Movement Disorders: A Clinical and Therapeutic Approach Shyamal Kumar Das
INDEX
ABCDEFGHIKLMNOPQRSTUVW
A
Abnormal eye movements 104
Acquired basal ganglia disorders 126
acquired hepatocerebral syndrome 126
basal ganglia calcification 127
hypoxic-ischemic encephalopathy 127
magnetic resonance spectroscopy 127
methanol toxicity 127
Wernicke-Korsakoff syndrome 127
Aetiopathogenesis of Parkinson’s disease 176
Alternative therapies 206
acupuncture 211
ayurvedic medicine 206
exercise 212
folic acid and folinic acid 209
manual therapy 214
nutritional supplements 211
physical therapies 208
speech therapy 215
vitamin D and vitamin K2 210
vitamins 209
Amyotrophic lateral sclerosis 126
Approach to movement disorders 17
Approaches to a patient with sleep related movement disorder 145
history 145
investigations 145
physical examination 145
B
Basal ganglia 7
anatomy 7
amygdaloid complex 8
caudate nucleus 7
claustrum 8
lentiform nucleus 8
basal ganglia circuits 13
biochemistry 14
components 7
cross-sectional view 7
physiology 12
Behcet’s disease 44
Bell’s phenomenon 102
Bobble-head doll syndrome 525
Botulinum toxin 467
adverse effects 471
basic pharmacology 468
clinical applications 473
dosage range 475
immunogenicity 470
mechanism of action 469
Bradykinesia 27
C
Cardiac dysrhythmias 245
Cardiovascular dysfunction 242
Caudal vermis syndrome 448
Causes of dystonia 36
Cell divison 54
Cerebellar cortical degeneration 124
Cerebellar degenerative disorder 123
Cerebellar syndromes 448
Cervical dystonia 479
Chorea 25
Chromosomal basis of heredity 53
human chromosome 54
Churg-Strauss syndrome 43
Classification of genetic disorders 53
chromosomal disorder 53
multifactorial disorder 53
single-gene disorder 53
Cogwheel rigidity 27
Cortical control of saccades 95
Cortico-basal-ganglionic degeneration 102, 123, 171
differential diagnosis 173
imaging 172
pathology 173
D
Degenerative cerebellar disease 450
Diffuse Lewy body disease 170
differential diagnosis 171
imaging 171
management 171
pathology 171
Dimentia in Parkinson disease 182
Binswanger encephalopathy 192
clinical features 189
cognitive dysfunction 186
diagnosis 189
differential diagnosis 192
diffuse Lewy body disease 192
investigation 193
neurochemistry 188
neuropathology 188
pathophysiology 187
risk factors 187
treatment 193
Dopa responsive dystonia 279
clinical features 284
differential diagnosis 285
genetic basis 282
pathology 283
prognosis 289
treatment 289
Dopamine agonists 202
Dopamine receptor agonists 489
Dopamine receptor blockers 336
atypical neuroleptics 336
catecholamine β-agonist 337
targeted combined pharmacotherapy 337
typical neuroleptics 336
Drug induced dyskinesia 378
clinical features 379
epidemiology 379
management 385
pathogenesis 380
Dystonia 22, 78, 253
classification 253
clinical features 253
differential diagnosis 255
dystonia-plus syndromes 82
DYT11 85
generalized dystonia 256
genetics 78
mixed dystonias 82
predominantly focal 82
DYT7
predominantly generalized dystonia 80
DYT1 81
DYT2 81
DYT4 81
rapid-onset dystonia parkinsonism 86
secondary dystonia 258
segmental dystonias 256
types 255
x-linked dystonia parkinsonism 87
paroxysmal dyskinesias 88
E
Electrophysiology 459
dystonia 459
myoclonus 460
Epidemiology of movement disorders 33
Extrapontine osmotic myelinolysis 131
Eye movements 94
importance 94
F
Fahn-Marsden scale 299
Familial Parkinson’s disease 177
Friedreich ataxia 451
G
Gait disorders 107
examination 110
falls 118
collapsing falls 118
toppling falls 118
tripping falls 118
history 109
investigation 118
management 118
mechanism 108
neural control 109
physiology 108
syndromic classification 117
slow shuffling gait 117
veering gait 118
Gaze holding 97
Gene mapping 67
Genetic variation in individuals 65
mutations and polymorphisms 65
causes of mutations 67
DNA repair 67
mutation affecting gene-expression 66
nucleotide substitutions 66
somatic and germ-line mutation 67
unstable mutation 66
Genetics of Parkinson’s disease 69
alpha-synuclein 70
DJ-1 73
leucine rich repeat kinase 2 74
lysosomal type 5P-type ATPase 75
Parkin 71
pink 1 72
ubiquitin C-terminal hydrolase 72
Geste-antagonistique 20
Gilles de la Tourette syndrome 103
H
Hallervorden-Spatz disease 125, 312
diagnostic criteria 314
differential diagnosis 314
genetics 312
management 315
neuropathology 312
pathogenesis 313
Hemiballism 418
causes 418
clinical features 420
epidemiology 418
pathophysiology 419
prognosis 422
treatment 420
Hereditary chin tremor 396
Hoehn and Yahr scale 149
Holmes’s tremor 395
Human genome 55
central dogma 58
DNA structure 55
fundamentals of gene expression 57
gene structure 55
genetic code 60
organization 55
post-translational modification 60
transcription 58
translation 58
Human genome project 67
Huntington’s disease 103, 409
assessment 412
clinical presentation 409
diagnosis 412
epidemiology 409
genetics 413
management 415
pathogenesis 414
pathology 414
I
Idiopathic Parkinson’s disease 149
clinical features 151
clinical subgrouping 150
depressive illness 157
diagnosis 154
differential diagnosis 155
Dopa responsive dystonia 156
drug induced parkinsonism 156
investigation 157
biochemical investigation 157
imaging 157
genetic study 157
management 158
early 159
late 159
normal pressure hydrocephalus 157
Parkinsonism plus syndrome 156
post encephalitic parkinsonism 156
surgical management 160
vascular parkinsonism 157
Immunologic chorea 42
Infantile tremor syndrome 326
Involuntary movement disorders 133
K
Keyser-Fleischer ring 21, 266
Kinetic tremor 29
L
Leigh disease 128
Lewy bodies 151
M
Management of motor complications 198
Moersch-Woltmann syndrome 437
Molecular pathogenesis of Parkinson’s disease 75
Movement disorders 3
characteristics 19
clinical features 20
abnormal posturing 20
associated peripheral neuropathy 21
associated pyramidal tract involvement 21
associated sphincter disturbance 22
bradykinesia 20
cognitive dysfunction 21
gait abnormality 20
ocular examination 21
ocular movement disorders 20
rigidity 20
speech abnormality 20
systemic illness 22
tremor 20
physiology 3
Multiple system atrophy 122, 167
differential diagnosis 169
imaging 168
management 169
pathology 168
role of electrophysiology 168
Muscle cramp 433
Myoclonus 23, 400
classification 400
diagnosis 402
management 405
pharmacotherapy 407
N
Neurodegenerative disorder 48
dystonia 49
Parkinson disease 48
Neuroleptic malignant syndrome 381
Neuromyotonia 46
Non-neuroleptic induced dyskinesia 385
Non-schizophrenic disorders 370
O
Obsessive compulsive disorder 38
Occupational dystonias 293
aetiopathogenesis 293
clinical subtypes 295
diagnosis 297
musician’s focal dystonia 296
treatment 297
Ocular flutter and opsoclonus 100
Opsoclonus- myoclonus 46
Optokinetic eye movements 99
Organization of the saccadic system 96
Oromandibular dystonia 480
P
Pancerebellar syndromes 449
Parkinson’s disease 34
Parkinson’s disease and depression 228
characteritics 232
management 233
pathophysiology 232
prevalence 231
Parkinsonism plus syndromes 162
course and prognosis 167
differential diagnosis 164
etiology 166
imaging 164
pathology 164
Paroxysmal dyskinesias 303
diagnosis 309
management 309
pathophysiology 308
Paroxysmal exertion induced dyskinesia 306
Paroxysmal hypnogenic dyskinesia 306
Paroxysmal movement disorders in sleep 27
Paroxysmal non-kinesigenic dyskinesia 305
Patterns of single gene inheritance 63
autosomal dominant inheritance 64
autosomal recessive inheritance 64
mendelian inheritance 64
mode of transmission 63
X-inactivation 65
X-linked inheritance 65
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection 344
clinical features 345
etiopathogenesis 348
management 350
pathogenesis 347
Pediatric movement disorders 319
investigations 326
prognosis 329
surgical management 328
Pharmacological approach to treatment of movement disorders 487
choreic disorders 496
pathology 496
pharmacotherapy 497
dystonias 491
neurotransmitter abnormalities 491
pharmacotherapy 491
essential tremor 494
pathology 495
pharmacotherapy 495
myoclonus 492
neurotransmitter abnormalities 493
pharmacotherapy 494
Parkinson’s disease 488
pharmacotherapy 489
tic disorders 497
neurotransmitter abnormalities 497
pharmacotherapy 497
Post stroke movement disorders 510
blepharospasm 512
epidemiology 510
hand tremor 516
head tremor 516
hemiakathisia 513
hemiballism and biballism 512
hemibody tremor 516
hemichorea 511
limb shaking TIAs 514
myoclonus 512
post stroke dystonia 513
rubral tremor 516
thalamic tremors 515
tremor 515
vascular Parkinsonism 514
violent tremor 516
Post traumatic movement disorders 519
Postural tremor 28
Progressive supranuclear palsy 102, 122
Psychogenic movement disorders 357
clinical features 359
diagnostic criteria 367
epidemiology 357
management 368
prognosis 368
Q
Quality of life 503
R
Rabbit syndrome 385
Rest tremor 28
Restless leg syndrome 141
Rett syndrome 429
differential diagnosis 431
genetic studies 431
laboratory studies 430
management 432
prognosis 430
reasons 431
Role of brainstem nuclei 97
Role of genetics in medicine 52
Role of levodopa 199
Rostral vermis syndrome 448
S
Saccadic eye movement 94
Schönlein purpura 43
Schwartz-Jampel syndrome 437
Serotonin syndrome 382
Sexual dysfunction 241
Sleep and movement disorders 137
chemistry 138
neurobiology 138
stages of sleep 137
Sleep bruxism 462
Sleep related movement disorders 139
individual disorders 139
clinical features 140
epidemiology 140
etiology 140
pathophysiology 140
REM sleep behavior disorder 139
treatment 140
Smooth pursuit 98
Spasmodic dysphonia 483
Specific disorders related clinical approach 22
Spontaneous eye movements 104
Sporadic Parkinson’s disease 179
inflammatory action 182
mitochondrial dysfunction 181
oxidative stress 180
proteolytic stress 182
Stereotypic movement disorder 426
differential diagnosis 427
epidemiology 427
etiology 427
prognosis 428
treatment 429
Stiff-leg syndrome 46
Stiff-person syndrome 45
Sudomotor dysfunction 246
Surgery for Parkinson’s disease 219
deep brain stimulation 228
direct and indirect pathways 221
procedure of deep brain stimulation 225
globus pallidus interna 226
nucleus of thalamus 226
subthalamic nucleus 225
ventral intermedio-lateral 226
role of dopamine 221
role of surgery in Parkinson’s disease 222
pallidotomy 223
subthalamotomy 224
thalamotomy 223
stimulator hardware 226
target localization 227
Sydenham’s chorea 339
epidemiology 339
etiopathogenesis 339
pathology 340
pathophysiology 340
clinical features 341
diagnosis 342
treatment 343
Sydenham’s chorea 42
T
Tardive 383–385
akathisia 385
dyskinesia 383
dystonia 384
myoclonus 385
stereotype 383
tourettism 384
tremor 384
Tics disorders 24, 330
classification 331
clinical characteristics 331
differential diagnosis 334
treatment 335
Tools of human molecular genetics 60
molecular diagnosis 60
application of PCR in molecular diagnosis 62
restriction fragment length polymorphism 61
Southern blot 61
Tourette’s syndrome 42, 332
Tremor 28, 76, 391
differential diagnosis 397
treatment 397
types 393
cerebellar tremor 395
dystonic tremor 395
odd or atypical tremor 395
orthostatic tremor 396
palatal tremor 396
primary writing tremor 396
psychogenic tremor 396
voice tremor 395
U
Urinary dysfunction 240
V
Vergence system 99
Vertical gaze 99
Vestibular system 98
W
Whipple disease 104
Wilson’s disease 38, 103, 124, 261
ceruloplasmin 263
clinical presentations 264
diagnosis 267
differential diagnosis 270
epidemiology 262
hepatic manifestations 266
metallothionein 263
neuroimaging 267
pathogenesis 263
pathology 264
prognosis 277
treatment 273
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Chapter Notes

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1Movement Disorders: A Clinical and Therapeutic Approach2
Movement Disorders: A Clinical and Therapeutic Approach
Shyamal Kumar Das MD, DM Professor and Head, Department of Neurology In-charge, Movement Disorders Clinic Bangur Institute of Neurosciences and Psychiatry Kolkata, West Bengal, India
3
Published by
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Jaypee Brothers Medical Publishers (P) Ltd
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Movement Disorders: A Clinical and Therapeutic Approach
© 2008, Shyamal Kumar Das
All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editor and the publisher.
This book has been published in good faith that the material provided by contributors is original. Every effort is made to ensure accuracy of material, but the publisher, printer and editor will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only.
First Edition: 2009
9788184485264
Typeset at JPBMP typesetting unit
4To
My parents who have brought me on this earth
and gave me the basic education
My maternal uncle and aunty who have supported my technical education
and without their help I would not be a doctor
and
To my wife who has always helped me in my positive activity
and stood by me in critical time
5Contributors 8Foreword
There have been significant advances in our understanding of problems associated with movement disorders during the last decade. Movement disorders now have rightful claim to be considered as a subspecialty in Neurology.
Clinicians now recognize the importance of not only having a precise knowledge about different types of movement disorder but also to have a clear idea about the anatomical, physiological and biological processes behind these disorders.
With pouring in of huge amount of information about the disorder, it has become somewhat complicated and at times confusing particularly for the initiators.
Movement Disorders: A Clinical and Therapeutic Approach is an attempt to present a methodical approach towards understanding these disorders. Starting from the basics which include epidemiology, immunology and genetics along with anatomical, physiological and biochemical backgrounds, different movement disorders have been presented separately in separate chapters in a unique fashion. Few chapters such as eye movement disorders, gait disorders, sleep, depression and autonomic dysfunction have been included due to common clinical accompaniments with movement disorders.
Discussion about a place of a clinical approach to assess movement disorders will be found very useful to readers. Inclusion of a chapter on post-stroke movement disorder is a new idea and useful one.
Management of movement disorders is often difficult and unsatisfactory. A therapeutic approach of management of movement disorders with presently available treatment facility has been presented in the book. Inclusion of a chapter on use of botulinum toxin and discussion on alternative therapies in Parkinson's disease will be useful to readers.
Contributors of this book have done a commendable job in presenting up-to-date information about movement disorders—useful for general physicians, neurologists and specialists in the field of movement disorders.
Dr NN Sarangi
Ex-Professor and Head
Department of Neurology
Bangur Institute of Neurology
(Presently, Bangur Institute of Neurosciences and Psychiatry)
Kolkata, India
9Preface
My interest in movement disorders grew when I was first confronted with managing a patient with blepharospasm to send her more than 1,300 kilometers away for botulinum toxin injection. I was initially posted in a district center which is about 300 kilometers away from the heart of eastern metropolis, Kolkata (erstwhile Calcutta), the nearest Neuro Centre. Since my new posting in the Premiere Institute of Neurosciences in the state of West Bengal, I realized the need to cater patients who need botulinum toxin injection and subsequently, I along with one of my colleagues started seeing patients with movement disorders and organizing a clinic for botulinum toxin injection. The hospital authority also approved the establishment of a Movement Disorders Clinic.
I am grateful to Prof D Sen, Ex-Director, Prof NN Sarangi, Prof B Maity and Prof Trishit Roy, all subsequent Heads of the Department of Neurology and my colleague Prof PK Ganguly for cooperation. My special thanks go to Prof MK Bhattacharya, Prof P Tripathy, Prof SP Ghorai, Dr P Deb from the Department of Neurosurgery, Prof Ajit Duttamunshi, from the Department of Neuroradiology and Prof A Goswami and Prof B Das from the Department of Neuroanesthesiology for cooperation in establishing the clinic.
Subsequently, my visit and training in the Institute of Neurology, London, UK and Movement Disorders Clinic, Department of Clinical Neurosciences, University of Calgary, Canada gave me the requisite expertise to practice movement disorders.
Development of movement disorders as a subspecialty of neurology is only about three decades old, which is almost parallel to the development of newer pharmacological agents such as botulinum toxin, tetrabenazine, etc. in the market and subsequently providing benefit to the ailing patients. The advent of molecular genetic has rapidly explored the basic understanding of the disease process and presymptomatic diagnosis is now possible and beneficial in some conditions such as Wilson's disease. I am happy that a dedicated Institute of Neurosciences under the University of Kolkata has recently been established in Kolkata and we are collaborating with this new institute for genetic study.
Most important aspect of the movement disorders is that it is clinical based and video-recording helps in rapid review and better understanding of the different phenomenology of abnormal movements. We have a very strong clinical bias and keep a record of all the interesting cases, seen in movement disorders clinic.
At present, there are very few available textbooks on movement disorders which are costly and elaborate and mostly reference books. During my regular teaching in clinic and ward round, I realized the need of a short textbook which will show the step-by-step approach clinically to diagnose a case with movement disorder and manage the patient. With this principle, I have started writing this book and collecting manuscripts from my colleagues interested in this subject. I have tried to present the different conditions in a lucid way, providing increasing number of tables and figures. I feel that this will be extremely helpful for the postgraduates, post-doctoral residents and junior consultants.10
This book contains chapters which will be primarily helpful for the neurologists, neurosurgeons, pediatricians, psychiatrists and internists. All the chapters have been written by persons who were involved in dealing with cases of movement disorders or have interest in movement disorders. Thus, the blending of experience and literature support has enhanced the value of this book. Some of my colleagues who are basic scientists have written few chapters and I am sure that those chapters will be very beneficial to the readers.
I am indebted to the overseas writers who have agreed to write for this book and I am grateful to Prof Mark Hallett and Prof Oksana Suchowersky particularly. My acknowledgement goes to Dr Ranjit Ranawaya, Associate Professor, Department of Clinical Neurosciences, University of Calgary who has always constructively criticized and reviewed all the chapters for consistency.
I am indebted to Prof SK Shankar, Head of the Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore for supplying important photographs and to Dr Lele, Consultant Radiologist, Mumbai for supplying SPECT photographs.
I know that it will be beyond the capacity to include all updated information regarding movement disorders in this world of explosive information, because that is not my intention. So, I shall request the readers to consult journals, large textbooks and Internet for further up-to-date information if they want to learn more.
My gratitude goes to eminent neurologists in the city such as Prof Shyamal Sen, Prof A Dasgupta, Prof M Bhattacharya, Prof PK Basu, Prof A Mukherjee, and also various consultant neurologists from the city, who have helped me in sending cases of interest to this clinic and enriching me with newer and challenging experiences. I remember my teacher late Prof Bhaskar Raychaudhuri under whom I received my initial training and subsequently I owe to Prof JS Chopra, Ex-Head, Department of Neurology, PGIMER, Chandigarh for my post-doctoral training in Neurology.
I must admit the help rendered by my colleagues in the institute where I am working presently and the post-doctoral residents who are always keen to learn the different phenomenology and exploring underlying etiology. I must say that the desire to write this book also came from the difficulties I faced during teaching and realized the need of a book which will assist the students to learn the basics of movement disorders as well as the logic of treatment and explore the underlying etiology.
Shyamal Kumar Das
11Acknowledgements
I am indebted to Dr Amit Vyas, Dr Ashis Dutta and Dr Moumita Bagchi—all DM residents in Neurology for literary assistance.
I must acknowledge the help of Ms Nibedita Roy for secretarial assistance, Dr Punyabrata Barma, Dr Deep Das and Mr Arup Biswas for assistance in photography and Mr Ramswarup for video-recording.
I think that possibly this book would not have been published without the help of Jaypee Brothers Medical Publishers (P) Ltd, New Delhi.