THERAPEUTICS of PARKINSON’S DISEASE and OTHER MOVEMENT DISORDERS Therapeutics of Parkinson’s Disease and Other Movement Disorders Edited by Mark Hallett and Werner Poewe © 2008 John Wiley & Sons, Ltd ISBN: 978-0-470-06648-5 THERAPEUTICS of PARKINSON’S DISEASE and OTHER MOVEMENT DISORDERS Edited by MARK HALLETT National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA and WERNER POEWE Department of Neurology, Medical University of Innsbruck, Austria This edition first published 2008 # 2008, John Wiley & Sons Ltd Wiley-Blackwell is an imprint of John Wiley & Sons, formed by the merger of Wiley’s global Scientific, Technical and Medical business with Blackwell Publishing Registered office: John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK Other Editorial Offices: 9600 Garsington Road, Oxford, OX4 2DQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA For details of our global editorial offices, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell The right of the author to be identified as the author of this work has been asserted in accordance with the Copyright, Designs and Patents Act 1988 All rights reserved No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher Wiley also publishes its books in a variety of electronic formats Some content that appears in print may not be available in electronic books Designations used by companies to distinguish their products are often claimed as trademarks All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners The publisher 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modifications, changes in governmental regulations, and the constant flow of information relating to the use of medicines, equipment, and devices, the reader is urged to review and evaluate the information provided in the package insert or instructions for each medicine, equipment, or device for, among other things, any changes in the instructions or indication of usage and for added warnings and precautions Readers should consult with a specialist where appropriate The fact that an organization or Website is referred to in this work as a citation and/or a potential source of further information does not mean that the author or the publisher endorses the information the organization or Website may provide or recommendations it may make Further, readers should be aware that Internet Websites listed in this work may have changed or disappeared between when this work was written and when it is read No warranty may be created or extended by any promotional statements for this work Neither the publisher nor the author shall be liable for any damages arising herefrom Library of Congress Cataloguing-in-Publication Data Therapeutics of Parkinson’s disease and other movement disorders/edited by Mark Hallett and Werner Poewe p ; cm Includes bibliographical references and index ISBN 978-0-470-06648-5 Parkinson’s disease–Treatment Movement disorders– Treatment I Hallett, Mark, 1943- II Poewe, W [DNLM: Parkinson Disease–therapy Movement Disorders–therapy WL 359 T3974 2008] RC382.T43 2008 2008022144 616.80 33–dc22 ISBN: 9780470066485 A catalogue record for this book is available from the British Library Typeset in 9/11 pt Times by Thomson Digital, India Printed and bound in Great Britain by Antony Rowe Ltd, Chippenham, Wiltshire Contents Preface Contributors PART I ix xi PARKINSON’S DISEASE AND PARKINSONISM The Etiopathogenesis of Parkinson’s Disease: Basic Mechanisms of Neurodegeneration C Warren Olanow and Kevin McNaught Physiology of Parkinson’s Disease Shlomo Elias, Zvi Israel and Hagai Bergman 25 Pharmacology of Parkinson’s Disease Jonathan M Brotchie 37 The Treatment of Early Parkinson’s Disease Olivier Rascol and Regina Katzenschlager 49 Treatment of Motor Complications in Advanced Parkinson’s Disease Susan H Fox and Anthony E Lang 71 Managing the Non-Motor Symptoms of Parkinson’s Disease Werner Poewe and Klaus Seppi 91 Surgery for Parkinson’s Disease Jens Volkmann 121 Future Cell- and Gene-Based Therapies for Parkinson’s Disease Tomas Bj€ rklund, Asuka Morizane, Deniz Kirik and Patrik Brundin o 145 Parkinson-Plus Disorders Martin K€ llensperger and Gregor K Wenning o 157 vi CONTENTS PART II TREMOR DISORDERS 10 Essential Tremor Rodger J Elble 179 11 Other Tremor Disorders G€ nther Deuschl u 193 PART III DYSTONIA, CRAMPS, AND SPASMS 12 Pathophysiology of Dystonia Mark Hallett 205 13 General Management Approach to Dystonia Cynthia L Comella 217 14 Botulinum Toxin for Treatment of Dystonia Dirk Dressler 227 15 Surgical Treatments of Dystonia Christopher Kenney and Joseph Jankovic 241 16 Wilson’s Disease George J Brewer 251 17 Cramps and Spasms Christine D Esper, Pratibha G Aia, Leslie J Cloud and Stewart A Factor 263 18 Stiff Person Syndrome Philip D Thompson and Hans-Michael Meinck 283 PART IV CHOREA, TICS AND OTHER MOVEMENT DISORDERS 19 Huntington’s Disease Kevin M Biglan and Ira Shoulson 295 20 Chorea Francisco Cardoso 317 21 Treatment of Tics and Tourette Syndrome Harvey S Singer and Erika L.F Hedderick 331 22 Therapeutics of Paroxysmal Dyskinesias Shyamal H Mehta and Kapil D Sethi 345 23 Treatment of Miscellaneous Disorders Marie Vidailhet, Emmanuel Roze and David Grabli 353 24 Myoclonus Shu-Ching Hu, Steven J Frucht and Hiroshi Shibasaki 363 CONTENTS vii PART V DRUG-INDUCED MOVEMENT DISORDERS 25 Neuroleptic-Induced Movement Disorders S Elizabeth Zauber and Christopher G Goetz 373 26 Other Drug-Induced Dyskinesias Oscar S Gershanik 389 PART VI ATAXIA AND DISORDERS OF GAIT AND BALANCE 27 Ataxia Thomas Klockgether 407 28 Treatment of Gait and Balance Disorders Bastiaan R Bloem, Alexander C Geurts, S Hassin-Baer and Nir Giladi 417 PART VII RESTLESS LEGS SYNDROME 29 The Restless Legs Syndrome Richard P Allen and Birgit H€ gl o 447 PART VIII PEDIATRIC MOVEMENT DISORDERS 30 Pediatric Movement Disorders Jonathan W Mink 471 PART IX PSYCHOGENIC MOVEMENT DISORDERS 31 Psychogenic Movement Disorders Elizabeth Peckham and Mark Hallett 479 Index 489 Preface Over the past few decades the field of neurology has seen spectacular developments in diagnostic techniques, most vividly exemplified by modern neuroimaging and molecular genetics Although not always at the same speed this evolution has gone hand in hand with an enlarging armentarium of effective therapies to treat neurological disease This is particularly true for the field of movement disorders, where one of the most exciting success stories of modern translational research in neuroscience unfolded more than 40 years ago: the discovery of dopamine deficiency in the striatum of patients with Parkinson’s disease and the subsequent introduction of levodopa as a dramatically effective therapy of this hitherto devastating illness Since then the therapeutic options for Parkinson’s disease have grown exponentially, often making treatment decisions difficult Moreover, there are now numerous therapies for other movement disorders with substantial impact on patients While many therapies remain symptomatic, a number normalize the condition such as de-coppering in Wilson’s disease and levodopa in dopa-responsive dystonia While there are a number of textbooks on movement disorders, none so far has emphasized treatment, and this current work attempts to fill this gap Practitioners want and need practical detailed advice on how to treat patients We have recruited a team of experts who have attempted to deal with most situations Wherever available, chapter authors have used evidence from randomized controlled clinical trials to develop practical recommendations for every day clinical practice As is the case for all of medicine there are many situations in the treatment of movement disorders where evidence from controlled trials is either insufficient or open to interpretation We have therefore deliberately encouraged the expert authors to share with the reader their personal clinical acumen and therapeutic wisdom Summary tables and algorithms are part of many chapters and will hopefully serve as a quick reference guide for practical treatment decisions in many different circumstances Of course, each patient presents unique circumstances, so physicians will need to use their judgement every step of the way, but having expert guidance should at least set the general direction We are grateful to the movement disorder experts whom we have recruited from all over the world to bring their knowledge to this textbook We appreciate their expertise and patience with our compulsive editing, as we have tried to give a uniform style to the recommendations, and occasionally added our own opinions We have tried to be up to date, but medications and other treatment options may change New agents appear and some may even be withdrawn because new adverse effects surface So, we hope that this book and its advice will be a helpful guide, but physicians must continue to be alert to any changes in practice that might arise MARK HALLETT WERNER POEWE 31: PSYCHOGENIC MOVEMENT DISORDERS patients than other terms In one study in patients with seizure-type movements, the terms “stress-related seizures” or “functional seizures” were found to be less offensive than the terms “psychogenic seizure,” “non-epileptic attack disorder” or “pseudoseizures” (Stone et al., 2003a) It is very important to describe the medical condition to the patient in a way that will help ensure trust in the physician, so there is a better chance for recovery In addition, we generally discuss that stress may play a contributing factor in the development of PMDs and try to identify any possible stressors If there is the ability to have long-term care with this patient, it may be best to see them for a couple of appointments, plant the idea early on that this is in the differential diagnosis and that you need to rule out some other conditions If the patient has seen other physicians they may have been told “it is all in your head,” “just stop doing it,” and so on Lack of sympathy for this diagnosis does not help your patient or help you to keep a patient that may actually get better Referral to a Psychiatrist It is very important to refer the patient to a psychiatrist who has an understanding of psychogenic movement disorders Commonly, the diagnosis is made, the patient is willing to undergo a psychiatric evaluation and then is told by the evaluating psychiatrist that they have an underlying neurological problem (and it can’t possibly be psychiatric) This is extremely frustrating for the patient to hear, will lose any credibility that you have with the patient and send them on an endless search for what is the “real cause” of their condition The psychiatrist has to understand that the referral is not made for the diagnosis of the condition, but for identification and treatment of any underlying psychiatric problem This should be a principal component of therapy CONCLUSION Psychogenic movement disorders are truly a crisis for neurology (Hallett, 2006) They occur in a relatively young population and can be reversible with early intervention and multi-modality treatment However, this requires an astute knowledge of this condition and appropriate referral to a psychiatrist and additional therapists To compound this difficult problem, the medical community at large is poorly educated on this topic, and as a result these patients are misdiagnosed References Baik, J.S and Lang, A.E (2007) Gait abnormalities in psychogenic movement disorders Movement Disorders, 22 (3), 395 399 487 Baker, J Psychogenic 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A critical review of previous intervention studies.Psychosomatic Medicine, 69 (9), 889 900 Suzuki, A., Mochizuki, H., Kajiyama, Y., Kimura, M., Furukawa, T., Ichikawa, G., Arai, H and Mizuno, Y (2001) A case of convergence spasm in hysteria improved with a brief psychiatric assessment No To Shinkei, 53 (12), 1141 1144 Thomas, M and Jankovic, J (2004) Psychogenic movement disorders: diagnosis and management CNS Drugs, 18 (7), 437 452 Van Nuenen, B.F., Wohlgemuth, M., Wong Chung, R.E., Abdo, W.F and Bloem, B.R (2007) Acupuncture for psychogenic movement disorders: Treatment or diagnostic tool Movement Disorders, 22, 1353 1355 Verhoeven, J., Marien, P., Engelborghs, S., D’Haenen, H and DeDeyn, P (2005) A foreign speech accent in a case of conversion disorder Behavioural Neurology, 16 (4), 225 232 Voon, V and Lang, A.E (2005) Antidepressant treatment outcomes of psychogenic movement disorder Journal of Clinical Psychiatry, 66 (12), 1529 1534 Zeuner, K.E., Shoge, R.O., Goldstein, S.R., Dambrosia, J.M and Hallett, M (2003) Accelerometry to distinguish psychogenic from essential or parkinsonian tremor Neurology, 61, 548 550 Index Note: Page references in italics refer to figures; those in bold refer to Tables abetalipoproteinemia 409 acetazolamide in myoclonus 366 acetylcholinesterase inhibitors in Huntington disease 303 action dystonia 205 adenylyl cyclase 39 a2 adrenergic receptors 42 adverse drug reactions (ADRs) 59 aggresomes 13 akathisia sub-acute, neuroleptic-induced 377–8 tardive 384 akinesia 25 alcoholic cerebellar degeneration 412 alopecia 60 Alzheimer’s disease (AD) 3, 164 amantadine drug-induced dyskinesias 397 in Huntington disease 302–3, 306–7 in multiple system atrophy 159 in Parkinsons’s disease 54, 55, 59, 64, 80, 82, 100, 430–1 amitryptiline 92 animal models of Parkinson’s disease and Parkinsonism 27 ankle–foot orthosis (AFO) 424, 424 antecollis 236 anti-cholinergic agents in corticobasal degeneration 169 in dystonia 219 in multiple system atrophy 159 in Parkinson’s disease 55, 64, 100 in progressive supranuclear palsy 164 anti-convulsants in restless legs syndrome 459–60 anti-copper drugs in Wilson’s disease 254–6 anti-depressants drug-induced dyskinesias 392–3 in dementia with Lewy bodies (DLB) 167 in Huntington disease 303 in Parkinson’s disease 92–5 anti-epileptic drugs drug-induced dyskinesias 389–92 anti-histamines drug-induced dyskinesias 395–6 anti-muscarinics 100 anti-psychotics in Parkinson’s disease 101 antibiotics in drug-induced dyskinesias 396 apomorphine in Parkinson’s disease 54, 57, 61, 77–8, 79, 108, 109 apoptosis in Parkinson’s disease 7, appetite, loss of 91 aripiprazole in Parkinson’s disease 100 in tic disorders 339 arm dystonia 236–7, 237 arm tremor 194 asterixis 363 ataxia 407–14 caused by acquired vitamin deficiency and metabolic disorders 412–13 classification 408 due to other toxic reasons 412 general principles of therapy 407 ataxia telangiectasia (AT) 408 ataxia with isolated vitamin E deficiency (AVED) 407, 409–10 ataxia with oculomotor apraxia, autosomal recessive type (AOA1) 409 type (AOA2) 409 atomoxetine 394 autonomic dysfunction in Parkinson’s disease 106–10 constipation 107 definition and clinical features 106–7 orthostatic hypotension (OH) 106 pathophysiology and clinicopathological correlations 107 practical management 107, 108 Therapeutics of Parkinson’s Disease and Other Movement Disorders Edited by Mark Hallett and Werner Poewe © 2008 John Wiley & Sons, Ltd ISBN: 978-0-470-06648-5 490 autonomic dysfunction in Parkinson’s disease (Continued ) prevalence 107 sexual dysfunction 107 urinary dysfunction 106–7 autosomal dominant Parkinson’s disease 7–10 autosomal recessive Parkinson’s disease 10–12 autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) 409 baclofen drug-induced dyskinesias 397 in dystonia 222 in tic disorders 337–8 balance disorders see gait and balance disorders Barry–Albright Scale 242 basal ganglia advanced neurosurgical treatments of Parkinson’s disease 30–1 cellular and molecular pharmacology 39–43, 40 acetylcholine 41 dopamine 39–40 glutamate 40–1 peptides 41–2 circuit-based model of pharmacology 37–9 circuitry 38 clinical anatomy 27–8 physiological studies in MPTP-treated primates and human Parkinson’s disease 29–30 in normal primates 28–9 population activity in dopamine-depleted animal models/human Parkinson’s disease 30 surgery 123–4 Bax 15 beginning and end-of-dose dyskinesia 80 Bell’s palsy 210, 263 belly dancer’s dyskinesia (BDD) 271–2, 357–8 benign hereditary chorea 318 benign idiopathic dystonic of infancy 474 benign myoclonus of early infancy (benign infantile spasms) 472 benign neonatal sleep myoclonus 472 benign paroxysmal torticollis 474 benzodiazepines in corticobasal degeneration 169 in dystonia 222 in drug-induced dyskinesias 397 in myoclonus 366 benztropine in progressive supranuclear palsy 164 bisacodyl 110 bladder dysfunction 107–9 blepharospasm 210, 242, 242, 354 bobble-head doll syndrome 356–7 botulinum toxin in corticobasal degeneration 169 in multiple system atrophy 160 in myoclonus 367 in Parkinson’s disease 109 INDEX in progressive supranuclear palsy 164 in tic disorders 340 see also botulinum toxin for dystonia botulinum toxin for dystonia 227–38 basic principles 231–3 dosing 231–2, 232 intramuscular placement 232–3 monitoring of therapeutic effect 233 multi-layer concept of treatment 231, 231 patient diary 234 planning of therapy 232 history 227 pharmacology 227–31 antigenicity 229–30 botulinum toxin drugs 228–9, 228 mode of action 227–8 properties of drugs 229 safety aspects and adverse effects 230 structure 227 therapeutic profile 230–1, 231 time course of action 228 specific indications 233–8 arm dystonia 236–7, 237 cervical dystonia 235–6, 236 cranial dystonias 233, 235 leg dystonia 237–8, 238 pharyngolaryngeal dystonia 234–5 segmental dystonic, generalized dystonia 238 bradykinesia in Parkinson’s disease 25 brain stem myoclonus 363 brain stem reticular myoclonus 363 Brain-Thyroid-Lung syndrome 322 British antilewisite (BAL) in Wilson’s disease 256 bromocriptine in multiple system atrophy 159 in Parkinson’s disease 49, 51, 54, 55, 57, 58, 59, 60, 61, 75, 92 in progressive supranuclear palsy 162 in sub-acute neuroleptic malignant syndrome 376 bruxism 355 buccolingomasticatory syndrome 323 bupropion drug-induced dyskinesias 397 in Parkinson’s disease 109 Burke–Fahn–Marsden–Dystonia Scale (BFMDS) 242 -burst stimulation 210 buspirone 397 cabergoline in multiple system atrophy 159 in Parkinson’s disease 49, 54, 55, 57, 59, 60, 61, 65, 75, 76 calcineurin inhibitors drug-induced dyskinesias 397 carbamazepine in restless legs syndrome 459–60 caspase 3, 15 catatonia 375 causalgia dystonia 208, 273–6 INDEX CB1 receptors 43 CDCrel-1 10 CDS hypothesis 55 cell transplantation therapy 145–50 fetal neural tissue transplantation 146–7 historical background 145–6 stem-cell based therapies 147–9 embryonic stem cells 148–9 neural stem cells 148 Spheramine 149–50 CEP-1347 53 cephalosporins 396 cerebellar ataxia 413 cerebellar tremor syndromes 195–7 cerebrotendinous xanthomatosis (CTX) 410 certazidine 396 cervical dystonia 205, 235–6, 236 chelators, problems with in Wilson’s disease 257–8 CHIP 11 CHIP/HSP70 11 cholinergic agents in progressive supranuclear palsy 164 cholinesterase inhibitors (ChE-Is) in dementia with Lewy bodies (DLB) 165–7 (ChE-Is) in Parkinson’s disease 97, 100, 104 chorea 317–27 clinical presentation and prevalence 317–24 drug-induced choreas 323–4, 324 genetic choreas 317–22, 319–21 infectious choreas 323 Sydenham chorea (SC) 322–3 vascular choreas 322 etiological classification 318 management 324–7, 325 genetic choreas 325 PAPS 326 SLE 326 Sydenham chorea 325–6, 326 chorea-acanthocytosis (ChAc), autosomal recessive 318 cimetidine 395 ciprofloxacin 396 clomipramine 92 clonazepam in dystonia 222 in myoclonus 366 in insomnia 112 in tic disorders 338 clonidine in tic disorders 337 clozapine in dementia with Lewy bodies (DLB) 167 in Parkinson’s disease 80, 101, 104 cocaine 393–4 coherence entrainment test 199 complex regional pain syndrome (CRPS) 208, 273–6 COMT inhibitors in Parkinson’s disease 49, 54, 58–9, 72–4, 80 constipation in Parkinson’s disease 109–10, 107 constraint induced movement therapy in dystonia 218 491 continuous dopamine stimulation 55 cortical myoclonus 363 corticobasal degeneration 168–70, 208 clinical diagnosis 169 definition and clinical features 168 non-medical therapy 170 pathophysiology and clinicopathological correlations 169 pragmatic management 170 prevalence 168–9 principles of management 169–70 symptomatic therapy 169 CR levodopa 55 crack-dancing 394 cranial dystonia 205, 233, 235 craniocervical dystonia 354 Creutzfeldt–Jakob disease 364 new variant 323 cyclin E a/b tubulin 10 cyclosporin A 396 dantrolene in sub-acute neuroleptic malignant syndrome 376 daytime sleepiness and sudden onset sleep 112–15 deep brain stimulation (DBS) 125–32 adverse effects on cognition, mood and behavior 132 surgery- and device-related 130–1 therapy-related 131–2 alternative targets 130 in insomnia 111–12 in myoclonus 367 pallidal 127–8, 127 physiological mechanisms 126 safety 130–2 subthalamic nucleus (STN-DBS) 121, 128–30, 129 targets, comparison 133–4 thalamic 126–7 in tic disorders 340 dementia with Lewy bodies (DLB) 7, 97, 164–8 clinical diagnosis 166 definition and clinical features 164–5 pathophysiology and clinicopathological correlations 165 prevalence 165 principles of management 165–8 autonomic symptoms 168 dementia and psychiatric features 165–7 neuroprotective therapy 165 non-medical therapy 168 Parkinsonism 167–8 pragmatic management 168 symptomatic therapy 165 dentatorubro-pallidoluyisian atrophy 410 desglymidodrine 107 desipramin in progressive supranuclear palsy 164 desmopressin in multiple system atrophy 160 in Parkinson’s disease 108 492 diazoxide drug-induced dyskinesias 397 digoxin drug-induced dyskinesias 397 dihydroergocryptine (DHEK) 53, 54, 57 dihydroergotamine 107 diphasic dyskinesia 80 diphenhydramine drug-induced dyskinesias 395 DJ-1 3, 11, 12 domperidone 59, 77, 78, 110 donepezil in dementia with Lewy bodies (DLB) 166–7 in Parkinson’s disease 97–100, 104 in progressive supranuclear palsy 164 dopamine agonists in Parkinson’s disease 54, 55–8, 64–5, 429–30 in restless legs syndrome 456–8, 457 in tic disorders 332–3 dopamine depletion in dystonia 221–2 dopamine dysregulation syndrome 60, 104, 106 dopamine receptor agonist in Parkinson’s disease 75 dopamine receptor antagonists in dystonia 221–2 dopamine replacement therapy 29 dopaminergic agents in corticobasal degeneration 169 in dystonia 219–21 in multiple system atrophy 159 in Parkinson’s disease 92 in progressive supranuclear palsy 162 dopa-responsive dystonia (DYT5) 206 drug-induced choreas 323–4, 324 drug-induced dyskinesias 389–98, 390 antibiotics 396 anti-depressants 392–3 anti-epileptic drugs 389–92 anti-histamines 395–6 immunosuppressants 396–7 miscellaneous 397–8 non-neuroleptic 390 principles of management 398–9 psychostimulants 393–5 sympathomimetics 395 dysgammaglobulinaemia 198 dyskinesia, tardive 379–82 dysphagia 83–4, 84, 236 dystonia, acute, neuroleptic-induced 373–4 botulinum toxin in 227–38 general management approach 217–24 non-pharmacologic interventions 217–18 geste antagoniste 217–18 limb cooling 218 repetitive transcranial magnetic stimulation 218 sensory motor retuning (SMR; constraint induced movement therapy) 218 pharmacologic treatments 219–22, 220–1 tardive 382–3 treatment guidelines 222–3 INDEX focal dystonia 223, 224 generalized dystonia 223, 223 see also dystonia, pathophysiology of; dystonia, psychogenic; dystonia, surgical treatments dystonia, pathophysiology of 205–13 definition and clinical classification 205 etiologic classification 205–9 genetic 205–8, 206 secondary 208–9 increased plasticity 210–11 loss of inhibition 209–10 physiology 209–13 sensory abnormality 211–13 dystonia, psychogenic 209, 482 dystonia, surgical treatments 241–7 background and rationale 241 central/ablation 243–4 focal dystonia 244 cervical 244 generalized dystonia 243–4 primary 243–4 secondary 244 central surgery/deep brain stimulation 245–7, 246 focal dystonia 247 cervical 247 generalized dystonia 245–7, 247 primary 245–7 secondary 247 intrathecal baclofen (ITB) 241–2 peripheral 242–3 dystonic tremor syndromes 195 E3 ubiquitin ligase 10, 11 early morning dystonia 80 ecological validity 421–2 ECT in Parkinson’s disease 95 embryonic stem cells 148–9 endocannabinoids 42–3 endomorphin-1 42 enhanced physiological tremor 193–4, 194 enkephalins 41 entacapone 49, 61, 65, 72–3, 74 epilepsia partialis 364 epilepsia partialis continua 354, 364 epileptic myoclonus 364 episodic ataxias 411 episodic focal lingual spasms 266 erectile dysfunction 109 ergot 61 erythromycin 396 essential myoclonus 364 essential palatal tremor (EPaT) 198 essential tremor 179–88 clinical characteristics 179 diagnosis and differential diagnosis 180–2 diagnostic criteria 181–2 pathophysiology 179–80 treatment 183–8 INDEX algorithm 188 non-surgical 185 pharmacologic 183–6 practical management 187 surgical 186–7 ethosuximide in myoclonus 366 etilefrine hydrochloride 107 etilevodopa 76, 79 evidence-based medicine (EBM) assessment of early Parkinson’s disease interventions 49–59 drug efficacy on disease progression 50–3 drug with symptomatic efficacy as monotherapy 53–5, 53 prevention of motor complications 55–9, 56 random controlled trials using placebo as a comparator 54 COMT inhibitors 54 dopamine agonists 54 levodopa 54 MAO-B inhibitors 54 random controlled trials using an active comparator 54–5 agonist monotherapy vs another agonist 54 agonist monotherapy vs levodopa 54 controlled released (CR) levopoda vs standard levodopa 54 excessive daytime sleepiness (EDS) 112, 114 exercise therapy, balance and gait 422 external global pallidus (GPe) 38 facial hemidystonia 354 facial movement disorders 353–6, 354 intermittent tonic contractions 355–6 jerky movement 353–5 rhythmic movements 355 facial palsy 355 falls 417 prevention of 420–1 familial paroxysmal choreoathetosis 345 fatigue 91 fentanyl 397 fetal neural tissue transplantation 146–7 fludrocortisone in Parkinson’s disease 107 fluoxetine in Parkinson’s disease 92, 61, 95 fluphenazine in tic disorders 338–9 fluvoxamine 61 focal dystonia 205, 208 focal myoclonus 358 foot drop 424 frataxin 408 freezing of gait (FOG) 419 Friedreich’s ataxia (FRDA) 407–8 functional electrical stimulation (FES) 424–5, 426 GABA in restless legs syndrome 460 in tic disorders 333 gabapentin drug-induced dyskinesias 391 in myoclonus 366 in restless legs syndrome 459–60 gait and balance disorders 417–37 assessment 419 disease-specific treatment strategies 427–36 generic treatment strategies 420–7, 420 drugs 421 mental imagery (MI) 425 multidisciplinary rehabilitation 425–7 occupational therapy 422–3 physiotherapy 421–2 prevention of falls 420–1 protective measures 425 surgery 421 treadmill training 423 virtual reality (VR) 425 walking aids 423–4 generic vs disease-specific treatment strategies 419–20 negative consequences 418 pathophysiology 418–19, 418 psychogenic 482 galantamine in Parkinson’s disease 100, 104 gangliosidoses 208 geldandamycin gene therapy for Parkinson’s disease 150–3 continuous DA delivery strategy 150–1 continuous DOPA delivery strategy 150 enhanced DA synthesis from peripheral L-DOPA 151 growth factor therapy 152–3 targeting the subthalamic nucleus 151–2 generalized dystonia 238 genetic choreas 317–22, 319–21, 325 geniospasm 355 geste antagoniste 182, 205, 217–18 Gilles de la Tourette syndrome see Tourette syndrome glial cell line-derived neurotrophic factor (GDNF) 152–3 globus pallidus 28 glutamatergic agents in progressive supranuclear palsy 164 in tic disorders 333 gluten ataxia 413 gracile axonal dystrophy (GAD) guanfacine in tic disorders 337 Guillain-Barr syndrome 198 e Hallervorden–Spatz disease 208 haloperidol in tic disorders 339 head banging 357 head nodding 473–4 heat shock proteins HSP70 11, 13 HSP90 13 heat stroke 375 hedonistic homeostatic dysregulation 104 Helicobacter pylori 78 hemiballism 125, 322, 324 hemichorea 125 hemidystonia 205 493 494 hemifacial spasm (HFS) 263–6, 264, 353–4 hemiglossal twisting 266 hereditary childhood onset dystonia (idiopathic torsion dystonia, DYT1) 205–6 hereditary distal muscle cramps without neuropathy 268–9 hereditary polyneuropathy (HMSN) 198 hereditary progressive dystonias 206 high-frequency deep brain stimulation (DBS) 122 historical background 145–6 HIV/AIDS, infectious chorea and 323 Holmes’ tremor 197 human retinal pigment epithelial (hRPE) 149–50 huntingtin 300–1 Huntington disease 295–308, 410 clinical care 306–8 behavioral features 307–8 cognitive impairment 307 motor features 306–7 participation in research 308 predictive and pre-natal testing 306 clinical characteristics 295–9 affective disorder 297 aggression 297 apathy 297 behavioral disturbances 297 body mass and metabolism 297–8 chorea 296 cognitive dysfunction 296–7 dysarthria 296 motor manifestation 295–6 natural history 298 oculomotor abnormalities 296 psychosis 297 total functional capacity scale 298 diagnosis 295 epidemiology 299 experimental therapeutics and care 301–8 neuroprotective 303–5, 303 restorative 305, 305 symptomatic therapies 302–3, 302 juvenile-onset 208, 298–9 pathogenesis 299–301 pathology 299 Huntington’s disease-like (HDL2) 318 g-hydroxybutyrate (GHB) in myoclonus 366 4-hydroxy-2-nonenal 13 5-hydroxytryptamine (serotonin) 42 5-hydroxytryptophan in myoclonus 366 6-hydroxydopamine (6-OHDA) 27 hyperekplasia 364 O.K.? hypersexuality 60 hyperthermia, malignant 375 hypokinetic dysarthria 83 hypophonia 83 hypothyroidism, ataxia and 413 INDEX idazoxan in progressive supranuclear palsy 164 idebenone 408 idiopathic torsion dystonia (DYT1) 205–6 imipramine 92 immune-mediated ataxias 413–14 immunosuppressants 396–7 impulse control disorders (ICDs) 104 infantile cobalamin 355 infectious choreas 323 insomnia 91, 111–12, 113 intention tremor 179 internal globus pallidus (GPi) 38 intrathecal baclofen (ITB) in dystonia 241–2 iron treatment in restless legs syndrome 460–1 Isaacs’ syndrome 269–70 jacitation 271 jerking stiff man syndrome 283 jitteriness, infantile 472–3 jumpy stumps 270–1 juvenile autosomal recessive Parkinson’s disease (AR-JP) 10 juvenile-onset Huntington disease 208, 298–9 kinetic tremor 179 knee-ankle-foot orthosis (KAFO) 424 Korsakov’s psychosis 412 L-dopa in corticobasal degeneration 169 in dementia with Lewy bodies (DLB) 167 in multiple system atrophy 159 L-threodihydroxy-phenylserine (L-threo-DOPS) in orthostatic hypotension 160 lactacystin 14 lactulose 110 lamotrigine drug-induced dyskinesias 392 in myoclonus 366 Lance–Adams syndrome 364 laryngeal dystonia 234–5 late-onset hereditary Parkinson’s disease 10 leg dystonia 237–8, 238 Leigh’s disease 208 Lesch-Nyan disease 357 lesional stereotaxy 124–5 vs deep brain stimulation 133 levetiracetam in myoclonus 366 in tic disorders 337 levodopa enteral 76, 81 liquid/soluble 76 non-responsive motor complications 72 in Parkinson’s disease 3, 31, 49, 52, 53, 54, 56, 57, 58, 59, 61, 63–4, 65, 71, 72, 76, 78, 80, 108, 428–9 INDEX in restless legs syndrome 455–6 toxicity 64 levodopa ethyl ester (etilevodopa) 76, 79 levodopa-induced dyskinesia (LID) 31, 71 levodopa-responsive motor complications motor fluctuations levodopa methylester 76 levodopa/benserazide (Madpoar dispersible) 76 levodopa/carbidopa (Parcopa) 76 levodopa/carbidopa in methyl cellulose (Duodopa) 76 levofloxacion 396 Lewy bodies Lewy neurites 3, 13 Lewy neuritis L’Hermitte’s sign 152 limb cooling in dystonia 218 limb movement disorders 358–60, 359 hyperkinetic movement disorders 358–9 hyperkinetic movement disorders 358–9 peripherally induced movement disorders 358 tonic spasms and abnormal postures 360 lisuride in multiple system atrophy 159 in Parkinson’s disease 49, 54, 57, 58, 59, 61, 65, 81 in progressive supranuclear palsy 162 lithium drug-induced dyskinesias 397 in Parkinson’s disease 61 long intracortical inhibition (LICI) 209 LRRK2 3, 10 lubag 207 lysine 48 10 lysine 63 10 macrogol 110 mandibular dystonia 233 MAO-B inhibitors in Parkinson’s disease 49, 54, 55, 58, 64, 74, 80 marijuana (d-9-tetrahydrocannabinol (THC)) 340 Marinesco-Sjogren syndrome (MSS) 410 masturbation, posturing during 474–5 medium-sized spiny neurons (MSNs) 38, 39 Meige syndrome 354 melevodopa (levodopa methyl ester) 79 memantine 100 mental imagery (MI) 425 methadone 398 methyphenidate 394 midodrine in orthostatic hypotension 160 in Parkinson’s disease 107 mirtazapine in Parkinson’s disease 95, 97 mitochondrial dysfunction in Parkinson’s disease 12 modafinil in daytime sleepiness and sudden onset sleep 112 mono-amine oxidase inhibitors in Parkinsons’s disease 430 morning akinesia 71 mosapride 109, 110 Mount-Reback syndrome 325 72 495 Movement Disorder Society (MDS) 49–59 moving umbilicus syndrome (belly dancer’s dyskinesia (BDD)) 271–2, 357–8 moxisylyte in multiple system atrophy 160 MPTP 12, 14 MPTP model of Parkinson’s disease 26, 123 multi-focal dystonia 205 multidisciplinary rehabilitation 425–7 multiple system atrophy (MSA) 157–61, 411–12 additional features 158 autonomic symptoms 160 consensus criteria 158 definition and clinical features 157 dystonia 160 erectile dysfunction 161 inspiratory stridor 161 neuroprotective therapy 158–9 non-medical therapy 161 non-pharmacological treatment 159–60 orthostatic hypotension 160 pathophysiology and clinicopathological correlations 157–8 pragmatic management 161 prevalence 157 principles of management 158–61 surgical therapy 159 symptomatic therapy 159 urinary dysfunction 160 myasthenia gravis 267 Myoamoya disease 322 myoclonic dystonia of the face 355 myoclonus 363–8 causes 365 clinical manifestations and localization of 363–4 etiology 364 psychogenic 482 tardive 384 treatment 365–8, 367, 368 myoclonus dystonia (DYT11) 207 myoclonus syndrome 364 myokymias 355 neck movement disorders 356–7, 356 neck tongue syndrome 356 nefazodone in Parkinson’s disease 95 negative myoclonus 363 neural stem cells 148 neuroacanthocytosis 318 neuroleptic malignant syndrome, sub-acute 374–7 neuroleptic-induced movement disorders 373–84 acute dystonia 373–4 sub-acute akathisia 377–8 sub-acute neuroleptic malignant syndrome 374–7 sub-acute parkinsonism 378–9 tardive akathisia 384 tardive dyskinesia 379–82 tardive dystonia 382–3 tardive myoclonus 384 tardive tourettism 384 496 neuroleptics commonly prescribed 374 in dementia with Lewy bodies (DLB) 167 neuromyotonia (Isaacs’ syndrome) 269–70 neuroprotective therapy in multiple system atrophy 158–9 in progressive supranuclear palsy 162 NMDA receptor antagonists in corticobasal degeneration 169–70 in dementia with Lewy bodies (DLB) 167 in multiple system atrophy 159 noradrenergic agents in progressive supranuclear palsy 164 norepinephrine in tic disorders 333 nortryptiline 92 occupational therapy 422–3 octreotide in orthostatic hypotension 160 oculogyric crisis 373 olanzapine in dementia with Lewy bodies (DLB) 167 in Parkinson’s disease 80, 101 in tic disorders 339 d-opioid receptors 41 k-opioid receptors 41, 42 mu-opioid receptors 41, 42 opioids in Parkinson’s disease 61 in restless legs syndrome 460 opsoclonus-myoclonus syndrome 364, 365 oral contraceptives 397 orthostatic hypotension (OH) 106–7 oxidative stress in Parkinson’s disease 12 oxybutinin in multiple system atrophy 160 in Parkinson’s disease 107 PA700 14 PA700 proteasome activator 10 painful legs and moving toes 272–3, 358 paired associative stimulation (PAS) 210 palatal myoclonus (palatal tremor) 197–8, 363 palatal tremor syndromes 197–8, 363 pallidotomy 31, 124–5 palsy (akinesia) in Parkinson’s disease 25 PANDA 334 PAPS 326 paraneoplastic cerebellar degeneration (PCD) 413 PARK 1–11 parkin 3, 11, 12, 14 parkin-associated endothelin receptor-like receptor (Pael-R) 10, 11 parkinsonism psychogenic 482 sub-acute, neuroleptic-induced 378–9 Parkinson-plus disorders 157–70 Parkinson’s disease causes 3, 4–6 INDEX clinical symptoms 25–7 pathology pathophysiology 123–4 physiology 25–47 of rigidity 26–7 of tremor 26 Parkinson’s disease, advanced, drug-responsive motor complications 71–85 beginning of dose worsening, end-of-dose rebound 79 diphasic dyskinesia 82–3 dose failure ‘No-ON’; delayed or partial ‘ON’ response 78–9 drug-resistant motor compilation 83–4 drug-resistant tremor 84 dyskinesia 79–80 peak-dose 79, 80–1 dysphagia 83–4, 84 early-morning OFF dystonia 82–3 motor fluctuations 71–83 clinical features 71 mechanisms 71–2 treatment 72–5 wearing-off 71–5 sialorrhea 84, 85 speech problems 83, 83 Sudden OFFs and ON-OFF fluctuations 75–8 unpredictable fluctuations 75–8 unpredictable motor fluctuations 79 unpredictable OFFs 75–8 Parkinson’s disease, early 49–66 adverse drug reactions of dopaminergic medication 59–60 abnormal daytime somnolence 60–1 caused by anti-cholinergics 61 caused by MAO-B inhibitors 61 central 60–1 fibrosis 59–60 gastro-intestinal and cardiovascular type A adverse reactions 59 leg oedema 59 mortality and early use of dopaminergic drugs 61–2 psychiatric symptoms 60 drug characteristics 64 evidence-based medicine (EBM) assessment 49–59 pragmatic approach in routine clinical practice 62–5, 62, 63 safety issues 59–62 Parkinson’s disease, gait and balance disorders in 427–33, 428 medical treatment 428–31, 430 adverse effects 431 dopamine agonists 429–30 for freezing of gait 431 mono-amine oxidase inhibitors 430 other non-dopaminergic drugs 431 occupational therapy 433 pathophysiology and classification 427–8 physiotherapy 432–3 surgery 431–2 Parkinson’s disease, non-motor symptoms of 91–115 anhedonia 91 anxiety 91 INDEX apathy 91 classification and clinical spectrum 91 cognitive dysfunction and dementia 97–100 dementia pragmatic management 98, 100 RCT trials 99 depression 91–7, 93–4 pragmatic management 95–7, 96 hallucinations 100 insomnia 91 management 92–5 medication-related impulse-dyscontrol\abnormal repetitive behaviors 104–6 neuropsychiatric features 95–106, 96 non-pharmacological treatments 95 panic attacks 91 psychosis 100–4, 102–3 pragmatic management 104, 105 Parkinson’s disease, surgery comparison of techniques 133–4 historical aspects 121–3 patient selection 134–6 pragmatic management 136, 136 parkinsonism, pharmacological treatment 43–7, 43 currently available dopamine receptor agonists 44 emerging non-dopaminergic approaches 44, 44 endocannabinoids 45 motor complications 46–7 treatment-related motor complications 45, 46–7 paroxetine in multiple system atrophy 159 paroxysmal dyskinesia 345–50 history 345 miscellaneous 350, 350 secondary causes 346 paroxysmal exertion (exercise)-induced dyskinesias (PED) 345, 349–50 paroxysmal hypogenic dyskinesias (PHD) 345, 350 paroxysmal kinesigenic choreoathetosis 345 paroxysmal kinesigenic dyskinesias (PKD) 345, 346–7, 359 paroxysmal movement disorders 359 paroxysmal non-kinesigenic dyskinesias (PNKD) 345, 347–9 paroxysmal tonic upgaze of infancy 473 pathological gambling 60 pathothenate-kinase associated neurodegeneration (PKAN) 318 pediatric autoimmune neuropsychological disorder (PANDA) 334 pediatric movement disorders 471–5 developmental 472–5 diagnosis 471–2 pemoline 394 penicillamine in Wilson’s disease 256, 258, 259 penicillin 396 pergolide in multiple system atrophy 159 in Parkinson’s disease 47, 49, 52, 53, 54, 55, 56, 57, 59, 60, 61, 75, 76, 109 in tic disorders 339–40 497 peribedil 57 pericentrin 13 periocular dystonia 233 perioral dystonia 233 peripheral dystonia 273–6 peripheral neuropathy, tremor syndromes in 198–9 phantom limb phenomena 358 pharyngolaryngeal dystonia 234–5 phenyl-propanolamine drug-induced dyskinesias 395 in orthostatic hypotension 160 phosphodiesterase-5 (Parkinson’s diseaseE-5) 109 physiologic myoclonus 364 physiotherapy 421–2 pimozide in tic disorders 338 PINK1 11–12 PINK1 piracetam in myoclonus 366 piribedil 47, 49, 53, 61 post-hemiplegic dystonia 208 post-hypoxic myoclonus (Lance–Adams syndrome) 364 post-paralytic facial syndrome 355 post-pump chorea 322 postural tremor 179 pramipexole in insomnia 112 in multiple system atrophy 159 in Parkinson’s disease 49, 51, 53, 54, 55, 57, 59, 60, 61, 63, 75, 92 in progressive supranuclear palsy 162 prazosin in multiple system atrophy 160 pre-proenkephalin-A (PPE-A) 41 pre-proenkephalin-B (PPE-B) 41 primary antiphospholipid antibody syndrome (PAP) 323 primary orthostatic tremor 194–5 primidone in myoclonus 366 progressive encephalomyelitis with rigidity (PER) 286 progressive myoclonic ataxia (Ramsay–Hunt syndrome) 364 progressive myoclonic epilepsy 364 progressive supranuclear palsy 10, 161–4 definition and clinical features 161–2 pathophysiology and clinicopathological correlations 162 pragmatic management 165 prevalence 162 principles of management 162–4, 165 anti-cholinergic agents 164 boulinum toxin A 164 cholinergic agents 164 dopaminergic agents 162 glutamatergic agents 164 neuroprotective therapy 162 NINDS-SPSP clinical criteria for diagnosis 163 non-medical treatment 164 noradrenergic agents 164 serotonergic agents 162 498 progressive supranuclear palsy (Continued) serotonin antagonists 164 surgical therapy 164 symptomatic therapy 162 propofol 398 propriospinal myoclonus 364 proteasome 20S 14 PA700 14 protofibrils pseudo athetosis 358–9 pseudoephedrine 395 PSI 14 PSMA2 14 PSMA3 14 PSMA5 14 psychogenic movement disorders 479–87 diagnostic criteria 479–81, 481 epidemiology 479 examination 480–1 general guidance 486–7 history 480 medical treatment 485–6 acupuncture 486 behavioral modification 485 biofeedback 486 hypnosis 486 in-patient 485 oral medication 485 out-patient 485–6 physical therapy 485 psychotherapy 486 trial of placebo 486 neurological evaluation 480–1 psychogenic signs 483–5, 484 referral to psychiatrist 487 telling the patient 486–7 treatment modalities 480, 485–7 types 481–2 psychostimulants 393–5 punding 104 quetiapine in dementia with Lewy bodies (DLB) 167 in Parkinson’s disease 80, 101, 104 in tic disorders 339 radiofrequency thalamotomy 187 Ramsay–Hunt syndrome 364 ranitidine 395 Rapid Eye Movement (REM) Sleep Behavior Disorder 110–11 rapid-onset dystonia-parkisonism (DYT12) 207 rasagiline 49, 52, 53, 54, 61, 63, 74 Rasmussen’s encephalitis 355, 364 reboxetine 109 reflex sympathetic dystrophy (RSD) 208, 273–6 Refsum’s disease 407, 410 INDEX repetitive transcranial magnetic stimulation (rTMS) in dystonia 218 in Parkinson’s disease 95 rest tremor 179 restless legs syndrome 60, 447–65 clinical presentation 450 descriptive terms 448 diagnosis 447–9, 448 differential diagnosis 449–50, 450 evaluation 450–1 genetics 454–5 history 447 major management categories 463–5 augmentation management 464–5 daily RLS 464 intermittent RLS 464 mild RLS 463–4 treatment resistant 464 management 455–65 evidence and rationale for treatment components 455–61 iron treatment 462–3 medical evaluation 455 medications and substances to be avoided 461 non-pharmacological treatment 461 pharmacological treatment 455–61 practical guidelines 461–5 treatment goals 463 pathophysiology 453–4 prevalence 451–3, 451, 451 reticular reflex myoclonus 364 Richardson’s syndrome 162 riluzole 53 rippling muscle disease (RMD) 266–7, 358 risperidone in dementia with Lewy bodies (DLB) 167 in Parkinson’s disease 80, 101, 104 in tic disorders 339 rivastigmine in dementia with Lewy bodies (DLB) 165–6 in Parkinson’s disease 97, 100, 104 ropinirol in insomnia 112 in multiple system atrophy 159 in Parkinson’s disease 49, 51–2, 53, 54, 55, 56, 57, 59, 61, 75, 76 rotenone 12 rotigotine in insomnia 112 in Parkinson’s disease 53, 54, 75 Roussy–Levy syndrome 199 RS-86 in progressive supranuclear palsy 164 safinamide 64 Schwartz–Jampel syndrome (SJS) 267–8 see also Tourette syndrome Segawa’s disease 206 segmental dystonia 205, 238 INDEX selective serotonin reuptake inhibitors (SSRIs) drug-induced dyskinesias 392–3 in multiple system atrophy 159 in Parkinson’s disease 61, 74, 95 selegiline in Parkinson’s disease 49, 51, 53, 54, 58, 61, 62, 74 in progressive supranuclear palsy 162 sensory motor retuning (SMR) in dystonia 218 serotonergic agents in progressive supranuclear palsy 162 serotonin antagonists in progressive supranuclear palsy 164 in tic disorders 333 serotonin syndrome 64, 74 sexual dysfunction in Parkinson’s disease 107 short intracortical inhibition (SICI) 209 shuddering, infantile 473 sialorrhea 84, 85 sildenafil in multiple system atrophy 161 in Parkinson’s disease 109 Sj€gren’s disease 360 o sleep disorders 110–15 sleep fragmentation 111–12 sodium picosulfate 110 somatosensory-evoked potential (SEP) testing 212, 212 sonic hedgehog (Shh) 43 spasmodic torticollis 195, 208 spasmus nutans 473 spastic gait and balance 433–6, 433, 435 chemodenervation 434–5 medical treatment 433 oral anti-spasticity agents 433–4 pathophysiology 433 surgical interventions 435–6 additional interventions 436 intrathecal baclofen 436 orthopedic surgery 436 selective dorsal rhizotomy 436 see also Parkinson’s disease, gait and balance disorders in; spastic gait and balance Spheramine 149–50 spinal myoclonus 357 spinal segmental myoclonus 364 spinobulbar muscular atrophy 410 spinocerebellar ataxia with axonal neuropathy (SCAN1) 409 spinocerebellar ataxias (SCAs) 410–11, 411 sporadic adult-onset ataxia of unknown origin (SAOA) 412 sporadic Parkinson’s disease 12–15 altered proteasomal function 14–15 apoptosis 15 Lewy bodies 13 pathogenic factors 12 proteolytc stress 12–13 square-wave dyskinesia 79 St John’s wort 61 startle syndrome 363, 364 stem-cell based therapies 147–9 stereotactic gamma-knife thalamotomy 187 stiff person syndrome 283–9 antibody-mediated endocrinopathies 285 anti-GAD antibodies 284 clinical presentation 283 diagnosis 287 differential diagnosis 286–7 immunopathology 284 neuropathology 285–6 neurophysiology 284 paraneoplastic encephalomelitis 286 pathophysiology 284 pharmacology 286 treatment 287–8, 289 variants 286 variation in clinical presentation 283–4 striosomes 38 stump dyskinesias 358 sub-acute akathisia 377–8 sub-acute parkinsonism 378–9 subcortical myoclonus 363 substantia nigra pars compacta (SNc) 38, 39 substantia nigra pars reticulata (SNr) 28 subthalamic nucleus (STN) 28 subthalamotomy 125 sudden onset sleep 112–15, 114 sulpiride in tic disorders 340 surround inhibition 210 Sydenham chorea (SC) 322–3 management 325–6, 326 sympathomimetics 395 symptomatic palatal tremor (SPT) 197–8 symptomatic therapy in progressive supranuclear palsy 162 synaptophysin 13 synaptotagmin X1 10 a-synuclein 3, 7–9, 12, 13, 14 aSp22 isoform 10, 11 b-synucleins 7, g-synucleins synphilin-1 10 systemic lupus erythematosus (SLE) 323, 326 tacrine in dementia with Lewy bodies (DLB) 167 in Parkinson’s disease 97 tacrolimus 396–7 tadlafil 109 tandospirone citrate in progressive supranuclear palsy 162 tardive akathisia 384 tardive dyskinesia 379–82 tardive dystonia 382–3 tardive myoclonus 384 tardive Tourettism 384 tau 7, 13 TCH346 52 tegaserod 109, 110 499 500 tetrabenazine in Huntington disease 302 in myoclonus 366–7 in tic disorders 340 d-9-tetrahydrocannabinol (THC) 340 tetrathiomolybdate in Wilson’s disease 255–6, 258 thalamic tremor 197 thalamotomy 124 tiapride in tic disorders 340 tic disorders 331 chronic 331 dystonic 355–6 indications 334 non-pharmacological treatment 334–5 contingency management 334–5 habit reversal 335 massed practice 335 relaxation 335 self-monitoring 335 neurobiological basis 332–3 pharmacologic treatment 335–41, 335 first tier 336, 337–8 other medications 339–40 potential future therapies 340–1 second tier 336, 338–9 surgical approaches 340 treatment of co-morbidities 341 transient 331, 475 treatment 334–41, 334 a-tocopherol 51 tolcapone 49, 58, 72, 73, 74 tolterodine 107 tongue-biting dystonia 325 topiramate in myoclonus 366 in tic disorders 337 torticollis 235 benign paroxysmal 474 post-traumatic 274, 274 spastic 208 Tourette syndrome 326, 331–2, 356 academic difficulties 332, 341 ADHD 332, 341 affective disorders: anxiety and depression 332, 341 associated problems 332 episodic outbursts and self-injurious behaviour 332, 341 general principles of treatment 333–4, 333 obsessive–compulsive disorder 332, 341 treatment of co-morbidities 341 tourettism, tardive 384 transcranial magnetic stimulation (TMS) in tic disorders 341 transcutaneous electrical nerve stimulation (TENS) 218 transdifferentiation 148 transplantation 132–3 treadmill training 423 INDEX tremor in Parkinson’s disease 25, 26 psychogenic 199, 481–2 tricyclic antidepressants (TCAs) drug-induced dyskinesias 392–3 in Parkinson’s disease 74, 92, 95, 100 trientine in Wilson’s disease 255, 258, 259 trimethobenzamide hydrochloride 77 trospium in multiple system atrophy 160 in Parkinson’s disease 107 trunk movement disorders 357–8, 356 b-tubulin 13 g-tubulin 13 tyramine cheese effect 64 Ubc6 10 UbcH7 10 UbcH8 10 ubiquitin 14 UCH-L1 3, 14 UCH-L1 9–10 urinary dysfunction in Parkinson’s disease 106–7 valproate drug-induced dyskinesias 391–2 in myoclonus 366 vanilloids 109 vardenafil 109 vascular choreas 322 vascular hemichorea (hemiballism) 125, 322, 324 venlafaxine in Parkinson’s disease 95 ventral tegmental area (VTA) 38 vesicular monoamine transporter 151 vigabatrin in myoclonus 366 virtual reality (VR) 425 vitamin B1 deficiency, ataxia and 412–13 vitamin B12 deficiency, ataxia and 413 vitamin E deficiency, ataxia and 413 wakefulness, disorders of 110–15 walking aids 423–4 Wernicke’s encephalopathy 412 West syndrome 472 Westphal variant of chorea 318 Whipple’s disease 355 Wilson’s disease 208, 251–60 hepatic presentation 252 pre-symptomatic siblings 253 screening and diagnosis 252–3 therapeutics 253–60 anti-copper drugs 254–6 hepatic failure 256–7, 256 hepatitis or cirrhosis 257 maintenance therapy 258–9, 259 neurologic/psychiatric presentation 252, 257–8, 257 INDEX pediatric patient 259 pregnancy 259–60 prognostic index 257 various phases 256–60 writer’s cramp 205, 209, 210, 236–7, 244 X-linked McLeod syndrome 318 yohimibine in orthostatic hypotension 160 zinc in Wilson’s disease 254–5, 258, 259 ziprasidone in Parkinson’s disease 104 in tic disorders 339 zolpidem in progressive supranuclear palsy 164 zonisamide in myoclonus 366 501 .. .THERAPEUTICS of PARKINSON’S DISEASE and OTHER MOVEMENT DISORDERS Therapeutics of Parkinson’s Disease and Other Movement Disorders Edited by Mark Hallett and Werner Poewe ©... 978-0-470-06648-5 THERAPEUTICS of PARKINSON’S DISEASE and OTHER MOVEMENT DISORDERS Edited by MARK HALLETT National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA and WERNER POEWE... Therapeutics of Parkinson’s Disease and Other Movement Disorders Edited by Mark Hallett and Werner Poewe © 2008 John Wiley & Sons, Ltd ISBN: 978-0-470-06648-5 The Etiopathogenesis of Parkinson’s Disease: