. Memory Memory games games and remedial remedial reminiscence reminiscence can to of rest and activity. can be used to cognitive function. function. maintain cognitive Evidence for the effectiveness effectiveness of 01 OT in in PD PD Unfortunately, there is very little published evidence of the efficacy of any of the efficacy of described above above in in the the management management of of PD. PD. There There can canbe beno nodoubt doubt that that approaches described the known problems associated OT 'works' given the associated with with PD. PD. Patients Patients with with PD PD are are still still infrequently 1997) and infrequently referred referred to OT OT (Meara (Meara and and Hobson 1997) and patients have have considerconsiderable first assessed assessed (Beattie (Beattie and Caird 1980), 1980). An early study of able unmet needs when first OT in in PD PD was wasinsufficiently insufficientlydetailed detailedtotointerpret interpretand andsuffered sufferedfrom fromserious serious methodmethodological ological flaws flaws(Gibberd (Gibberd etetal. al. 1981). 1981).One Onestudy studyusing usingaasingle singleblind blindand andrandomised randomised design design suggested suggestedpractical practicalbenefit benefitfrom fromgroup group therapy therapy intervention intervention that persisted for (Gauthier et et al. al. 1987). 1987). for over over aa year year in in the the intervention intervention group (Gauthier

Summary As As a member

of a multidisciplinary team the OT can can make make an an important important contribucontribution to to the the successful successful management of of the the physical, physical, functional, functional, social, social, psychologipsychological, and and emotional of PD. Some cal, emotional consequences consequences of Some of the the difficulties difficulties surrounding surrounding assessment discussed and some suggestions suggestions for and assessment in in PD PD have have been been discussed and some for treatment treatment and rehabilitation rehabilitation programmes programmes have havebeen been put put forward. forward. Whilst Whilst treatment treatment should should be be knowledge and evidence based for clinical clinical practice practice is is still still knowledge and evidence based a stronger stronger foundation for required.

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REFERENCES Anthony M (1997) (1997) Challenging Challenging assumptions: assumptions: Parkinson's Parkinson's disease disease and driving. driving. Occupational Therapy 12—13. TherapyNews, News,April April1997, 1997, 12-13.

Beattie A, A, Caird Caird FI Fl (1980) (1980) The The occupational occupational therapist and the Beattie the patient patient with withParkinson's Parkinson's disease. disease. British Medical 280, 1354—S. British MedicalJournal, Journal, 280, 1354-5. Cohn C, Collin C, Wade Wade DT, DT, Davis Davis S, S, Home Horne VV(1988) (1988) The The Barthel Barthel ADL ADL Index: Index: aa reliability reliability study. study. International Disability 10,10, 61—3. International DisabilityStudies, Studies, 61-3. Egan C Skilled Helper. Helper.California: California:Brooks BrooksCole ColePublishing PublishingCompany. Company. G (1990) (1990) The TheSkilled FIM (1993) Guide Guide to the Uniform Uniform Data DataSet Setfor for Medical MedicalRehabilitation Rehabilitation(Adult (AdultElM). FIM).Buffalo BuffaloNew New York: Research Research Foundation Foundation State StateUniversity UniversityofofNew NewYork YorkatatBuffalo. Buffalo. AC (1995) Assessment Three Star Fisher AG Assessment of of Motor Processing Processingand andSkills. Skills.Fort FortCollins CollinsCompany: Company: Three Star Press. Press. Cauthier L, 5, GauthierS Cauthier 5 (1987) (1987) The The benefits benefits of ofgroup group occupational occupational therapy therapy for patients Gauthier L, Dalziel DalzielS, with of of Occupational Therapy, 41, 360—S. AmericanJournal Journal Occupational Therapy, 41, 360-5. with Parkinson's Parkinson'sdisease. disease.American Cibberd FB, Page Page NGR, NCR, Spencer Spencer KM, KId, Kinnear Kinnear E, E, Hawksworth Hawksworth JB JB (1981) (1981) Controlled Controlled trial trial of of Gibberd physiotherapy 282, physiotherapy and andoccupational occupationaltherapy therapyfor forParkinson's Parkinson'sdisease. disease.British BritishMedical MedicalJournal, Journal, 282, 1t96. 1196. Poundations for for Practice. Models, Prames of Reference Hagedorn HH(1992) (1992)Occupational OccupationalTherapy Therapy Foundations Practice. Models, Frames of Reference and and Core CoreSkills. Skills. London: London:Churchill ChurchillLivingstone. Livingstone. Law Law M, M, Polatajko Polatajko H, H, Pollock Pollock N, N, McColl McColl M, M, Carswell Carswell A, A, Baptiste Baptiste S5 (1994) (1994) Pilot Pilot testing testing of the Canadian occupational Canadian occupational performance performance measure: clinical clinical and measurement measurement issues. issues. Canadian Journal Therapy, 61,61, t91—7. JournalofofOccupational Occupational Therapy, 191-7. Meara RJ, Hobson JP (1997) Levels of service service provision provision for for people with Parkinson's disease: Meara RJ, Hobson Levels of disease: a snrvey of community registered survey registered patients' patients'perceptions. perceptions.Journal Journalofofthe theBritish BritishAssociation Associationfor for Service 64,64, 3—tO. Servicetotothe theElderly, Elderly, 3-10. Churchill Livingstone. PhysicalManagement ManagementofofParkinson's Parkinson sDisease. Disease.London: London: Churchill Livingstone. Turnbull G G (1992) (1992) Physical Dysfunction. London: Williams andand Wilkins. Turner A (1992) Occupational OccupationalTherapy Therapyand andPhysical Physical Dysfunction. London: Williams Wilkins. Wade DT (1992) Measurementin University Press. Measurement inNeurological NeurologicalRehabilitation. Rehabilitation.Oxford: Oxford:Oxford Oxford University Press.

Rehabilitation, Rehabilitation, speech speech and language therapy and elderly patients with with Parkinson's Parkinson's disease disease Sheena Round

Introduction Speech and swallowing problems are common in in elderly elderly subjects subjects with Parkinson's Parkinson's disease (PD). (PD). Patients disease Patients and carers carers frequently frequently cite cite communication communication difficulties difficulties as causing the the greatest greatest disability disabilityand and handicap handicap in in PD PD (Oxtoby In one study causing (Oxtoby 1982). 1982). In around 70% of patients with PD complained complained of impairment of of speech speech and and voice voice and 41% difficulty with chewing and swallowing swallowing (Hartelius (Hartelius and and Svensson Svensson 41% reported difficulty 1994) and reported speech speech problems problems in in over over 60% of patients studied studied 1994) and another study reported et al.1985). al.1985). Dysphagia Dysphagia has has been been reported reportedinin15—50% 15-50% of patients with with PD, PD, (Gibberd et swallow being 95% of of cases cases with abnormalities on barium swallow being demonstrated demonstrated in up to 95% (Bramble etetal. al.1976, 1976, Robbins Robbins etet al. al.1986, 1986, Lieberman Lieberman etetal. al.1980). 1980).A A survey survey studied (Bramble carried out by the Parkinson's Disease Disease Society Society of found of the the United United Kingdom (UK) found 40% of of the the PD PD patients patients contacted contacted complained complained of drooling drooling and 26% 26% had had that 40% difficulty as a result result of of dysphadysphadifficulty swallowing swallowing (Oxtoby (Oxtoby 1982). 1982).Aspiration Aspiration pneumonia pneumonia as gia event in in late late stage stage PD. gia is is aa common common terminal event Despite the the high prevalence Despite prevalence of communication and and swallowing swallowing problems very few patients patients with with PD get get referred referred to speech and language therapists (Oxtoby few (Oxtoby 1982, 1982, 65% of patients in in their their Mutch et al. 1986). Mutch Mutch et al. al. (1986) (1986) found found that although 65% reported difficulties difficulties with speech speech less less than 5% 5% had had been beenassessed assessed by by aa speech speech study reported and language therapist. the efficacy efficacy therapist. A developing body of evidence exists supporting supporting the of speech 1984, Scott speech therapy therapy in PD PD (Scott (Scott and and Caird Caird 1983, 1983, Robertson Robertson and Thomson 1984, et al. 1990). This al. 1985, 1985, Johnson Johnson and and Pring Pring 1990). This chapter chapter aims aims to to examine examine the symptoms most frequently seen therapists working elderly patients the most frequently seen by by therapists working with with elderly patients with with PD, the methods of assessment assessment used, used, and and some some of of the the therapy therapy techniques techniques used used to to treat treat PD. PD.

What causes speechand and language languageimpairments impairments and and swallowing swallowing problems in causes speech PD? The impairments impairments that result result in in the the characteristic characteristic speech speech of of PD, PD, which which is is often often

present early in the natural history history of of the the disease disease and and the the involvement involvement of of swallowswallow226

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usually only seen seen in in late late stage stage disease, disease,are arepoorly poorly understood. understood. Speech ing, which is usually difficulties probably reflect reflect rigidity rigidity and and akinesia akinesia in in the the muscles muscles involved involved in in phonaphonadifficulties probably to and and execution execution of oflearnt learnt motor motor tion and respiration. Speech Speech requires rapid access access to motor deficit deficit of akinesia disrupts speech and impairs complans. In PD the central motor munication. Loss of akinesia must reflect reduced reduced munication. Loss of speech speech control control due due to to akinesia must in turn reflect striatal transmission. Clinically, Clinically, certain disturstriatal dopamine transmission, certain elements elements of the speech disturbance of PD do do improve improve after after dopaminergic dopaminergic therapy therapy though though the temporal relationship between effect is uncertain. Shea Shea et al. (1993) found that that selegiline selegiline between dose and effect could result significant improvements could result in in significant improvements in in articulation articulation and and other other speech speech probproblems. Swallowing Swallowing problems respond to to lems. problemsoccur occurinin late late stage stage disease diseaseand and do do not not respond dopaminergic drug treatment, though though isolated isolated case case reports reports have have claimed claimed benefit benefit dopaminergic from drug therapy. The association between swallowing problems, disease progression and late onset disease in older subjects subjects suggest suggest that that these problems may result from an interaction of of dopamine dopamine loss loss and and aging aging changes or to involvement involvement either from non-dopaminergic pathways pathways with with disease disease progression. progression. of other non-dopaminergic

Age and comorbidity elderly patient patient with with PD has to contend The elderly contend with with the therecognized recognized effects effects of of aging, aging, comorbidity and speech and swallowing mechaand the the effects effects of the disease disease itself itself on speech swallowing mechaThese include physical, physical, cognitive cognitive and and perceptual perceptual changes changes linked linked to to age age and and nisms. These disease. ItIt isisimportant PD patient patient to to be be aware aware of these disease. important for the therapist treating the PD complex interactions (see Table Table 14.1). 14,1). Communication Communication and dysphagia often often occur together in elderly elderly patients patients and and are are the the two two main main areas areas of of intervention intervention for for the the therapist.

therapy impairments impairments in in elderly elderly patients with PD Speech and language language therapy PD several aspects aspects of of speech speech and and language language that that need to be assessed assessed in in patients patients There are several with PD 14,2), Impairment Impairment of PD (see (see Table 14.2). of language language includes includes loss loss of facial facial expression, rate of of speech, speech, reduction reduction loss of volume, volume, reduced reduced control over respiration and the rate in the prosody and rhythm of of speech and the presence presence of dribbling. The resulting The resulting motor symptoms are are usually classified classified as asaahypokinetic hypokineticextrapyramidal extrapyramidal dysarthria. dysarthria.

Communication disorders in in PD PD Communication itself itself is is aa two two way way process process involving involvingthe the imparting imparting and exchange exchange of information verbally and nonverbally. nonverbally. In In PD PD communication communication can can be be disrupted verbally and ways from from deficits deficitsthat thatcan caninclude includerespiratory, respiratory,phonatory, phonatory, prosodic prosodic in a number of ways and articulatory features together with impaired body language.

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Table 14.1 The impact impact of of aging and disease disease on on speech speech and and swallowing swallowing in 14.1 The in elderly elderly patients patients with with PD PD

Age related related changes changesinclude include ossification ossification of of cartilages cartilages of ofthe thelarynx, larynx, atrophy atrophy of of the the muscles musclesof of •• Age the vocal cords and stiffening stiffening of the crico arytenoid joints. nonverbal verbalclues cluessuch suchasasfacial facial • Deteriorating Deteriorating vision vision may may affect affect the interpretation interpretation ofofnon expression. is often often aa barrier barrier to to social social interaction interaction and personal adjustment adjustment despite the advances • Deafness Deafness is advances in hearing aids. aids. Hearing aids, aids, even when provided, are not always always worn and even when in place are are sometimes sometimes in in aapoor poor state state of ofrepair. repait place • Any respiratory disease disease that impairs impairs vital vitalcapacity capacity can can affect affect speech. speech. Respiratory Respiratory disease disease isis common in in older older people and and will will further impair impair speech speech in in PD. PD. conversation in the elderly (Scott • Speech repetition and hesitation are are recognized features features of conversation elderly (Scott of speech often often noted in elderly et al. 1985). These Thesemay mayaugment augmentthe the disruption disruption in rhythm of patients. patients are more likely likelyto tohave havecognitive cognitiveimpairments impairmentsand anddementia dementiathan than the the • Elderly patients younger patients and communication communication skills skills are further impaired impaired by by dementia dementia (Powell (Powell et al. al. 1995). • Depression and and anxiety, anxiety, which are common features features of ofaging, aging, may may increase increase the the difficulties, social communication difficulties, social isolation isolation and and dependency dependency resulting resulting from from language language and and difficulties caused caused by by PD. PD. swallowing difficulties problems in PD may in elderly patients patients be further impaired of impaired by by side side effects effects of • Swallowing Swallowing problems concurrent medication (dry mouth), poor dentition, dentures and poor dentition, ill ill fitting fitting dentures oesophageal motility

Table 14.2 14.2 Areas Areas of of speech speech and and language and and dysphagic dysphagic assessment assessment Facial expression Facial • Rate Rate of of speech • Intonation • Volume • Intelligibility • Respiration Respiration • Swallowing Swallowing • Rhythm Rhythm •

Overall, the patients' patients' communication communication difficulties may make makethem themhard hard to to underunderOverall, the difficulties may stand and and tiring tiring to to listen listen to. to.Reduced Reduced facial facial expression expression can can be misinterpreted misinterpreted by by carers, friends friends and professionals hostility, coldcoldcarers, professionals as indicating a lack of intelligence, hostility, ness, intended ness, depression, depression, deafness deafnessororlack lackofofinterest interest on on the the patient's patient's part. part. The intended message of patient can, therefore, therefore, be loss or message of the the PD PD patient be misinterpreted misinterpreted due due to to the loss

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of communication communication skills skills that that were were previously previously autoautoreduction in the effectiveness effectiveness of matic. Respiratory associated impairments in elderly patients with associated impairments with PD PD symptoms that suggest an underlying underlying respiraElderly patients frequently frequently exhibit exhibit symptoms suggest an tory deficit. Decreased Decreased expiratory expiratory air flow, flow, short, shallow shallow clavicular clavicular breathing, breathing, tory deficit. reduced lung capacity and air wastage wastage before before initiating initiating an an utterance utterance are are features features of of the impaired As a result, the impaired respiratory respiratory mechanism mechanism in elderly elderly patients. patients. As result, respiratory respiratory associated functional speaking associated functional symptoms symptoms may may include include shortness shortness of of breath breath when speaking and a reduction reduction in in volume. volume. Difficulties Difficulties coordinating and coordinating respiration respiration and and phonation may result in reduction in control control over over the pace of speech, which which can, can, in in turn, turn, result in abnormalities abnormalities in rhythm. Breath Breath may may run out before before a phrase or or sentence sentence has been completed. completed. Voice, Voice, volume and respiration Respiration, in terms of of breath breath support support and andbreathing breathing patterns, patterns,isisinextricably inextricably

linked to to the pacing of of speech and phonation phonation of of the linked the volume, volume, pacing speech and the PD PD patient patient (Montgomery et et al. al. 1972). 1972). (Montgomery Clavicular breathing patterns can result in short, shallow episodes episodes of of inspiration inspiration Clavicular breathing and expiration. Breath Breath support support can can then then be be inadequate inadequate to to maintain maintain phonation phonation over over results, or, typically, a complete sentence sentence and short phrasing results, typically,breath breath runs runs out before the end of the sentence and residual air is used used whilst whilst the the patient patient continues continues to try to phonate. The voice quality is breathy, breathy, indicating indicating inefficient inefficient use use of of breath breath for phonation. voice quality phonation. Phonation cannot cannot be be maintained maintainedand andthere thereisisaaloss loss of of vocal vocal intensity. intensity. If tension of voice quality quality can canbecome becomeharsh harsh and and erratic erratic the laryngeal musculature is evident, the voice and tension. tension. Voice Voice production can can be be as a result result of of aa combination of 'breathiness' and intermittent, with slowed slowed onset onset of of voice voice and pitch breaks. intermittent, with Articulation Although the PD patient is capable of of producing producing speech speech sounds sounds in in isolation is usually capable or within within single single words words correctly correctly with with no nophonological phonological difficulties, difficulties, inaccuracies inaccuracies often become apparent in conversation. Ill Ill fitting dentures can also influence influence articoften ulatory accuracy accuracy and and rate. The range and accuracy of the sophisticated, sophisticated, fine fine coorcoordinated needed for for speech speech are are reduced reduced due due to to muscular muscular rigidity rigidity and and dinated movements movements needed tremor together with with difficulties difficulties in initiation. This This affects affects movements movements of the lip, tongue and and jaw. jaw. Multisyllabic words and and consonant consonant clusters Multisyllabic words clusters are often often articulated articulated imprecisely imprecisely articulatory placements placements not notalways always accurately accurately achieved. achieved. Plosives Plosives (p,b,k,g,t,d) with articulatory syllables may can be weak weak due due to to an an impoverished impoverished air air stream stream and some sounds and syllables may

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Table 14.3 14.3 Prosodic Prosodic features features in in PD PD

The rate of speech in PD can be increased or decreased compared to age age matched subjects subjects and can exhibit festination as as utterance utterance length length increases. increases. • There There is is often a lack of pausing or a reduced length of of pausing. pausing. • The incomplete production production of of sounds, sounds,together together with with lack lack of of pausing pausing and and the the elision elision of of parts parts of consonant clusters or reduction of give the the impression impression that that aa of the the number number of ofsyllables syllables can give faster rate of speech is being used. • Voicing can can be be continuous. • There stress and There can can be be aa loss loss of of word stress stress in in polysyllabic polysyllabic words and aa loss of syllable stress contrastive stress. stress. • Use of stress stress can can be be inappropriate inappropriate and there can be difficulty difficulty in in both both imitating and achieving different patterns. different stress patterns. •

be omitted. Articulatory contacts can be incomplete incomplete and repeated sounds can can be

produced with too short produced with short aa duration duration and andcontrasts contrastsusually usually made made between, between, for for example, voiced voicedand and unvoiced unvoicedphonemes phonemes (e.g. (e.g.p/b) p/b) can can be beblurred, blurred, with with continuexample, ous comous voicing voicing evident. evident.There There isis often often aa reduced reduced duration duration of of some some phonemes phonemes compared to those produced produced by by normal normalspeakers. speakers. Some Some phonemes, phonemes,syllables syllables and words words can together with with complete complete elision elision of of some some phonemes phonemes or orsyllables. syllables. The The can be be run together resultant speech is often described as slurred or indistinct and assessed and treated speech is often described as slurred or indistinct assessed and treated as aa hypokinetic hypokinetic dysarthria. Prosody

Prosody is defined defined as the rhythm of of speech. speech. Prosodic Prosodic features features often often affected affected in in PD PD

stress and and intonation intonation (see stress patterns are rate, stress (see Table Table 14.3). The placing of correct stress within words, words, phrases and and sentences sentences can can affect affect intelligibility and semantics, including conveying conveying emotion emotion without without altering the syntax. The overall overall result result of ofthe the impairimpairment prosody can can be aa flat, flat, monotonous monotonous voice, voice, which which further further reduces reduces the ment of prosody effectiveness (1990) found found effectivenessofofthe thepatient's patient's ability abilityto to communicate. communicate. Pitcairn Pitcairn et al. (1990) that the the tape tape recorded recorded voice voice of of patients patients with with PD PD was was interpreted interpreted by by observers observers as as cold, and to to relate relate poorly poorly to to the the interviewer. interviewer. cold, withdrawn withdrawn and anxious and Rate of speech speech Speech rate can be variable, often often increased increased and festinant or slow with problems in initiation. Palilalia, the involuntary repetition of of single single words, may also also be be present. }-Iammen and Yorkston Yorkston (1996) (1996)found found that that subjects subjects with with PD PD had a greater proporHammen and proporof pauses pauses occurring occurring at at syntactically syntactically inappropriate inappropriate locations locations in comparison comparison tion of with age matched subjects. When speech rates were reduced, both groups groups showed showed a decrease decrease in in pauses pauses located located at at appropriate appropriate syntactic boundaries.

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Assessment of communication in Assessment of in PD PD Assessments devised to tailor tailor communication commnnication assessment devised to assessment to the individual profile profile

are not not yet yet widely widely used or or available. available. The Parkinson's Disease Society Society of the patient are devised a Speech Speech and Language Therapy of the UK has devised and Language Therapy checklist checklist for for PD PD patients. This assessment is service modality specific specific assessassessThis assessment service specific specific and and in in common with modality ments dysarthria assessments) assessments) does full account or ments (e.g. dysarthria does not not take take full account of the patients' or carers' perceptions difficulties in carers' perceptions of of communication difficulties in functional functional terms. At At present, standard multidisciplinary multidisciplinary assessments assessments that would would allow allow us to to form form collaborative collaborative goals for exist. As multidisciplicare pathways pathways or or establish establish mutual mutual goals for patients, do not exist. nary teams emerge and develop, then so will assessments that will the need for assessments that can be incorporated easily easily into this this model model of of care. care. Ideally, Ideally, assessment would be aimed at at finding areas of finding areas of difficulty difficulty from from each each discipline's discipline's perspective perspectiveand and then then determining therapeutic goals. goals. Goals specific Goals should should be be based based not not only only on on the performance in a specific assessment test, perception and wishes of and carer. carer. assessment test,but but also also on on the the perception and wishes of the the patient and are the patient patient and and carer's carer'saims aims and andaspirations? aspirations?Are Arethey they the the same same as as the thertherWhat are apist's? These patient, apist's? These aspirations aspirations maybe may be at at aa different different point point of the denial curve for patient, family at carer and family at any any one one point point in in time. A A patient patient may may be be at at the the point point of of denying denying the diagnosis, whilst the carer may have accepted accepted it, it, causing causing aa different different emphasis emphasis in in rehabilitation, which which may may affect affect functional functional outcome. Speech Speech therapy attitude to rehabilitation, outcome measures measuressuch suchas asquality quality of of life, life, even assessments rarely include functional outcome though these are arguably arguably the issues for the patient and and carer. carer. the most most important issues Speech and language language therapy treatment in in PD PD

practice is is for for early early intervention intervention by by therapists therapists at at the the time time of diagnosis to Present practice diagnosis to to restore restore function. function. This This maintain function rather than than intervening intervening at at aa later later stage to has been subject subject to to any any formal formal evaluation evaluation of ofeffectiveness, effectiveness, but represents represents aa has not been growing clinical stage can also also growing clinical consensus consensusofof best best practice. practice. Intervention Intervention at at this this stage prevent unhelpful unhelpful habits from from forming. forming. Early Early intervention is is particularly imporimportant in in elderly elderly subjects subjects who funcwho are at greatest risk of early decline decline in language language function. Skills Skills that relearned as as that were were previously previously carried carried out automatically need to be relearned taught behaviours. These These include include facial facial expression, expression, diaphragmatic diaphragmatic breathing, breathing, taught behaviours, prosody, ability to self self monitor. Video and audio recordings recordings are prosody, volume volume and and the the ability monitor. Video often used to overcome often overcome problems problems patients may have have in inself-monitoring self-monitoring speech speech proproand quality. quality. duction and Speech therapy treatment treatment as as part part of ofan anexercise exercise programme programme in inPD PD

PROPATH programme (Montgomery (Montgomery et al, The PRO PATH programme al. 1994) 1994) was designed designed to improve improve functional patients through programme of education education and and functional outcome outcome for for PD PD patients through a programme

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exercise. The study demonstrated that that disease disease symptoms symptoms could could be be stabilized stabilized and and self-efficacy andhealth health confidence confidencecould couldbe be increased increased by by this this approach. self-efficacy and approach. This suggests that outcomes outcomes from from therapy therapy in PD may be improved linking therapy therapy suggests that improved by linking exercise proexercise to to more more general general physical physical exercise exerciseininaamultidisciplinary multidisciplinary rehabilitation rehabilitation programme speech therapy therapy in PD PD Intensive speech

A number number of of studies studies have have suggested suggested that that therapy therapy may may be be more more effective effective when given given intensivelyrather ratherthan than in in the the traditional once weekly therapy model model (Scott (Scott and and intensively weekly therapy Caird 1983, 1984, 1984, Le Dorze Dorze et al. al. 1992). 1992). Caird 1981, 1983, 1984, Robertson Robertson and and Thomson, Thomson, 1984, These studies found that periods periods of of intensive intensive therapy therapy can can produce produce significant significant These studies found improvements in speech as assessed assessed by by scores scores for forprosodic prosodic abnormality abnormality and intelligibility. Such ligibility. Such improvements improvements can can last last for for several severalmonths months after after therapy therapy stops. Carer perceptions in these studies studies also also indicated improvement in communication communication perceptions indicated that that improvement skills had patients. skills had occurred in the patients.

Treatment of respiratory related symptoms Respiratory related related symptoms symptoms can affect affect volume, volume, pitch, segmentation segmentation of of speech speech into phrases, rate of speech rhythm of of speech. speech. speech and the ability to to control the rate and rhythm The treatment treatment of of respiratory respiratory related related symptoms symptoms is aimed aimed at at increasing increasing breath breath The the control control of of respiration respiration and and the the relaxation relaxation of of respiratory respiratory musculature. musculature. support, the Diaphragmatic breathing and total body relaxation are often often used to achieve achieve this. this. Treatment of prosodic features Patients with with PD appear appear to to experience experience difficulty difficulty in perceiving perceiving different different prosodic prosodic features of of intonation intonation (Scott Group therapy therapy is often helpful since since itit features (Scott et al. 1984). 1984). Group often helpful allows the the patient patient to experience allows experience multiple examples examples of the target and and receive receive feedgroup members, members,which which aids aids self-monitoring. self-monitoring.The TheVocalite, Vocalite, aa voice back from other group operated light source, has has been been used used successfully successfullytotoimprove improveprosodic prosodicabnormality abnormality increasing the awareness of difficulties and by increasing the patient's awareness of difficulties and allowing allowing the the practice practice of of of intonation intonation and and conversational conversational speech. speech. more normal patterns of

Treatment of abnormal abnormal speech rate Treatment of Pacing techniques techniques are usually used to aid control over the delivery of speech. These Pacing include pacing boards (Helm (Helm 1979), 1979), with physical physical markers for the patient patient to to use use to to pace their speech. Beukelman (1977) also also targeted targeted rate rate reduction by Beukelman and Yorkston Yorkston (1977) where the the first first letter of of each each word spoken was pointed to using an alphabet board where on the alphabet board. They found that this this slowed slowed the rate and increased increased intelligibility. gibility.Rate Rateofofspeech speechcan canoften oftenimprove improvewith withthe theuse useofofdiaphragmatic diaphragmaticbreathing breathing and increased control provides due to the relaxation relaxation and the increased control over over phonation phonation that this provides

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of the muscles used for for respiration and phonation. phonation.Relaxation Relaxation of of the the laryngeal laryngeal musmusculature their range culature may may increase increase their range and ease ease of of movement movement and and consequently consequently the the patient's level level of control and pacing abilities in speech. Hanson and Metter Metter (1980) (1980) used the technique of of delayed delayed auditory feedback feedback for the same purpose. Pacing boards and syllabic speech, where where speech speech is is paced paced in in synchrony synchrony with with a Pacing boards syllabic speech, slow, tapped tapped beat, beat, both both serve serve to to help help establish establish aalearned, learned, patterned patterned response response where where slow, there were introduce were previously previously automatic automatic responses. responses. Another Another method method used is to introduce short pauses between between words. speech rate may not itself itself lead to words. Improvement Improvement in speech meaningful (Downie etet al. al. 1981). 1981). meaningful gains in functional performance (Downie

Every technique requires requiresthe the motivation motivation and and cooperation of the Every technique cooperation of the patient. patient. Without adequate be apparent apparent to to adequate self self monitoring abilities, abilities, improvement may only be given by by aa member member of of the the family family or or by by the the therapist. therapist. the patient when feedback feedback is given Treatment to improve intonation

often reduced in the elderly patients due to a decreased to alter alter pitch, This is often decreased ability to may improve improve with therapy. therapy. The The underlying underlying deficit deficit will remain unaltered, but but but may rigidity is is evident, therapy therapy to to reduce reduce such such rigidity rigidity can can be be effective. effective. where muscular rigidity If the laryngeal laryngeal muscles muscles are arerigid rigidthen then the the elongation elongation and and contraction of the vocal cords longitudinally longitudinally is reduced reduced and therefore therefore the ability to alter pitch is impaired. Relaxation diaphragmatic breathing, breathing,laryngeal laryngeal massage, massage, Relaxation of of these these muscles muscles through diaphragmatic therapy and prosodic prosodic exercises exercises can use of'hot of 'hot packs', hydro hydrotherapy can help help to to restore restore the the range of movement of the muscles by by relaxing relaxing them them and and reducing muscle spasm. This This can can result in an increased pitch range, range, which which will will have a positive positive effect effect upon upon pitch use for emphasis in sentences and stress stress within words. words. The The ability ability to to sing, sing, tell tell jokes, jokes, for emphasis recite conversation of the patient recite poems poems and provide interest for the listener in the conversation can all be be affected affectedby byloss lossofofintonation intonation and and respond following prosodic therapy. If there difficulties in group there are difficulties in recognizing recognizingand and imitating imitating aa normal normal model model then group therapy can again again be helpful. helpful. Visual Visual feedback to improve improve self self monitoring monitoringof ofspeech speech

Caligiuri and and Murray Murray (1983) (1983) found found that that visual visual feedback feedback allowed allowed patients patients to changes in in intensity, intensity,duration duration or or intraoral intraoral pressure pressure of of the monitor self-generated self-generated changes stressed syllable syllableororword. word.In In the the UK UK the the Royal Royal National National Institute Institute for for the the Deaf's stressed Deaf's Visispeech and Speechviewer computer are used to to provide provide Visispeech and the IBM Speechviewer computer programmes are visual feedback feedback with with regard regard to to pitch, pitch, volume volume and and intonation intonation patterns. How effective is prosodic therapy?

Research into into prosodic prosodic therapy therapy often often describes describes the use use of of prosodic prosodic exercises exercises without aa description description of of any any programme of of therapy therapy to to reduce reduce muscular muscular rigidity. rigidity. Scott Caird (1983) (1983) examined examined the effects effects of therapy therapy on on specific specific prosodic prosodic Scott and and Caird

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Table 14.4 Environmental Environmental adaptations adaptations to to improve communication communication

Use contextual contextual clues cluesto to aid aidthe the listeners listeners understanding understanding of the communicated •• Use communicated message message level of of background background noise • Ensure aa low level • Maintain good good lighting on the the face face of the speaker speaker speakers short • Keep Keep the distance between speakers • Adopt a good posture • Use Use external aids (e.g. amplifiers) amplifiers)

features. They found found that that abnormalities abnormalities of of intonation intonation and rhythm, although unifeatures. They versally present, only only marginally marginally improved improved after versally present, after therapy. Abnormalities Abnormalities of vocal quality, rate rate and and volume volume were were present present in in 80% 80% of of patients patients and and improved improved substansubstanquality, tially and improvement was was largely largely maintained. Abnormalities Abnormalities of pitch and and tially and this improvement tone were present in around half of the the patients patients studied and showed showed some some improvement after after therapy, though this this was not maintained. maintained. Treatment to improve improve volume The elderly PD PD patient characteristically has a quiet voice. voice. Fear Fear of of being being inaudible can cause with cause anxiety, anxiety, which which increases increases tension. tension, The The volume volume that that can be produced with a sudden energy such such as a shout is often often far removed removed from sudden burst of energy from the patient's functional ability ability when conversing. conversing. This measured using using aa Sound Sound Level Level functional This can be measured assessment to determine habitual volume. Meter during assessment above aids aids the the patient patient not only in creatDiaphragmatic breathing as described described above ing good breath support but volume for but also also in in facilitating facilitating maximum maximum achievable achievable volume for conversation, conversation, as as respiration respiration isis synchronized synchronizedwith with phonation. phonation. al. (1995) (1995) compared the effects of intensive intensive speech speech therapy therapy on respiraRamig et al. effects of tion and concluded that intensive and on on voice voice and respiration in in PD. PD. They concluded intensive treatment of effective in of voice voice and and respiration respiration was was more more effective in improving improving volume volume than than the the treatment of respiration alone. alone. Several Several environmental adaptations can can facilitate facilitate comcommunication munication when whenvoice voice volume volume isis reduced reduced (see (see Table Table 14.4). 14.4). Where lack of volume is the main deficit, deficit, amplification amplification systems systems for for the patient patient have been found found to be and Watson When this this type type of have been be beneficial beneficial (Greene (Greene and Watson 1968). 1968). When speech will continue speech is amplified, amplified, aa client client will continue to match match speech speech with with the the amplified amplified output for for some some time time after after the the latter latter has has been been switched switched off off (Greene (Greene 1980). 1980). The The benefits are reduction of of anxiety, anxiety, which reduces rigidity, rigidity, tremor and akinesia, akinesia, the benefits psychological psychological value valueand and increased increased ability abilitytoto monitor monitor their own speech speech due to the the auditory feedback. Sound Level Level Meters Meters and visual displays displays of of volume volume on computer programmes, such the IBM IBM Speechviewer, Speechviewer, indicate glance the volume volume programmes, such as the indicate atat a glance obtained by the patient, providing visual feedback feedback and reinforcement to encourage encourage

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Speech and and language language therapy Speech self monitoring of speech is is not articuself of volume volume as as aa therapeutic therapeutic goal. goal. However, However, if speech lated well, increasing the volume may reduce the intelligibility. intelligibility. There is now an an impressive impressive range range of of amplifiers amplifierson on the the market market that that include include portable amplifiers work. able amplifiers and and static static more more powerful powerful amplifiers amplifiers for for use use atat home home or or for for work. Amplifiers are useful where the spouse has has aa hearing hearing loss. loss. Telephone Telephone Amplifiers are particularly useful amplifiers are also available. Adams and and Lang (1992) found found that ifif white masking presented at a Adams Lang (1992) masking noise was was presented decibels then then there there was was aa marked marked increase increase in in voice voice intenintensound pressure pressure level level of 90 decibels sity. Portable Portable voice voice activated activated maskers maskers such such as as the the Edinburgh Masker may help to sity. transfer this skill skill to daily conversation. The The Lightwriter Lightwriter allows allows aa simple message message to the use use of of aa voice synthesizer. Easy Keys be conveyed with the Keysisisaalaptop laptopcomputer computer with any a voice voice synthesizer, synthesizer, which which can can convey convey complex complex messages messageswithout without the need for any effort. vocal effort.

Nonverbal aspects aspects of communication in in PD PD Facial Facial expression in in PD PD Bradykinesia in conjunction conjunction with with rigidity rigidity produces produces the the typical typical mask-like mask-like facial facial

expression. Pentland (1988) found found that that reduced reduced facial facial expression expression made expression. Pentland etet al. al. (1988) patients with with PD PD appear appear to to have have no no strong strongfeelings feelings and and seem seem less likeable patients likeable to to observers, appearing unfriendly. Patients also appeared appeared to be more more anxious, anxious, ers, appearing bored bored and unfriendly. hostile, suspicious, suspicious, introverted introverted and passive passive than than the the age age matched matched subjects subjects without without PD. Overall patients were rated rated as as 'less 'less likeable', likeable'. Smiles Smiles were were regarded regarded as as 'false 'false PD. Overall smiles'. smiles'. Consequently, Consequently, the the message messagethat that aaPD PDpatient patient isis communicating communicating is is not not necessarily that essarily that which which was was intended. The improvement improvement of of facial facial expression expression is is encouraged encouraged through through facial facial exercises, exercises, relaxation and occasionally hydrotherapy to reduce the muscle relaxation occasionally hydrotherapy muscle rigidity. rigidity. Scott and Caird (1981) (1981) found found that proprioceptive proprioceptive neuromuscular neuromuscular facilitation facilitation (PNF) (PNF) techCaird niques were effective effective in improving impoverished impoverished facial facial expression. expression. The kinaesniques were in improving thetic awareness of patient's own facial facial expression expression is reduced, decreasing decreasing the thetic awareness of a patient's is reduced, the effectiveness of effectiveness ofself-monitoring self-monitoringskills. skills.Mirrors Mirrorsmaybe maybe used used to to aid aid self-monitoring. self-monitoring. monitor each each other other and and so soincrease increase awareawareGroup therapy encourages patients to monitor ness of facial facial expression, expression, relearning relearning a previously previously automatic skill.

Additional nonverbal features Altered posture may stooped position with the upper body bent forward may result in a stooped forward deviating from midline. Head Head control, control, eye ability to or deviating from the the midline. eye contact contact and and the the ability to use normal gesture may all be be affected affected in in aa way waythat that reduces reduces the the ability ability to to communicate. Communication variable, depending factors such such as fatigue, fatigue, the cate. Communication can be variable, depending on factors

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time within their drug drug cycle, cycle, or stress. The patient can can become aa passive passive communicator, losing losing the the desire desire and/or and/or ability to initiate a conversation, argue or debate; nicator, conversation, argue responding to to the the communication communication of of others others rather rather than taking a shared responding shared responsibility for speak 'for' the the sibility for conversation. conversation.The Thetemptation temptation for for the the carer carer is is then then to speak further increasing increasing the the patient's patient'srole roleas asaapassive passive communicator. communicator. patient, further

The role of carers carers in speech speech therapy found that carers' frustrations arose from problems caused Scott and Caird (1983) found reduction in in volume volume of ofthe thevoice, voice,difficulty difficulty maintaining maintaining aa conversation conversation by the reduction responses,distress distressatatthe thequality qualityof ofthe thevoice, voice, difficulty difficulty folfolbecause of monotonous monotonous responses, at their their own own inability inability to to lowing the disordered disordered rate of speech, and embarrassment at cope. cope. When When intervening intervening therapeutically therapeutically to to increase increase functional functional performance, performance, these aspects wider context context of of assessing assessing the patient's aspects must must be be taken taken into account in the wider communication they are active active participants communication needs. needs. Carers Carersneed need toto feel feel that that they participants in in programmes and and only only then then will the maximal potential benefit therapy programmes benefit from from therapy and be achieved. Carers also also need need to to have have aaclear clearunderstanding understanding of what therapy can and cannot achieve achieve and and feel feelconfident confidentininsupporting supporting the the patient patient through through the course of therapy.

Assessment and treatment of of dysphagia dysphagia in in PD PD Dysphagia results from tongue tongue tremor with reduced results from reduced initiation initiation of of lingual lingual movemove-

ment, repetitive repetitive tongue pumping action action and and lingual lingual festination. festination. Excessive Excessive tremor and dyskinesia dyskinesia may may disguise disguisethe the fact factthat that aabolus bolus isisimmobile. immobile.AAdry drymouth mouth due due to to drug also affect affect the drug therapy can also the ability ability to to form form a bolus. bolus. Delayed Delayed initiation initiation of of the the swallow and swallow progression swallow swallow rehearsals, rehearsals, when when the swallow swallow isis repeated repeated without without progression of the difficulty the bolus, bolus, are are common common findings. findings. Poor Poor postural postural control can increase increase the the difficulty in in successfully successfullytransporting transportingthe thebolus bolusposteriorly posteriorlyininthe themouth mouth in in order order to to initiate swallow reflex, reflex. As can be be aadelayed delayed pharyngeal pharyngealswallow, swallow, the swallow As a consequence, there can pharyngeal peristalsis, peristalsis, multiple multiple swallows swallows to clear the pharynx of of the bolus, bolus, reduced pharyngeal laryngeal elevation inadequate laryngeal elevation and/or closure, laryngeal laryngeal penetration, penetration, the presence of ineffective cough increase the of an ineffective cough and and pharyngeal pharyngeal pooling. These These impairments increase risk infrerisk of of aspiration aspiration and and aspiration aspiration pneumonia. Aspiration and choking are not infrequent in the late late stage PD. The therapist can can advise advise on safe safe consistency of diet and swallowing rehabilitation techniques. techniques. swallowing difficulties include includethe thewider wider range rangeof ofdifficulties difficultiesencountered encounteredrather rather than than Eating difficulties the swallowing process process alone. alone. Tremor Tremor and and symptoms described below can increase the time itit takes takes to eat eat and and can can cause cause embarrassment embarrassment when when eating eating in in public. public. This This

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Speech and and language language therapy Speech

results in reduced visits to restaurants or or for for social social meals leading to social isolation and overprotection by by the carer. carer. Dysphagia assessment would normally normally involve involvethe the therapist therapist carrying carrying out out a stanDysphagia assessment assessment would dardized formal or informal informal assessment, including possible referral for videofluoroscopy. Videofluoroscopy assessment findings videofluoroscopy. Videofluoroscopycan canconfirm confirm the the assessment findings and to the the dysphagia dysphagia management management of ofthe thepatient. patient.The Theeffects effects of ofswallowing swallowing contribute to rehabilitation assessed while undergoing rehabilitation techniques techniques can can also also be be assessed while the the patient patient is undergoing videofluoroscopy (e.g. different position when videofluoroscopy (e.g. the the patient patient moving moving their their head head to a different swallowing). The The formal formal assessment assessment may may include include oro-motor oro-motor assessment, assessment, assessment assessment of posture, and and respiratory respiratory status. status.This Thiswould would normally normallybe befollowed followed by by assessment assessment of posture, the oral and pharyngeal pharyngeal stages stages of the swallow swallow to to determine whether the patient isis different consistencies consistencies and at risk of aspiration using different and amounts amounts of of liquids liquids and/or solids. solids, Recommendations on the safe consistency consistency of of food food and and liquid liquid for for the patient Laryngeal indicators laryngeal eleare then made. Laryngeal indicators of of aspiration aspiration can include poor laryngeal eleabsence of voice, voice, 'wet' or 'gurgly' voice voice quality, vation, absence quality, the the absence absence of of aa voluntary voluntary and // or or coughing coughingas asaaresult resultof ofswallowing. swallowing. cough, a weak, weak, unprotective cough and Multidisciplinary management of dysphagia dysphagia

Physiotherapists and speech therapists have developed developed techniques to improve improve the the difficulty retainretainmobility of the tongue. Better mobility of the tongue will reduce difficulty ing the bolus in the midline and will improve the retrieval of food from the bucchal bucchal sulcii of the the bolus bolus to totrigger trigger the theswallow swallow reflex. reflex. Dieticians and sulcii and and transportation of speech closely together in managing dysphagia, dysphagia, with the diediespeech therapists must work closely tician providing an individually tailored tailored diet for for the the patient. patient. Speech Speechtherapists therapists must must also liaise also liaise with with occupational occupational therapists therapists to to establish establish the the most most appropriate appropriate posture for feeding and need for for any any feeding feeding aids (see (see Chapter 13). 13). Changes Changes in the the for feeding and the need timing of antiparkinsonian medication medication may may improve improve dysphagia dysphagia (Bushmann et al. 1989, 1995). 1989, Fonda Fonda and Schwarz 1995).

Conclusions therapist has has an an important role improve the communicaThe speech speech therapist role in helping to improve tion and swallowing swallowing problems of elderly patients with PD. PD. This role is constantly constantly evolving as effective communication developed, multidisciplinary multidisciplinary evolving as more more effective communication aids are developed, becomes available available and more research is underunderworking improves, new treatment becomes taken the clinical clinical effectiveness effectiveness of therapy therapy interventions. interventions. Multidisciplinary Multidisciplinary taken into the

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needed to to underpin underpin these developments. The perceptions and goals assessments are needed goals of patients patients and carers are increasingly increasingly recognized recognizedas asbeing beingimportant important in in directing directing of effective therapy. effective

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Speech and and language language therapy Speech Lieberman A, Singh Singh G, G, Fries FriesJF JFon onbehalf behalfof ofthe the Propath Propath Board (1994) Patient Montgomery EB, EB, Lieberman education and health promotion can randoniised conconeducation can be be effective effective in Parkinson's Parkinson's disease: disease: aa randomised trolledtrial. trial.American AmericanJournal Journal Medicine, 429-35. trolled of of Medicine, 97,97, 429—35. Montgomery EB, Lieberman Lieberman A, A,Singh Singh G, G, O'Reilly O'Reilly F, F, Fallwright 5, S, Davey Smith G, Ben-Obenour Ben-Obenour MontgomeryEB, WH, PM, Cohen The causes of abnormal WH, Stevens Stevens PM, Cohen AA AA (1972) (1972) The causes of abnormal pulmonary pulmonary function function in Parkinson's disease. 105, 3, 382—7. Parkinson's disease.American AmericanReview ReviewofofRespiratory RespiratoryDisease, Disease, 105, 3, 382-7. Mutch Mutch WJ, WJ, Strudwick Strudwick A, A, Roy Roy SK, SK, Downie AW AW (1986) Parkinson's Parkinson's disease: disease: disability, disability, review review and and management. British 293, 675—7. management. BritishMedical MedicalJournal, Journal, 293, 675-7. andand their Social Needs. London: Parkinson's Disease Parkinson'sDisease DiseasePatients Patients their Social Needs. London: Parkinson's Disease Oxtoby M M (1982) (1982) Parkinson's Society. Pentland B, Pitcairn WJR (1988) The effects of reduced reduced expression Pentland Pitcairn TK, Gray Gray JM, Riddle Riddle WJR (1988) The effects of expression in Parkinson's disease disease on on impression formed by Parkinson's by health health professionals. professionals. Clinical ClinicalRehabilitation, Rehabilitation,1,1, 307—13. 307-13. Clemie S, 5, Gray Gray JM, JM, Pentland Pentland B B (1990) (1990) Impressions Impressions of of parkinsonian parkinsonian patients from Pitcairn TK, Clemie from their recorded recordedvoices. voices.British BritishJournal Journal Disorders of Communication, 25,85—92. 1, 85-92. their of of Disorders of Communication, 25, 1, Powell JA, JA,Hale HaleMA, MA,Bayer BayerAJ AJ(1995) (1995)Symptoms Symptomsofofcommunication communication breakdown breakdown in in dementia: Powell of Disorders of Communication, 30, 65—75. EuropeanJournal Journal of Disorders of Communication, 30, 65-75. carers' perceptions. perceptions.European Raniig LO, LO,Countryman Countryman S, 5, Thompdon Thompdon LL, Horii YY (1995) (1995) Comparison Comparison of of two two forms forms of of intenintenRamig LL, Horii sive speech speech treatment treatment for sive for Parkinson's Parkinson's disease. disease. Journal Journalof ofSpeech Speechand andHearing HearingResearch, Research,38, 38,6,6, 1232—51. 1232-51. JA, Logemann Logemann JA, JA,Kirshner KirshnerHS HS(1986) (1986)Swallowing Swallowingand andspeech speechproduction productionin in Parkinson's Parkinson's Robbins JA, disease. disease. Annals AnnalsofofNeurology, Neurology,19,19,282—7 282-7. Thomson F (1984) Speech Robertson SJ, SJ, Thomson Speech therapy in Parkinson's Parkinson's disease: a study of the efficacy efficacy and long-term ofof Disorders of of Communication, long-term effects effects of of intensive intensive treatment. treatment.British BritishJournal Journal Disorders Communication, 19, 213—24 19,213-24 Caird FI Fl (1981) (1981) Speech Speech forpatients forpatients with Scott S,5, Caird with Parkinson's Parkinson'sdisease. disease. British BritishMedical MedicalJournal, Journal,283, 283, 1088. 1088. Scott 5, Scott S, Caird Caird Fl FI(1983) (1983)Speech Speechtherapy therapyfor forParkinson's Parkinson'sdisease. disease.Journal JournalofofNeurology, Neurology, Neurosurgery, and Psychiatry, 46, 46, 140—4. Neurosurgery, and Psychiatry, 140-4. Scott S, 5, Caird Caird FI Fl (1984) (1984) The The response response of of the apparent receptive speech disorder disorder of of Parkinson's Scott receptive speech Parkinson's disease to speech andand Psychiatry, 47, 302—4. speech therapy. therapy.Journal JournalofofNeurology, Neurology,Neurosurgery, Neurosurgery, Psychiatry, 47, 302-4. Scott S, 5, Caird Caird Fl, BO (1984) (1984) Evidence Evidencefor for an an apparent apparent sensory sensory speech speech disorder disorder in in Scott FI, Williams Williams BO JournalofofNeurology, Neurology, Neurosurgery, Psychiatry, 47, 840-3. Parkinson's disease.Journal Parkinson's disease. Neurosurgery, and and Psychiatry, 47, 840—3. Scott S, 5, Caird Fl, Scott FI, Williams BO (1985) Communication Communication in Parkinson's Parkinson's Disease. Disease.London: London:Croom Croom Helm. Shea BR, BR, Drummond Drummond SS, Metzer WS, WS, Krueger Krueger KM KM (1993) (1993)Effect Effectofofselegiline selegiine on on speech speech perforperforShea SS, Metzer 45,45, 1, 40—6. mance in in Parkinson's Parkinson'sdisease. disease.Folia FoliaPhoniatrica, Phoniatrica, 1, 40-6.

Index

Note: Note: page numbers in in itolics italicsrefer refer to tofigures figuresand andtables tables

acetazolamide acetazolamide 89 89 acetylcholinesterase inhibitors acetylcholinesterase inhibitors 42 Achilles tendon 206 Achilles tendon stretch stretch 206 activities of of daily dailylivfrig living (ADL) (ADL) activities assessment 218, 218, 220 220 scales 186 186 affect—arousal affect-arousal 173 173 age/aging 27 27 age/aging at diagnosis diagnosis of ofPD PD28—9 28-9 clinical heterogeneity heterogeneityofofPD PD2 7—30 27-30 essential tremor 82 essential tremor 82 neurodegenerative disease disease 111 111 prevalence of PD PD 112 prevalence of 112 risk of of PD PD114—15, 114-15, 118 118 speech and language problems 227, 228 language problems 227,228 agranulocytosis, agranulocytosis,fatal fatal65—6 65-6 akinesia 1,1,4,5,24,25,227 4, 5, 24, 25, 227 diagnosis 6 diagnostic criteria criteria 77 dopamine agonists 149 149 progressive progressive supranuclear palsy 99 swallowing mechanism 46 swallowing mechanism 46 tremor15, 15,85—6 85-6 alcohol, essential tremor alprazolam 89 alprazolam 7, 8, 9 Alzheimer's disease disease (AD) (AD) 7, age associated risk 111 111 concurrent concurrent 88 dementia with with Lewy Lewy bodies 41 41 diagnostic errors 88 extrapyramidal extrapyramidalsigns signs117—18 117-18 Lewy bodies bodies 23 23 senile plaques 23 23 amantadine145—6, 145-6, 157 157 amantadine essential tremor tremor treatment treatment89—90 89-90 72 amiodarone 72 amisulpride 66 66 amisulpride 65, 154 154 amitriptyline 65, B, DIP 73 amphoterocin B, DIP 73 235 amplifiers 235

240

anaesthesia 193 193 anismus 158, 158,192 192 ankle dorsiflexor dorsiflexor stretch stretch 207 207 reflex reflex 208 208 71 anti-emetics 71 anticholinergic drugs 146 146 anticholinergic dysfunction 10 bladder dysfunction constipation 10 10 gastric motility motility inhibition inhibition 158 158 hallucinations hallucinations 157 157 neuroleptic-induced parkinsonism treatment treatment 71 neuroleptic-induced parkinsonism 71 psychosis 42 42 urge incontinence 154, 154,155 155 38, 224 224 antidepressants 38, impotence impotence 156 156 anxiety anxiety 223—4 223-4 assessment assessment 200 200 dependency 168 168 disability determinant 173 disability determinant 173 apomorphine34, 34,45, 45,50—3, 50-3, 135 135 apomorphine administration 51, 52 51,52 challenge test 52, 52, 187 187 injection nodules 53 injection site nodules patient assessment assessment 187 187 patient patient selection selectioncriteria criteria51—2 51-2 surgical patients 194 194 selegiline inhibition 145 apoptosis, selegiline inhibition 145 articulation229—30 229-30 articulation impairment 227 227 230 articulatory contacts 230 226, 236 236 aspiration 226, laryngeal laryngeal indicators 237 237 perioperative perioperative 193 193 Assessment of of Motor Processing Processing and Skills Skills 220 Assessment assessment of 30,32 assessment of patients patients 30,32 assessment tools, 186 assessment tools, standardized standardized 186 168 assets 168 Association of Chartered Physiotherapists Physiotherapists Interested in Neurology (ACPIN) (ACPIN) 199 199

241 241

Index Association of Chartered Physiotherapists Physiotherapists with a

Special (ACSIEP) Special Interest Interest in Elderly People (ACSIEP) 199 199 atenolol 87 atenolol Attendance Allowance Allowance 190 190 attention attention deficit, deficit, selective selective 173 173 autonomic dysfunction, severe 10 10 autonomic failure failure 35 function 43 autonomic function 43 Babinski sign, sign, vascular vascular parkinsonism parkinsonism 13 13 47 balance 47 evaluation 208—9 evaluation 208-9 impaired 190 impaired 190 patient status assessment assessment 200 200 physiotherapy 208—9 physiotherapy 208-9 elderly 33 problems in elderly Balance Performance 209 Balance Performance Monitor 209 balance problems 34, 34, 49 49 Barthel Scale/Index 31, 186, 186,220 Scale/Index 31, 220 102,103, 103,104 104 basal ganglia 102, gene regulation regulation 34 dopamine mediated gene 221 bathing 221 benign essential tremor 83 essential tremor 83 benserazide 135 135 benzodiazepines88—9 88-9 benzodiazepines 71 benzquinamide 71 146 benztropine 146 208 Berg Scale 208 beta beta adrenergic adrenergicblockers blockers87—8 87-8 betahistine, betahistine, neuroleptic neurolepticsubstitute substitute70—1 70-1 72 bethanechol 72 13,101 Binswanger's disease 13, 101 43 blood pressure 43 201 Bobath approach 201 227 body language, impaired 227 191 body weight maintenance 191 toxin injection injection 90 90 botulinum toxin bowel symptoms symptoms45—6 45-6 bowel 235 bradykinesia 235 medication strategy strategy 149 medication 149 breathing clavicular patterns patterns 229 clavicular control 187 187 diaphragmatic 232 diaphragmatic 232 see also also respiration respiration bromocriptine137, 137,138—9 138-9 bromocriptine 38 bupropion 38 cabergoline 137, cabergoline 137, 140, 140, 154 calcium channel channel blockers blockers 72 calcium CAMCOG cognitive function function assessment assessment 31, CAMCOG cognitive 32 Performance Measures Measures Canadian Occupational Performance (COPM) 218 218

carbidopa 135, 135, 136 136 release 137 controlled release 137 carbonic carbonic anhydrase anhydrase inhibitors inhibitors 89 care quality 30 quality rehabilitation activity activity 166 rehabilitation carers asset value value 168 asset 168 communication for for patient patient 236 communication dependency 168 dependency 168 distress 39 distress ill health health 179 ill 179 needs 128—9 needs 128-9 palliative care care 131 palliative 131 physiotherapy implications implications 204 physiotherapy psychological support support 186 psychological 186 role in speech speech and and language language therapy therapy 236 role catechol-O-methyltransferase (COMT) catechol-O-methyltransferase (COMT) central inhibition inhibition142—3 142-3 inhibitors 142—4, inhibitors 142-4, 153 strategy 149 medication strategy 149 peripheral inhibition inhibition 142 peripheral 142 cephaloridine, cephaloridine, DIP DIP 73 cerebral cortex, Lewy Lewy bodies bodies 23 cerebral multi-infarct multi-infarct states states(CMIS) (CMIS)98, 98,100—1, 100-1, 104—5 104-5 cerebrospinal fluid fluid (CSF) (CSF) shunt shunt 14 cerebrospinal 14 chin, tremor tremor 85 chin, 85 chlorpromazine chlorpromazine 65 chromosome 4q21-22 116—17 116-17 chromosome 4q21—22 cinnarizine 72 cinnarizine 72 cisapride cisapride 46 gastric motility motility 158 gastric 158 clasp knife knife rigidity rigidity 5, clasp 5, 13 clinical clinical criteria, criteria, diagnostic diagnostic 7 clinical effectiveness 129 effectiveness evidence evidence 129 clinical 199 clinical guidelines guidelines 199 clinical clinical manifestations, manifestations, essential essentialtremor tremor 82 clinical trials trials 134—5 134-5 127, 130 130 clinics, specialist 126, 127, specialist 126, clonazepam clonazepam 89 clozapine 42, 42,65—6 65-6 hallucination control control 157 hallucination 157 tremor control control 148 tremor 148 co-beneldopa 135, 135, 137 137 co-careldopa 135 135 controlled release release (CR) (CR) 136—7, 136-7, 149 co-danthramer 46 co-danthramer 46 cogentin cogentin 146 146 cognitive cognitive function function assessment assessment 200 occupational therapists therapists 224 occupational 224 cognitive impairment impairment 34, cognitive 34,39, 39,134, 134, 199, 199, 224 cortical 41 cortical 41 dementia risk 40 depression 36—7 depression 36-7

242 242

Index impairment (cont.) cognitive impairment motor disability disability link 173 173 palliative care 49 palliative care subcortical 41 41 cognitive therapy 224 224 coguitive 3,15 15 cogwheel rigidity 3, lithium 72 72 colon, myenteric plexus 158 158 communication assessment 200, 231 200,231 disorders disorders226, 226,227—30 227-30 environmental adaptations 234 environmental adaptations 234 nonverbal nonverbal aspects aspects235—6 235-6 nursing care 187, 187,189 189 passive passive 236 sexuality sexuality192—3 192-3 variability variability235—6 235-6 communicative impairment 173 173 communicative comorbidity, speech and language language problems problems 227 227 strategies, physiotherapy 204 compensatory strategies, physiotherapy 204 179 complementary therapies 179 (CT) 16, 16,17 computed tomography (CT) 17 201,222 222 conductive education programmes 201, confusion, benzodiazepines 89 89 confusion, constipation10, 10,45—6, 45-6, 158 158 constipation nursing care 192 192 occupational therapy interventions 221 221 continence advisor 45 45 198 corollary discharges 198 cortical loops loops24—5 24-5 cortical corticobasal ganglionic ganglionic degeneration degeneration (CBDG) (CBDG) 8 dopaminergic drugs 10 10 motor sign sign asymmetry 11 11 neuroimaging neuroimaging 17 17 223 counselling 223 Creutzfeldt-Jakob Creutzfeldt—Jakobdisease disease 8 inclusion bodies bodies see seeLewy Lewybodies bodies cytoplasmic inclusion delayed auditory feedback 233 233 delirium 134 134 drug-induced 42 42 delusional ideas/delusions 36, 36,39, 39,42 42 dementia dementia39—42, 39^2, 173 173 causes 40 40 clinical characteristics characteristics8—9 8-9 early early 8—9 8-9 epidemiology epidemiology39—40 39-40 levodopa/PDI levodopa/PDI 149 149 with Lewy Lewy bodies bodies 3,3,8,8,9,9,40—1 40-1 management management 42 42 neuropsychological neuropsychological features features41—2 41-2 normal pressure pressure hydrocephalus 13, 13,14 14 nursing home admission 114 114 risk factors factors 40 40 demographic demographic change change122—3 122-3

dentures 191 191 speech 229 229 dependency t67—8 167-8 depeudeucy recognition recognition 176 176 144 deprenyl 144 depression35—9, 35-9,157—8, 157-8,223—4 223^ depressiou assessment assessment 200 200 associated features with with PD PD36—7 36-7 cognitive impairment impairment 36—7 36-7 dementia risk 40 40 dependency 168 168 detecting detecting 38 38 diagnostic criteria 35 diagnostic criteria 35 disability determinant 173 disability determinant 173 dopamine 37 37 DSM-III criteria 36 DSM-III criteria epidemiology epidemiology35—6 35-6 medial prefrontal prefrontal cortex cortex 38 psychotic features features 36 sleep disorders disorders 154 154 treatment treatment38—9 38-9 desmopressin 45, 45,155 155 diagnosis accuracy accuracy 6—7 6-7 atypical atypical features features7—12 7-12 communication communication177—8 177-8 milestones 177—9 milestones in inPD PD176, 176,177-9 progression progression to todisability disability177—9 177-9 23,111 111 diagnostic accuracy 23, diagnostic criteria criteria 30 predictive value value 7 232 diaphragmatic breathing 232 151,152 152 diary keeping 151, diazepam DIP 73 73 essential tremor treatment treatment 89 essential tremor dietician 221 221 diphasic dystonia/dyskinesia dystonia/dyskinesia (D-I-D) dystonia 153 153 disability early 179, 179, 180 180 impact of ofdailylife daily life 176 176 late stage 179, 179, 181 181 variability 173—4 variability 173-4 176 disability organizations 176 see seealso alsoParkinson's Parkinson'sDisease DiseaseSociety Society disadvantage 166, 166,167 167 116 disease aetiology aetiology theories theories 116 treatmentstrategy strategy149—50 149-50 disease progression, treatment treatment70—1 70-1 dizziness, treatment 33,135 135 domperidone 33, blood pressure pressure 44 levodopa absorption 136 levodopa absorption 136 nausea control control 136 136 neuroleptic neuroleptic substitute substitute70—1 70-1 surgical patients 194 194

243

Index donazepil 42 42 dopamine deficiency 1, deficiency 1, 23, 23, 198 198 depression 37 depression 37 loss 25 loss storage inhibitors inhibitors71—2 71-2 depletion 69 striatal depletion striatal levels with COMT COMT inhibitors inhibitors142—3 142-3 transport inhibitors transport inhibitors71—2 71-2 dopamine agonists dopamine agonists137—8, 137-8, 153 akinesia 149 akinesia 149 hypotension 44 orthostatic hypotension psychosis 42 psychosis effects 138 side effects see also also apomorphine apomorphine dopamine-mediated gene gene regulation, basal ganglia ganglia 34 dopaminergic drugs10—11 10-11 dopaminergic drugs adverse reactions reactions in PD 65 adverse 65 dorsiflexion, dorsiflexion, active active 207 dorsiflexor dorsiflexor stretch, stretch, reflex reflex 208 dosette 187 dosette 187 dreams, vivid vivid 47, 154 dressing 220-1 dressing 220—1 drinking 221 221 driving ability190, 190,222—3 222-3 driving ability 223 Driving Assessment Centres 223 drooling sialorrhoea droolkig see sialorrhoea drug therapy134—5 134-5 drug therapy cost/cost benefits benefits 129 cost/cost 129 medication strategies 1, 152, medication strategies148—5 148-51, 152,153—8 153-8 surgical patients patients 194 surgical 194 see also alsoindividual individualdrugs drugs drug-induced parkinsonism parkinsonism (DIP) (DIP)2,2,49, 49,64, 64,72—3, 72-3, 117 117 calcium channel blockers 72 calcium 72 dopamine storage/transport storage/transport inhibitors inhibitors71—2 71-2 gender 69 gender genetic influence influence 69 genetic defect 69 metabolic defect neuroleptic-induced 65—7 1 neuroleptic-induced 65-71 PD distinction distinction 68 PD subclinical PD PD 69—70 69-70 dry mouth 191 mouth 191 dyskinesia 153 dyskinesia 47, 153 peak dose dose 150—1 150-1 sleep disturbance disturbance 154 sleep 154 dysphagia 226, 226,236—7 236-7 see see also alsoswallowing swallowingdifficulties difficulties dysthymic dysthymic disorder disorder 35 dystonia athsmus 158 anismus 158 off period period 34 off physiotherapy 209—10 physiotherapy 209-10 wearing off 151, 151,153 153

Keys 235 Easy Keys eating 221 221 aids 237 aids 236—7 difficulties 191, 191,236-7 posture 237 posture 237 see also also nutrition nutrition Edinburgh Masker Masker 235 235 educational 126 educational programmes programmes 126 Eldepryl™ EldeprylTM144 144 elderly patients, assessment assessment of of PD PD30—2 30-2 electroconvulsive 33, 38 electroconvulsive therapy therapy (ECT) (ECT) 33, neuroleptic-induced parkinsonism parkinsonism treatment treatment neuroleptic-induced 71 71 emotionalism 35 emotionalism 35 end of dose deterioration 32—3 deterioration 32-3 motor fluctuations fluctuations34, 34,150—1 150-1 entacapone 142, 142, 144 144 environment 166 environment 166 modification 167 modification 167 safe 221 safe 221 environmental adaptations, adaptations, communication communication 234 environmental environmental risk factors 115 factors for for PD PD 115 epidemiology of parkinsonism parkinsonism 117—18 117-18 evaluation of studies studies 112 evaluation epidemiology of PD PD111—12 111-12 epidemiology of risk factors 114—17 114-17 equilibrium apraxia apraxia 106 equilibrium 106 equipment feeding aids aids 237 feeding provision 222 provision 222 erectile dysfunction dysfunction 155, erectile 155, 192 192 essential tremor (ET) 3,3,14—15 14-15 tremor (ET) age 82 age alcohol 15, 85—6 alcohol 15,85-6 beta beta adrenergic adrenergicblockers blockers87—8 87-8 clinical manifestations manifestations 82—5 82-5 clinical variants variants 84—5 84-5 differential diagnosis diagnosis 84 differential disability 83—4 disability 83-4 epidemiology 80 epidemiology factors influencing influencing 82—3 82-3 genetics 80—1 genetics 80-1 prevalence 80, prevalence 80, 81 81 treatment 85—91 treatment 85-91 progression 82 tremor progression etatcrible 13 etat crible 13 life sustaining sustaining measures measures 182 ethics, life 182 exercise 189 exercise early PD PD 202—3 202-3 mortality 212 mortality 212 physiotherapy 201 physiotherapy 201 stretching 206 stretching exercise 178 exerciseprogramme programme 178 1—2 speech and and language languagetherapy therapy23231-2

244

Index exercise tolerance 210 exercise tolerance 210 assessment assessment 200 200 108 external cues 108 106 externally cued cned movements movements 106 72 extrapyramidal reactions, piperazine derivatives 72 extrapyramidalsigns signs117—18 117-18 extrapyramidal neuroleptic-induced extrapyramidal syndromes, neuroleptic-induced 70 17 eye of of the tiger sign 17 facial facial expression 235 235 loss 227 227 faecal impaction 46 faecal impaction 46 9-10, 34, 47, 190 190 falls 9—10, benzodiazepines benzodiazepines 89 late stage PD 179 179 management management 209 209 multisystem degeneration parkinsonism parkinsonism 49 multisystem degeneration 49 orthostatic hypotension hypotension 43 43 physiotherapy physiotherapy 208 208 prevention 172 172 familial parkinsonism, autosomal autosomal dominant dominant 111 familial parkinsonism, 111 116 family history, history, risk risk factor factor 116 features, atypical atypical7—12 7-12 features, nutrition feeding see eating; meals; nutrition 123 financing of health care 123 Flewitt-Handford approach 201 Flewitt—Handford approach 201 44, 156 156 fludrocortisone 44, 155,156 156 fluid intake 155, 72 flunarizine 72 73 5-fluorouracil, DIP 73 38 fluoxetine 38

DIP 73 73 flurbiprofen flurbiprofen 44 44 focal infarction, single 13 13 focal 189,208 foot care 189, 208 179 fractures, late stage PD 179 free radical theory of cell death in PD 116 116 free freezing 9,9,153—4 153-4 freezing episodes 6, 6, 47 47 fields 102 102 frontal fields functional disability, disability, symptomatic symptomatictherapy therapy148—9 148-9 Functional Independence Measure Measure 220 220 Profile 200 200 Functional Limitations Profile systems 129 129 funding systems gait gait 6,6,9—10 9-10

deterioration deterioration 47 47 disturbance disturbance206—7 206-7 freezing freezing 9 problems 33, 33, 34 34 re-education re-education207—8 207-8 senile 15—16 15-16 14, 98 98 gait apraxia 3,3,14, syndrome 98—100 syndrome 98-100

gait disorders classification classification 101—2 101-2 normal normal pressure pressure hydrocephalus hydrocephalus13—14 13-14 severe isolated isolated 99 treatment treatment 107 107 disease 50 vascular disease vascular parkinsonism parkinsonism100—1 100-1 see see also alsovascular vascularhigher-level higher-levelgait gaitdisorders disorders ignition102—4 102-4 gait ignition impairment 105 105 gastric motility 158 gastric motility 158 reflex loss 46 gastrocolic reflex 13 gegenhalten 5,5,13 123 general practitioner (GP) 123 81 genetic anticipation, essential tremor 81 risk of of PD PD116—17, 116-17,118 genetic risk 118 32, 35 Geriatric Depression Scale (GDS-15) (GDS-15) 32, 208 'Get up and Go' test 208 24,25, 25, 26 26 globus pallidus 24, eye of of the tiger sign 17 17 210 glottal area tremor tremor 210 glycopyrrolate glycopyrrolate 155 155 201 goal setting, setting, joint joint 201 grooming220—1 220-1 grooming 204 group programmes 204 222,223 223 group therapy 222, facial facial expression awareness 235 235 prosody prosody 232, 232,233—4 233-4 Hallervorden—Spatz disease 12, 17 Hallervorden-Spatz disease 12,17

hallucinations 9,9,36, 36, 39, 39, 47 drug-induced drug-induced 157 157 levodopa/carbidopa levodopa/carbidopa CR 137 137 nursing home admission 114, 134 114,134 42 hallucinosis 42 impaired vision/hearing 190 190 levodopa-induced levodopa-induced 34 65 haloperidol 65 essential tremor 82, 83 hand, essential tremor 82, 189 handwriting 189 skills 223 223 see see also alsowriting writingtremor tremor head control 235 235 essential essential tremor 82, 82, 84, 84, 87 87 injury risk factor factor 116 116 tremor 11 11 health promotion 172 172 Health Status Questionnaire Questionnaire 12 (HSQ-12) (HSQ-12) 32 123 healthcare delivery 123 122,171 171 healthcare needs 122, professionals healthcare professionals patient relationships relationships 177 177 roles roles 174—5 174-5 hearing 190 190

245

Index heel strike 207 heel strike 207

hemiballism hemiballism 26 hemiparkinsonism 37 37 hemiparkinsonism 11 hemiparkinsonism hemiatrophy 11 disorders lOt—2, 101-2, 108 108 higher-level gait disorders see oho vascnlar see also vascular higher-level higher-level gait gait disorders disorders HLA B44 69 HLAB44 hospital hospital admission, admission,nnrsing nursingcare care193—4 193-4 disease 12 12 Huntington's disease patient needs 171 171 hydrocephalus, normal pressnre pressure8,8,13—14 13-14 hydrocephalns, normal levodopa 10—11 levodopa 10-11 nenroimaging 17 neuroimaging 17 205 hydrotherapy 205 hyperkinesia, tetrahenazine therapy therapy 71 hyperkinesia, tetrabenazine 71 hyperreflexia, vascular vascnlarparkinsonism parkinsonism 13 hyperreflexia, hypnotic drngs drugs47—8, 47-8, 154 154 hypnotic hypotension, orthostatic orthostatic43—4 43-4 hypotension, hyrocephalus, normal pressure hyrocephalns, pressnre 9 ignition ignition apraxia apraxia105—6, 105-6, 108 108 immobility 47 47 155,192 192 impotence 155, 111,113 113 incidence of PD 111, seeLewy Lewy bodies bodies inclusion bodics bodiessee inclnsion incontinence see urinary urinary incontinence independence medical management management quality qnality 172 medical 172 PILS scheme 167—8, 167-8, 170 information information access to to 176 access 176 availability 176—7 availability 176-7 inaccnrate 177 inaccurate 177 leaflets t76—7, leaflets 176-7, 179, 179, 189, 189, 203 misconstrned 177 misconstrued 177 nnrse provision provision 189 nurse 189 provision to to patients patients 172 provision 172 institutionalcare care113—14, 113-14,134 institntional 134 interventions effectiveness 169—70 169-70 mnltidisciplinary 169 multidisciplinary 169 palliative 166 palliative 166 participation 169 participation 169 physiotherapy 198 physiotherapy 198 relevance 165 relevance 165 intnbation intubation 193 193

kinaesthetic kinaesthetic information information198—9 198-9

lactnlose 46 lactulose language larignage impairments 226—7 impairments 226-7 see also olso speech and hmgnage language problems; problems; speech speech and larignage language therapy laryngeal muscle mnscle relaxation relaxation 233 laryngeal 233

laxatives 46, laxatives 46, 192 192 lead-pipe rigidity 5, 5, 13 13 leaflets, information information 176—7, leaflets, 176-7, 179, 179, 189, 189, 203 203 leg cramps cramps 154 leg 154 levodopa 10—11, levodopa 10-11,135—7 135-7 bioavailability bioavailability135—6 135-6 controlled release controlled release 135, 135,136—7, 136-7, 149, 153 dementia management management 42 dementia level 33, 136 dosage level formnlations 33, formulations 33, 34 long-term therapy therapy 134 long-term 134 metabolism 135 metabolism 135 mnltisystem degeneration degeneration parkinsonism parkinsonism 49 multisystem orthostatic hypotension hypotension 44 orthostatic decarboxylase inhibitor inhibitor combination peripheral decarboxylase 135—6, 135-6, 149, 153 153 effects 33, side effects 33, 136 136 snstained release sustained release 154 154 care 49 terminal care L-tryptophan addition addition 37 l-tryptophan vascnlar parkinsonism 13 vascular parkinsonism 13 Lewy bodies bodies 1,1,3,23 3, 23 Lewy Alzheimer's disease disease 23 23 bralnstem brainstem 41 41 cortical 41 cortical 41 dementia 40—1 dementia 40-1 motor nenrone neurone disease disease 23 myenteric plexus plexns of of colon colon 158 myenteric see also olso dementia, dementia,with withLewy Lewybodies bodies life 115, 116 116 life event event risk risk factors factors for for PD 115, life sustaining snstaining measures, measnres, ethics ethics 182 life 182 lifestyle PILS scheme scheme 167, PILS 167, 168, 168, 169, 169, 170 170 riskfactorsforPD risk factors for PD 115, 115,116 116 Lightwriter 235 Lightwriter 235 limb oedema 211 211 lithium 72-3 lithinm 72—3 long-term care113—14 113-14 long-term care Machado—Josephdisease disease 12 Machado-Joseph MadoparTM135 135 Madopar™ resonance imaging imaging (MRI) (MRI) 16, magnetic resonance 16, 17 voice activated activated 235 masker, voice meals 221 221 prefrontal cortex, cortex, depression depression 38 medial prefrontal 38 medication nnrsing care care 187 nursing 187 strategies 148—51, strategies 148-51, 152, 152, 153—8 153-8 DIP 73 meperidine, DIP 73 metclopramide 136 metclopramide 136 methazolamide methazolamide 89 see 11-methyl-4-phenyl-methyl-4-phenyl- 1,2,3,6-tetrahydropyridine 1,2,3,6-tetrahydropyridine see MPTP alpha-methyldopa 71—2 alpha-methyldopa 71-2 metoclopramide 67 metoclopramide

246

Index metoprolol 87

micturition urgency 221 221 see also nocturia; nocturia; urinary urinaryincontinence incontinence midodrine156—7 156-7 midodrine mineralocorticoids 156 156 mineralocorticoids State Examination (MMSE) (MMSE) 31, 31,32, 32, Mini-Mental State 200 misdiagnosisofPD 117, 118 117,118 mixed gait gait apraxia apraxia106—7 106-7 mobility189—90, 189-90,222 mobility 222 deterioration deterioration 190 190 fluctuation fluctuation 190 190 medical management quality 172 172 nursing nursing care care189—90 189-90 visual visual cues cues207—8 207-8 monoamine monoamineoxidase-B oxidase-Binhibitors inhibitors144—5, 144-5,146—7 146-7 mood disorders 200 200 disturbances 37, 37,173 173 mortality exercise 212 212 from PD 111, fromPD 111,113 113 blocks 6 motor blocks 37 motor disability 37 cognitive impairment link link 173 cognitive impairment 173 rehabilitation 33 33 motor motor disorder, disorder, pathophysiology pathophysiology198—9 198-9 motor fluctuations fluctuations33—4 33-4 motor medication 1, 152, 149-51, 152,153—4 153-4 medicationstrategy strategy149—5 motor neurone neurone disease disease age associated risk 111 111 Lewy bodies bodies 23 23 patient needs 171 171 failure 104 104 motor programming failure signs motor signs asymmetry 11 11 drug resistant 33 33 motor syndromes syndromes32—5 32-5 motor motor motor tricks tricks153—4 153-4 movement internal sense 198 198 patient status assessment assessment 200 practice practice 207 207 rotational rotational components components205—6 205-6 movement disorders clinics 126, 126, 127 social isolation 203 203 MPTP MPTP 146—7 146-7 disease 13 13 multi-infarct disease 123,126 126 multidisciplinary team 123, goals 179 179 physiotherapist liaison 204 204 171 multiple pathologies 171 multiple multiple sclerosis, sclerosis, patient patientneeds needs171—2 171-2

multiple system system atrophy (MSA) (MSA) 7,7,9, 9, 10 10 autonomic function function 43 43 epidemiology 117 117 levodopa response 10 10 parkinsonism 49, 49, 50 50 rehabilitiation 180, 180, 182 182 striato nigral variant 50 50 diseases, epidemiology multisystem degenerative diseases, 117 117 multisystem neurodegenerative disease, disease, parkinsonism parkinsonism2—3 2-3 muscle changes in immobilized immobilized 206 206 flexor 206 206 strengthening 207 207 stretch 206 206 tone 200 200

nadolol 87 87 natural history of PD 111 111 nausea treatment treatment70—1 70-1 nausea needs, perceived perceived 176 176 41 neurofibrillary tangles 41 neuroimaging16—18 16-18 neuroimaging agents neuroleptic agents atypical atypical 66 DIP DIP 65—7 65-7 neuroleptic-induced neuroleptic-inducedparkinsonism parkinsonism64—5, 64-5,65—71 65-71 agents agents 65—7 65-7 clinical features features 67—8 67-8 duration 68 68 incidence incidence 69 pathogenesis pathogenesis 69—70 69-70 susceptibility susceptibility 69 69 treatment treatment70—1 70-1 neuropathology coexisting with PD PD 23 coexisting with 23 PD PD 22—3 22-3 neurophysiological neurophysiologicaltests tests16—18 16-18 neurophysiology neurophysiologyofofPD PD24—6 24-6 neurosurgery34—5 34-5 neurosurgery cost of procedures 129 129 see also also surgery surgery neutropenia, neuroleptic-induced neuroleptic-induced 66 66 154 nightmares 154 nigrostriatal tract 23, 23,25, 25,26 26 72 nimodipine 72 154,155 155 nocturia 154, nurse, specialist specialist PD PD126, 126,127, 127, 174-5,195 195 nurse, 174—5, inpatient management guidelines 194 194 nurses community 175 175 psychological support 186 psychological support 186 nursing assessment assessment 186—7 186-7

241 247

Index plim 188 plan service purchasing 195 195 team 185 team 185 nursing nursingcare care185—6 185-6 communication 187, 187, 189 189 components 187, components 187,188, 188,189—95 189-95 continuity 185 185 elimination 19191-2 1—2 elimination hospital admission admission 193—4 193-4 information provision provision 189 information 189 medication 187 medication 187 mobility 189—90 mobility 189-90 nutrition 191 191 palliative care care 195 palliative 195 planning 185—6 planning 185-6 sexuality 192—3 sexuality 192-3 residents 114, 134 nursing home residents 114,134 nutrition nursing care 191 191 problems in late stage PD 179 179 see see also alsoeating; eating;meals meals

occupational therapy therapy 126, occupational 126, 127,217 127, 217 aims 217,218 aims assessment 218, 220 assessment 218,220 dysphagia 237 dysphagia for effectiveness effectiveness 224 evidence for reference 218, 219 frame of reference 218,219 interventions 220—4 interventions 220^ practice 218, 219 model of practice 218,219 off period off disability 35 disability dystonia 34 dystonia olanzapine 42, olanzapine 42, 66 on-off on—offmood mood swings swings 37 ondansetron 71 ondansetron 71 oral hygiene 191 hygiene 191 Orem's Model Model of of nursing nursing care care 187 187 oro-motor assessment assessment 237 237 orthostatic hypotension 211 211 oxybutynin oxybutynin 45 palilalia 230 230 palliative palliativecare care48—9, 48-9,130—1, 130-1, 166 nursing care 195 195 physiotherapy 204 physiotherapy parkinsonism parkinsonism 11 autosomal dominant dominant 12 autosomal 12 diagnosis 4, 5 diagnosis drug-induced 12, drug-induced 12, 38 lower body body 9, lower 9, 13 13 multisystem neurodegenerative neurodegenerativedisease disease2—3 2-3 post-encephalitic 12 post-encephalitic 12 pyramidal tract signs 11 11 rest tremor tremor 6 rest

unilateral acute acute onset onset 11 unilateral 11 vascular 2, 2, 13 13 levodopa 10-11 levodopa 10—11 see also drug-induced drug-induced parkinsonism parkinsonism(DIP) (DIP) Parkinson's disease (PD) (PD)1—2 1-2 Parkinson's disease age at diagnosis diagnosis 28—9 28-9 basal ganglia ganglia disordered disordered cueing cueing 104 104 clinical expression expression 28 clinical clinical signs signs 4—7 4-7 coexisting neuropathology neuropathology 23 coexisting 23 disease 8 concurrent Alzheimer's Alzheimer's disease conditions mimicking conditions mimicking12—16 12-16 with dementia 40 40 DIP distinction distinction 68 DIP heterogeneity 29 heterogeneity late onset onset 2,2,29—30, 29-30, 33 stage 2, 28 late stage neuropathology 22—3 neuropathology 22-3 neurophysiology 24—6 neurophysiology 24-6 progressing 29 rapidly progressing subclinical 69—70 subclinical 69-70 subtypes 28—9 subtypes 28-9 tremor dominant dominant 7 tremor Parkinson's Disease Society 176, 176, 193, 193,212 212 physiotherapy assessment assessment 200 physiotherapy scale 200 scale Speech and and Language Language Therapy Therapy checklist checklist 231 Speech paroxetine 38 paroxetine DIP 73 DIP 73 participation 169 participation 169 of PD 111 pathology of 111 patients care professionals professionals relationships relationships 177 health care psychological support support 186 psychological 186 PDQ-39 32, 32, 200 200 PDQL 32, 32, 200 percutaneous endoscopic endoscopic gastrostomy gastrostomy (PEG) (PEG) feeding 182 feeding 182 pergolide 45, pergolide 45,137, 137,139—40 139-40 peripheral decarboxylase decarboxylase inhibitor inhibitor (PDI) (PDI)135—6 135-6 permissive 116 permissive exposure, exposure, latent latent period period 116 perphenazine perphenazine 65 pesticide 116 pesticide use, use, risk risk factor factor 116 pharynx, dysphagia 236 dysphagia 236 phenelzine 73 phenelzine 73 phenobarbital phenobarbital 89 phenothiazines phenothiazines 66 fluorinated 65 fluorinated phonation 229 phonation 229 phonemes, elision elision 230 physical with psychological psychological physical deficit deficit combination combination with deficit 173 deficit 173 physical physical therapy therapy 126 physiotherapy 34, 34,126, 126,127, 127,166, 166,178 178 assessment 199—201 assessment 199-201

248

Index physiotherapy (cont.) (cont.) balance balance 208—9 208-9 breathing control control 187 187 chnical gnidehnes 199 clinical guidelines 199 compensatory compensatorystrategies strategies204, 204,207—8 207-8 disease progression progression203—4 203-4 domiciliary domiciliary204—5 204-5 dystonia dystonia 209—10 209-10 earlyPD early PD202—3 202-3 environment environment204—5 204-5 evidence evidence base base211—12 211-12 exercise tolerance 210 exercise tolerance falls falls 208 208 gait disturbance disturbance206—7 206-7 hospital 205 205 intervention 198 198 late stage PD 204 stage PD management cycle 202 movement component componenttreatment treatment205—11 205-11 206 range 206 rotational 205—6 205-6 rotational muscle shortening 206 206 muscle strengthening strengthening 207 207 occupational therapy interventions 222 222 palliative care 204 palliative care 204 respiratory respiratoryfunction function2 10—11 210-11 review 202 202 tongue mobility 237 237 treatment treatment approaches approaches200—4 200^ walking aids 209 209 PILS scheme scheme 167—8 167-8 evidence for intervention interventioneffectiveness effectiveness 169—70 169-70 87 pindolol 87 piperazine derivatives 72 72 neuroleptic-induced parkinsonism 65 neuroleptic-induced parkinsonism pneumonia, aspiration aspiration 226, 226,236 236 189, 208 208 podiatry 189, positron positron emission emissiontomography tomography(PET) (PET)17—18 17-18 postural postural hypotension hypotension156—7 156-7 multiple system system atrophy 182 182 9-10 postural instability instability 6,6,9—10 postural reflexes reflexes postural absent 6 impaired 47 47 posture advice 206 206 altered 235 235 assessment assessment 237 237 feeding feeding 237 237 patient status assessment assessment 200 pramipexole pramipexole137, 137,140—1 140-1 41 pre-dementia 41 premotor premotorarea area(PMA) (PMA)102, 102,103—4 103^ pressure sores 190 190 late stage PD 179 179

prevalence of PD 112—13 PD 111, 111,112-13

prevention, PILS PILS scheme 167, 167, 170 170 primary health care teams teams 195 195 primary motor cortex (Ml) (M1) 102, 102,103, 103, 104 104 primidone, essential tremor treatment treatment 88 essential tremor private sector t76 private sector 176 procaine 73 73 prochlorperazine prochlorperazine 136 136 progressionofdisease progression of disease171—2 171-2 progressive neurological 1—2 neurologicaldisorders, disorders,needs needs17171-2 progressive progressive supranuclear palsy (PSP) 7, 7, 8, 8, 9 epidemiology 117 117 levodopa response response 10 neuroimaging neuro imaging 17 17 parkinsonism parkinsonism 49—50 49-50 rehabilitiation rehabilitiation 180 180 231-2 PROPATH programme programme 231—2 propiverin 155 155 71 propranolol 71 essential tremor treatment 87, essential tremor 87, 88 88 facilitation 235 235 proprioceptive neuromuscular facilitation 173 prosodic cue perception perception 173 prosodic therapy therapy232, 232,233—4 233-4 prosodic 230 prosody 230 173 psychiatric disorders 173 psychiatric disturbance, 139 psychiatric disturbance, bromocriptine bromocriptine 139 psychological deficit psychological deficit combination combination with physical deficit deficit 173 173 psychosis 42—3 42-3 psychosis drug-induced drug-induced 42 42 putamen, cortical input 25 25 72 pyridostigmine 72 71 pyridoxine 71

quality of care 30 30 of life life quality of essential tremor 83 essential tremor 83 health related measures 32, 32, 130 130 quetiapine 66 66 Reed and Sanderson Sanderson model 218, 219 218,219 referral, progressive progressive paradigm paradigm 223 165 rehabilitation 165 apomorphine 52 52 assets 168 168 complexity complexity168—9 168-9 concepts concepts165—6 165-6 degenerative degenerative parkinsonian syndromes 180, 180, 182 182 disadvantage 166, 166,167 167 effectiveness 169—70 169-70 goals 166 166 implementation implementation166—9 166-9 intensive inpatient 176 intensive inpatient 176 motor disability disability 33 33 multisystem degeneration parkinsonism parkinsonism 50 multisystem degeneration 50 needs specific specific to PD/parkinsonism PD/parkinsonism 172—4 172-4

249

Index nursing assessment assessment 186—7 186-7 resources 174—6 resources 174-6 service matching to to needs needs 170—6 170-6 speech and language therapy therapy exercise exercise programme23231-2 programme 1—2 strategies 176—9, strategies 176-9,180—1 180-1 structured process 169 169 swallowing 236 swallowing virtual virtual reality realityimages images104—5 104-5 writing 223 223 relaxation therapy 223 223 total body 232 232 REM behaviour behaviour disorder disorder 48 reserpine 71 reserpine 71 resources, rehabilitation rehabilitation174—6 174-6 respiration associated impairments impairments 229 associated reduced control control 227 reduced 227 related symptoms and speech related speech and language language therapy 232 232 respiratory function function assessment 200 assessment physiotherapy 210—11 physiotherapy 210-11 respiratory muscle muscle training training211—12 211-12 respiratory status status assessment assessment 237 237 respiratory speech 211 211 respiratory support, speech rest tremor tremor 77 absence 11—12 absence 11-12 vascular parkinsonism parkinsonism 13 13 rigidity rigidity 1,4—5, 1, 4-5, 24 24 diagnostic criteria criteria 7 diagnostic essential tremor tremor 15 essential 15 facial expression expression 235 facial 235 lithium 72 lithium 72 medication strategy strategy 149 medication 149 swallowing mechanism mechanism 46 swallowing vascular parkinsonism parkinsonism 13 13 see see also alsogegenhalten gegenhalten risk factors for for PD PD 114—17 114-17 66 risperidone 66 137,141 141 ropinirole 137,

seborrhoea 155 seborrhoea 155 secondary care 126 care 126 selective selectiveattention attention 173 173 selective serotonin serotonin reuptake reuptake inhibitors (SSRIs) selective (SSRIs) 38, 39, 157, 158 DIP 73 73 impotence 156 impotence 156 syndrome 145 serotonin syndrome 145 selegiline 38, 38,144—5, 144-5, 148, 154, 157 neuroprotection 146—7 neuroprotection 146-7 hypotension 44 orthostatic hypotension psychosis 42 psychosis speech and and language language problems problems 227 speech

self-efficacy, subjective sense self-efficacy, sense 172, 172,178—9 178-9 self-funding 129 self-funding treatment treatment 129 senile plaques 41 41 Alzheimer's disease disease 23 23 serotonin 37, 38 precursors 37 syndrome 145 syndrome 145 sertindole 66 sertindole 66 sertraline 38, 38, 158 158 service organization/provision 123,124—5, 124—5, 126 126 organization/provision 123, economic appraisal appraisal 129 economic 129 qualityassessment quality assessment129—30 129-30 utilization 128 utilization services access to to 176 access 176 diagnosis specific diagnosis specific 170—4 170-4 matching to to needs needs170—6 170-6 requirements in in progressive progressive neurological disorders 172 disorders 172 sexual desire 192 desire 192 sexual dysfunction 48, 48,155—6 155-6 sexuality, nursing nursing care care192—3 192-3 Short-Form 36 (SF-36) (SF-36) 32, 32, 130 sialorrhoea 42, 42,46, 46,146, 146,155, 155,191, 191, 226 226 Sinemet™ SinemetTM135 135 sitting exercises exercises 189 189 skin care 191 191 sleep sleep abnormalities 154 abnormalities 154 disturbance/fragmentation 47—8 disturbance/fragmentation 47-8 smoking 116 116 social 203, 223 social isolation isolation 203, social 122,171 social needs needs 122, 171 social 126, 128 128 social networks networks 126, social 167, 168, 168, 170 social resources, resources, PILS PILS scheme scheme 167, social 36,126, 126, 128 128 social support support 36, soft 200 soft tissue tissue length/extensibility length/extensibility 200 sotalol 87 sotalol 87 Meter 234 Sound Level Level Meter spasticity spasticity 5 speech abnormal rate treatment treatment232—3 232-3 articulation 229—30 articulation 229-30 coordfriation 229 coordination difficulties 46 difficulties progressive supranuclear supranuclear palsy palsy 50 50 disorders in progressive impairments 226—7 impairments 226-7 intonation improvement improvement 233 233 rate 230 rate 230 respiratory support support 211 respiratory 211 self monitoring monitoring 233 self 233 visual feedback feedback 233 233 volume volume234—5 234-5 loss 227, 227, 229 229 speech speech and language language problems age 227, age 227, 228

250

Index speech and language problems (conL) (cont.) comorbidity 227 227 speech and language language therapist 187 187 speech and language therapy 126, 126, 127, 127,210, 210,226 226 carer role 236 236 exercise programme 23 1—2 exercise programme 231-2 intensive 232 respiratory related symptom symptom treatment 232 232 treatment treatment231—4 231-4 Speechviewer (IBM) (IBM)234—5 234-5 spino 81 spino bulbar bulbar muscular atrophy, X-linked X-linked 81 standing exercises exercises 189 189 state 190 state disability allowances allowances 190 state 129 state funding funding 129 stress 36,223 stress 36, 223 stress 192 stress incontinence incontinence 192 substantia sub stantia nigra nigra cell loss 22 22 gliosis 22 22 nigrostriatal tract tract 23 23 pars compacta 22, 22,23, 23,25 25 cell degeneration degeneration 11 pars reticulata 22, 25 22, 24, 24,25 subthalamic nucleus nucleus 25—6, 25-6, 26 supplementary motor area area (SMA) (SMA) 102, 102, 103 103 infarction infarction 104 104 supraglottal supraglottal area area tremor tremor 210 surgery 193 193 drug treatment 194 194 see also also neurosurgery neurosurgery 228, 236—7 swallowing difficulties difficulties 226—7, 226-7,228, 236-7 disease progression 46 disease progression dopaminergic therapy 155 155 drooling 155 155 impairment in in progressive progressive supranuclear palsy 50 50 late stage PD 179 179 multiple system system atrophy 182 182 rehabilitation techniques 236 236 syllables,elision elision 230 syllables, symptom 151,152 152 symptom diary 151, syncope 43, 44 syncope 43, 117 alpha-synuclein 117

tablet containers 187 187 tacrine 42 42 tardive dyskinesia 70, 70,71 71 teamwork174—6 174-6 teamwork telephone amplifiers amplifiers 235 235 terminalillness illness130—1 130-1 terminal also palliative see also palliativecare care tetrabenazine 71 71 thalamic inhibition inhibition24—5 24-5 thalamic thalamic stimulation essential tremor treatment 91 essential tremor 91 refractory tremor 148 refractory tremor 148

thalamotomy essential tremor treatment treatment 90 essential tremor refractory tremor 148 refractory tremor 148 thioridazine 65 thioxanthenes 66 87 timolol 87 147 tocopherol 147 221 toileting 221 tolcapone 34, 142, 143 143 155 tolterodine 155 tongue essential tremor 87 essential tremor 87 mobility 237 tremor 85, 85, 236 torsion dystonia, dystonia, autosomal autosomal dominant dominant idiopathic 81 81 training provision provision to to patients patients 172 172 (TENS) 211 211 transcutaneous nerve stimulation (TENS) treatment individualized individualized plan 201 201 nursing care regimes 187 187 problem problem solving solving approach approach200—1 200-1 24 tremor 1,1,24 diagnostic criteria 7 diagnostic criteria drug treatment 148 148 essential essential 7,7,11—12 11-12 glottal area 210 210 head 11 11 lithium 72 72 medically undiagnosed 117 medically undiagnosed 117 medication strategy strategy 148, 148, 149 149 pill-rolling pill-rolling 6 postural 72 72 rest rest 5—6 5-6 supraglottal area 210 210 task-specific task-specific 84, 85 tongue 236 236 writing 84 writing 84 see also essential tremor tremor (ET); (ET);rest rest tremor tremor tricyclic tricyclic antidepressants 38, 38,154, 154, 157 157 146 trihexylphenidyl 146 trunk mobility mobility exercises exercises 210 trunk 207 trunk mobilization techniques techniques 207 l-tryptophan 37 37 L-tryptophan Unified Parkinson's Parkinson's Disease Disease Rating RatingScale Scale (UPDRS) (UPDRS) 30—1, 30-1, 186 score score 211 211 urethral sphincter electromyography electromyography 10 urge incontinence incontinence154—5, 154-5,191-2 urge 191—2 symptoms 45 urinary bladder symptoms urinary incontinence incontinence10, 10,154—5 154-5 urinary multiple system atrophy 180 system atrophy 180 normal pressure pressure hydrocephalus 13, 13,14 14 nursing 1—2 nursingcare care19191-2 studies 10 10 urodynamic studies

251 251

Index vaginismus 192

vascnlar vascular higher-level higher-level gait gait disorders disorders98, 98,102, 102,104—5 104-5 classification 105—7, 105-7, 108 108 disequilibrium 105 105 equilibrium apraxia 106 106 ignition ignitionapraxia apraxia105—6 105-6 mixed mixed gait gait apraxia apraxia106—7 106-7 physiotherapy 107 107 treatment treatment107—8 107-8 vascular parkinsonism parkinsonism100—1 100-1 193 ventilation during surgery 193 videofluoroscopy 237 237 videofluoroscopy 189, 203 203 videotapes 189, vincristine/adriamycin vincristine/adriamycin combination, DIP 73 73 virtual reality reality images, images, rehabilitation rehabilitation104—5 104-5 virtual 190 vision 190 visual cues, cues, mobility mobility207—8 207-8 visual feedback, feedback, speech speech 233 visual impairment, progressive progressive supranuclear supranuclear palsy palsy 50, 180 for PD 116 vitamin C, C, protective effect effect for 116 vitamin EE see see tocopherol tocopherol vocal 210 vocal cords, cords, abduction/adduction abduction/adduction 210 Vocalite Vocalite 232 voice essential tremor 87 essential tremor 87 tremor 82, 82,83, 83,85 85 volume volume 227, 227,229, 229,234—5 234-5

voluntary motor control loss 24 24 voluntary organizations 176 176 walking 189 189 aids aids 204, 209 confidence confidence 206 206 elderly elderly15—16 15-16 209 walking frames frames 209 sticks 209 209 walking sticks washing 221 221 wearing off dystonia 151, 151,153 153 motor motor fluctuations fluctuations150—1 150-1 Scale 200 Webster Scale welfare workers 176 welfare workers 176 wheelchair 222 222 12 Wilson's disease 12 World Health Organization Organization Classification Classification of Impairments, Disabilities Disabilities and Handicaps 166 writing tremor primary 84 84 see see also alsohandwriting handwriting

ziprasidone ziprasidone 66 zolpidem zolpidem 154 154 zotepine 66 66