Neurology and clinical neuroscience

1572 Pages·2007·83.571 MB·English

by  A. Schapira, et al.,

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NEUROLOGYAND CLINICALNEUROSCIENCE ISBN-13: 978-0-323-03354-1
ISBN-10: 0-323-03354-7
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Library of Congress Cataloging-in-Publication Data
Neurology and clinical neuroscience / editor, Anthony H.V. Schapira; associate editors, Edward Byrne...
[et al.].
p. ; cm.
ISBN 0-323-03354-7
1. Neurology—Textbooks. 2. Neurosciences—Textbooks. 3. Nervous system—Diseases—Textbooks.
I. Schapira, Anthony H. V. (Anthony Henry Vernon). II. Byrne, Edward, M.D.
[DNLM: 1. Nervous System Diseases. 2. Neurology. WL140 N49269 2007]
RC346.N4514 2007
616.8—dc22 2006046665
Acquisitions Editor: Susan Pioli
Developmental Editor: Heather Krehling
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Senior Project Manager: Peter Faber
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To my wife, Laura, and my daughter, Sarah
C O N T R I B U T O R S
Rasheed A. Afinowi, MBBS, MSc Doris-Eva Bamiou, MD, MSc, MPhil
Clinical Research Fellow, Victor Horsley Department of Honorary Senior Lecturer, Institute of Child Health,
Neurosurgery, National Hospital for Neurology and University College London; Consultant in Audiological
Neurosurgery, London, United Kingdom Medicine, National Hospital for Neurology and
Intracranial Hemorrhage: Aneurysmal, Idiopathic, and Hypertensive Neurosurgery, London, United Kingdom
Vestibular System Disorders
Rexford S. Ahima, MD, PhD
Associate Professor of Medicine, Department of Medicine, Christopher Bass, MA, MD, FRCPsych
Division of Endocrinology, Diabetes, and Metabolism, Honarary Senior Clinical Lecturer in Psychiatry, University
University of Pennsylvania School of Medicine; Department of Psychiatry, Warneford Hospital;
Endocrinologist, Hospital of the University of Consultant in Liaison Psychiatry, John Radcliffe
Pennsylvania, Philadelphia, Pennsylvania Hospital, Oxford, United Kingdom
Neurology of Endocrinology Conversion and Dissociation Syndromes
Marta Altieri MD, PhD
Michel Baulac, MD
Department of Neurological Sciences, 1st Faculty of
Professor of Neurology and Head, Epileptology Unit,
Medicine and Surgery, University of Roma La Sapienza,
Clinique Paul Castaigne, Hôpital de la Pitié-Salpêtrière,
Rome, Italy
Paris, France
Cerebral Venous Thrombosis
Clinical Spectrum [Epilepsy]
Hortensia Alvarez, MD
Antonio Belli, MD, FRCS(SN)
Neuroradiologist, Diagnostic and Therapeutic Vascular,
Neurologist, Victor Horsley Department of Neurosurgery,
Neuroradiology, Bicetre Hospital, Kremlin Bicetre,
National Hospital for Neurology and Neurosurgery,
France
London, United Kingdom
Arteriovenous Malformations of the Brain and Spinal Cord
Intracranial Hemorrhage: Aneurysmal, Idiopathic, and Hypertensive
Shalini A. Amukotuwa, MBBS(Hons), BMedSci
Paul Bentley, MA, MRCP
Departmental Research Fellow, Department of
Specialist Registrar–Neurology, St. Mary’s Hospital,
Neurosciences, St. Vincent’s Hospital, Melbourne,
London, United Kingdom
Victoria, Australia
Prothrombotic States and Related Conditions
Spinal Disease: Neoplastic, Degenerative, and Infective Spinal Cord Diseases
and Spinal Cord Compression
Italo Biaggioni, MD
Yaacov Anziska, MD Professor of Medicine and Pharmacology, Vanderbilt
School of Medicine, Nashville, Tennessee
Associate in Neurology, Columbia University College of
Physicians and Surgeons; Resident in Clinical Orthostatic Hypotension
Neurophysiology, NewYork–Presbyterian Hospital,
New York, New York Marcelo E. Bigal, MD, PhD
Metabolic, Immune-Mediated, and Toxic Neuropathies Assistant Professor of Neurology, Albert Einstein College
of Medicine of Yeshiva University; Director of Research,
Gregory J. Artz, MD Montefiore Headache Center, Bronx, New York; Director
Fellow, Department of Neurotology, Michigan Ear of Research, The New England Center for Headache,
Institute, Farmington Hills, Michigan Stamford, Connecticut
Auditory System Disorders Chronic Daily Headache
Messoud Ashina, MD, PhD, DMedSci Rolfe Birch, MChir, FRCS, FRCS(Eng)
Associated Professor of Neurology, University of Professor in Neurological Orthopaedic Surgery, University
Copenhagen School of Medicine; Staff Neurologist, College London, London; Head, Peripheral Nerve Injury
Danish Headache Center—University of Copenhagen, Unit, Royal National Orthopaedic Hospital, Stanmore,
Copenhagen, Denmark United Kingdom
Tension-Type Headache Tumors of the Peripheral Nerve
vii
viii Contributors
Bastiaan R. Bloem, MD, PhD Georgina Burke, BSc, MBBS, MRCP
Neurologist, University Medical Center, Nijmegen, The Specialist Registrar–Neurology, Wessex Neurological
Netherlands Centre, Southampton General Hospital, Southampton,
Gait Disturbances and Falls United Kingdom
Neuromuscular Junction Disorders
Bradley F. Boeve, MD
Associate Professor of Neurology, Mayo Clinic College of Jean Lud Cadet, MD
Medicine; Consultant, Divisions of Behavioral Neurology Chief, Molecular Neuropsychiatry Branch, National
and Movement Disorders and Mayo Sleep Disorders Institute on Drug Abuse, National Institutes of Health,
Center, Mayo Clinic, Rochester, Minnesota Bethesda, Maryland
Dementia with Lewy Bodies Environmental Toxins
Lysa Boissé, MD, MSc
John N. Caviness, MD
Resident in Neurology, Queen’s University/Kingston
Professor of Neurology, Mayo Clinic College of Medicine;
General Hospital, Kingston, Ontario, Canada
Consultant, Division of Movement Disorders, Mayo
Neurological Disorders Associated with Human Immunodeficiency Virus
Clinic Scottsdale, Scottsdale, Arizona
Infection
Huntington Disease
Karen I. Bolla, PhD
Stephen D. Cederbaum, MD
Associate Professor of Neurology, Psychiatry and Behavior
Professor of Pediatrics and Human Genetics, David
Sciences, and Environmental Health Sciences,
Geffen School of Medicine at UCLA, Los Angeles,
Neurology, Johns Hopkins School of Public Health;
California
Director of Neuropsychology, Johns Hopkins Bayview
Medical Center, Baltimore, Maryland Urea Cycle Disorders
Environmental Toxins
Patrick Chauvel, MD
John G. F. Boughey, MBChB, MRCP Professor of Physiology, Faculty of Medicine, Université
Behavioral Neurology Fellow, Department of Neurology, Aix-Marseille; Head, Department of Clinical
Mayo Clinic, Jacksonville, Florida Neurophysiology, Hôpital de la Timone; Director, U751
Alzheimer’s Disease Epilepsy and Cognition, INSERM, Marseille, France
Developmental Defects and Pathophysiology [Epilepsy]
Kevin B. Boylan, MD
Assistant Professor, Department of Neurology, Mayo Patrick F. Chinnery, MBBS(Hons), PhD, MRCPath,
Medical School; Mayo Clinic, Jacksonville, Florida FRCP
Amyotrophic Lateral Sclerosis Professor of Neurogenetics, The University of Newcastle
upon Tyne; Honorary Consultant Neurologist, Newcastle
Robert Brenner, MD, FRCP
Hospitals NHS Trust, Newcastle upon Tyne, United
Consultant Neurologist, Department of Academic
Kingdom
Neuroscience, Royal Free Hospital, London, United
Metabolic Myopathies (Including Mitochondrial Disorders)
Kingdom
Investigations in Multiple Sclerosis
Soke Miang Chng, MD
Neuroradiologist, Diagnostic and Therapeutic Vascular
Edward B. Bromfield, MD, MEd
Neuroradiology, Bicetre Hospital, Kremlin Bicetre,
Associate Professor of Neurology, Harvard Medical School;
France
Chief, Division of Epilepsy and Sleep, Department of
Arterionenous Malformations of the Brain and Spinal Cord
Neurology, Brigham and Women’s Hospital, Boston,
Massachusetts
Mark J. Cook, MD
Drug Treatment [Epilepsy]
Professor of Neurology, University of Melbourne School
John C. M. Brust, MD of Medicine, Parkville; Director, Department of
Professor of Clinical Neurology, Columbia University Neurology, St. Vincent’s Hospital, Melbourne,
College of Physicians and Surgeons; Director, Victoria, Australia
Department of Neurology, Harlem Hospital Center, Disorders ofTaste and Smell; Spine and Spinal Cord: Developmental
New York, New York Disorders; Spinal Disease: Neoplastic, Degenerative, and Infective Spinal
Neurology of Drug and Alcohol Addictions Cord Diseases and Spinal Cord Compression
Camilla Buckley, BM, BCh, MRCP, DPhil John R. Crawford, PhD
Lecturer in Clinical Neurology, Department of Clinical Professor, School of Psychology, University of Aberdeen,
Neurology, University of Oxford; Honorary Specialist Aberdeen, UK; Consultant Clinical Neuropsychologist,
Registrar, Neurosciences Group, Radcliffe Infirmary, Grampian University Hospitals NHS Trust, Aberdeen,
Oxford, United Kingdom United Kingdom
Neuromuscular Junction Disorders Executive Function and Its Assessment
contributors ix
Eric A. Crombez, MD Günther Deuschl, MD
Assistant Professor, Department of Pediatrics, Division of Professor of Neurology, University of Kiel; Head of
Medical Genetics, David Geffen School of Medicine at Department, Klinik für Neurologie, Universitats
UCLA, Los Angeles, California Klinikum Schleswig-Holstein, Campus Kiel, Germany
Urea Cycle Disorders Tremor
David W. Dodick, MD
Jeffrey L. Cummings, MD
Professor of Neurology, Mayo Clinic College of Medicine;
The Augustus S. Rose Professor of Neurology, David
Neurology Consultant, Mayo Clinic, Scottsdale, Arizona
Geffen School of Medicine at UCLA; Director, UCLA
Other Secondary Headache Disorders
Alzheimer’s Disease Center; Director, Deane F. Johnson
Center for Neurotherapeutics, UCLA Medical Center, Los Erika D. Driver-Dunckley, MD
Angeles, California Clinical Instructor, Mayo Clinic College of Medicine,
Affective Disorders Scottsdale; Senior Associate Consultant, Mayo Clinic
Hospital, Phoenix, Arizona
Huntington Disease
Marinos C. Dalakas, MD
Chief, Neuromuscular Diseases Section, National
James Evans, MD
Institutes of Health, Bethesda, Maryland; Professor of
Assistant Professor, Department of Neurosurgery, Jefferson
Neurology, University of Athens Medical School; Chief,
Medical College of Thomas Jefferson University,
Section of Neuroimmunology and Neuromuscular
Philadelphia, Pennsylvania
Diseases, Department of Pathophysiology, University
Trigeminal Neuralgia and Other Facial Pain
Hospital, Athens, Greece
Inflammatory Myopathies Steven K. Feske, MD
Associate Professor of Neurology, Harvard Medical
Josep Dalmau, MD, PhD School; Chief, Division of Stroke and Neurovascular
Diseases, Department of Neurology, Brigham and
Associate Professor of Neurology, University of
Women’s Hospital, Boston, Massachusetts
Pennsylvania School of Medicine; Neurologist, Hospital
of the University of Pennsylvania, Philadelphia, Neurology of Pregnancy and the Puerperium
Pennsylvania
John K. Fink, MD
Paraneoplastic Disorders of the Nervous System
Professor, Department of Neurology, University of
Michigan Medical School; Physician-Scientist, Geriatric
Pe¯teris D¯arzin¸ˇs, BMBS, PhD, FRACP, FRCPC Research, Education, and Clinical Center, Ann Arbor
Associate Professor in Geriatric Medicine, Faculty of Veterans Affairs Medical Center, Ann Arbor, Michigan
Medicine, Nursing and Health Sciences, Monash Hereditary Spastic Paraplegias
University; Consultant Physician, Rehabilitation and
Aged Services Program, Southern Health; Monash April L. Fitzsimmons, MD
Ageing Research Centre, Kingston Centre—Monash Clinical Fellow, Department of Neurology, Harvard Medical
University, Melbourne, Victoria, Australia School; Fellow in Neuro-oncology, Brigham and
Delirium Women’s Hospital, Boston, Massachusetts
Tumors of the Spinal Cord
Bruce Day, MBBS, FRACP Deborah I. Friedman, MD
Honorary Lecturer, Department of Medicine, Monash Associate Professor of Ophthalmology and Neurology,
University; Director of Neurophysiology, Alfred Hospital, University of Rochester School of Medicine and
Melbourne, Victoria, Australia Dentistry; Attending Physician, Strong Memorial
The Persistent Vegetative State (Prolonged Postcoma Unresponsiveness) and Hospital, Rochester, New York
Posthypoxic Brain Injury Idiopathic Intracranial Headache
Ilan S. Freedman, MBBS
H. Gordon Deen, MD
Orthopaedic Registrar, Department of Orthopaedic
Associate Professor of Neurosurgery, Mayo Clinic College
Surgery, Western and Alfred Hospitals, Melbourne,
of Medicine, Rochester, Minnesota; Consultant in
Victoria, Australia
Neurosurgery, Mayo Clinic Jacksonville, Jacksonville,
Spinal Trauma
Florida
Head Trauma
Simon C. Gandevia, MD, PhD, DSc
Professor, University of New South Wales School of
Rajas Deshpande, MD, DM Medical Sciences, Sydney; Deputy Director, Prince of
Senior Consultant and Head, Neurology Department, Wales Medical Research Institute, Randwick, New South
Aditya Birla Memorial Hospital, Therzaon, India Wales, Australia
Clinical Spectrum: Definition and Natural Progression [Multiple Sclerosis Organization: Pyramidal and Extrapyramidal System [Motor System and
and Demyelinating Disorders] Motor Diseases]
x Contributors
Felix Geser, MD, PhD Richard Greenwood, MB, BCh, MD, FRCP
Visiting Scholar, Center of Neurodegenerative Disease Recognised Teacher, Institute of Neurology, University of
Research, Department of Pathology and Laboratory London; Consultant Neurologist, Acute Brain Injury
Medicine, University of Pennsylvania School of Service, National Hospital for Neurology and
Medicine/Hospital of the University of Pennsylvania, Neurosurgery, and Regional Neurological Rehabilitation
Philadelphia, Pennsylvania Unit, Homerton University Hospital NHS Trust, London,
Primary Autonomic Failure United Kingdom
Rehabilitation of Stroke
Nir Giladi, MD
Amparo Gutierrez, MD
Senior Lecturer, Sackler School of Medicine, Tel Aviv
Associate Professor of Clinical Neurology, Louisiana State
University; Director, Movement Disorders Unit, Tel Aviv
University Health Sciences Center School of Medicine,
Sourasky Medical Center, Tel Aviv, Israel
New Orleans, Louisiana
Gait Disturbances and Falls
Anatomy and Physiology of Muscle and Nerve
M. John Gill, BSc, MB, ChB, MSc Maxime Guye, MD, PhD
Professor, Departments of Medicine, Microbiology, and Neurophysiology and Neuropsychology Laboratory, Faculty
Infectious Disease, University of Calgary Faculty of of Medicine, Université de la Méditerranée; Neurologist,
Medicine, Calgary, Alberta, Canada Clinical Neurophysiology Service, CHU la Timone, and
Center for Biological and Medical Magnetic Resonance
Neurological Disorders Associated with Human Immunodeficiency Virus
(CNRS UMR 6612), CHU la Timone, Marseille, France
Infection
Developmental Defects and Pathophysiology [Epilepsy]
Peter J. Goadsby MD, PhD, DSc Glenda M. Halliday, PhD
Professor, Institute of Neurology and National Hospital for Professor of Medicine, University of New South Wales
Neurology and Neurosurgery, London, United Kingdom School of Medical Sciences; National Health and
TACs and SUNCT Medical Research Council Principal Research Fellow,
Prince of Wales Medical Research Institute, Sydney, New
South Wales, Australia
Graeme M. Gonzales, MD
Organization: Pyramidal and Extrapyramidal System
Department of Neurology,University of Melbourne,
Parkville, Victoria, Australia
Cynthia L. Harden, MD
Disorders ofTaste and Smell
Associate Professor of Neurology, Weill Medical College of
Cornell University, New York, New York
Neill R. Graff-Radford, MBBCh, FRCP(Lond) Management of Status Epilepticus
Professor of Neurology, Mayo College of Medicine; Mayo
Clinic Jacksonville, Jacksonville, Florida Jeffrey M. Hausdorff, BSE, MSME, PhD
Alzheimer’s Disease Senior Lecturer, Sackler School of Medicine, Tel Aviv
University, Tel Aviv, Israel; Lecturer in Medicine,
Harvard Medical School, Boston, Massachusetts;
Elizabeth Graham, FRCP, FRCOphth
Director, Laboratory of Gait and Neurodynamics, Tel
Consultant Medical Ophthalmologist, National Hospital Aviv Sourasky Medical Center, Tel Aviv, Israel
for Neurology and Neurosurgery, London, United
Gait Disturbances and Falls
Kingdom
Retinal Disease Robert C. Hermann, Jr., MD
Clinical Professor of Medicine and Neurology and Director,
Neurology EMG Laboratory, University of Texas Health
Robin Grant, MBChB, MD, FRCP(Glasg),
Science Center at San Antonio, San Antonio, Texas
FRCP(Edin)
Peripheral Nerve Injury
Honorary Senior Lecturer in Neurology, Division of
Clinical Neurosciences, University of Edinburgh Faculty
Rhonda O. Holmes, BSpPathol
of Medicine; Consultant Neurologist, Western General
Senior Clinician, Neurosciences, and Speech Pathologist,
Hospital, Edinburgh, United Kingdom
Austin Health, Heidelberg, Victoria, Australia
Neurological Complications ofTreatments
Motor Speech and Swallowing Disorders
John E. Greenlee, MD Alan C. Jackson, MD, FRCPC
Professor and Interim Chair, Department of Neurology, Professor of Medicine (Neurology) and Microbiology and
University of Utah School of Medicine; Neurologist, Immunology, Queen’s University Faculty of Medicine;
Veterans Administration Medical Center, Salt Lake City, Attending Staff, Department of Medicine (Neurology),
Utah Kingston General Hospital, Kingston, Ontario, Canada
Bacterial Meningitis (Including Lyme Disease, TB, and Syphilis) Viral Meningitis and Encephalitis
contributors xi
Malaka B. Jackson, MD John B. Kerrison, MD
Endocrinology Fellow, Division of Pediatric Endocrinology, Charleston Neuroscience Institute; St. Francis Hospital
University of Pennsylvania/Children’s Hospital of and Trident Hospital; Retina Consultants of Charleston,
Philadelphia, Philadelphia, Pennsylvania Charleston, South Carolina
Neurology of Endocrinology Genetic Causes of Blindness
Kim Jeffs, MBBS, FRACP Joshua C. Kershen, MD
Honorary Fellow, Faculty of Medicine, Dentistry and Assistant Professor, Department of Neurology, Tufts
Health Sciences, University of Melbourne, Melbourne; University School of Medicine; Neurologist, Tufts-New
Consultant Geriatrician, The Northern Hospital, Epping; England Medical Center, Boston, Massachusetts
Research Fellow, Northern Clinical Research Centre, Infective Neuropathies
The Northern Hospital, Melbourne, Victoria, Australia
Delirium Desmond Kidd, MD, FRCP
Honorary Senior Lecture, Royal Free and University
Richard T. Johnson, MD, FRCP College School of Medicine; Consultant Neurologist,
Distinguished Service Professor of Neurology, Royal Free Hospital, London, United Kingdom
Microbiology, and Neuroscience, Johns Hopkins Examination of the Visual System
University School of Medicine and Bloomberg School of
Public Health; Neurologist, Johns Hopkins Hospital, Glynda Kinsella, DiplPhysiol, MSc, PhD
Baltimore, Maryland Associate Professor of Psychological Science, La
Acute Disseminated Encephalomyelitis and Progressive Multifocal Trobe University; Clinical Neuropsychologist,
Leukoencephalopathy Caulfield General Medical Centre, Melbourne, Victoria,
Australia
Heinz Jungbluth, MD, PhD Principles of Neuropsychometric Assessment; Executive Function and Its
Assessment
Clinical Research Fellow, Dubowitz Neuromuscular
Centre, Imperial College, Hammersmith Hospital;
Consultant Paediatric Neurologist, Evelina Children’s Laurence J. Kinsella, MD, FAAN
Hospital, St. Thomas’ Hospital, London, United Professor of Neurology, Saint Louis University School of
Kingdom Medicine; Chief, Neurology and Neurophysiology, Forest
Congenital Myopathies Park Hospital, St. Louis, Missouri
Vitamin Deficiencies
Padmaja Kandula, MD
Assistant Attending Neurologist, Weill Medical College of Neil Kitchen, MD, FRCS(SN)
Cornell University, New York, New York Consultant Neurosurgeon and Associate Clinical Director,
Management of Status Epilepticus National Hospital for Neurology and Neurosurgery,
London, United Kingdom
Intracranial Hemorrhage: Aneurysmal, Idiopathic, and Hypertensive
Raju Kapoor, DM, FRCP
Consultant Neurologist, National Hospital for Neurology
Autumn M. Klein, MD, PhD
and Neurosurgery, London, United Kingdom
Instructor in Neurology, Harvard Medical School; Fellow
Pathophysiology of Multiple Sclerosis: Demyelination and Axonal Injury
in Clinical Neurophysiology, Division of Epilepsy and
Sleep, Department of Neurology, Brigham and Women’s
Horacio Kaufmann, MD
Hospital, Boston, Massachusetts
The Alex and Shirley Aidekman Professor of Neurology,
Neurology of Pregnancy and the Puerperium
Mount Sinai School of Medicine; Director, Autonomic
Disorders Research and Treatment Program, Mount
Andrew J. Kornberg, MD
Sinai Medical Center, New York, New York
Professor of Paediatrics, Faculty of Medicine, Dentistry and
Orthostatic Hypotension
Health Science, University of Melbourne; Director,
Department of Neurology, Royal Children’s Hospital;
Andrew H. Kaye, MBBS, MD, FRACS Attending, St. Vincent’s Hospital, Parkville, Victoria,
Professor and Head, Department of Surgery, University of Australia
Melbourne, Parkville; Director of Neurosurgery, Royal Spine and Spinal Cord: Developmental Disorders
Melbourne Hospital, Parkville, Victoria, Australia
Hydrocephalus Including Normal-Pressure Hydrocephalus
Thomas Kossmann, MD, FRACS
Professor and Director, Department of Trauma Surgery,
Noojan J. Kazemi, MBBS and Director, National Trauma Research Institute, The
Research Fellow, University of Melbourne and Royal Alfred Hospital/Monash University, Melbourne, Victoria,
Melbourne Hospital, Parkville, Victoria, Australia Australia
Hydrocephalus Including Normal-Pressure Hydrocephalus Spinal Trauma
xii Contributors
John W. Krakauer, MD Richard B. Lipton, MD
Assistant Professor of Neurology, Columbia University Professor of Neurology, and Epidemiology and Population
College of Physicians and Surgeons; Assistant Attending Health and Vice-Chair, Department of Neurology,
Neurologist, NewYork–Presbyterian Hospital, New York, Albert Einstein College of Medicine of Yeshiva
New York University; Director, Montefiore Headache Center,
Ischemic Stroke: Mechanisms, Evaluation, and Treatment Bronx, New York
Chronic Daily Headache
Marcelo Kremenchutzky, MD
Assistant Professor, Department of Clinical Neurological Jan D. Lünemann, MD
Sciences, University of Western Ontario; Neurologist,
The Rockfeller University Christopher H. Browne Center
London Health Sciences Centre, London, Ontario,
for Immunology and Immune Diseases, Laboratory of
Canada
Viral Immunobiology, New York, New York
Clinical Spectrum: Definition and Natural Progression [Multiple Sclerosis
Epidemiology and Genetics of Multiple Sclerosis
and Demyelinating Disorders]
Hanns Lochmüller, MD
Brian C. Kung, MD
University of Munich Hospital, Neurology Clinic and
Resident, Otolaryngology—Head and Neck Surgery,
Outpatient Service, Friedrich-Baur Institute, Munich,
Thomas Jefferson University Hospital, Philadelphia,
Germany
Pennsylvania
Muscular Dystrophies
Examination of Hearing and Balance
Richard P. Lango, MD James R. Lupski, MD, PhD
Instructor in Neurology, Department of Cullen Professor of Molecular and Human Genetics and
Neurology/Neuroimmunology, University of Rochester Professor of Pediatrics, Baylor College of Medicine;
School of Medicine and Dentistry; Fellow in Neurology, Attending Pediatrician and Consulting Medical
Strong Memorial Hospital, Rochester, New York Geneticist, Texas Children’s Hospital and Ben Taub
Treatment of Multiple Sclerosis General Hospital, Houston, Texas
Inherited Neuropathies
Pierre Lasjaunias, MD, PhD
Neuroradiologist, Bicetre Hospital, Kremlin Bicetre, Linda M. Luxon, BSc, MBBS, FRCP
France Professor of Audiological Medicine, University of London;
Arteriovenous Malformations of the Brain and Spinal Cord UCL Institute of Child Health, Great Ormond Street
Hospital for Children; Consultant Audiological Physician
Ramon C. Leiguarda, MD and Lead Clinician, Department of Neuro-Otology,
Professor of Neurology, University of Buenos Aires School National Hospital for Neurology and Neurosurgery,
of Medicine; Chairman, Department of Cognitive London, United Kingdom
Neurology, Institute of Neurological Research–Fleni, Vestibular System Disorders
Buenos Aires, Argentina
Apraxia John M. Lynch, MB, MRCPI
Clinical Research Fellow, National Hospital for Neurology
Peter J. Lennarson, MD and Neurosurgery, London, United Kingdom
Neurologic Surgeon, Department of Surgery, University of Genetics [Epilepsy]
Nebraska Medical Center, Omaha, Nebraska
Coma and Brain Death
Richard A. L. Macdonnell, MD, FRACP
Associate Professor, Faculty of Medicine, University of
Gian-Luigi Lenzi, MD, FRCP
Melbourne, Melbourne; Deputy Director of Neurology,
Professor, Department of Neurological Sciences, 1st
Austin Health, Heidelberg, Victoria, Australia
University of Roma La Sapienza, Rome, Italy
Motor Speech and Swallowing Disorders
Cerebral Venous Thrombosis
Eric T. MacKenzie, PhD
Francois Lette, MD
Director of Research, University of Caen; Staff, University
Senior Associate Consultant, Division of Executive and
Medical Center, Caen, France
International Medicine, Mayo Clinic, Jacksonville,
Florida Anatomy and Physiology of Cerebral and Spinal Cord Circulation
Parasitic and Fungal Infections
Boby Varkey Maramattom, MD, DM
Bernardo Liberato, MD Consultant, Division of Critical Care Neurology,
Director, Neurovascular Service, Hospital Copa D’Or, Rio Department of Neurology, Lourdes Hospital, Kochi,
de Janeiro, Brazil Kerala, India
Ischemic Stroke: Mechanisms, Evaluation, and Treatment Neurology of Pulmonology and Acid-Base Disturbance
contributors xiii
Gad A. Marshall, MD Francesco Muntoni, MD
Clinical Instructor in Neurology, David Geffen School of Professor of Pediatric Neurology, Department of Pediatrics,
Medicine at UCLA, Los Angeles, California Hammersmith Hospital Campus, Imperial College,
Affective Disorders London, United Kingdom
Congenital Myopathies
Roland Martin, MD
National Institutes of Health, National Institute of Kaye Murray, MBChB, BMedSci
Neurological Disorders and Stroke, Neuroimmunology Research Registrar, National CJD Surveillance Unit,
Branch, Bethesda, Maryland; Universita Autonoma de University of Edinburgh; Specialist Registrar in
Barcelona/Hospital Universitari Vall d’Hebron, Unitat de Neurology, Department of Clinical Neurosciences,
Neuroimmunologia Clínica, Barcelona, Spain Western General Hospital, Edinburgh, United
Epidemiology and Genetics of Multiple Sclerosis Kingdom
Prion Diseases
John H. Menkes, MD
Professor Emeritus of Neurology and Pediatrics, David
Sakkubai Naidu, MD
Geffen School of Medicine at UCLA; Director Emeritus
Professor of Pediatrics and Neurology, Johns Hopkins
of Pediatric Neurology, Cedars Sinai Medical Center, Los
University School of Medicine; Director, Neurogenetics
Angeles, California
Unit, Kennedy Krieger Institute, Baltimore, Maryland
Wilson Disease
Leukodystrophies
Alessio Mercurio, MD
Department of Neurological Sciences, 1st University of Peter J. Nestor, MBBS, PhD, FRACP
Roma La Sapienza, Rome, Italy Senior Research Associate, University of Cambridge;
Cerebral Venous Thrombosis Honorary Consultant Neurologist, Addenbrooke’s
Hospital, Cambridge, United Kingdom
Disorders of Memory
Geoffrey Miller, MA, MB, ChB, MPhil, MD, FRCP,
FRACP
Professor of Pediatrics and Neurology, Department of Nancy J. Newman, MD
Pediatrics, Yale University School of Medicine; Clinical Leo Delle Jolley Professor of Ophthalmology, Professor of
Director, Pediatric Neurology Service, Yale–New Haven Ophthalmology and Neurology, and Instructor in
Children’s Hospital, New Haven, Connecticut Neurological Surgery, Emory University School of
Neurology of Cerebral Palsy Medicine, Atlanta; Lecturer in Ophthalmology, Harvard
Medical School, Boston; Director of Neuro-
Ophthalmology, Emory Eye Center, Atlanta, Georgia
Neil R. Miller, MD
Genetic Causes of Blindness
Professor of Ophthalmology, Neurology, and Neurosurgery
and Frank B. Walsh Professor of Neuro-Ophthalmology,
Johns Hopkins University School of Medicine; Full-Time Fiona L. M. Norwood, MD
Faculty Member, Johns Hopkins Hospital, Baltimore, Consultant Neurologist, King’s Neuroscience Centre,
Maryland King’s College Hospital, London, United Kingdom
Optic Neuropathies Channelopathies of Muscle (Including Myotonic Dystrophy)
Bahram Mokri, MD
Wolfgang H. Oertel, MD
Professor of Neurology, Mayo Clinic College of Medicine,
Professor of Neurology, Philipps University School of
Rochester, Minnesota
Medicine, Marburg, Germany
Low CSF Headache
Restless Legs Syndrome
Maria Cristina Morganti-Kossmann, PhD
Michael D. Osborne, MD
Assistant Professor and Associate Director, Basic Research,
National Trauma Research Institute, The Alfred Assistant Professor in Physical Medicine and Rehabilitation
Hospital/Monash University, Melbourne, Victoria, and Assistant Professor in Anesthesiology, Mayo Clinic
Australia College of Medicine, Rochester, Minnesota; Consultant,
Mayo Clinic, Jacksonville, Florida
Spinal Trauma
Neurorehabilitation
Hugo W. Moser, MD
Professor of Neurology and Pediatrics, Johns Hopkins Michael L. Oshinsky, PhD
University School of Medicine; Director, Neurogenetics Assistant Professor of Neurology, Jefferson Medical College
Research Center, Kennedy Krieger Institute, Baltimore, of Thomas Jefferson University, Philadelphia,
Maryland Pennsylvania
Leukodystrophies Headache Pathogenesis