International Neurology

International Neurology A Clinical Approach

EDITED BY

ROBERT P. LISAK MD FAAN FRCP Parker Webber Chair in Neurology Professor and Chair of Neurology Professor of Immunology and Microbiology Wayne State University School of Medicine; Neurologist-in-Chief, Detroit Medical Center Chief of Neurology, Harper University Hospital Detroit, MI, USA

DANIEL D. TRUONG MD FAAN Head of The Parkinson and Movement Disorder Institute Memorial Neuroscience Institute Orange Coast Memorial Medical Center Fountain Valley, CA, USA

WILLIAM M. CARROLL MBBS MD FRACP FRCP(E) Head of Neurology and Clinical Neurophysiology Sir Charles Gairdner Hospital Nedlands, Perth, Australia

ROONGROJ BHIDAYASIRI MD FRCP Director Chulalongkorn Comprehensive Movement Disorders Center Chulalongkom University Hospital Bangkok, Thailand; University of California at Los Angeles Los Angeles, CA, USA

FOREWORD BY JOHN WALTON (LORD WALTON OF DETCHANT)

©WILEYBLACKWELL A John Wiley &. Sons, Ltd., Publication

Contents

List of contributors, xi

Foreword, xxv

Endorsement from the World Federation of Neurology, xxvi

Preface, xxvii

Part 1 Vascular disease

1 Stroke: an overview, 1 Christopher Li-Hsian Chen and Chung Y. Hsu

2 Transient ischemic attacks, 2 Samir H. Shah and Bruce Ovbiagele

3 Atherothrombotic disease, 6 Nijasri C. Suwanwela

4 Occlusive disease of small penetrating arteries, 9 Takeshi Iwanaga, Hock L. Teoh, Jorge A. Zavala, and Geoffrey A. Donnan

5 Binswanger's disease, 12 Dean A. Le and Ferdinando S. Buonanno

6 Brain embolism, 14 Bernard PL. Chan and Chung Y. Hsu

7 Borderzone cerebral infarction, 18 Thomas W. Leung

8 Dissection of the cervicocerebral arteries, 20 Nijasri C. Suwanwela and Chung Y. Hsu

9 Coagulation disorders in stroke, 23 Kay Sin Tan

10 Hemorrhagic strokes, 25 Josef Schill and Thorsten Steiner

11 Strokes in children and young adults, 33 Alfred Lindner

12 Other cerebrovascular syndromes, 36 David Blacker

13 Diseases of the cerebral venous system, 39 Patrick Carney and Stephen M. Davis

14 Spinal cord stroke, 42 Manu Mehdiratta and Louis R. Caplan

Inflammatory vasculopathies

15 Extracranial granulomatous arteritis (giant cell arteritis), 45 Sandeep Randhawa and Gregory P. Van Stavern

16 Intracranial granulomatous arteritis (primary angiitis of the CNS)/idiopathic CNS vasculitis, 49 Donald Silberberg

17 Takayasu's arteritis, 51 Yasuhisa Kitagaiva

18 Polyarteritis nodosa, Churg-Strauss syndrome, overlap and related syndromes, 54 Stanley van den Noort and Gaby T. Thai

19 Wegener's granulomatosis, 57 Gerard Said

20 Cerebrovascular disease associated with antiphospholipid antibodies, 59 Megan Alcauskas and Steven R. Levine

21 Thromboangiitis obliterans - Buerger's disease, 61 Kumar Rajamani

Part 2 Autoimmune and inflammatory disease

22 Systemic lupus erythematosus, rheumatoid arthritis, and Sjogren's syndrome, 63 Marc Gotkine and Oded Abramsky

23 Systemic sclerosis, 70 Ho Jin Kim and Min Su Park

24 Mixed connective tissue disease, 72 Ho Jin Kim and Min Su Park

25 Behcet's syndrome and the nervous system, 74 Aksel Siva and Sabahattin Saip

26 Sarcoidosis, 79 Barney J. Stern

27 Inflammatory spondyloarthropathies, 83 Asmahan Alshubaili

v

vi Contents

Part 3 Seizure disorders and epilepsy

28 Epilepsy: overview, 85 Andres M. Kanner

29 Cryptogenic and symptomatic generalized epilepsies and syndromes, 90 Marco T. Medina, Antonio V. Delgado-Escueta, and Luis C. Rodriguez-Salinas

30 Genetic (primary) idiopathic generalized epilepsy (IGE), 95 Afawi Zaid

31 Localization-related epilepsies, 99 Hirokazu Oguni and Chrysostomos P. Panayiotopoulos

32 Neurodiagnostic tools for the paroxysmal disorders, 104 Barbara E. Swartz

33 Antiepileptic drugs, 107 Paul B. Pritchard III

34 Surgical treatment of epilepsy, 112 Ivan Rektor and Barbara E. Swartz

Part 4 The dementias

35 The dementias: an overview, 116 Howard H. Feldman and Najeeb Qadi

36 Isolated memory disorders, 120 Elka Stefanova and Vladimir Kostic

37 Mild cognitive impairment, 123 Elka Stefanova and Vladimir Kostic

38 The degenerative dementias, 126 Irena Rektorovä, Robert Rusina, Jakub Hort, and Radoslav Mate]

39 Other dementias, 137 John M. Ringman and Arousiak Varpetian

Part 5 Movement disorders

40 Movement disorders: an overview, 144 Roongroj Bhidayasiri and Daniel D. Truong

41 Tremor, 146 Jan Raethjen and Günther Deuschl

42 Parkinsonism, 152 Bradley J. Robottom, William J. Weiner, and Lisa M. Shulman

43 Parkinson's disease, 159 Daniel D. Truong and Roongroj Bhidayasiri

44 Dystonia, 166 Stanley Fahn

45 Chorea and related disorders, 173 Roongroj Bhidayasiri and Daniel D. Truong

46 Myoclonus, 179 John N. Caviness and Daniel D. Truong

47 Tics and Tourette, 184 Valerie Suski and Mark Stacy

48 Ataxia, 187 Sergei N. Illarioshkin

49 Drug induced movement disorders, 191 Rick Stell

50 Gait disorders, 194 Mr Giladi

51 Psychogenic movement disorders, 197 Brandon Barton, Esther Cubo, and Christopher G. Goetz

Part 6 Other neurodegenerative diseases

Upper and lower motor neuron degenerative disorders

52 Amyotrophic lateral sclerosis, 199 Björn Oskarsson, Yvonne D. Rollins, and Steven P. Ringel

53 Primary lateral sclerosis, 203 Yvonne D. Rollins, Björn Oskarsson, and Steven P. Ringel

54 Hereditary spastic paraplegia, 205 Ildefonso Rodriguez-Leyva

55 Spinal muscular atrophies, 208 Sabine Rudnik-Schöneborn and Klaus Zerres

56 Post-polio syndrome, 212 M/s Erik Gilhus

Degenerative muscle disorders

57 Limb girdle muscular dystrophies, 215 Ignacio M. Carrillo-Nunez, Anneke J. van der Kooi, and Marianne de Visser

58 Dystrophinopathies, 219 S.M. Schade van Westrum and Marianne de Visser

59 Facioscapulohumeral muscular dystrophy, 223 George W. Padberg

60 Scapuloperoneal syndrome, 225 Georges Serratrice

61 Myotonic dystrophy, 227 Slobodan Apostolski and Vidosava Rakocevic-Stojanovic

Contents vii

62 Oculopharyngeal muscular dystrophy, 231 80 Cerebral malaria, 285 Luis A. Chui and Tahseen Mozaffar Polrat Wilairatana and Srivicha Krudsood

63 Emery-Dreifuss muscular dystrophy, 233 81 Trypanosomiasis, 290 Ronnie Karayan and Tahseen Mozaffar Francisco Javier Carod-Artal

64 Distal myopathies, 235 Rickettsiae and parasitic infections Nigel G. Lain? and Phillipa J. Lamont _„ „ . , , . , , . . . , . „ „

5 & r ' 82 Rickettsial and parasitic infections, 293 Oscar H. Del Brutto

Part 7 Infect ious d i s e a s e s 83 Cestodes, 294 Oscar H. Del Brutto

Bacterial infections

65 Acute bacterial meningitis, 238 Sudesh Prabhakar

66 Brain abscess, 242 Gagandeep Singh

67 Subdural empyema, 245 Sandi Lam and Tien T. Nguyen

68 Epidural abscess, 248 Sandi Lam and Tien T. Nguyen

69 Intracranial septic thrombophlebitis, 250 D. Nagaraja and D.K. Prashantha

70 Encephalitis due to bacterial infections, 253 Karen L. Roos and Jennifer Durphy

Mycobacterial infections

71 Mycobacterium tuberculosis, 258 Einar P. Wilder-Smith

11 Mycobacterium avium, 261 Einar P. Wilder-Smith

73 Leprosy, 262 Minh Le

Spirochetal infections

74 Neurosyphilis, 266 Jonathan Can

75 Lyme disease, 269 Patricia K. Coyle

76 Fungal infections of the central nervous system, 274 Thomas С Cesario

Protozoan diseases

77 Introduction to protozoans of the central nervous system, 278 Marylou V. Solbrig

78 Amoebic disease of the central nervous system, 279 Melanie Walker

79 Toxoplasmosis of the central nervous system, 282 Marylou V. Solbrig

84 Trematodes: schistosomiasis, 297 Sureshbabu Sachin and Manjari Tripathi

85 Nematodes, 300 Manjari Tripathi and Sureshbabu Sachin

86 Rickettsial disease, 303 Clarisse Rovery and Didier Raoult

Viral infections

87 Acute, recurrent, and chronic viral meningitis, 308 Larry E. Davis

88 The syndrome of acute encephalitis, 314 Heng Thay Chong and Chong Tin Tan

89 Post-viral cerebellitis, 329 Heng Thay Chong and Chong Tin Tan

90 Subacute and chronic viral infections, 331 Ellen Gelpi and Herbert Budka

Prion diseases

91 Prion diseases, 336 Ellen Gelpi and Herbert Budka

HIV and acquired immunodeficiency syndrome

92 HIV and the acquired immunodeficiency syndrome: an overview of neurological complications, 340 Bruce J. Brew

93 Human immunodeficiency virus: biology and general overview of seroconversion and early infection, 342 Alexandros С Tselis

94 HIV-related CNS disorders, 345 Girish Modi, Kapila Hari, and Andre Mochan

HIV-related peripheral nervous system and myopathic disorders

95 HIV neuropathy, 351 Giorgia Melli and Ahmet Höke

96 Myopathies in HIV infection, 354 Mirela E. Toma, Alejandra Gonzalez-Duarte, and David M. Simpson

viii Contents

97 HIV-associated cerebral opportunistic infections, 356 Bruce J. Brew

98 HIV-associated lymphoma: neurologic complications, 360 Bruce }. Brew

The neurologic complications ofHTLV-1 myelopathy syndromes

99 Peripheral nervous system complications of HTLV-1 myelopathy: HAM/TSP and related syndromes, 362 Abelardo Araüjo, Marco A. Lima, and Marcus Julius T. Silva

Part 8 Demyelinating disorders

100 Multiple sclerosis, 366 Robert P. Lisak and Jun-Ichi Kira

101 Neuromyelitis optica (NMO) or Devic's disease, 375 William M. Carroll

102 Isolated inflammatory demyelinating syndromes (IIDS), 378 Ernest W. Willoughby

103 Acute disseminated encephalomyelitis, 381 Brenda L. Banwell

104 Osmotic demyelination syndromes, 386 Ovidiu Bajenaru

105 Concentric sclerosis (Balö's disease), 389 Takeshi Tabira

Part 9 Toxicology

106 Neurotoxicology, 391 Nai-Shin Chu and Chin-Chang Huang

Part 10 Nutritional deficiency

107 Vitamin deficiency, 401 Jacques Serratrice

108 Starvation, Strachan's syndrome, and postgastroplasty polyneuropathy, 408 he Quang Cuong

109 Alcohol-related neurological disorders, 410 Yuri Alekseenko

Part 11 Peripheral neuropathies

110 Peripheral neuropathies: overview, 416 Friedhelm Sandbrink

Til Hereditary neuropathy, 419 Liying Cui and Mingsheng Liu

112 Acquired neuropathies, 425 Friedhelm Sandbrink

113 Plexopathies and mononeuropathies, 432 Friedhelm Sandbrink

Part 12 Muscle and neuromuscular junction disorders

Disorders of neuromuscular junction

114 Myasthenia gravis, 438 Richard A. Lewis

115 Lambert-Eaton syndrome, 442 Johan A. Aarli

116 Neuromuscular transmission disorders caused by toxins and drugs, 445 Zohar Argov

117 Critical illness myopathy, 447 Muhammad Al-Lozi and Alan Pestronk

Myopathy and myositis

118 Progressive muscle dystrophies, 449 Stephan Zierz

119 Familial periodic paralyses, 454 Chokri Mhiri

120 Congenital disorders of the muscle, 457 Young-Chul Choi

121 Muscle cramps, 461 Raymond L. Rosales

122 Dermatomyositis, 465 Marinas С. Dalakas

123 Polymyositis, 469 Marinos C. Dalakas

124 Inclusion body myositis, 474 Frank L. Mastaglia and Merrilee Needham

125 Myoglobinuria, 477 John Vissing

Contents ix

Part 13 Neurogenetics

126 Genetics in neurology, 480 Karen P. Frei

Part 14 Neuro-otology

127 Neuro-otology, 485 Kevin A. Kerber

Part 15 Neuro-ophthalmology

128 Clinical approaches in neuro-ophthalmology, 495 Anuch.it Poonyathalang

Part 16 Neuro-oncology

Glial tumors

129 High grade astrocytomas, 506 Olivier L. Chinot

130 Low grade astrocytoma, 510 Martin J. van den Bent

131 Low grade and anaplastic oligodendroglioma, 514 Ayman I. Omar and Warren P. Mason

132 Brain stem glioma, 519 Ira J. Dunkel and Mark M. Souweidane

133 Intracranial ependymoma, 522 Sajeel Chowdhary and Marc Chamberlain

Non-glial tumors

134 Nerve sheath tumors, 525 Nathan J. Ranalli and Eric L. Zager

135 Meningiomas, 528 Laurie Rice and Jeffrey Raizer

136 Medulloblastoma, 531 Regina I. Jakacki

137 Primary central nervous system lymphoma (CNSL), 535 Deborah T. Blumenthal

Metastatic tumors

138 Brain metastases, 539 Silvia Hofer and Michael Brada

139 Leptomeningeal metastases, 543 Elizabeth R. Gerstner and Tracy T. Batchelor

140 Spinal epidural metastases, 546 Lee I. Kubersky and David Schiff

Neurologic complications of systemic cancer

141 General approach to the diagnosis and treatment of paraneoplastic neurologic disorders, 550 Myrna R. Rosenfeld and Josep O. Dalmau

Part 17 Sleep disorders

142 Insomnia, 554

Colin A. Espie and Delwyn J. Bartlett

Primary disorders of daytime hypersomnolence

143 Narcolepsy, 557 Marcel Hungs and Emmanuel Mignot

144 Idiopathic hypersomnia, 561 Marcel Hungs and Jed Black

145 Obstructive sleep apnea, 563 Christine Won, Jee Hyun Kim, and Christian Guilleminault

146 Restless legs syndrome, 566 Birgit Högl, Birgit Frauscher, and Claudio Sergio Podestä

147 Orcadian sleep disorders, 569 Sergio Tufik, Monica L. Andersen, Lia R.A. Bittencourt, and T. De Mello

Parasomnias

148 Disorders of arousal, 571 Li Ling Lim

149 REM behavior disorder (RBD), 573 Li Ling Lim

150 Paroxysmal nocturnal dystonia, 575 Cynthia L. Cornelia

151 Sleep abnormalities in neurological disorders, 577 Margaret Park and Cynthia L. Cornelia

Part 18 Spinal cord disorders

152 Disc disease, 579 David B. Vodusek and Simon Podnar

153 Syringomyelia, 587 Alia Guekht

Part 19 Pediatric neurology

154 Pediatric neurotransmitter diseases, 590 Stephen Deputy

155 Neonatal neurology, 594 Mary Payne and Ann Tilton

x Contents

156 Floppy infant syndrome, 596 Jong-Hee Chae

157 Storage disorders, 600 Jeffrey Ekstrand and Raman Sankar

158 Disorders of amino acid, organic acid, and ammonia metabolism, 609 Stephen Cederbaum

159 Mitochondrial encephalomyopathies, 617 Stacey K.H. Tay and Salvatore DiMauro

160 Fatty acid oxidation disorders, 631 Thomas Wieser and Thomas Deufel

161 Disorders resulting from transporters, 635 David Gloss

162 Lesch-Nyhan disease, 638 Allison Conravey and Ann Tilton

163 The porphyrias, 640 Frank}.E. Vajda and Carlo Solinas

164 Wilson's disease, 644 Peter A. LeWitt and Anna Cztonkowska

Part 20 CNS trauma

165 Traumatic brain injury, 648 Christopher С Giza

166 Spinal injury, 652 Enver I. Bogdanov

167 Whiplash injury, 655 Anthony Ciabarra

168 Decompression sickness, 657 Leon D. Prockop

169 Post-traumatic movement disorders, 659 Louis C.S. Tan and Daniel D. Truong

Part 21 Pain syndromes

170 Pain, 661 Guillermo Garcia-Ramos, Bernardo Cacho Diaz, and Bruno Estanol Vidal

Part 22 Headache and neck/facial pain

171 Headache, 669 Stephen D. Silberstein

172 Facial and neck pain, 675 Kammant Phanthumchinda

173 Fibromyalgia, 679 Saeed Bohlega

Appendix: Treatment algorithm for Parkinson's disease, 681

Index, 683

Color plate section is found facing p. 484