Multiple Sclerosis 2

376 Pages·2003·4.435 MB·iii-xv, 1-377\376

by  W. Ian McDonald and John H. Noseworthy (Eds.)

Multiple Sclerosis 2
Edited by
W. Ian McDonald, Ph.D., F.R.A.C.P., F.R.C.P.
Professor Emeritus of Neurology, Institute of Neurology,
University College, London
and
John H. Noseworthy, M.D., F.R.C.P.C.
Department of Neurology, Mayo Clinic/Mayo Foundation, Rochester,
Minnesota
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Library of Congress Cataloging-in-Publication Data
Multiple sclerosis 2 / edited by W. Ian McDonald and John H. Noseworthy.
p. ; cm. — (Blue books of practical neurology ; 27)
Includes bibliographical references and index.
ISBN0-7506-7348-6
1. Multiple sclerosis. I. Title: Multiple sclerosis two. II. McDonald, W. Ian.
III. Noseworthy, John H. IV. Series.
[DNLM: 1. Multiple Sclerosis. WL360 M96255 2003]
RC377 .M8393 2003
616.8¢34—dc21 2002026294
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To those with multiple sclerosis who have taught us about it and inspire us to
seek lasting answers to the challenges they face each day.
Contributing Authors
Douglas L. Arnold, M.D., F.R.C.P.(C)
Professor of Neurology and Neurosurgery, McGill University; Director,
Clinical Research Unit and Magnetic Resonance Spectroscopy Unit, Montreal
Neurological Institute, Montreal, Quebec, Canada
Alexei N. Boiko, M.D.
Professor,Department of Neurology, Moscow University, Moscow, Russia
W. Brück, M.D.
Department of Neuropathology, Charité, Humboldt University, Berlin, Germany
Zografos Caramanos, M.A.
Research Assistant, Department of Neurology and Neurosurgery, McGill Uni-
versity; Research Assistant, Magnetic Resonance Spectroscopy Unit, Montreal
Neurological Institute, Montreal, Quebec, Canada
Alastair Compston, M.D.
Department of Neurology, University of Cambridge, Addenbrooke’s Hospital,
Cambridge, United Kingdom
Patricia K. Coyle, M.D.
Professor of Neurology; Director, SBMS Comprehensive Care Center, School
of Medicine, State University of New York Stony Brook; Attending Neurolo-
gist, Department of Neurology, University Hospital Medical Center, Stony
Brook
George C. Ebers, M.D.
Action Professor of Clinical Neurology, University of Oxford; Head of Depart-
ment, Department of Clinical Neurology, Radcliffe Infirmary, Oxford, United
Kingdom
ix
x Contributing Authors
Franz Fazekas, M.D.
Department of Neurology and MR Institute, Karl-Franzens University, Graz,
Austria
Jacqueline Foong, M.R.C.Psych, M.Phil.
Honorary Senior Lecturer, Department of Clinical Neurology, Institute of
Neurology; Consultant, Department of Neuropsychiatry, The National Hospital
for Neurology and Neurosurgery, London, United Kingdom
Jennifer A. Freeman, B. App. Sci., Ph.D., M.C.S.P.
Honorary Lecturer, Institute of Neurology, London; Senior Lecturer, Institute
of Health Studies, Plymouth University, Devon, United Kingdom
Anke Hensiek, M.D.
Department of Neurology, University of Cambridge, Addenbrooke’s Hospital,
Cambridge, United Kingdom
Reinhard Hohlfeld, M.D.
Chairman and Director, Department of Neurology, Institute for Clinical
Neuroimmunology, University of Munich, Germany
Jürg Kesselring, M.D.
Professor of Clinical Neurology and Neurorehabilitation, Universities of Bern
and Zürich; Head of the Department of Neurology, Rehabilitation Center, Bern,
Switzerland
Hans Lassmann, M.D.
Division of Neuroimmunology, Brain Research Institute, University of Vienna,
Austria
Fred D. Lublin, M.D.
Corinne Goldsmith Dickinson Center for Multiple Sclerosis, Mount Sinai
Medical Center, New York
Claudia F. Lucchinetti, M.D.
Assistant Professor of Neurology, Mayo Clinic and Foundation, Rochester
Roland Martin, M.D.
Chief, Cellular Immunology Section, Neuroimmunology Branch, NINDS, NIH,
Bethesda
W. Ian McDonald, Ph.D., F.R.A.C.P., F.R.C.P.
Professor Emeritus of Neurology, Institute of Neurology, University College,
London, United Kingdom
Luanne Metz, M.D.
Associate Professor, Department of Clinical Neurosciences, University of
Calgary; MS Clinic Director, Foothills Hospital, Calgary, Canada
Contributing Authors xi
David H. Miller, M.D.
Institute of Neurology and The National Hospital for Neurology and Neuro-
surgery, London, United Kingdom
John H. Noseworthy, M.D., F.R.C.P.C.
Department of Neurology, Mayo Clinic/Mayo Foundation, Rochester
Donald W. Paty, M.D.
Professor, Division of Neurology, University of British Columbia, Vancouver,
Canada
Stephen C. Reingold, Ph.D.
Research Programs Department, National Multiple Sclerosis Society, New
York
Moses Rodriguez, M.D.
Department of Neurology, Mayo Clinic, Rochester
Maria A. Ron, Ph.D., M.R.C.P., F.R.C.Psych.
Professor of Clinical Neurology, Institute of Neurology; Consultant, Depart-
ment of Neuropsychiatry, The National Hospital for Neurology and Neuro-
surgery, London, United Kingdom
Richard Roxburgh, M.D.
Department of Neurology, University of Cambridge, Addenbrooke’s Hospital,
Cambridge, United Kingdom
Peter Rudge, M.D.
Honorary Senior Lecturer, Institute of Neurology; Consultant Neurologist,
National Hospital for Neurology and Neurosurgery, London, United Kingdom
A. Carlos Santos, M.D.
Assistant Professor, Medical Clinic—Image Center, University of Sao Paulo—
Ribeirao Preto Medical School; Neuroradiologist, Department of Radiology,
Ribeirao Preto Clinical Hospital, Sao Paulo, Brazil
Kenneth J. Smith, M.D.
Neuroinflammation Research Group, Guy’s, King’s, and St. Thomas’School of
Medicine, King’s College, London, United Kingdom
Mats Söderström, M.D.
Associate Professor, Department of Ophthalmology, Karolinska Institute;
Senior Consultant, Department of Ophthalmology, Huddinge University Hos-
pital, Huddinge, Sweden
Alan J. Thompson, M.D., F.R.C.P., F.R.C.P.I.
Garfield Weston Professor of Clinical Neurology and Neurorehabilitation, Insti-
tute of Neurology, University College, London, United Kingdom
xii Contributing Authors
G.K. Vorobeychi, M.D.
Resident, Division of Neurology, University of British Columbia, Vancouver,
Canada
Brian G. Weinshenker, M.D., F.R.C.P.C.
Department of Neurology, Mayo Clinic, Rochester
Dean M. Wingerchuk, M.D., F.R.C.P.C.
Department of Neurology, Mayo Clinic, Scottsdale
Series Preface
The Blue Books of Practical Neurology denotes the series of monographs pre-
viously named the BIMR Neurology series, which was itself the successor of
the Modern Trends in Neurologyseries. As before, the volumes are intended for
use by physicians who grapple with the problems of neurological disorders on
a daily basis, be they neurologists, neurologists in training, or those in related
fields such as neurosurgery, internal medicine, psychiatry, and rehabilitation
medicine.
Our purpose is to produce monographs on topics in clinical neurology in
which progress through research has brought about new concepts of patient
management. The subject of each book is selected by the Series Editors using
two criteria: first, that there has been significant advance in knowledge in that
area and, second, that such advances have been incorporated into new ways of
managing patients with the disorders in question. This has been the guiding
spirit behind each volume, and we expect it to continue. In effect, we empha-
size research, both in the clinic and in the experimental laboratory, but princi-
pally to the extent that it changes our collective attitudes and practices in caring
for those who are neurologically afflicted.
Arthur K. Asbury
Anthony H.V. Schapira
Series Editors
xiii
Preface
The past decade has been arguably the most exciting ever in terms of progress
in our understanding of multiple sclerosis and how to treat it. To give a com-
prehensive account of the disease and its management now requires a large clin-
ical reference. This is not the aim of the present book. Rather, it is to concentrate
on selected topics in which our understanding has advanced at the basic or the
clinical levels. In particular we have aimed to provide a practical approach to
diagnosis and management of the different forms of the disease encountered by
the practicing neurologist.
A notable change in the past decade has been the placement of the person
with multiple sclerosis at the center of discussions about the disease and its
management and the recognition that decisions about priorities in research
should derive from a partnership between investigators and people with the
disease. This change in orientation is reflected in the contents of the book. In
addition to chapters on drug treatment, there are accounts of the role of reha-
bilitation and of the cognitive, psychological, and social impacts of the disease,
an appreciation of which is essential to good management.
In structuring the book we have kept in mind the needs of neurologists in
training as well as those in practice. We hope that both groups will find this
new volume on Multiple Sclerosis of value.
W.I. McDonald
J. Noseworthy
xv
1
The Diagnosis of Multiple Sclerosis
W. Ian McDonald, Franz Fazekas, and Alan J. Thompson
The diagnosis of the established case of multiple sclerosis (MS) is easy: It
simply requires the demonstration of abnormal physical signs indicating the
presence of lesions at two necessarily separate sites in the central nervous
system (CNS), in an individual with a history of at least two episodes of neu-
rological disturbance of the kind seen in MS, and that there is no better expla-
nation for the clinical picture. These essential classical criteria for the diagnosis
of MS1 can be fulfilled by clinical assessment alone. Most neurologists,
however, like to have the reassurance of confirmatory investigations, usually an
abnormal magnetic resonance imaging (MRI) study, though abnormal evoked
potentials (EPs) and cerebrospinal fluid (CSF) analysis are also helpful.
Diagnostic problems arise in two main contexts. The first, particularly impor-
tant when the question of disease-modifying therapy is being considered, occurs
when the patient is seen early in the course of the disease, at a stage when
the clinical evidence for dissemination in time and space is inconclusive. The
second occurs when there are unusual features present, such as progression from
onset,2 aphasia, or epilepsy.
To overcome these problems, a number of sets of diagnostic criteria have
been proposed over the years. The principles are the same in all. Since 1983,
the results of EP and CSF examination have been formally included in what
became known as the Poser criteria.3
These criteria, which have served well, were developed just as MRI became
available. Though its diagnostic usefulness seemed obvious, there was insuffi-
cient experience with it to incorporate the results of its use. Experience with
MRI in MS since then has been enormous, and for this reason a meeting was
convened in London in July 2000 under the auspices of the National MS Society
of the United States and the Multiple Sclerosis International Federation. The
charge to the International Panel was to review existing criteria and to refine
them in the light of present knowledge. It was agreed that the criteria should
be evidence based. Two consequences follow. First, because of present limita-
tions in our knowledge, particularly about temporal factors in the evolution
of lesions, the criteria for establishing dissemination in time appear rather
1