diseases of the nervous system - assets · diseases of the nervous system the third edition of a...

Arthur K. Asbury is Van Meter Professor Emeritus ofNeurology, Hospital of the University of Pennsylvania,Philadelphia, USA

Guy M. McKhann is Professor of Neurology, The ZanvylKrieger Mind and Brain Institute, Johns HopkinsUniversity, Baltimore, Maryland, USA

W. Ian McDonald is Professor Emeritus, Institute ofNeurology, The National Hospital for Neurology andNeurosurgery, Queen Square, London, UK

Peter J. Goadsby is Professor of Neurology, Institute ofNeurology, The National Hospital for Neurology andNeurosurgery, Queen Square, London, UK

Justin C. McArthur is Professor of Neurology andEpidemiology, Johns Hopkins University School ofMedicine, Baltimore, Maryland, USA

Diseases of the Nervous System

The third edition of a neurology classic, this two-volumetext is the most comprehensive neurology reference avail-able. It encompasses epidemiology, pathology, pathophys-iology, and clinical features of the full range of neurologicaldisorders. The basic principles of neurological dysfunctionare covered at cellular and molecular level by leadingexperts in the field. Disease mechanisms are reviewedcomprehensively, with particular relevance to the princi-ples of therapy.

Sections cover the general principles of neurologicaldisease, disorders of higher function, motor control,special senses, spine and spinal cord, bodily function,headache and pain, neuromuscular disorders, epilepsy,cerebrovascular disorders, neoplastic disorders, autoim-mune disorders, disorders of myelin, infections, traumaand toxic disorders, degenerative disorders, and neurolog-ical manifestations of systemic conditions. Each section,under the direction of one of the distinguished editors, is atext-within-a-text, offering the most reliable account of itstopic currently available.

Contributors to this work include the leading cliniciansand clinical neuroscientists internationally. Current, com-prehensive and authoritative, this is the definitive refer-ence for neurologists, neurosurgeons, neuropsychiatrists,indeed everyone with a professional or research interest inthe neurosciences.

From reviews of previous editions:

‘A scholarly, well-crafted and attractive book . . . Diseases of the

Nervous System deserves a place in the library of every medical

school, neurology department, and neuroscience department’

New England Journal of Medicine

‘All clinical neurologists and trainess in neurology will find it

invaluable as a reference and a way into the latest scientific

literature’ British Medical Journal

‘This is a superb book’

Journal of Neurology, Neurosurgery and Psychiatry

© Cambridge University Press www.cambridge.org

Cambridge University Press0521793513 - Diseases of the Nervous System: Clinical Neuroscience and Therapeutic Principles, Third EditionEdited by Arthur K. Asbury, Guy M. McKhann, W. Ian McDonald, Peter J. Goadsby and Justin C. McArthurFrontmatterMore information

http://www.cambridge.org/0521793513

http://www.cambridge.org


Diseases of the Nervous System

Clinical Neuroscience and Therapeutic Principles

Third Edition

VOLUME 2

Edited by

Arthur K. Asbury, MDUniversity of Pennsylvania School of Medicine

Philadelphia, USA

Guy M. McKhann, MDThe Zanvyl Krieger Mind and Brain Institute

Johns Hopkins University, Baltimore, MD, USA

W. Ian McDonald, MB, ChB, PhDInstitute of Neurology

The National Hospital for Neurology and Neurosurgery, London, UK

Peter J. Goadsby, MD, PhDInstitute of Neurology

The National Hospital for Neurology and Neurosurgery, London, UK

Justin C. McArthur, MB BS, MPHDepartments of Neurology and Epidemiology

Johns Hopkins University School of Medicine, Baltimore, MD, USA






The Pitt Building, Trumpington Street, Cambridge, United Kingdom

The Edinburgh Building, Cambridge CB2 2RU, UK

40 West 20th Street, New York, NY 10011-4211, USA

477 Williamstown Road, Port Melbourne, VIC 3207, Australia

Ruiz de Alarcón 13, 28014 Madrid, Spain

Dock House, The Waterfront, Cape Town 8001, South Africa

http://www. cambridge.org

Third edition © Cambridge University Press, 2002

This book is in copyright. Subject to statutory exception

and to the provisions of relevant collective licensing agreements,

no reproduction of any part may take place without

the written permission of Cambridge University Press.

First and second editions published by W.B. Saunders 1986, 1992

Third edition published by Cambridge University Press 2002

Printed in the United Kingdom at the University Press, Cambridge

Typeface Utopia 8.5/12pt. System QuarkXPress™ [ ]

A catalogue record for this book is available from the British Library

Library of Congress Cataloguing in Publication data

Diseases of the nervous system / edited by Arthur K. Asbury . . . [et al.]. – 3rd ed.

p. ; cm.

Includes bibliographical references and indexes.

ISBN 0 521 79351 3 (hardback)

1. Nervous system – Diseases. I. Asbury, Arthur K., 1928–

[DNLM: 1. Nervous System Diseases. WL 140 D611 2001]

RC346 .M33 2001

616.8 – dc21 2001043713

ISBN 0 521 79351 3 hardback in two volumes

Every effort has been made in preparing this book to provide accurate and up-to-

date information which is in accord with accepted standards and practice at the

time of publication. Nevertheless, the authors, editors and publisher can make no

warranties that the information contained herein is totally free from error, not

least because clinical standards are constantly changing through research and

regulation. The authors, editors and publisher therefore disclaim all liability for

direct or consequential damages resulting from the use of material contained in

this book. Readers are strongly advised to pay careful attention to information

provided by the manufacturer of any drugs or equipment that they plan to use.






This text is dedicated to our many friends and col-

leagues who wrote the chapters, for their diligence,

for their intellectual rigour, and for their belief in

the vision of scientific and clinical excellence on

which these volumes are predicated.






Contents

List of contributors xiiiEditor’s preface xxxiii

V O LU M E 1

PA RT I I N T RO D U C T I O N A N D G E N E R A LP R I N C I P L E S

1 Pathophysiology of nervous system diseases 3Justin C. McArthur, Guy M. McKhann, W. Ian McDonald, Peter J. Goadsby and Arthur K. Asbury

2 Genetics of common neurological disorders 14Orest Hurko

3 Repeat expansion and neurological disease 32J. Paul Taylor, Andrew P. Lieberman andKenneth H. Fischbeck

4 Cell birth and cell death in the central nervoussystem 55Samuel J. Pleasure and David E. Pleasure

5 Neuroprotection in cerebral ischemia 62Jin-Moo Lee and Dennis W. Choi

6 Promoting recovery of neurological function 79Ted Abel and Michael E. Selzer

7 Measurement of neurological outcomes 105Jeremy Hobart and Alan J. Thompson

8 Principles of clinical neuro-epidemiology 118Michael D. Hill and Thomas E. Feasby

9 Principles of therapeutics 127Peter J. Goadsby

vii






10 Windows on the working brain: functional imaging 131John A. Detre and Paul D. Acton

11 Windows on the working brain: magneticresonance spectroscopy 146James W. Prichard, Jeffrey R. Alger, Douglas Arnold, Ognen A.C. Petroff

and Douglas L. Rothman

12 Windows on the working brain: evokedpotentials, magnetencephalography and depthrecording 160Hans-Joachim Freund and Hans J. Heinze

PA RT I I D I S O R D E R S O F H I G H E RF U N C T I O N

13 Congenital disorders of cerebral corticaldevelopment 177Ganeshwaran H. Mochida and Christopher A. Walsh

14 The aging brain: morphology, imaging andfunction 195Marilyn S. Albert and Guy M. McKhann

15 Neurodegenerative diseases 210Stanley B. Prusiner

16 Aging and dementia: principles, evaluation anddiagnosis 237Sudha Seshadri and David A. Drachman

17 Alzheimer’s Disease 252Raymond J. Kelleher and John H. Growdon

18 Dementia with Lewy bodies 267Pamela J. McLean, Estrella Gómez-Tortosa,Michael C. Irizarry and Bradley T. Hyman

19 Frontotemporal dementia 283Bruce L. Miller, Howard J. Rosen and Michael Greicius

20 Consciousness and its disorders 289Antonio R. Damasio

21 Mechanisms of memory and amnestic syndromes 301John D.E. Gabrieli and Silvia A. Bunge

22 Acquired disorders of language 317John Hart, Jr and Ola A. Selnes

23 Neglect 331Kenneth M. Heilman

24 Brain death 348James L. Bernat

25 Disorders of mood 364Dean F. MacKinnon and J. Raymond DePaulo

26 Schizophrenia 374Paul J. Harrison

27 Obsessive–compulsive disorder 385Bavanisha Vythilingum, Dan J. Stein and Scott L. Rauch

28 Autism and autistic spectrum disorders 406Barry Gordon

29 Attention deficit hyperactivity disorder:spectrum and mechanisms 422Martha Bridge Denckla

30 The neurobiology of drug addition 431Charles A. Dackis and Charles P. O’Brien

PA RT I I I D I S O R D E R S O F M OTO RCO N T RO L

31 Mechanisms of motor control 447James Ashe and Apostolos P. Georgopoulos

32 The apraxias 461Ramón C. Leiguarda

33 Parkinson’s disease 476Marian L. Evatt, Mahlon R. DeLong and Jerrold L. Vitek

34 Other extrapyramidal syndromes: 489parkinsonism-plus and other forms ofsecondary parkinsonismKailash P. Bhatia

35 Tremors 512Jyh-Gong Hou and Joseph Jankovic

36 Myoclonus 523Peter Brown

37 Dystonia 532Barbara Illowsky Karp, Susanne Goldsteinand Mark Hallett

38 Tourette syndrome 551Donna J. Stephenson and Harvey S. Singer

viii Contents






39 Cerebral palsy 568Alexander H. Hoon, Jr and Michael V. Johnston

40 Gait and balance disorders 581John G. Nutt and Fay B. Horak

PA RT I V D I S O R D E R S O F T H E S PE C I A LS E N S E S

41 Smell 595Richard L. Doty and Steven M. Bromley

42 Taste 610Steven M. Bromley and Richard L. Doty

43 Disorders of vision 621Antony Morland and Christopher Kennard

44 Oculomotor control: normal and abnormal 634David S. Zee and R. John Leigh

45 Disorders of the auditory system 658Borka Ceranic and Linda M. Luxon

46 Vertigo and vestibular disorders 678Thomas Brandt

PA RT V D I S O R D E R S O F S PI N E A N DS PI N A L CO R D

47 Spinal cord injury and repair 695John W. McDonald

48 Myelopathies 713Philip D. Thompson and Peter C. Blumbergs

49 Diseases of the vertebral column 727Simon F. Farmer and Lucinda J. Carr

50 Cervical pain 742Nikolai Bogduk

51 Diagnosis and management of low back pain 760Donlin M. Long and Mohammed BenDebba

PA RT V I D I S O R D E R S O F B O DYF U N C T I O N

52 Autonomic function and dysfunction 773Christopher J. Mathias

53 Human brain–gut interactions: mechanismsof swallowing, visceral perception, and analcontinence in health and disease 795Shaheen Hamdy

54 Eating disorders: neurobiology andsymptomatology 808David C. Jimerson and Barbara E. Wolfe

55 Sleep and its disorders 816Robert Y. Moore and Eric A. Nofzinger

56 Bladder and sexual dysfunction 833Oliver J. Wiseman and Clare J. Fowler

57 Hypothalamic/pituitary function and dysfunction 853Roberto Salvatori and Gary S. Wand

PA RT V I I H E A D AC H E A N D PA I N

58 Peripheral nociception and the genesis of persistent clinical pain 873Richard J. Mannion and Clifford J. Woolf

59 Central nervous system mechanisms of pain 888Frederick A. Lenz, Patrick M. Dougherty and Joel D. Greenspan

60 Management of chronic pain 906Russell K. Portenoy

61 Migraine 920Michel D. Ferrari and Joost Haan

62 Cluster headache, other trigeminal autonomicsyndromes and the short-lived headaches 927Peter J. Goadsby

63 Orofacial pain 939Steven B. Graff-Radford

64 Chronic daily headache 955Stephen D. Silberstein and Richard B. Lipton

Complete two-volume index 973

Contents ix






VO LU M E 2

PA RT V I I I N E U RO M U S C U L A RD I S O R D E R S

65 Pathophysiology of nerve and rootdisorders 1075Jonathan D. Glass

66 Toxic and metabolic neuropathies 1092Shawn J. Bird and Arthur K. Asbury

67 Guillain–Barré syndrome 1110John D. Pollard and Judith M. Spies

68 Hereditary neuropathies 1129Mary Reilly and P.K. Thomas

69 Disorders of neuromuscular junction transmission 1143Vinay Chaudhry and Daniel B. Drachman

70 Disorders of striated muscle 1163Richard W. Orrell and Robert C. Griggs

71 Pathophysiology of myotonia and periodicparalysis 1183Stephen C. Cannon and Alfred L. George, Jr.

72 Pathophysiology of metabolic myopathies 1207Salvatore DiMauro and Ronald G. Haller

PA RT I X E PI L E P S Y

73 The cellular basis of epilepsy 1229Robert S. Fisher and Jie Wu

74 The clinical spectrum of epilepsy 1248Renzo Guerrini, Lucio Parmeggiani and Pierre Thomas

75 Status epilepticus 1271Hannah Cock and Simon Shorvon

76 Assessment of the patient with epilepsy 1281John S. Duncan

77 Principles of pharmacotherapy of theepilepsies 1301Emilio Perucca

78 Epilepsy surgery: disease treatment andinvestigative opportunity 1313Guy M. McKhann II and Matthew A. Howard III

PA RT X C E R E B ROVA S C U L A R D I S O R D E R S

79 Physiology of the cerebral circulation 1337Peter J. Goadsby and Richard P. White

80 Stroke syndromes 1345Louis R. Caplan

81 The treatment of acute ischemic stroke 1361Robert A. Felberg and James C. Grotta

82 Behavioural manifestations of stroke 1371Ricardo E. Jorge and Robert G. Robinson

83 Intracerebral hemorrhage 1383Juan R. Carhuapoma and Daniel F. Hanley

84 Aneurysms and arteriovenous malformations 1392Marcus A. Stoodley and Bryce K.A. Weir

85 Hereditary causes of stroke 1405Martin Dichgans

86 Preventive management of stroke 1415Gillian E. Mead and Charles P. Warlow

PA RT X I N E O P L A S T I C D I S O R D E R S

87 Primary brain tumours in adults 1431John Laterra and Henry Brem

88 Brain tumours in children 1448Peter C. Phillips and Michael A. Grotzer

89 Brain metastases 1462Roy A. Patchell

90 Paraneoplastic syndromes 1474Jerome B. Posner

91 Harmful effects of radiation on the nervous system 1489Jeremy H. Rees and Michael Brada

PA RT X I I AU TO I M M U N E D I S O R D E R S

92 Immune mechanisms in neurological disease 1501Hans-Peter Hartung, Klaus V. Toyka and Bernd C. Kieseier

93 The basis of immunotherapy in neurologicaldisease 1527Reinhard Hohlfeld

x Contents






94 Vasculitis of the central nervous system 1547David B. Hellmann and John H. Stone

95 Sarcoidosis of the nervous system 1558John Zajicek

96 Collagen–vascular disease and thenervous system 1569Michael J. Regan and John H. Stone

PA RT X I I I D I S O R D E R S O F M Y E L I N

97 Myelination, demyelination andremyelination 1593Alastair Compston

98 Multiple sclerosis and its pathophysiology 1606W. Ian McDonald, Maria A. Ron and Gavin Giovannoni

99 The diagnosis and management of multiplesclerosis 1620David H. Miller and Alan J. Thompson

100 Leukodystrophies 1645Hugo W. Moser and Kunihiko Suzuki

PA RT X I V I N F E C T I O N S

101 Host responses in central nervous systeminfection 1651David N. Irani and Diane E. Griffin

102 Viral diseases of the nervous system 1660Larry E. Davis

103 Neurological manifestations of HIV infection 1683Justin C. McArthur

104 Neurological manifestations of HTLV-Iinfection 1710Mitsuhiro Osame

105 Clinical features of human prion diseases 1716Robert G. Will

106 Bacterial infections 1728Dakshin Gullapalli and Thomas P. Bleck

107 Parasitic disease 1745Julio Sotelo and Kaethe Willms

108 Lyme disease 1754Patricia K. Coyle

109 Neurosyphilis 1766Louis Reik, Jr

110 Tuberculosis 1777Yukari C. Manabe and Richard E. Chaisson

PA RT X V T R AU M A A N D TOX I CD I S O R D E R S

111 Head trauma 1793M. Sean Grady and Tracy K. McIntosh

112 Environmental toxins and neurologicaldisease 1805Jennifer B. Sass, Donna Mergler and Ellen K.Silbergeld

113 Alcohol neurotoxicity 1814Ivan F. Diamond and Steven L. McIntire

114 Neurological consequences of drug abuse 1827John C.M. Brust

PA RT X V I D E G E N E R AT I V E D I S O R D E R S

115 Genetically engineered models ofneurodegenerative diseases 1841Philip C. Wong, Huaibin Cai, David R. Borcheltand Donald L. Price

116 Motor neuron disease 1863Pamela J. Shaw

117 The hereditary ataxias 1880Puneet Opal and Huda Y. Zoghbi

118 Huntington’s disease 1896Christopher A. Ross and Russell L. Margolis

119 Progressive cerebral degeneration of childhood 1911Gerald V. Raymond

120 Mitochondrial encephalomyopathies 1922Salvatore DiMauro and Anthony H.V. Schapira

PA RT X V I I N E U RO LO G I C A LM A N I F E S TAT I O N S O F S Y S T E M I CCO N D I T I O N S

121 Neurological aspects of pregnancy 1939Peter W. Kaplan

Contents xi






122 The brain and the cardiovascular system 1952James L. Januzzi, Jr and Guy M. McKhann

123 Neurological complications of hepatic andgastrointestinal disease 1970Benjamin H. Eidelman

124 Renal disease and electrolyte disturbances 1987G. Bryan Young and Charles F. Bolton

125 Disorders of intracranial pressure 2016Romergryko G. Geocadin and Michael A. Williams

126 Neurologic manifestations of endocrinedisease 2033Gary M. Abrams and Cheryl A. Jay

127 Neurological manifestations of hematologicaldiseases 2044Martin A. Samuels

128 Neurocutaneous syndromes 2061E. Steve Roach

129 Neurological complications after organtransplantation 2082Eelco F.M. Wijdicks

Complete two-volume index 2093

xii Contents






Contributors

E D I TO R S

Arthur K. AsburyDepartment of NeurologyHospital of the University of Pennsylvania3400 Spruce StreetPhiladelphiaPA 19104-4283USA

Guy M. McKhannThe Zanvyl Krieger Mind and Brain InstituteJohns Hopkins University338 Krieger Hall3400 N. Charles StreetBaltimoreMD 21218-2689USA

W. Ian McDonaldThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BG, UK

Peter J. GoadsbyInstitute of NeurologyNational Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

xiii






Justin C. McArthurDepartments of Neurology and EpidemiologyJohns Hopkins University School of Medicine600 N. Wolfe StreetBaltimoreMD 21287-7609USA

C O N T R I BU TO R S

Ted AbelDepartment of BiologyUniversity of Pennsylvania319 Leidy LaboratoriesPhiladelphiaPA 19104USA

Gary M. AbramsDepartment of NeurologyUCSF Mount Zion Medical Center1600 Divisadero StreetSan FranciscoCA 94115USA

Paul D. ActonDepartment of RadiologyUniversity of Pennsylvania School of MedicineRoom 3053700 Market StreetPhiladelphiaPA 19104-3147USA

Marilyn S. AlbertDepartment of Geriatrics – Massachusetts General Hospital EastDepartment of Psychiatry and NeurologyHarvard Medical School149 13th StreetCharlestownMA 02129USA

Jeffrey R. AlgerDepartment of RadiologyUniversity of California at Los AngelesLos Angeles, CAUSA

Douglas ArnoldDepartment of NeurologyMontreal Neurological InstituteCanada

Arthur K. AsburyDepartment of NeurologyHospital of the University of Pennsylvania3400 Spruce StreetPhiladelphiaPA 19104-4283USA

James AsheBrain Sciences Center, VAMCDepartment of Neurology and NeuroscienceUniversity of MinnesotaOne Veterans DriveMinneapolisMN 55417USA

Mohammed BenDebbaDepartment of NeurosurgeryJohns Hopkins Medical InstituteMeyer 7-109600 N. Wolfe StreetBaltimoreMD 21287USA

James L. BernatNeurology SectionDartmouth-Hitchcock Medical Ctr.1 Medical Center DriveLebanonNH 03756USA

Kailash P. BhatiaNational Hospital for Neurology and NeurosurgeryInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

xiv List of contributors






Shawn J. BirdDepartment of NeurologyHospital of the University of Pennsylvania3 W Gates3400 Spruce StreetPhiladelphiaPA 19104-4283USA

Thomas P. BleckDepartment of Neurology, 394University of Virginia Health Sciences CenterMcKim Hall 2025CharlottesvilleVA 22908-0001USA

Peter C. BlumbergsDepartment of NeuropathologyInstitute of Medical and Veterinary ScienceUniversity of AdelaideAdelaide 5000South Australia

Nikolai BogdukNewcastle Bone and Joint InstituteUniversity of NewcastleRoyal Newcastle HospitalPO Box 664JNewcastleNSW 2300Australia

Charles F. BoltonUniversity Western OntarioLondon Health Sciences Centre375 South StreetLondonOntario N5A 4G5Canada

David R. BorcheltDepartment of PathologyJohns Hopkins University School of Medicine558 Ross Research Building720 Rutland AvenueBaltimoreMD 21205-2196USA

Michael BradaDepartment of Clinical OncologyThe Institute of Cancer ResearchThe Royal Marsden NHS TrustDowns RoadSuttonSurrey SM2 5PTUK

Thomas BrandtDepartment of NeurologyKlinikum GrosshadernUniversity of MunichMarchioninistr. 15Munich 81377Germany

Henry BremDepartment of NeurosurgeryJohns Hopkins Medical Institutions466 CarnegieBaltimoreMD 21287USA

Steven M. BromleyNeurological Institute of New YorkColumbia-Presbyterian Medical CenterNew YorkNY 10032USA

Peter BrownSobell Department of Motor NeuroscienceInstitute of NeurologyQueen SquareLondon WC2N 3BGUK

John C.M. BrustCollege of Physicians & Surgeons of Columbia UniversityHarlem Hospital Center506 Lenox AvenueNew YorkNY 10037USA

List of contributors xv






Silvia A. BungeDepartment of PsychologyStanford University School of MedicineStanfordCA 94305USA

Huaibin CaiDepartment of PathologyJohns Hopkins University School of Medicine558 Ross Research Building720 Rutland AvenueBaltimoreMD 21205-2196USA

Stephen C. CannonDepartment of Neurobiology Harvard Medical SchoolDepartment of NeurologyMassachusetts General Hospital55 Fruit StreetBostonMA 02114USA

Louis R. CaplanHarvard Medical School and Department of NeurologyBeth Israel Deaconess Medical Center330 Brookline AvenueBostonMA 02215USA

Juan Ricardo CarhuapomaDivision of Critical Care NeurologyNeurological InstituteNew York Presbyterian HospitalColumbia University College of Physicians and SurgeonsNew YorkNYUSA

Lucinda J. CarrConsultant Pediatric NeurologistGreat Ormond Street Hospital for ChildrenNHS TrustGreat Ormond StreetLondon WC1N 3JNUK

Borka CeranicNeuro-otology DepartmentThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC2N 3BGUK

Richard E. ChaissonJohns Hopkins Medical Institutions455 1830 East Monument StreetBaltimoreMD 21205USA

Vinay ChaudhryDepartment of NeurologyJohns Hopkins School of Medicine600 N. Wolfe StreetMeyer 6-119BaltimoreMD 21287USA

Dennis W. ChoiDepartment of NeurosciencesMerck Research LaboratoriesMerck & Co. Inc., 770 Sumneytown PikeWest Point, PA 19486, USA

Hannah CockInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

Alastair CompstonUniversity of Cambridge Clinical SchoolAddenbrooke’s HospitalHills RoadCambridge CB2 2QQUK

Patricia K. CoyleDepartment of NeurologySUNY at Stony BrookHSC T12/020Stony BrookNY 11794-8121USA

xvi List of contributors






Charles A. DackisTreatment and Research CenterDepartment of PsychiatryUniversity of Pennsylvania3900 Chesnut StreetPhiladelphiaPA 19104-6178USA

Antonio R. DamasioDepartment of NeurologyUniversity of IowaCollege of Medicine200 Hawkins DriveIowa CityIA 52242-1053USA

Larry E. DavisNeurology ServiceVA Hospital2100 Ridgecrest Drive S.E.AlbuquerqueNM 87108USA

Mahlon R. DeLongDepartment of NeurologyEmory University School of MedicineWoodruff Memorial BuildingSuite 6001639 Pierce DriveAtlantaGA 30322USA

Martha Bridge DencklaKennedy–Krieger Institute707 North BroadwaySuite 516BaltimoreMD 21205USA

J. Raymond DePauloDepartment of PsychiatryJohns Hopkins University School of Medicine600 N. Wolfe StreetBaltimoreMD 21287USA

John A. DetreDepartment of NeurologyHospital of the University of Pennsylvania3400 Spruce StreetPhiladelphiaPA 19104-4283USA

Ivan F. DiamondErnest Gallo Clinic and Research CenterUniversity of California, San Francisco5858 Horton StreetSuite 200EmoryvilleCA 94608USA

Martin DichgansDepartment of NeurologyKliniken GrosshadernMardiounivistrasse 15Munchen, D-81377Germany

Salvatore DiMauroDepartment of NeurologyColumbia University College of Physicians and Surgeons630 West 168th StreetNew YorkNY 10032USA

Richard L. DotySmell and Taste CenterHospital of the University of Pennsylvania5 Ravdin Building3400 Spruce StreetPhiladelphiaPA 19104-4283USA

Patrick M. DoughertyDepartment of AnesthesiologyM.D. Anderson HospitalHoustonTXUSA

List of contributors xvii






David A. DrachmanDepartment of NeurologyUniversity of Massachusetts Medical School55 Lake Avenue NorthWorcesterMA 06155USA

Daniel B. DrachmanNeurology/Neuromuscular UnitJohns Hopkins School of Medicine6-119 Meyer Building600 N. Wolfe StreetBaltimoreMD 21287-0001USA

John S. DuncanInstitute of NeurologyQueen SquareLondon WC1N 4BGUK

Benjamin H. EidelmanDepartment of NeurologyMayo Clinic, Jacksonville4500 San Pablo RoadJacksonvilleFL 32224-1865USA

Marian L. EvattDepartment of NeurologyEmory University School of MedicineWoodruff BuildingSuite 600139 Pierce DriveAtlantaGA 30322USA

Simon F. FarmerNational Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

Thomas E. FeasbyDepartment of Clinical NeurosciencesUniversity of CalgaryFoothills Hospital1403 29th Street NWCalgaryAB T2N 2T9Canada

Robert A. FelbergDepartment of NeurologyOchsner Clinic Foundation1514 Jefferson HighwayNew OrleansLA 70121USA

Michael D. FerrariDepartment of NeurologyLeiden University Medical CentrePO Box 96002300 RC LeidenThe Netherlands

Kenneth H. FischbeckNeurogenetics BranchNational Institute of Neurological Disorders and StrokeNational Institutes of HealthBuilding 10, Room 3B-1410 Center DriveBethesdaMD 20892-1250USA

Robert S. FisherDepartment of NeurologyStanford University School of MedicineRoom H3160300 Pasteur DriveStanfordCA 94305-5235USA

Clare J. FowlerInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

xviii List of contributors






Hans-Joachim FreundMedizinische EinrichtungenUniversitat DusseldorfNeurologische KlinikMoorenstrasse 5Dusseldorf 40225Germany

John D.E. GabrieliDepartment of PsychologyStanford UniversityStanfordCA 94305USA

Romergryko G. GeocadinDepartments of Neurology and Anesthesiology Critical

Care MedicineJohns Hopkins University Hospital400 N. Wolfe StreetBaltimoreMD 21287USA

Alfred L. George, JrDepartments of Medicine and PharmacologyVanderbilt University Medical Center451 Preston BuildingNashvilleTN 37232-6304USA

Apostolos P. GeorgopoulosVeterans Administration Medical CenterDepartment of NeurologyNeuroscience and PsychiatryUniversity of MinnesotaOne Veterans DriveMinneapolisMN 55417USA

Gavin GiovannoniInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

Jonathan D. GlassDepartment of NeurologyEmory University School of MedicineWhitehead Biomedical Research Building615 Michael StreetWWB 600Atlanta GA 30322USA

Peter J. GoadsbyInstitute of NeurologyThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

Susanne GoldsteinHuman Motor Control SectionNational Institute of Neurological Disorders and StrokeNational Institutes of HealthBldg 10, Room 5N22610 Center Drive, MSC 1428BethesdaMD 20892-1428USA

Estrella Gómez-TortosaServicio de NeurologíaFundación Jiménez DíazAvda. Reyes Católicos 2Madrid 28040Spain

Barry GordonDivision of Cognitive NeurologyThe Johns Hopkins University School of Medicine222 Meyer, 600 N. Wolfe St.BaltimoreMD 21287-7222USA

M. Sean GradyDepartment of NeurosurgeryHospital of the University of Pennsylvania5th floor, Silverstein Pavilion3400 Spruce StreetPhiladelphiaPA 19104-4283USA

List of contributors xix






Steven B. Graff-RadfordCedars–Sinai Medical Center444 South San Vicente, Suite 1101Los AngelesCA 90048USA

Joel D. GreenspanDepartment of OCB5Dental School University of MarylandBaltimore, MD, USA

Michael D. GreiciusDepartment of Psychiatry and Behavioral SciencesStanford University416 Quarry RoadCA 94305USA

Diane E. GriffinDepartment of Molecular Microbiology and ImmunologyJohns Hopkins University Bloomberg School of Public

Health615 N. Wolfe StreetBaltimoreMD 21205USA

Robert C. GriggsDepartment of NeurologyUniversity of Rochester School of Medicine and Dentistry601 Elmwood AvenueRochesterNY 14642USA

James C. GrottaDepartment of NeurologyUniversity of Texas HoustonMedical School Bldg. MSB 7-0446431 Fannin StreetHoustonTX 77030USA

Michael A. GrotzerDivision of Oncology/HematologyUniversity Children’s HospitalSteinwiesstrasse 75CH-8032ZurichSwitzerland

John H. GrowdonDepartment of NeurologyHarvard Medical School Memory Disorders UnitNeurology ServiceMassachusetts General HospitalWACC 830BostonMA 02115USA

Renzo GuerriniNeurosciences UnitInstitute of Child Health and Great Ormond Street

HospitalUniversity CollegeThe Wolfson CentreMecklenburgh SquareLondon WC1N 2AP, UK

Dakshin GullapalliDepartment of NeurologyUniversity of Virginia School of MedicineCharlottesvilleVA 22908USA

Joost HaanDepartment of NeurologyLeiden University Medical CentrePO Box 96002300 RC LeidenThe Netherlands

Ronald G. HallerNeuromuscular CenterInstitute for Exercise and Environmental MedicinePresbyterian Hospital7232 GreenvilleDallasTX 75231USA

Mark HallettNINDSNational Institutes of HealthBuilding 10, Room 5N 22610 Center Drive, MSC 1428BethesdaMD 20892-1428USA

xx List of contributors






Shaheen HamdyDepartment of GI ScienceClinical Sciences BuildingHope HospitalEccles Old RoadSalfordManchester M6 8HDUK

Daniel F. HanleyDepartment of NeurologyJohns Hopkins Hospital600 N. Wolfe StreetMeyer 6-113BaltimoreMD 21287USA

Paul J. HarrisonUniversity Departments of Psychiatry and Clinical

Neurology (Neuropathology)Warneford HospitalNeurosciences BuildingOxford OX3 7JXUK

John Hart, JrDonald W. Reynolds Center on AgingUniversity of Arkansas Medical School4301 W. Markham St.Little RockAR 72205, USA

Hans-Peter HartungDepartment of NeurologyHeinrich-Heine-UniversityDuesseldorfMoorenstr. 5D-40225 DuesseldorfGermany

Kenneth M. HeilmanDepartment of NeurologyUniversity of Florida College of MedicineBox 100238GainesvilleFl 32610USA

Hans-Joachen HeinzeKlin. NeurophysiologieOtto-von-GuerickeUniversitat MagdeburgLeipziger StreetMagdeburg 44,3910Germany

David B. HellmannDepartment of MedicineJohns Hopkins Bayview Medical CenterJohns Hopkins University School of Medicine4940 Eastern AvenueBaltimoreMD 21224USA

Michael D. HillDepartment of Clinical NeurosciencesUniversity of Calgary6020 Dalcastle Crescent N.W.CalgaryAB T3A 1S4Canada

Jeremy HobartDepartment of Neurology The Peninsula Medical SchoolDerriford HospitalPlymouth, Devon, UK

Reinhard HohlfeldInstitute for Clinical Neuroimmunology and Department

of NeurologyLudwig-Maximillians UniversityKlinikum GrosshadenMarchioninistrasse 15D-81366 MunichGermany

Alexander H. Hoon, JrDepartment of PediatricsJohns Hopkins University School of MedicineKennedy–Krieger Institute707 N. BroadwayBaltimoreMD 21205USA

Fay B. HorakNeurological Sciences InstituteDepartment of Pharmacology and PhysiologyOregon Health Sciences UniversityPortlandOR 97201USA

List of contributors xxi






Jyh-Gong HouParkinson’s Disease Center and Movement Disorders

ClinicDepartment of NeurologyBaylor College of Medicine6550 Fannin, Suite 1801HoustonTX 77030USA

Matthew A. Howard IIIDivision of NeurosurgeryUniversity of Iowa Hospitals and Clinics200 Hawkins DriveIowa CityIA 52242USA

Orest HurkoDepartment of Investigational MedicineGlaxoSmithKline PharmaceuticalsNeurology Centre for Excellence in Drug DiscoveryNew Frontiers Science Park North H17 2-233Third AvenueHarlowEssex CM19 5AWUK

Bradley T. HymanDepartment of NeurologyMassachusetts General Hospital East 149 13th StreetCharlestownMA 02129USA

David N. IraniDepartment of Molecular Biology and ImmunologyBloomberg School of Public HealthJohns Hopkins University615 N. Wolfe StreetBaltimoreMD 21205USA

Michael C. IrizarryDepartment of NeurologyMassachusetts General Hospital East149 13th StreetCharlestownMA 02129USA

Joseph JankovicParkinson’s Disease Center and Movement Disorders

ClinicDepartment of NeurologyBaylor College of Medicine6550 Fannin, Suite 1801HoustonTX 77030USA

James L. Januzzi, JrCardiology Division and Department of MedicineMassachusetts General HospitalHarvard Medical School55 Fruit StreetBoston MA 02114USA

Cheryl A. JayUCSF Department of NeurologyNeurology Service, 4M62San Francisco General Hospital1001 Potrero AvenueSan FranciscoCA 94110USA

David C. JimersonDepartment of PsychiatryBeth Israel Deaconess Medical Center and Harvard

Medical School330 Brookline AvenueBostonMA 02215USA

Michael V. JohnstonKennedy–Krieger InstituteDepartments of Neurology and PediatricsJohns Hopkins University School of MedicineSuite 610707 North BroadwayBaltimoreMD 21205USA

Ricardo E. JorgeDepartment of PsychiatryUniversity of Iowa College of Medicine200 Hawkins DriveIowa CityIA 52242USA

xxii List of contributors






Peter W. KaplanDepartment of NeurologyJohns Hopkins Medical Center at Bayview4940 Eastern AvenueBaltimoreMD 21224-2780USA

Barbara Illowsky KarpOffice of the Clinical Director and Human Motor Control

Section NINDSNational Institutes of HealthBuilding 10, Room 5N 22610 Center Drive, MSC 1428BethesdaMD 20892-1428USA

Raymond J. Kelleher, IIIMassachusetts Institute of Technology77 Massachusetts AvenueBldg. E25-435CambridgeMA 02139-4307USA

Christopher KennardDivision of Neurosciences and Psychological MedicineImperial College School of MedicineCharing Cross HospitalSt Dunstan’s RoadLondon W6 8RPUK

Bernd C. KieseierDepartment of NeurologyHeinrich-Heine-UniversityMoorenstrasse 5D-40225 DüsseldorfGermany

John LaterraDepartment of NeurologyJohns Hopkins Medical InstitutionsCarnegie Room 490600 N. Wolfe StreetBaltimoreMD 21287-7709USA

Jin-Moo LeeDepartment of Neurology Box 8111Washington University School of Medicine660 South Euclid AvenueSt. LouisMO 63105USA

R. John LeighDepartment of NeurologyUniversity Hospitals of Cleveland11100 Euclid AvenueClevelandOH 44106USA

Ramon C. LeiguardaRaúl Carrea Institute of Neurological ResearchF.L.E.N.I.Montanueses 2325Buenos Aires1428 1112Argentina

Frederick A. LenzDepartment of NeurosurgeryJohns Hopkins Hospital7-113 Meyer600 N. Wolfe StreetBaltimoreMD 21287USA

Andrew P. LiebermanNeurogenetics BranchNational Institute of Neurological Disorders and StrokeNational Institutes of HealthBuilding 10, Room 3B-1410 Center DriveBethesdaMD 20892-1250USA

Richard B. LiptonDepartment of Neurology, Epidemiology and Social

MedicineAlbert Einstein College of MedicineNew YorkUSAand Innovative Medical Research1200 High Ridge RoadStamford, CT 06905, USA

List of contributors xxiii






Donlin M. LongDepartment of NeurosurgeryJohns Hopkins Hospital600 N. Wolfe StreetMeyer 7-109BaltimoreMD 21287USA

Linda M. LuxonNeuro-otology DepartmentThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC2N 3BGUK

Dean F. MacKinnonDepartment of PsychiatryJohns Hopkins University School of Medicine600 N. Wolfe StreetBaltimoreMD 21287USA

Yukari C. ManabeDepartment of MedicineJohns Hopkins Medical Institutions455 1830 East Monument StreetBaltimoreMD 21205USA

Richard J. MannionNeural Plasticity Research GroupDepartment of Anesthesia and Critical CareMassachusetts General Hospital East and Harvard

Medical School149 13th StreetCharlestownMA 02129USA

Russell L. MargolisDepartment of Psychiatry and Behavioral SciencesJohns Hopkins University600 N. Wolfe StreetBaltimoreMD 21205USA

Christopher J. MathiasImperial College School of MedicineNeurovascular Medicine UnitSt. Mary’s HospitalPraed StreetLondon W2 1NYUK

Justin C. McArthurDepartments of Neurology and EpidemiologyJohns Hopkins University600 N. Wolfe StreetBaltimoreMD 21287-7609USA

W. Ian McDonaldInstitute of NeurologyThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BG, UK

John W. McDonald, IIIDepartment of Neurology and Neurological SurgeryCenter for the Study of Nervous System InjuryMcMillan Bldg.Washington University School of MedicineCampus Box 8111, Room 204660 South Euclid AvenueSt. LouisMO 63110-1010USA

Steven L. McIntireDepartment of Neurology, UCSFSuite 2005858 Horton StreetEmoryvilleCA 94608USA

Tracy K. McIntoshDepartment of NeurosurgeryHospital of the University of Pennsylvania5th floor, Silverstein PavilionPhiladelphiaPA 19104-4283USA

xxiv List of contributors






Guy M. McKhannThe Zanvyl Krieger Mind & Brain InstituteJohns Hopkins University338 Krieger Hall3400 N. Charles StreetBaltimoreMD 21218-2689USA

Guy M. McKhann, IIDepartment of Neurological SurgeryColumbia UniversityNeurological Institute Room 428710 168th StreetNew YorkNY 10032USA

Pamela J. McLeanDepartment of NeurologyMassachusetts General Hospital East149 13th StreetCharlestownMA 02129USA

Gillian E. MeadDepartment of Clinical and Surgical Sciences (Geriatric

Medicine)University of Edinburgh21 Chalmers StreetEdinburgh EH3 9EWUK

Donna MerglerCINBOISEUniversity of Quebec at MontrealCP 8888 succ Centre-villeMontrealQuebec H3C 3P8Canada

Bruce L. MillerDepartment of NeurologyUCSF School of MedicineParnassus AvenueSan Francisco CA 94143USA

David H. MillerUniversity Department of Clinical NeurologyInstitute of NeurologyQueen SquareLondon WC2N 3BGUK

Ganeshwaran H. MochidaDepartment of NeurologyBeth Israel Deaconess Medical CenterHarvard Institutes of Medicine, Room 81677 Avenue Louis PasteurBostonMA 02115USA

Robert Y. MooreDepartment of NeurologyUniversity of PittsburghLiliane S. Kaufmann Building3471 5th Avenue, Suite 811PittsburghPA 15213USA

Antony MorlandDepartment of PsychologyRoyal Holloway CollegeEghamSurrey TW20 0EXUK

Hugo W. MoserDepartments of Neurology and PediatricsJohns Hopkins UniversityKennedy–Krieger Institute707 N. BroadwayRoom 503BaltimoreMD 21205USA

Eric A. NofzingerDepartment of Psychiatry and NeuroscienceUniversity of Pittsburgh School of Medicine3811 O’Hara StreetPittsburghPA 15213USA

List of contributors xxv






John G. NuttDepartment of Neurology L226Oregon Health Sciences University3181 SW Sam Jackson Park RoadPortlandOR 97201USA

Charles P. O’BrienTreatment and Research CenterUniversity of Pennsylvania Department of Psychiatry3900 Chesnut StreetPhiladelphiaPA 19104-6178USA

Puneet OpalInstitute for Molecular GeneticsDepartment of PediatricsBaylor College of MedicineOne Baylor PlazaHoustonTX 77030USA

Richard W. OrrellDepartment of Clinical NeurosciencesRoyal Free and University College Medical SchoolRoyal Free CampusRowland Hill StreetLondon NW3 2PFUK

Mitsuhiro OsameThe Third Dept. of Internal MedicineFaculty of MedicineKagoshima University HospitalSakuragaoka 8 35-1Kagoshima 890-8520Japan

Lucio ParmeggianiInstitute of Child Health and Great Ormond Street

Hospital for ChildrenUniversity College LondonThe Wolfson CentreMecklenburgh SquareLondon WC1N 2APUK

Roy A. PatchellDepartment of Neurosurgery and NeurologyUniversity of Kentucky Medical Center800 Rose StreetLexingtonKY 40536USA

Emilio PeruccaClinical Pharmacology UnitDepartment of Internal Medicine and TherapeuticsUniversity of PaviaPiazza Botta 1027100 PaviaItaly

Ognen A.C. Petroff

Department of NeurologyYale University Medical School333 Cedar StreetNew Haven, CT 06510USA

Peter C. PhillipsDepartment of NeurologyChildren’s Hospital of Philadelphia34th & Civic Center Blvd.4th Floor Wood Bldg.PhiladelphiaPA 19104-4399USA

David E. PleasureDepartments of Neurology and PediatricsChildren’s Hospital of PhiladelphiaRoom 516H Abramson Research Building3517 Civic Center BoulevardPhiladelphiaPA 19104USA

Samuel J. PleasureDepartment of NeurologyUniversity of California, San FranciscoRoom S-256C513 Parnassus AvenueSan FranciscoCA 94143USA

xxvi List of contributors






John D. PollardInstitute of Clinical NeurosciencesRoyal Prince Alfred HospitalUniversity of SydneyCamperdownSydney NSW 2050Australia

Russell K. PortenoyDepartment of Pain Medicine and Palliative CareBeth Israel Medical CenterFirst Avenue at 16th StreetNew YorkNY 10003USA

Jerome B. PosnerDepartment of NeurologyMemorial Sloan-Kettering Cancer Center1275 York AvenueNew YorkNY 10021USA

Donald L. PriceDivision of NeuropathologyDepartments of Neurology, Pathology and NeuroscienceJohns Hopkins Medical Institutions558 Ross Research Bldg.720 Rutland AvenueBaltimoreMD 21205USA

James W. PrichardDepartment of Neurology Box 60 Yale Medical SchoolWest TisburyMA 02575USA

Stanley B. PrusinerInstitute for Neurodegenerative DiseasesDepartments of Neurology and of Biochemistry and

BiophysicsBox 0518, Parnassus AvenueUniversity of California, San FranciscoSan FranciscoCA 94143-0518USA

Scott L. RauchDepartment of Psychiatry, Harvard Medical SchoolMassachusetts General Hospital East149 13th StreetBostonMA 02129USA

Gerald V. RaymondDepartment of NeurologyJohns Hopkins University School of MedicineKennedy–Krieger Institute707 N. BroadwayBaltimoreMD 21205USA

Jeremy H. ReesNational Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

Michael J. ReganDivision of RheumatologyJohns Hopkins University School of Medicine1830 East Monument StreetSuite 7500BaltimoreMD 21205USA

Louis Reik, Jr.Department of NeurologyUniversity of Connecticut Health Center263 Farmington AvenueFarmingtonCT 06030USA

Mary ReillyDepartment of Clinical NeurologyThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC2N 3BGUK

E. Steve RoachDepartment of NeurologyUniversity of TexasSouthwestern Medical Center5323 Harry Hines Blvd.DallasTX 75235USA

List of contributors xxvii






Robert G. RobinsonDepartment of PsychiatryUniversity of Iowa College of Medicine200 Hawkins DriveIowa CityIA 52242USA

Maria A. RonInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

Howard J. RosenDepartment of NeurologyUCSF School of Medicine1600 Divisadero StreetSan Francisco CA 94115USA

Christopher A. RossDepartments of Psychiatry and NeuroscienceJohns Hopkins University School of Medicine720 Rutland AvenueBaltimoreMD 21205USA

Douglas L. RothmanDepartment of Diagnostic RadiologyYale University Medical School333 Cedar StreetNew Haven, CT 06510USA

Roberto SalvatoriDivision of Endocrinology and MetabolismDepartment of MedicineJohns Hopkins University School of MedicineSuite 3331830 E. Monument StreetBaltimoreMD 21287USA

Martin A. SamuelsDepartment of NeurologyBrigham and Women’s Hospital75 Francis StreetBostonMA 02115USA

Jennifer B. SassProgram in Human Health and the EnvironmentUniversity of Maryland School of Medicine9–34 MSTF10 South Pine StreetBaltimoreMD 21201-1596USA

Anthony H.V. SchapiraUniversity Department of Clinical NeurosciencesRoyal Free & UCH Medical SchoolRowland Hill StreetLondon NW3 2PFUK

Ola A. SelnesDepartment of NeurologySchool of MedicineDepartment of Cognitive ScienceKrieger Mind/Brain InstituteJohns Hopkins University600 N. Wolfe StreetBaltimoreMD 21287-7222USA

Michael E. SelzerDepartment of NeurologyHospital of the University of Pennsylvania3 W. Gates3400 Spruce StreetPhiladelphiaPA 19104-4283USA

Sudha SeshadriFramingham Heart StudyDepartment of NeurologyBoston University School of MedicineFraminghamBostonMA 01702USA

Pamela J. ShawDepartment of NeurologyE FloorMedical School University of SheffieldBeech Hill RoadSheffield S10 2RXUK

xxviii List of contributors






Simon ShorvonInstitute of NeurologyQueen SquareLondon WC1N 3BGUK

Ellen K. SilbergeldDepartment of Environmental Health SciencesBloomberg School of Public HealthJohns Hopkins University615 North Wolfe StreetBaltimoreMD 21205USA

Stephen D. SilbersteinJefferson Headache CenterDepartment of NeurologyThomas Jefferson University Hospital111 South 11 StreetSuite 8130PhiladelphiaPA 19107USA

Harvey S. SingerDepartment of NeurologyJohns Hopkins University600 N. Wolfe StreetHarvey 811BaltimoreMD 21287USA

Julio SoteloNational Institute of Neurology of MexicoInsurgentes Sur 3877Mexico City 14269Mexico

Judith M. SpiesInstitute of Clinical NeuroscienceUniversity of SydneyRoyal Prince Alfred HospitalMissenden RoadCamperdown NSW 2050Australia

Dan J. SteinMRC Anxiety Disorders UnitDepartment of PsychiatryUniversity of StellenboschCape TownSouth Africa

Donna J. StephensonDepartment of NeurologyJohns Hopkins University600 N. Wolfe StreetHarvey 811BaltimoreMD 21287USA

John H. StoneJohns Hopkins Vasculitis CenterJohns Hopkins School of Medicine1830 East Monument StreetSuite 7500BaltimoreMD 21205USA

Marcus A. StoodleyUniversity of New South WalesInstitute of Neurological SciencePrince of Wales HospitalRandwickNSW 1031Australia

Kunihiko SuzukiNeuroscience Center-CB 7250University of North CarolinaChapel HillNC 27599-7250USA

J. Paul TaylorNeurogenetics BranchNational Institute of Neurological Disorders and StrokeNational Institutes of HealthBuilding 10, Room 3B-1410 Center DriveBethesdaMD 20892-1250USA

List of contributors xxix






Peter K. ThomasDepartment of Clinical NeurologyInstitute of NeurologyQueen SquareLondon WC2N 3BGUK

Pierre ThomasService de Neurologie and Consultation d’EpileptologieCHU de NICEFrance

Alan J. ThompsonNeurological Outcome Measurement UnitInstitute of NeurologyQueen SquareLondon WC2N 3BGUK

Philip D. ThompsonDepartment of NeurologyRoyal Adelaide HospitalUniversity Department of MedicineUniversity of AdelaideAdelaide 5000South Australia

Klaus V. ToykaDepartment of NeurologyJulius-Maximilians-UniversityJosef-Schneider-Strasse 1197080 WürzburgGermany

Jerrold L.VitekDepartment of NeurologyEmory University School of MedicinePO Box Drawer VSuite 6001639 Pierce DriveAtlantaGA 30322USA

Bavanisha VythilingumMRC Anxiety DisordersDepartment of PsychiatryUniversity of StellenboschCape TownSouth Africa

Christopher A. WalshDepartment of NeurologyBeth Israel Deaconess Medical CenterHarvard Medical School77 Avenue Louis PasteurBostonMA 02115USA

Gary S. WandDepartment of MedicineThe Johns Hopkins University School of MedicineSuite 3331830 E. Monument StreetBaltimoreMD 21205USA

Charles P. WarlowDepartment of Clinical NeurosciencesThe University of EdinburghEdinburghUK

Bryce K.A. WeirSection of Neurosurgery, Department of SurgeryUniversity of Chicago Pritzker School of Medicine5841 S. Maryland AvenueChicagoIL 60637USA

Richard P. WhiteInstitute of NeurologyNational Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

Eelco F.M. WijdicksDepartment of NeurologyMayo Clinic and Mayo Foundation200 First Street, S.W.RochesterMN 55905USA

xxx List of contributors






Robert G. WillNational CJD Surveillance UnitWestern General HospitalUniversity of EdinburghUK

Michael A. WilliamsDepartment of NeurologyJohns Hopkins University School of Medicine600 N. Wolfe StreetBaltimoreMD 21287USA

Kaethe WillmsDepartment of Microbiology Faculty of MedicineNational University of MexicoInsurgentes Sur 3877 C.P.Mexico City 14269Mexico

Oliver J. WisemanThe National Hospital for Neurology and NeurosurgeryQueen SquareLondon WC1N 3BGUK

Barbara E. WolfeDepartment of PsychiatryBeth Israel Deaconess Medical CenterHarvard Medical School330 Brookline AvenueBostonMA 02215USA

Philip C. WongDivision of NeuropathologyDepartment of PathologyJohns Hopkins University School of Medicine558 Ross Research Building720 Rutland AvenueBaltimoreMD 21205-2196USA

Clifford J. WoolfNeural Plasticity Research GroupDepartment of Anesthesia and Critical CareMassachusetts General Hospital East and Harvard

Medical School149 13th StreetCharlestownMA 02129USA

Jie WuDepartment of NeurologyBarrow Neurological InstituteSt. Joseph Hospital and Medical Center350 W. Thomas RoadPhoenixAZ 85013-4496USA

G. Bryan YoungClinical Neurological SciencesUniversity Western OntarioLondon Health Sciences Centre375 South StreetLondonOntario N5A 4G5Canada

John ZajicekDepartment of NeurologyLevel 10, Derriford HospitalDerriford RoadPlymouthDevon PL6 8DHUK

David S. ZeeDepartment of NeurologyJohns Hopkins Hospital600 N. Wolfe StreetBaltimoreMD 21287USA

Huda V. ZoghbiDepartment of Molecular and Human GeneticsHoward Hughes Medical InstituteBaylor College of Medicine, T807One Baylor PlazaMail Stop 225HoustonTX 77030USA

List of contributors xxxi






Preface

In the 10 years since the second edition of Diseases of theNervous System appeared, extraordinary change has takenplace in the field of neurosciences, both basic and clinical.In accordance with what is happening in the neuro-sciences, major changes have occurred with this thirdedition. Organization of subject matter and ordering oftopics has changed substantially, in some cases dramati-cally. Of the contributors, a number of the authors for theprevious two editions have written again in their specialfields of expertise, but overall, more than three-quarters ofthe contributors are new to these pages. Two new editorshave been added, Peter J. Goadsby and Justin C. McArthur;Cambridge University Press is the new publisher; and anew subtitle, Clinical Neuroscience and TherapeuticPrinciples has supplanted the previous one.

Despite these changes, the purpose of these referencevolumes remains the same, namely to summarize what thescientific method, as applied to problems of neurologicaldysfunction, has taught us about the pathophysiology ofneurological disorders. To say it differently, our purpose isnot to focus on incidence, natural history, phenomenologyand semeiology of neurological disorders, although theseaspects are touched upon, but rather to focus on the mech-anisms of neurological disease and the principles thatform the basis for management and therapeutics. In addi-tion to the emphasis on pathophysiology and principles oftherapy, three other axioms guided the planning of thisedition, just as they did for the previous editions. First, con-tributors were chosen who brought clinical expertise tobear as well as scientific authoritativeness. Second, wetried to assure that the depth with which each topiccovered was relatively uniform from one chapter to thenext. There is a corollary to this principle of uniformity.Given that these volumes are a general reference coveringthe entire field of neurological disorders, each chapter is,by necessity, a relatively brief summary of where matters

xxxiii






diseases of the nervous system - assets · diseases of the nervous system the third edition of a...

Documents