PARKINSON''''S DISEASE AND MOVEMENT DISORDERS: DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN potx

CURRENT CLINICAL PRACTICE PARKINSON’S DISEASE AND MOVEMENT DISORDERS DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN Edited by CHARLES H ADLER, MD, PhD J ERIC AHLSKOG, PhD, MD HUMANA PRESS PARKINSON'S DISEASE AND MOVEMENT DISORDERS: DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN CURRENT CLINICAL PRACTICE Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician, edited by CHARLES H ADLER AND J ERIC AHLSKOG, 2000 Bone Densitometry in Clinical Practice: Application and Interpretation, by SYDNEY LOU BONNICK, 1998 Diseases of the Liver and Bile Ducts: Diagnosis and Treatment, edited by GEORGE Y WU AND JONATHAN ISRAEL, 1998 The Pain Management Handbook: A Concise Guide to Diagnosis and Treatment, edited by M ERIC GERSHWIN AND MAURICE E HAMILTON, 1998 Sleep Disorders: Diagnosis and Treatment, edited by J STEVEN POCETA AND MERRILL M MITLER, 1998 Allergic Diseases: Diagnosis and Treatment, edited by PHIL LIEBERMAN AND JOHN A ANDERSON, 1997 Osteoporosis: Diagnostic and Therapeutic Principles, edited by CLIFFORD J ROSEN, 1996 PARKINSON'S DISEASE AND MOVEMENT DISORDERS DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN Edited by CHARLES H ADLER, MD, PHD Consultant and Co-Director, Parkinson’s Disease and Movement Disorders Center, Department of Neurology, Mayo Clinic Scottsdale, Scottsdale, AZ; Associate Professor of Neurology, Mayo Medical School, Rochester, MN and J ERIC AHLSKOG, PHD, MD Chair, Division of Movement Disorders, and Consultant, Department of Neurology, Mayo Clinic Rochester, Rochester, MN; Professor of Neurology, Mayo Medical School, Rochester, MN HUMANA PRESS TOTOWA, NEW JERSEY Copyright © 2000 Mayo Foundation for Medical Education and Research For additional copies, pricing for bulk purchases, and/or information about other Humana titles, contact Humana at the above address or at any of the following numbers: Tel: 973-256-1699; Fax: 973-256-8341; E-mail: humana@humanapr.com or visit our website at http://humanapress.com All rights reserved This book is protected by copyright No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without written permission from the Mayo Foundation As new scientific information becomes available through basic and clinical research, recommended treatments and drug therapies undergo changes The authors and publisher have made all reasonable attempts to make this book accurate, up to date, and in accord with accepted standards at the time of publication The authors, editors, and publisher are not responsible for errors or omissions or for consequences from application of the book, and make no warranty, expressed or implied, in regard to the contents of the book Any practice described in this book should be applied by the reader in consultation with a physician and taking regard of the unique circumstances that may apply in each situation The reader is advised always to check product information (package inserts) for changes and new information regarding dose and contraindications before administering any drug Caution is especially urged when using new or infrequently ordered drugs Nothing in this publication implies that Mayo Clinic endorses the products or equipment mentioned in this book All articles, comments, opinions, conclusions, or recommendations are those of the author(s) and not necessarily reflect the views of the publisher or of Mayo Clinic This publication is printed on acid-free paper ANSI Z39.48-1984 (American National Standards Institute) Permanence of Paper for Printed Library Materials Photocopy Authorization Policy: Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by Humana Press Inc., provided that the base fee of US $10.00 per copy, plus US $00.25 per page, is paid directly to the Copyright Clearance Center at 222 Rosewood Drive, Danvers, MA 01923 For those organizations that have been granted a photocopy license from the CCC, a separate system of payment has been arranged and is acceptable to Humana Press Inc The fee code for users of the Transactional Reporting Service is: [0-89603-406-2/98 $10.00 + $00.25] Printed in the United States of America 10 PREFACE The field of movement disorders is relatively broad, encompassing disorders of increased movement, such as tremors, dystonia, and tics, to disorders characterized by a paucity of movement, such as Parkinson’s disease Our understanding of the pathogenic mechanisms and our treatment options are expanding at a rapid pace This expansion ranges from the medical and surgical advances in treating Parkinson’s disease to the flood of genetic abnormalities that have now been found to cause various movement disorders Although many patients are seen by the movement disorders specialist in neurology clinics around the country, most of these patients receive their follow-up care from a primary care physician or “general” neurologist who must be versed in the characteristics and treatment plans of this diverse group of disorders The major goal of Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician is to distill this immense amount of information and to educate the practitioner about the many facets of the movement disorders field We believe that this book fills a large void, since most texts on movement disorders are more detailed and geared toward the specialist We have asked the chapter authors to emphasize the clinical characteristics of each disorder, discuss the differential diagnosis and the diagnostic testing, and then outline the various treatment options, as if they were teaching during a preceptorship in their clinic To this end, we have not designed the book to be an exhaustive review of each topic; rather, it takes a general approach to each subject We have avoided referencing each statement; a short list of further recommended reading sources is given at the end of each chapter The purpose of this text is to help the practitioner distinguish which disorder is being encountered, give a basic understanding for test and treatment options that are required, and synthesize any recommendations made by a consulting specialist As the movement disorders specialist becomes busier and insurance regulations limiting specialty referrals increase, the burden of caring for these patients by the primary care physician will continue to grow Thus, we hope that this text will offer the reader full confidence in approaching patients with movement disorders The text is organized into five sections: basic diagnostic principles, Parkinson’s disease, other parkinsonian disorders, hyperkinetic movement disorders, and other movement disorders In Chapter of Section A: Basic Diagnostic Principles, Dr J Eric Ahlskog provides an extensive overview on the neurologic examination Since movement disorders can involve all parts of the neurologic system, a detailed neurologic examination is often imperative when these patients are evaluated Changes in speech often occur in many of the disorders, and the speech characteristics may provide important diagnostic clues In Chapter 2, Dr Joseph Duffy describes the varieties of motor speech abnormalities that may be encountered and provides a systematic approach to their recognition Given the frequency of Parkinson’s disease (PD), the tremendous advances made over the past two decades in understanding the disease and its treatment, and the debates on the “best” way to treat the patient, we have devoted 12 chapters to this entity in Section B: Parkinson’s Disease In Chapter 3, Dr Howard Hurtig discusses the pathophysiology, neurochemistry, and neuropathology of PD This is followed by Dr Richard Dewey’s v vi Preface description of the clinical characteristics of PD in Chapter 4, outlining not only the typical features, such as rest tremor and slowness of movement, but also clinical signs suggesting an atypical form of parkinsonism What causes PD? The intriguing search for etiologic answers has generated volumes of studies and papers, sometimes with conflicting results This information is distilled in two chapters The epidemiologic studies of PD, which have generated multiple clues to the etiology, are reviewed by Dr Demetrius Maraganore in Chapter This chapter covers reported risk factors and the role of genetics (including the defects in the synuclein gene) in addition to basic incidence and prevalence data On the other hand, basic bench research has produced multiple lines of evidence for a variety of possible causal factors Etiologic hypotheses generated from knowledge of biochemical mechanisms are comprehensively reviewed by Dr Peter LeWitt in Chapter This chapter includes discussion of the roles of oxidative stress, mitochondrial dysfunction, and neurotoxins and addresses etiologic mechanisms that have received less publicity, such as possible autoimmune and infectious causes We have divided the discussion of the medical treatment of PD into three chapters The issue of whether our current drug arsenal includes medications that will slow the progression of PD is hotly debated, and in Chapter 7, Dr Ahlskog tackles the theories and practical issues for the practitioner Chapter 8, also by Dr Ahlskog, is devoted to the various treatment options available for the patient with newly diagnosed PD This includes decision-making regarding the use of levodopa, dopamine agonists, and numerous other agents The complicated issue of how to treat the patient with more advanced PD is covered by Dr Ryan Uitti in Chapter Patients with PD also have nonmotor manifestations that can be as disabling as the tremor and bradykinesia The sleep problems of PD, including insomnia and daytime drowsiness, are addressed by Dr Cynthia Comella in Chapter 10 Neurogenic bladder and bowel problems and symptomatic orthostatic hypotension are common issues in the PD clinic; these autonomic problems and treatment strategies are covered by Dr Bradley Hiner in Chapter 11 Dementia, psychosis, and depression can be overwhelming factors in the patient with more advanced PD, and Dr Erwin Montgomery discusses these in Chapter 12 Currently, the most visible topic concerning PD is surgical treatment, which has made newspaper and television headlines for the past several years In Chapter 13, Dr Kathleen Shannon discusses neurosurgical intervention, including pallidotomy, thalamotomy, deep brain stimulation, and cerebral transplantation, and reviews the prospects for the future She provides guidelines on which patients may benefit and which of the different procedures may be appropriate for a given patient Therapy for patients with PD does not end with medications and surgery; Chapter 14, written by Drs Padraig O’Suilleabhain and Susan Murphy, addresses adjunctive treatments They include nutrition and dietary issues, which are especially important in advancing disease They also address the role of physical therapy in management of parkinsonian motor problems All disorders characterized by slowness of movement are not PD, and in Section C we have devoted six chapters to discussing these other disorders In Chapter 15, Drs Eric Molho and Stewart Factor cover secondary causes of parkinsonism, such as vascular, toxic, and traumatic etiologies; they also provide a practical strategy for the workup of parkinsonism Among the more common neurodegenerative disorders sometimes mistaken for PD is progressive supranuclear palsy; the key clinical signs and points that Preface vii separate this disorder from PD are covered by Dr Mark Stacy in Chapter 16 When patients have cerebellar signs, prominent autonomic dysfunction, or resistance to dopaminergic therapy, one must consider the multiple system atrophies discussed by Dr James Bower in Chapter 17 Inherited cerebellar disorders, including the autosomal dominant spinocerebellar ataxias, sometimes resemble sporadic multiple system atrophyand occasionally PD; these familial ataxic syndromes are reviewed by Dr Bower in Chapter 18 Corticobasal degeneration may resemble PD early in the course The clinical hallmarks that allow differentiation are covered by Dr Brad Boeve in Chapter 19 The final chapter in this section, Chapter 20, by Dr Richard Caselli, describes the various primary dementing disorders, including Alzheimer’s disease, that often include components of parkinsonism Section D begins the discussion of disorders characterized by too much movement, or hyperkinetic movement disorders All the chapters in this section address the characteristics of the individual disorders, diagnostic considerations, and treatment options We begin by a discussion of the most commonly encountered movement disorder, tremor Chapter 21, by Dr Joseph Matsumoto, describes the different types of tremor and how to differentiate and treat them Chapter 22, by Dr Jean Hubble, goes into further detail about the most commonly seen tremor, essential tremor Dystonia is a more common disorder than is often appreciated, occurring in adulthood as torticollis, blepharospasm, writer’s cramp, and other focal or segmental dystonias These along with generalized dystonic conditions, including primary torsion dystonia developing in childhood, are reviewed by Dr Daniel Tarsy in Chapter 23 Hemifacial spasm is sometimes mistaken for facial dystonia; this disorder, due to compression of the seventh cranial nerve, is discussed by Dr Mark Lew in Chapter 24 The dancing movements of chorea have origins ranging from inherited (Huntington’s disease) to infectious (Sydenham’s chorea) causes Clinical characterization and treatment are covered by Dr John Caviness in Chapter 25 Tardive dyskinesias are sometimes confused with primary choreiform syndromes These iatrogenically induced conditions are discussed by Dr Kapil Sethi in Chapter 26 The lightning-like jerks of myoclonus occasionally cause diagnostic confusion; if repetitive, they may resemble tremor or the phasic movements seen in some dystonic conditions In Chapter 27, Dr Caviness discusses diagnostic criteria, categorization, and treatment of myoclonus Simple spasm of muscle may have a wide variety of causes, ranging from peripheral to central nervous system origins In the most elementary sense, the concept of muscle spasm should include the sustained muscle contraction state of dystonia Primary disorders characterized by muscle spasm, however, have their own distinguishing features, which separate them from primary dystonias These disorders of muscle spasm, including the prototypical condition, stiff-man syndrome, are discussed by Dr Michel Harper in Chapter 28 The most common movement disorder of childhood is that of tics This problem, however, is not confined to children and occasionally confronts internists with adult practices The spectrum of motor and other tics, as well as the constellation of symptoms that make up Tourette’s syndrome, is the topic of Chapter 29 by Drs Kathleen Kujawa and Christopher Goetz A disorder that has gained much recognition in the past few years is restless legs syndrome, discussed by Drs Virgilio Evidente and Charles Adler in Chapter 30 Dr viii Preface Adler then covers the various uses of botulinum toxin (Chapter 31), an injectable agent that reduces movement and has application for treating multiple different movement disorders This drug has revolutionized the treatment of dystonia and certain other hyperkinetic movement disorders We conclude this book with Section E, which covers other movement disorders, those that not fit well into previous sections Dr Katrina Gwinn-Hardy, in Chapter 32, discusses the autosomal recessively inherited disorder Wilson’s disease, which can present with hyperkinetic or bradykinetic features This is critical to diagnose, since treatment is available; if unrecognized, it can result in irreversible neurologic damage and even death by hepatopathy Abnormal gait is a common component of many neurologic disorders and especially the conditions covered in this text Recognition of the prototypical types of gaits is critical to diagnosis Dr Frank Rubino applies his years of clinical savvy in Chapter 33, which addresses the broad topic of gait disorders Commonly, patients attribute their condition to some prior trauma How often does this occur? Although subject to much debate, the topic of trauma-induced movement disorders is covered in Chapter 34 by Dr Sotirios Parashos Possibly the most difficult problem for clinicians is that of a psychogenic movement disorder In Chapter 35, Drs David Glosser and Matthew Stern have written a very reader-friendly review of what the practitioner should consider when approaching these patients We conclude this book with an appendix that lists many of the organizations and foundations devoted to the disorders discussed in the book We wish to thank all the authors for their hard work and excellent contributions We thank the Mayo Clinic Section of Scientific Publications, specifically Roberta Schwartz, Marlené Boyd, Reneé Van Vleet, and John Prickman, and Humana Press, specifically Paul Dolgert, for their diligent effort in publishing this text We both would especially like to thank our wives, Laura Adler and Faye Ahlskog, as well as our children, Ilyssa and Jennifer Adler and Michael, John, and Matthew Ahlskog, for their support, encouragement, and patience during the long hours it took to complete this book We hope that our combined efforts have created a readable text for the primary care physician that has distilled the tremendous advances made in the movement disorders field leading up to the millenium Charles H Adler, MD, PHD J Eric Ahlskog, PHD, MD CONTENTS Preface v A BASIC DIAGNOSTIC PRINCIPLES 1 Approach to the Patient With a Movement Disorder: Basic Principles of Neurologic Diagnosis J Eric Ahlskog, PhD, MD Motor Speech Disorders: Clues to Neurologic Diagnosis 35 Joseph R Duffy, PhD B PARKINSON’S DISEASE 55 What Is Parkinson’s Disease? Neuropathology, Neurochemistry, and Pathophysiology 57 Howard Hurtig, MD Clinical Features of Parkinson’s Disease 71 Richard B Dewey, Jr., MD Epidemiology and Genetics of Parkinson’s Disease 85 Demetrius M (Jim) Maraganore, MD Parkinson’s Disease: Etiologic Considerations 91 Peter A LeWitt, MD Medication Strategies for Slowing the Progression of Parkinson’s Disease 101 J Eric Ahlskog, PhD, MD Initial Symptomatic Treatment of Parkinson’s Disease 115 J Eric Ahlskog, PhD, MD Advancing Parkinson’s Disease and Treatment of Motor Complications 129 Ryan J Uitti, MD 10 Sleep and Parkinson’s Disease 151 Cynthia L Comella, MD, ABSM 11 Autonomic Complications of Parkinson’s Disease 161 Bradley C Hiner, MD 12 Treatment of Cognitive Disorders and Depression Associated With Parkinson’s Disease 175 Erwin B Montgomery, Jr., MD 13 Surgical Treatment of Parkinson’s Disease 185 Kathleen M Shannon, MD ix Index motor control systems, 23t neurologic examination, 21-25 Cephalic tetanus, 354-356 Cerebellar ataxia, MSA and, 238 Cerebellar disorders, 23 gait, W RAMS, 12 Cerebellar outflow tremor, 275 Cerebellar tremor, severe, 279 Cerebe t ium, 6f Cerebral transplantation, PD and, 128, 192194 Cerulaplasrnin, WD and, 402,403 Cervical dystonia, 30CL302 abnormal head postures, 300,301f BTX, 389-390 Chaddock sign, 10 Charcot-Mane-Tooth disease, 360 Chorea acquired hepatocerebral degeneration 328 assessment, basal ganglia lesions, 326 causes, 322t Graves' disease, 327 hyperkinetic dysarthria, 47 hyperthyroidism, 327 levodopa, 123-1 24 lupus erythematosus, 327-328 neuroacanthocytosis 329 nonprogressive, 329 oral contraceptives, 326-327 paroxysmal, 329 polycythemia Vera, 328 senile, 329 Sydenharn's, 327 Chorea gravidarum, 328 Choreic gait disorders, 42 Choreifom dyskinesias, 139 Chronic acquired hypoparathyroidism, 22 Cinnarizine, 214 Cirrhosis, WD and, 398 Cisapride, 168 Clasp-knife reflex, I0 Classic orobuccolingual dyskinesia (OBLD), 332, 333t Cionazepam dystonia, 308 ET, 29 hereditary hyperekplexia, 357 Huntington's disease, 325 myoclonus, 350 RBD,157 RLS, 382 sti ff-man syndrome,354 TI?, 336 WD tremor, 280 46 Clonidine ET, 292 GTS, 367,370 RLS, 383 Clonus, 10 Clostridium bo~tdlinum, 385 C/ostridium tetani, 354 Clozapine adverse effects, 158 ET,292 PD, 213 refractory psychosis, 180 TD, 335,337-338 Clozaril, see Ctozapine Codeine, RLS and, 382 Cogentin, see Benztropine Cognitive impairment, 65, 78 Cogwheel rigidity,9 Cogwheeling, 74, Communicating hydrocephalus, 18 Communication, PD and, 205 Compazine, see Prochlorperazine Compression stockings, 166 Corntan, see Entacapone Conjugate gaze disorders, 15-1 Constipation diet, 201-202 PW, 167-168, 168t Constructionat apraxia, 269 Cowersationai speech, motor speech disorders an439 Coordination, assessment of, 12 Copper food content, 406t WD, 402,403,404-408 Coproialia, 366 Copropraxia, 366 Cortical degeneration syndromes, asymmetrical, 266-267 Cortical dementia, 263-264 Cortical reflex myoclonus, 343 Cortical sensory lass, CBD and, 255 Corticobasal drgeneratjon (CBD), 253-26 clinical features, 254-257,254, 257t clinicopathologic heterogeneity, 260 differential diagnosus, 254-260 laboratory findings, 258 [ management, 26&26 neuropathology, 258-259 parkinsonism, 268-269 PD,83 Corticobasal ganglionic degeneration, 232 Corticospinal system, 23 Corticospinal tracr, origins of, 5f Cramp-fasclculat~on syndrome (CFS), 358-359 Cramps, 33,357-358,359t benign, 358 occupational, 303 pwipberal neuropathy,19 writer's, 33, 303 Cranial dystdriia, 302-303 Cranial musculature, ET and, 284 Cranial reflexes, 10-1 Creutzfeldt-Jakobdisease, 250 myoclonus, 342 Cyanide intyxicahon, PD and, 215 Cycad flour, PD and, 88,202 Darvon, see Propoxyphene DATATOP, 1 0-1 1,200 Day-night reversal, 158-159 Deep brain stimuiation, Pn and, 128, 19%192 Deep tendon reflex, 10 Delayed onset sleep, PIS and, 152-153 Delirium tremens, 278 Dementia, 263 CBD,255 PD, 78,101, 135 Dementia complex, parkinsonism and, 267 Dementia syndromes, 2641 Dernser, see Metyrosine Dentatorubropallidoluysian atrophy (D'RPLA), 249,326 Depakote, see Valproic acid DeprenyI, PD and, 110-1 1 Depression Huntington's disease, 325 PD, 78,181-182 sleep disorders, 156 Desipramrne, tics and, 37 Detrol, see Toltedine Diazepam RLS, 382 stiff-man syndrome, 354 DID response, 140-141, I41f Diet, PD and 197-202 Dietary supplernentatlon,osteoporosis and, 20 Diffuse Lewy body disease (DLRD) myoclonus, 342-343 parkinsonism, 26S270 Dilantin, see Phenytoin Diltiazem, TD and, 336 Dirty tie sign, 230 Disequilibrium, PI3 and, 163, 163f Dishing, 323 Dizziness, PD and, 163, A3f Domperidone, nausea from, 123 Dopamine, 63-44 Dopamine agomsts adverse e f f d s , PD, 107-108 advancing treatment, 147-148 early use, 12 initial treatment, 125-1 26, 125t with levodopa, 108 Dopamine blocking agenrs (DBAs), TD and, 331-332 Dopamine receptor blockers dystonia and, 308 Dopaminerg~c agents, sleep and, 155 Dopaminergic agonists, adverse effects of 179 Dopatnmergic synapse, d i a ~ a m 74f , Dopa-responsive dysroni~ (DRD), 306 Double simultaneous stimulation, 15 Doxazosin, 71 Doxorubicin, HFS and, 17 Dressing, PD and, 205 Dressing apraxia, 26%269 Driving, PD and, 205 Drooling excessive, 169 WD, 398 h g - i n d u c e d dystonia, Drug-induced parkinsonism, 80, 88,211-2 14, 213t-214t, 227 Dysarthria, 3%50,40t ataxic, 45 flaccid, 3%40,44 hyperkinetic, 47-49 mixed 50 spastic, 4445 unilateral upper motor neuron, 49-50 WD, 398,40 Dyskinesias, 123-124 belly dancer, extremity trauma from, 433 hiphasic, 140-141, 14 1f choreiform, 139 classic orobuccolingual, 332, 3 dystonic, 139 levdopa-induced, 134, 139-140 orobucwlingual, 32-33 paroxysmal nocturnal, from head trauma, 430 pe&-dose, I3+140,140f-14 f trttdive, 32,33 1-338 epidemiology 33 1-332 national organizations, 456 treatment, 139-142 Dyskinesias while awake (DWA), 375 Dysphagia CBD, 257 PD,1 68- 169 Dysphonia adductor spasmodic, 303 spasmodic, 48 Dystonia, 10,293-310 assessment, 30 CBD, 255 cervical, s abnormal head postures, 300,30 f BTX, 389-390 ckassification, 298,298t-299t Index cranial, 302-303 defined, 27 I dopa-responsive, 306 drug-induced, evaluation, 307 extremity trauma, 432 finger flexion, 304 focal, 304-305 head trauma, 429 hereditary progressive, 306 hyperkinetic dysarthria, 48 idiopathic torsion, 297 PMD, 4 M intorted foot, laryngeal, 48, 303 BTX, 390-391 limb, 303-304 ETX, 391 lingual, 303 national organizations, 453-454 oromandibular, 302 BTX, 390 peripheral neumpathy, 19 pharyngeal, 303 primary, 29R-304 primary focal, 299-304 lower extremity, 304 primary generailzed, 298499 secondary, 304307 acquired structural lesions, 304-305 spinal trauma, 4 tardive, 334 national organizations, 456 treatment, 336337 tarsion tics, 367 vs tardive d ystonia, 34 treatment, 307-308, 309t truncal, 304 VS, cramps, 33 vs tremor, 28 281 WD,40 writer's, 33 Dystonia musculorum deforrnans, 29%299 Dystonic dyskinesias, 139 Dyston~cgait disorders, 42 Dystonic tremor, 30 Early Parkinson's disease (PD), presenting features of, 72-73, 72f Echolalia, 366 Eldepryl, see Selegilint Elderly anticholinergics, 130, 179 disequilibrium, 163, 163F falls, 41 gait disorders, 412 hypotension, 13&13 Electroconvulsive therapy (ECT), 182 Encephalitis lethargica, 58-59, 81,89,220 Entacapone 148-1451 drug interacbons, 165 Environmental factors, PD and, 93 Environmental toxins, PD and, 88 Eosinophilia-myalgia syndrome, myoclonus and, 348 Ephedrine, 166 Epilepsia partialis continua, vs tremor, 28 Epilepsy focal, vs HFS 15 juvenile myoclonic, 34 progressive mywlanic, 342 Epflgtic rnyoclanus, diagnosis, 34 1-342 Episodic ataxias, 249-250 Erythropoictin, 167 Essential blepharospasrn, 302 Essential myoclonus, diagnosis, 341 Essential tremor (ET), 278, 283-295 clinlcal presentation, 284-287 body regions affected, 284,283 disease onset and progression, 285 factors influencing, 285 clinical variants, 286287,287t diagnostic criteria, 28Tc diagnostfc pitfalls, 286 disabilities, 286, 286t etiology, 283-284 genetics, s pathophysiology, 287-288 PD, 287,288t prevalence, 283 twatment, 289-294 Estrogen, PI3 and, 87-88 Exaggeratedstartle syndromes, 349 Excessive drooling, PD and, 169 Executive function, 65 Exercise, PD and, ~ Extinction burst, 443 Extrapyrarnidal disorders, motor neuron disease and, 20 Extrapyramidal system, 7f, 25 Extraventricular obstructive hydrocephalus (EVOH),218 Extremity trauma, 43 3 Eye movements, 15-1 apraxia, 16 conjugate gaze disorders, S F nystagmus, 17 opsoclonus, 17 F Facial nerve, flaccld dysarthrfa and, 40,44 Falls elderly, 17 PD, 204 PSP,230-23 I Familla1 adult+nset spinocerebellar degcnmations, 243-252 c1mical classification, 243244,244t genetic classification, 244-245 therapy, 25 1-252 types, 245-25 Famil~al basal ganglia calcification, 222 Familial episodic ataxia, 360 Family hishry, see Genetic factors Fasciculations, 358,359t Fingcr flexion dystonia, 304 Flaccid dysarthria, 39-40,44 Elavoxatc hydrochloride, 170 Flomax, see Tamsulosin Fiorinef, see Fludrocortrsoric Fludrocortisone, 166 Flunarizine, 14 ET, 292 Fluphenazine GTS, 370 Huntington's disease, 325 Flycatcher" tongue, 323 Focal dystonia, 304-305 Focal epilepsy vs HFS, 15 Focal rnyokymia, 359-360 Foot, intorted, dystonia and, Fragmented sleep, PD and, 152-153 Freezing, 142,204 Freezing gait, 76-77, 76f 135 Friedre~ch's ataxia, 245-247 Frontal gait disorder, Frontoparletal praxis circuits, 23-25 F~ontotempord syndromes, parkinsonism and, 266 F waves, HFS and, 14 PD,7&77 peripheral neuromuscular system, 42 4 PMD, 447 primary progressive freez~ng 419 psychogenic, 424 voluntary muscle disorders 42-24 Galt freezing, 76-77, 76f, 135 Gait syndromes, classification of, 41 8t Gait training, PD and, 204 Gastrointestinal dysautonomia, 167-1 69 Gaze disorders, conjugate, Gegenhalten, 10,74,2 17 Gender, PD and, 88 Gene-environment interaction, PD and, 89 Generalized rnyokymia 359-360 Gene therapy, PD and, 194-195 Genetic factors o f PD,88-89,97-98,206 Genetic testing for WD, 404 Gerstmann-Strussler-Scheinker disease, 250 Gestctral apraxia 268 Gilles de la Tourette's syndrome (GTS), 365-37 clinical features, 365-366 d~agnostic assessment, 368 differential diagnosis, 367368,368t etiology, 366 pathophysiology, 367 prognosis, 37 ttearment, 369t, 370-371 Glabellar response, 1E Glutamate excitoxicity, PD and, 105, 12 Graphesthesia, 15 Graves' disease, chorea, 327 Guam disease, 202 Guillain-Bad syndrome, 280 Guillain-Mollaret triangle, 49 G H Haldol, see Haloperidol Hallucinations, PD and, 78: 176 Halluc~natlons syndrome, sleep and, 157 158 Haloperidol GTS,367,370 Huntington's disease, 325 Handedness, ET and 285 Hands, ET and, 284 Handwriting, ET and 284 FIarding classification, ADCA and 4 Harmal~ne, 288 Head trauma, 42 8-43 Hemidystonia, 304305 Remi facia t spasm (HFS) I 3-3 assessment, 19-20 BTX, 316317,391 diagnostic testing, 14 differential diagnosis, 15-3 16,315t medical therapy 16.3 17t pathophysiology, 14-3 I5 surgery, 17-3 18 Gabapentin ET, 292 RLS, 383 WD tremor, 2R0 Gait agc effects, 17,4 6f cautious 419 cerebeliar dtsardcrs, M freezing, 7677, 76f parkinsonian, 420 peripheral neuropathy, 19 spastlc, 19-420 spastic hemrparetic, 419 Gait disorders, 41 1412,414t choreic, 421 dystonic, 42 elderly, 412 frontal, 41 7-419 initial evaluation, 15-415.41 5t management algorithm, 422f 423f neuropathy, 42 1-422 Index Hemiparkinsonism-hemiatrophy, 221 ~ernolytlc anemia, WD and, 400 Herbicides PD and, 88 Hereditary hyperekplexia, 357 Hereditary progressive dystonia, 306 Heredodegenerative diseases, 305,305t Hoehn and Yahr Scale 79, 79t Huntington's disease, 222, 32 1-326 autosomal dominant tnher~tance, 323 chorea, 322-323 diagnosis, 324 mental status, 323 rnyoclonus, 342 national organizations, 454 paskinsonism, 268 pathophysiology, 323-324 tics, 367 treatment, 6 Hydrocephalus communicating, 18 extraventn'cular obstructive, I S intraventricular obstructive, 18 nonobstructive, 18 normal pressure, secondary parkinsonism, 21&220 h-Hydroxydopamine, 94 5-I-Iydroxytryptophan, myoclonus and, 350 Hyoscyamine, I70 Hyperactive detmsor, PD and, 170 Hyperekplexia head trauma, 430 hereditary, 357 Hyperhidrosis PD and, 172 Hyperkinetic disorders, 2S34 irresistible internal urge, outside conscious awareness, 27-34 Hyperkinetic dysarthria, chorea, 47 dystonia 48 palatopharyngolaryngeal myocEonus, 49 tremor, 4-9 Flyperkmetic movement disorders, 271-272 Hyperthyroidism, chorea and, 327 Ilypoact~ve detmsor, PD and, 1.70-171 HypogIossal nerve, flaccid dysarthria and, 44 Hypokinetic dysarthria, 46 CBD, 257 Hypometric saccadcs, 15-16 Hypoparathyroidism, chronic acquired, 22 Hypotension age factors, 130-13 orthostatic, see Orthostatic hypotension postural, MSA and, 237-238 Hypothermia, PD and, I72 Hypotonia, Hypoxla, rnyocfonus and, 343 Hytrin, 171 465 I Idazoxan, see Terazosin Idiopathic parkinsonism, see Parkinson's disease Idiopathic forsion dystonia, 297 PMD, 44-47 Idiopathic torticollis, clinical characteristics of, 431,4311 Imipramine, 170 RLS, 383 Impaired muscle relaxation syndromes, 333-3 34 hdomethacin, 167 Infectious parkinsonism, 19-22 I , 220t rnfluenza A, 99 Intention tremor, 275-276 Insomnia PD, 143, 153, 154t, 155 secondary, 55-1 56 Internet, 45 PD, 206207 Intorted foot, dystonia, Intraventticuiar obstructive hydrocephalus (IVOH), 218 Iron deficiency anemia, RLS and, 336 Isaacs' syndrome, 360 Isoproterenol, drug interactions with, 165 Isoptin, see Verapamil Isuprel, see Esoproterenol J Jerk nystagmus, Jobst stockings, 166 Jumping stump extremity trauma, 433 Juvenile myoclonic epilepsy, 34 K Kayser-Fleischer rings, 368 WD, 399;403 Klonopin, see Clonazepam L Laryngeal dystonia, 48, 303 BTX, 390-391 Laterocollis, 300 Lead-pipe rigidity, 74 Leg jitters, 373 Levodopa AD, parkinsonism and, 265 adverse effects, 130-13 , 179 CBD,255,260 insomnia, 154t MSA, 240 PD administration, 131-1 32 delaying, 106I07 early use, 12 466 formulation, 132 initial therapy, 17 initial treatment, 11%I22 limiting dosage, 107 liquid form, 138, 147 suboptimal response, 136137,137t wearing off, 137-138 PSP, 233 side effects, I2%124 -'sleep, 155 toxicity, 108-109 Levodopa-induced dyskinesias, 134, f 3%14Q Lewy bodies, 50, 61f, 232 parkinsonism, 264-266 Limb apraxia, 268 Limb dysronia, 303304 BTX, 391 Lingual dystonia, 303 Liorewl, see Baclofen Livw biopsy, WD and, 403 Liver transplantation, WQ and, 408 Local tetanus, k Locomotion, anatomy and physiology of, 12-4 13 Lodosyn, see Carbidopa Lower body parkinsonism, 217 Lupus erythematosus, chorea and, 327-328 Lynnox-Gaustaut syndrome, 34 M Machado-Joseph disease, 222 Madopar, see Benserazide-levodopa Male impotence MSA, 241 PD, 171 Malignant vasovagal syndrome, 164 Manganese poisoning, PD and, I Meals, timing of with PD, 198-200 Meige's syndrome, 302 vs HFS, 16 Melatonin, 156 Mephenesin, ET and, 292 Mestinon, see Pyridostignine MetaboIic disease, tremors and, 278 Metabolic myopathies, 36 Methanol intoxication, PD From, 216 Methazolamide, ET and, 291 Methylpbenidate, 159, 6 tics, 37&371 Metoclopramide nausea, 123 parkinsonism exacerbation, 132 TD, 335 Netoprolol, ET and, 290 Metronomes, motor speech disorders and, 52 Metyrosine dystonia, 308 TD, 336 Index Mexilitine, CFS and, 359 Midodrine, 166 MSA, 240-241 Milkmaid's grip, 323 Mini-Mental State Examination, 176, 77t Minipress, see Prazosin Minotop, ET and, 292 Mirapex, see Pramipexole Mirror movements, CBD and, 256 Mitochondria1 defects, PD and, 97 Mixed cortical-subcortical dementia, parkinsonism and, 268-270 Mixed dysarthrias, 50 Mdafinil, 159 Modeling, of PMD, 442 Monoamine axidase (MAO), 63, 103 Motilium, see Domperidone Motor axon hyperexcitability, clinical syndromes and, 358t Motor circuit, diagram, 66f Motor impersistence, Motor neuron disease, neurologic exam~nation for, 20 Motor pathways, diagram, 67f Motor speech disorders, S apraxia of speech, 50-51 clinical assessment, 37-49, 39t dysarthrias, 3%50 managemen5 1-53 nonspeecb oral mechanism findings 41 t speech characteristics, 42t43t Motor system diagram, 63f examination, +L4 extrapyramidal, 7f neuroanatomical distinctions, 6f Motor tics, 365-366 Movement disorders hyperkinetic, 271-272 post-traumatic, 427-433 psychogenic, see Psychogen~c movement disorders Moving toes, extremity trauma and, 433 MPTP (1-methyl-4-phenyl-l,2,3,6tetrahydropyrid~ne), 1, 88,94,97,202,2 15 glutamate receptor inhibitor, 12 PD, lW105 Multiple system atrophy (MSA), 235-241 clinical features, defined, 237 diagnosis, 239 differential diagnosis, E 73 history and epidemiology, 235-236 pathology, 238-239 PD, subtypes, 235-236,236f treatment, 23%241, 240t - - - - Multiv~tamins, and, 20&20 PD Mumbling, 77 Muscle channclopathies, 366361,361 t tMuscle stiffness cause, 352t metabolic myopathies, Myasthenia gravas, muscle weakness and, 20 Myelopathy, signs and symptoms of, 21-22 hlyoclonus, 27-28,339-350 CBD,255 classification, 344t-346i defined, 27 diagnosis, 34&349, 346t drug-induced, 343, 347t essential, d~agnosis 341 of, focal and segmental causes, head trauma, 430 infectious, 343 national organizations, 454 spinal trauma, 432 treatment, 337, 350 vs tremor, 28 Myopathy, neurologic examination for, 20-2 Myorhythmia, assessment of, 31-32 Myo~onic disorders, 360-361,362t Mysoline, see Primidone NcuromuscuIar junction defects, neurologic examinatton for 2&21 Neurontin, see Gabapentin Neuropathic restless Iegs syndrome, 376377 Neumtrophic factor deficiency PD and, 97 N~cardipine, and, 292 ET Nifedipine TD, 336 tics, 370 N Oculomasticatory myorhythmia, 32 Oculostapedial synkinesis, 18 Olanaapine, 158 179 Nadolol, ET and, 290 National organizations, 45 Nausea, 123 Negative reinforcement of PMD,44-46 Neostriatum, 60 Neptawne, see Methazolarnide Neuroacanthocytosis chorea, 329 tics, 367 Neuroanatomy, terminology, 8f Neurocardiogenic syncope syndrome, 164 Neuragenic bladder MSA, 241 PD, 169-170 Neuroleptic agents, drug-induced parkinsonism and, 80 Neurologic examination, 4-17 central nervous system, 21-25 eye movements, I 51 gait, 4-9 hyperkinetic disorders, 25-34 motor examination, 9-14 motor neuron disease, 20 myopathy, 21rZ I neummuscular junction defects, - C2 peripheral signs, 17-2 1, 18t sensorl, examination, 14-1 Neuromelanin, 96, 103 Nimodipine, ET and, 292 Nimotop, see Nimodipine Nitoman, see Ttrrabenazine Nocardia astemides, 99 Nonobstructive hydrocephaIus, 18 Wonprogressive chorea, 329 Nonverbal oral movements, motor speech disorders and, Norepinephrine, GTS and, 367 Nermat pressure hydrocephalus, Norpramin, see Desipramine Nyssagmus, 17 Obsessive-compulsive disorder (OCD), 366 Occupational cramp disorders, 303 Occupational therapy CBD, 260 PD,205 PD,213 TD, 335 Olivopontocerebellar amph y (OPCA), 235 differential diagnosis, 173 PD,83 On-off phenomenon, 142-143 Operant conditioning, PMD and, 442-446 Opsoclonus, 17 Opsoclonus-myoclonus syndrome, 348 Oral cnntraceptivcs, chorea and, 326-327 Oral mechanism of motor speech disorders, 38 Orblcularis oculi, 13 Organic tremor, vs psychogenic, 28 Orobuccokingual dyskinesia, 32-33 Oromandibular dystonia, 302 BTX, 390 Orthostatic hypotension (OH) diet, 202 MSh, 240-24 PD, 78, 124,162-167 blood pressurc determination, 163-165 clinical s~gnificance, 163-164, I64f patient history, 162-163 treatment, 165167, 166t-167t Orthostatic tremor, 27%280 Osteoporosis, dietary supplementation and, 20 I Index Oxidative stress, PD and, 96, 103-104 Oxatremorine, 288 Oxybutynin, 170, 241 Oxycodone, RLS and, 382 Clxyradicals, toxicity of, 96 Pacing boards, motor speech disorders and, 52 Painful hand and moving tingers, extremity trauma and 433 Painfbl legs, extremity trauma and, 433 Painful tic convulsif, 1.S-3 16 Palatal lift prosthesis motor speech disorders and, 52 Palatal myoclonus, 28,280-28 assessment, 1-32 Palatal tremor,49,286281 assessment, 1-32 Palatopharyngolaryngeal myoclonus hyperk~ netic dysarthria and, 49 Palilalia, 366 Pallidal nucid, 192 Pal lidotsmy parkinsonian tremor, 68 PD, 128, 186-188 Paraneoplastic syndromes, rnyaclonus and, 348 Paratonia, 74 Parkinsonian gait, 420 Parkinsonian terms, 12t Parkinsonian tremor, 278-279 Parkinsonism atherosclerotic, 21 atypical, assessment of, 22 defined, 20%210 drug-induced, SO 1-214,2 13t-2141,227 extremity trauma, 432 head aauma, W idiopathic, see Parkinson's disease infectious, E %221,220t lower body, 217 postencephalitrc, 58-59 post~nfect~ous, %221 21 psychogenic, PMD and, 448 secondary, set?Secondary parkinsonism signs, 241, 25 toxic, 214 216,215t vascular, 80 Parkinsonism plus syndrome assessment of, 22 Parkinsonism-plus syndromes, vs PSP, 232 Parkinson's dementia, parkinsonism and, 267 Parkinson's disease (PD), 57-84 advancing causes, 35 clinical trjals, 144 drug costs, 143 drug ~nteractions,144 history-taking, 129-1 35, 130t-13 it, 133t134t medication compliance, 143-1 44 motor ~roblem therapy: 136-144 physical examination, 135-136, 136t autonomic involvement, 78 basai ganglia, 6 bradykinesia, 75 cause, 88-89 central nervous infection, 99 clinical phenomenology, 68-70 cognitive compl~cations, 180 differential diagnosis, 1T X laboratory evaluat~on, 178 therapy, 178-1 80 cognitive impalnnent, 78 dementia, 135 diagnosis, 58 diet, 197-202 differential diagnosis, N , It early, presenting features of, 72-73, 72f epidemiological studies, 90 etiologic hypotheses, 93-99 galt disorders, 7 immune system achrvaiion, 96 incidence, 85 in~tiaitreatment, 1 amantadine, 1.27 anticholinergic drugs, 128 dopamine agonists, 125-126, levodopa, I 17, 118-122 medication ophons, 11&I 18 selegiline, 126127 rninrmal symptoms treatment of, 17 national organizatians, 206,207t, 455 natural history, 57-58.7WY neurochemiatry, 62-64 neuropathology, 5-1 nonphmacoIogic therapy, I28 OH,162-167 pathology, 91-92 pathophysiology, 64-70 postural instability, 76 precl~nical, 89 prevalence, 86 prevention, 88 progression, 101-103, 102t-103t protective factors, 87-88 psychoiogic and social support, 206-207 psychologic disarders, 78 rehabiIitation, 202-2196 rigidity, 74-15 risk factors, 8 screenmg, 89 significance, 86 sleep, 51-1 59 speech impairment, 77 - stag~ng 79,79t surgery, 1E-196 survival, 86 treatment antioxidants, 109-1 10 disease progression, O W 12 etiologic hypotheses, 102-105 glutamate excitotoxic~t~r, E 105, seiegiline, l e i 1 tremor, 73-74 utilization, 86 vs CBD, 259 Parrodel, see Bromocriptine Paroxysmal chorea, 329 Paroxysmal dystonic choreoathetosis (PDC), 329 P~roxysmalkinesigenic choreoathemsi.5 (PKC), 329 Paroxysmal nocturnal dyskioesia, head trauma and, 430 Patient compl~ance, and, 143-144 PD Peak-dose dyskinesias, 139-140, 140f-14 f Peak-dose freezing, 142 Pediatric psychogenic movement disorder, 448 Pendular nystagmus, 16 Penicillamine, WD and, 407408,409 Perceptual-motor syndromes, parkinsonism and, 266 Porcocet, see Oxycodone Percodan, see Oxycodone Pergolide PD, 103-104, 147 RLS, 381 side effects, 125-1 26 vs levodopa, PI3 and, 107 Periodic limb movcmcnts in sleep (PLMS), 315 Periodic limb movemenls while awake, 375 Peripheral dlsorders, RAMS and, I2 Peripheral tleuropathy characteristic signs, 17-19 hemifacial spasm, 19-20 tremor, 280 Pcrmax, see PcrgoIide Perphenazine, TD and, 335 Pesticides, PD tind, 88 Pharyngeal dystonia 303 Phenobarbital ET, 290-291 WD tremor, 280 Phenylpropenolam~ne.166 Phenytoin, myoclonus and, 350 Physical t h a n p y CBD, 260 Huntington's disease, 326 PD, 12.8 Phys~ologic myoclonus, diagnosis, 340 Pick's disease, 232 park~nsonlsm, 266 Pill-rolling, 274 469 - Pirnozide, GTS and, 370 Piracetam, myoclonus and, 350 Plasma catecholamines, 173 Polycythemia Vera, chorea and, 328 Polyminimyoclonus vs tremor, 28 Positive reinforcement, PMD and, 4 4 Postencephalitic parkinsonism, 5&-59,X 1,99,220 Posthypoxic rnyoclonus, 343 Postinfectious parkinsonism, 19-22 L Post-traumattc movement dlsorders, 427-433 extremity trauma, 43 1-433 head trauma, 428-43 spinal muma, 430113 Post-traumatic parkinsonism, 219 Post-traumatic torticallis, clinical characteristics of, 431,431 t Postural hypotension, MSA and, 237-238 Postural instability, PD and, 76 Postural orthostatic tachycardia syndrome, 164 Posmral tremor, 29 Posrure, PD and, 69 PramipcxoIe adverse effccts, 179 PD, 104, 148 RLS, 382 side effects, 126 vs lcvodopa, PD and 107 Praxis, assessment of, t 2-14 Praxis disorders, RAMS and, I2 Prazos~n,17 Primary dystonia Primary focal dyston~a,294 304 lower extremity, 304 Primary gcneraiized dystonia, 29%299 Primary progressive h e z i n g gat disorder, 41 Primidone, 279 ET, 290-291 ProAmatine, see Midodrine Procardia, see Nifedipine Proc hlorperazine nausea, 123 parkinsonism exacerbation I Programmed cell death PD and, 97 Progressive aphasia, parkinsonism and, 266 Progressive asyrnrncirical rigidity and apraxia (PARA) syndrome, 254-255,268-269 Progressive ataxia evaluation of, 25&-25 Progressive encepl~alomyelitiswith rigidity, stiffman syndrome and, 353-354 Progressive myoclnnic epilepsy, 342 Progress~ve supranuclear palsy national organizations, 455 PD, 82 Progressive supranuclear palsy (PSP), 229-233 clinrcal features, 22%23 1, 230t axial rigidity, 23 cognitive impa~nnent 23 early farls, 23&32 speech and swaIlowing problems, 231 supranuclear gaze paresis, 230 wide-eyed, unblinking face, 230 differential diagnosis, 23 1-232 evaluation and management, 233 parkinsonism, 267 pathology, 232-233 prognosis, 233 support groups, 233 vs CBD, 260 Prolixin, see Fluphenazine Propanthcl~nc bromide, 170, 24 Propoxyphene, RLS and, 382 Propranolol contraindications, 289-290 ET, 289-290 RLS, 383 Protein restriction diet, PD and 199-200,200t Provigil, see Modafinil Provocation rests, for PMD, 446 Pseudoathetoid, 256 Psychic tension, 339 Psychogenic gait disorders, 424 Psychogenic moveincnt disurders (PMD), 43545 assessment, 438439 clinical characteristics, 439-440 defined, 436 diagnostic accuracy, 437438 diagnostic certainty, 440 diagnostic presentation, 44&451 epidemiology, 436-437 gait disorders, 447 idiopathic torsion dystonia, 446-443 modeling, 442 negative reinforcement, 44&446 pediatric psychogenic movement disorder, 448 positive reihforcement, 442-444 provocation tests, 446 psychogenic parkinsonism, 448 psychological assessment, 440-441 , risk factors, 443t social learning interpretation, 441442 Psychogenic parkinsonism,PMD and, 448 Psychogenic tremor, vs organic, 28 Psychosis, Ievodopa and, 124 PuH test, I36 Punch-drunk syndrome, 430 Pyridostigmine, dystnnra and, 308 Q Quetiapine, 158 179 PD 213 T ) 335 Z, Quinine CFS and, 359 Radioactwe copper, WD andnd, 403-404 Rapid alternating movements (RAMS),11-12, 13t Rapid eye movement sleep behavior disorder (RED), 154-157 Rashes, Ievodopa and, 124 Reading aloud;motor speech disorders and, 39 Reflex sympathetic dystrophy, extremity trauma and, 432-433 Reflexes, 10-1 Reglan, see Metocloprarnide Renal tubular necrosis, WD and, 400 Repetitive action myoclonus, vs tremor, 28 Repetitive digit tapping task, 11-12 Requip, see RopiniroIe Reserpine Fluntington's disease, 325 TD, 336 Respiratory stridor, MSA and, 238 Rest tremor, 29,73,274,278 differential diapnsrs, Restless legs syndrome (RLS),1.55156, 373-384 akathis~a, 27 associated caudiiions, 377t clinical features 73-3 76 course, 375 defined, 73-3 74 diagnosis, 374t, 378-379 et~ology, 7 family history, 376 motor restlessness, 375 national organizations, 456 neurologic examination, 376 nocturnal exacerbation, 374 nonpharmacologic treatment, 379.379t pathogenesis, 377-378 pharrnacologic management, 8 , 38 1t sluep disturbance, 375 Restoril, peeTemazepam RericuIar reflex myoclonus, 348 Rerrocollis, 300 Rovcrse obstruction sleep apnea syndrome, 334 Rhythmic myoclonus, aa tremor, 339 Rigid~ty,B cogwheel, PD, , 7 R~ppIing muscle diseasc, 361 R~sperdal, Risperidoiie see Risperidone Huntington's disease, 325 PD, 213 TD, 336 Ritalin see Methylphcn~date Romberg's tesl, 412 Rop~n~role adverse effects, 179 47 Index PD,104, 148 RLS, 382 side effects, 126 Roussy-Levy syndrome, 280 Rnbral tremor, 275 s Saccadic pursuit, 15-1 h Salt tablets, 166 Schizophrenia, TD and, 33 Schwartz-Jampel syndrome, 360 Secondary dystonia 3Qd-307 acquired structural lesions, 304-305 Secondary parkinsonism, 1-2 19 drug-induced, 21 f-214,213t, 214t genetic disorders, 22 1-222,225t-227t hydrocephalus, %2 19 infectious, 19-221,220t investigation, 222, 226427, 226t metabolic disease, 221,222t postinfectious, 19-221 post-traumatic, 21 structural brain lesions, 1&219,2 17t toxic parkinsonism, 214-216, treatment 227 Segawa's disease, 306 Selective serotonin reuptake inhibitors (SSRIs), 182 Selegiline adverse effects, 179 drug interactions, 182 insomnia, 155 PD,105, i l e i I I, 126-127,148 Senile chorea, 329 Sensory ataxia, 422423 Sensoy system, examination of, 14-1 Serpasil, see Reserpine Serial dysmetriq 276 Semquel, see Quetiapine Serum ceruloplasmin IeveIs, WD and, 402,402t Serum copper, WD and, 404 Severe cerebellar tremor, 279 Sex factors, PD and, 88 Sexual trauma, PMD and, 444-445 ShuMing, 76 Shy-Drager syndrome {SDS), 78,235 differential diagnosis, 173 PD, 82 Sildenafil, 241 Sinemet, RLS and, 8 Sleep dopaminergic agent adverse effects, 155 hallucinations syndrome, 157-1 58 PD,151-159, 152t Sleep apnen, IS6 Sleep apnea syndrome, reverse obstruction and, 334 Slit-lamp examination, for WD, 403 Smoking PD.$7 RtS, 378 Social activities, PD and, 205 Swial learning variables, PMD and, 4 4 Spasmodic dysphonia, 48,303 BTX, b Spasmodic torticollis, 300-302 Spasms assessment, 33-34 cause, 352t metabolic rnyopathies, Spastic dysarthria, 44-45 Spastic gaits, 41-20 Spastic hemiparetic gait, 19 Spastic paraparesis, 19-420 Spasticiiy, SlQ, 1-352 23,35 Speech apraxia, 50-5 conversational, motor speech disorders and, 39 PD,205 PSP, 23 Speech alternating motion rates, motor speech disorders and, 38 Speech characteristics apraxia of speech, 51 ataxic dysarthria, 45 hyperkinetic dysarthria, hypokinetic dysarthria, 46 motor speech disorders, 42t-43t spastic dysarthrin, 45 unilateral upper motor neuron dysarthrin, 4% 50 Speech disordersTS motor, see Motor speech disordm Speech lmpalrment, PD and, 77 Speech production, defined, 36f Speech sequential motion rates, motor speech disorders and, 38 Speech therapy CBD, 260 Huntington's disease, 325-326 Spinal cord, assessment of, 21-22 Spinal trauma, 430-43 Spinocercbellar ataxia type 1.247 Spinocerebellar ataxia type 2, 247 Spinocerebeilar ataxia type 3,222, 247-248 Spinocerebellar ataxia type 4,248 Spinocerebellar a m i a type 5,248 Spinocerebellar ataxia type 6,248 Spinocerebellar ataxia type 7,24%249 Start hesitation, 14 Stereognosis, 15 Stiff-man syndrome differential diagnosis, 355t progressive encephalomyelitis with rigidity, 353-354 Index Stocking-glave,distribution, 1&I9 Strength testing, 11 Striatonigral degeneration (SND), 235236 differentla1 diagnosis, 173 PD, Stride, diagnosis of, Strychnine puisorrirrg, 356-357 Subacute arteriosclerotic enccphalopathy, 217 Subcortical dementia, parkinsonism and, 267-268 Substantia nigra (SN), 58,59f, 60f, 103 Subthalamic nuclei, 192 Subthalamotomy, PD and, 189-190 Suicidal ideation, Huntington's disease and, 325 Sunflower cataract, WD and 399 Superoxide dismutase (SOD), 109 Support goups PD, 206,207k PSP, 233 Supranuclear gaze paresis, 16,230 Swallowing, PSP and 23 Sydenham's chorea, 327 Symmetrcl, see Amantadme Symptomatic rnyoclonus, diagnosis, 342-343 Symptomatic palatal myoclonus, 348 T Tamsulostn, 171 Tardive akathisia, 33-334 treatment, 336 Tardive dyskinesia (TD), 32,331-338 epidemiology, 33 1-332 national organrzations, 456 vs tardive dystonia, 334 Tardive dystonia, 334 national organizations, 456 treatment, 336337 Tardive myoclonus, 334 Tardive tics, 334 treatment, 337 Tardive tourettism, 334 Tardive tremor, 335 Tasrnnr, see Tolcapone TD classification, 3321 clozapine, 337-338 drug-induced, 33 1-332,333t mechanism, 335 prognosis, 335 treatment, 3 S 3 , 337t Tegretol, see Carbama~epine Ternazepam, RLS and, 392 Terazosin, 17 Terminal accentuation tremor and, 29 Terminal tremor, 275 Terminology, of neuroanatomy, 8f Tctanus, 5 Tetany, 33 symptomatic, 357-358 Tetrabenazine dystonia, 308 Huntington's disease, 325 myoclonus, 350 TD, 336 tics, 370 Tetrahydroisoquinoline (THIQ), 94 Thalamic stimulation ET, 294 severe cerebellar mrnor 279 Thalamotomy dystonia, 309-3 10 ET, 293-294 parkinsonian tremor, 68 PD, 18&189 severe cerebellar tremor, 279 Thermoregulatory dysfunction, PD and, 171-172 Thermotabs, see Salt tablets Tic convulsif, 13 painhl, 16 Tic disorders, assessment of, 26-27 Tics BTX-A, 391-392 clrnical features, 365-366 defined, 271 diagnusls, 367-368,368t etiology, 366 pathophysiology 367 prognosis, 371 treatment, 369t,37&37 vs, dystonia, 30 Tigan see Trirnethobenzamide Tipof-the-tongue syndrome, PD and, 78 Tituhation, 276 T~opherol, and, 1 &111 PD Tofranil, see Imipramfne Tolcapone, 147-1 48 drug interactions, 165 Tolterodine, 170,241 Tone, 9-1 Torsion dystonia idiopathic, 297 PMD, 446447 tics, 367 vs tardive dystonia 334 Torticollis, 300 BTX 389-390 idiopathic, clinical characteristics of, 43 1,43 I t Tourette's syndrome, national organizations, 456 Toxic disease, tremors and, 278 Toxic parkinsonlsm, 21&2 16,2 1st Tramadoi, RLS and, 383 Transfers, PD and, 204 Tremor, 273-28 action, 29, 73 473 Index action-postural 27&275 assessment, 28-30 CRD, 253.260 cerebeilar outflow, 275 clmzficat~on, 274-276 defined, 271 differential diagnosis, 281 dystonic, 30 Valproic acid myoclonus, 350 RLS, 383 stiff-man syndrome, 354 WI1 tremor, 280 Vascular parkinsonism, 80 Ventralis intemedius nucleus (VIM), 191 Verapamil, TD and, 336 essenttal, see Essenlial tremor exaggerated physiologic, 277.277t Viagra, see Sildenafil extrem~ty trauma 432 frequency, 276 head trauma, hyperkinetic dysarthria, 48-49 intention, 275-276 laboratory evaluation, 277 nntional organizations, 456 neurologic signs, 7 organic us psychogenic 28 I orthostatic, 279-280 palatal, 49,28&2& assessment, 1-32 parkinsonian, 278-279 Vitamin E, 109 I'D h8,73-74 peripheral neuropathy, 19,280 postural, 29 rest, 29,73,274,278 rubral, 275 severe cerebellar 279 spinal trauma, 432 tardive, 335 terminal, 275 lerminal accentuation, 29 toxic-metabolic disease, 278 WD,398 writing 280 Triavil, TD and, 335 Tricyclic anftdepressants, 182 Trientrne WD and, 408, 409 Trigeminal nerve, flaccid dysarthria and, 40 Trihexyphenidyl myoclon~is, 350 PD, 127 128, 144, 146 Trimefkobenzamide, nausea from, I23 Truncal dystonia 304 Unified Parkinson's Disease Rating Scale, 79 Unilateral upper motor neuron dysarthria, 4%50 Urinary copper WD and, 402 Urispas, see Flavoxnte hydrochloride Urologic disorders, PD and, 169-1 71 Utilimtion behavior, 256 Vagus nerve, flaccid dysarthria and, 44 Valium, see Diazepam Vitamin D, osteoporosis nnd, 20 Vitamins, 200-20 I Vocal exercise, motor speech disorders and, 5251 Vocal tics, 365-366 Vocational therapy, PD and 205 Vo~ce amplifiers, motor speech disorders and, 52 Vowel prolongation, inotor speech disorders and, 39 Walking, 4,411 requirements, 14t Web sites, 453-457 PD,206-207 Wellhutrin, see Bupropion Whipple's discase, myoclonus and, 348 Wilson's disease (WD), 222, 397-409 clinical features, 397400 hematologic disorders, 400 hepatic manifestations, 398 neurologic dysfunction 398-399,399t ocular manifestations, 38-00 psychiatric manifestations, 399 renal disease, 400 rheurnatologic symptoms, 400 diagnostic tests, 40 4 differential dingnos~s, 40 epidemiology, 400 genetics, 400 national organizations, 457 pathoph ysiology, 400 tics, 367 treatment 404409,405t copper absorption reduction, U copper intake reeducation, 404-406 increased copper elrrn~nation, 407408 liver transplantation, 408 monitoring 40W09 tremor, 279 vs dystonia, 307 vs PD, 83 Worldwide Education and Awareness for Movement Disorders, national organizations 457 Writes's cramp, 33, 303 Writer's dystonia, 33 Writing tremor, 280 474 Index z Xanax, see ~ ~ ~ r a z o l a m Zinc, W D and, 406, Young-onset Parkinson's disease dopamine agonists, 125 treatment 17 Zolpidem, 156 Zona compacta, 60, 60f Zyprexa, see Olanzapine 408 C U R R E N T C L I N I C A L P R A C T I C E Parkinson’s Disease and Movement Disorders Diagnosis and Treatment Guidelines for the Practicing Physician Edited by Charles H Adler, MD, PhD Consultant and Co-Director, Parkinson’s Disease and Movement Disorders Center, Department of Neurology, Mayo Clinic Scottsdale, Scottsdale, AZ; Associate Professor of Neurology, Mayo Medical School, Rochester, MN J Eric Ahlskog, PhD, MD Chair, Division of Movement Disorders, and Consultant, Department of Neurology, Mayo Clinic Rochester, Rochester, MN; Professor of Neurology, Mayo Medical School, Rochester, MN With the rapid expansion of research on the pathogenesis of movement disorders, the growing availability of novel medical and surgical treatment options for Parkinson’s disease, and the increasing prevalence of such disorders in an aging population, physicians face an especially difficult task in keeping their practices up-to-date In Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician, highly experienced clinician–researchers distill this immense amount of new information to create a practice-oriented tutorial for all nonspecialists treating movement disorders Their book helps physicians to distinguish each disorder, providing a basic understanding of both the test and treatment options needed in active practices, as well as the effective use of the therapeutic recommendations of consulting specialists The first half of the book is devoted to Parkinson’s disease and conditions masquerading as parkinsonism, and the remainder details the recognition and treatment of tremor, dystonia, chorea, myoclonus, tics, gait disorders, restless legs syndrome, the ataxias, conditions resulting in spasms, and the use of botulinum toxin in movement disorders In all cases the book provides sufficient background so that even relatively inexperienced clinicians can readily master the diagnosis and treatment of these neurologic conditions Concise, informative, and eminently practical, Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician brings today’s primary care physician, neurologist, gerontologist, and internist the critically evaluated knowledge and up-to-date expertise needed to treat patients with movement disorders in full confidence FEATURES • Synthesis of the most recent advances in understanding and treating movement disorders • Comprehensive coverage of Parkinson’s disease and other movement disorders • Practical guide for differential diagnosis, testing, treatment options, and prognosis • Written by practicing clinician–researchers chosen for their broad clinical experience • Extensive treatment guidelines and numerous clinical pearls • Presented as if the authors were sitting and instructing a physician doing a preceptorship CONTENTS A BASIC DIAGNOSTIC PRINCIPLES Approach to the Patient With a Movement Disorder: Basic Principles of Neurologic Diagnosis Motor Speech Disorders: Clues to Neurologic Diagnosis B PARKINSON’S DISEASE What Is Parkinson’s Disease? Neuropathology, Neurochemistry, and Pathophysiology Clinical Features of Parkinson’s Disease Epidemiology and Genetics of Parkinson’s Disease Parkinson’s Disease: Etiologic Considerations Medication Strategies for Slowing the Progression of Parkinson’s Disease Initial Symptomatic Treatment of Parkinson’s Disease Advancing Parkinson’s Disease and Treatment of Motor Complications Sleep and Parkinson’s Disease Autonomic Complications of Parkinson’s Disease Treatment of Cognitive Disorders and Depression Associated With Parkinson’s Disease Surgical Treatment of Parkinson’s Disease Adjunctive Therapies in Parkinson’s Disease: Diet, Physical Therapy, and Networking C PARKINSONISM BUT NOT PARKINSON ’S DISEASE (OTHER AKINETIC-R IGID SYNDROMES ) Introduction Secondary Causes of Parkinsonism Progressive Supranuclear Palsy Multiple System Atrophy Familial Adult-Onset Spinocerebellar Degenerations Current Clinical Practice™ PARKINSON’S DISEASE AND MOVEMENT DISORDERS: DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN ISBN: 0-89603-607-3 Corticobasal Degeneration Parkinsonism in Primary Degenerative Dementia D MOVEMENT DISORDERS CHARACTERIZED BY E XCESSIVE M OVEMENT (HYPERKINETIC ) Introduction Tremor Disorders: Overview Essential Tremor: Diagnosis and Treatment Dystonia Hemifacial Spasm Huntington’s Disease and Other Choreas Tardive Dyskinesias Myoclonus Spasms and Stiff-man Syndrome Gilles de la Tourette’s Syndrome and Tic Disorders Restless Legs Syndrome Botulinum Toxin Treatment of Movement Disorders E O THER MOVEMENT DISORDERS Wilson’s Disease Gait Disorders: Recognition of Classic Types Posttraumatic Movement Disorders Psychogenic Movement Disorders: Theoretical and Clinical Considerations Appendix Index 0 0> 780896 036079 ... DISEASE AND MOVEMENT DISORDERS: DIAGNOSIS AND TREATMENT GUIDELINES FOR THE PRACTICING PHYSICIAN CURRENT CLINICAL PRACTICE Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines. .. The major goal of Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician is to distill this immense amount of information and to educate the. .. Parkinson’s Disease and Movement Disorders: Diagnosis and Treatment Guidelines for the Practicing Physician Edited by: C H Adler and J E Ahlskog © Mayo Foundation for Medical Education and Research,