Table Of ContentCONTRIBUTORS
Numbers in parentheses indicate the pages on which the authors' contributions begin.
HERMANN ACKERMANN (245, 249), Department ROLFE BIRCH (1325), The Royal National
of Neurology, University of Tubingen, 71076 Orthopaedic Hospital Trust, Peripheral Nerve Injury
Tijbingen, Germany and Children's Hand Unit, Stanmore, Middlesex HA7
4LP, United Kingdom
GREGORY W. ALBERS (349), Stanford Stroke Center,
Palo Alto, California 94304 T. R BLECK (529), University of Virginia, Health Sci
ences Center, School of Medicine, Charlottesville,
PRAVEEN ANAND (1325), ICSM at Hammersmith
Virginia 22908
Hospital Campus, London W12 OHS, United
Kingdom GIAN DOMENICO BORASIO (813, 1165), Interdis
ciplinary Palliative Care Unit and Department of
D. E. ANDRICH (1479), Department of Neurology, Neurology, Ludwig-Maximilians University, 81377
The National Hospital for Neurology and Neuro Munich, Germany
surgery, London WCIN 3BG, United Kingdom
KAI BOTZEL (1099), Department of Neurology,
STANLEY H. APPEL (1165), Department of Neurology, Ludwig-Maximilians University, 81377 Munich,
Baylor College of Medicine, Houston, Texas 77030 Germany
ALLEN L ARIEFF (991), Department of Medicine, MARIE-GERMAINE BOUSSER (447), Service de Neu
University of California School of Medicine at San rologic, Hopital Lariboisiere, Paris Cedex 10, 75475,
Francisco, San Francisco, California 94143 France
TIPU AZIZ (1233), Division of Neuroscience and Psy THOMAS BRANDT (17, 67, 139), Department of
chological Medicine, Imperial College School of Med Neurology, Ludwig-Maximilians University, 81377
icine, London W6 8RF, United Kingdom Munich, Germany
M. BAHR (947), Department of Neurology, STEVEN M. BROMLEY (171), Neurological Institute,
University Hospital Gottingen, 37075 Gottingen, Department of Neurology, Columbia Presbyterian
Germany Medical Center, New York, New York 10040
PETER BAIN (1233), Division of Neuroscience and JOHN C. M. BRUST (791), Department of Neurology,
Psychological Medicine, Imperial College School of Harlem Hospital Center, New York, New York
Medicine, London W6 8RF, United Kingdom 10037
ULRICH BUTTNER (165), Department of Neurology,
HENRY J. M. BARNETT (939), Department of Clini
cal Neurology, The University of Western Ontario, Ludwig-Maximihans University, 81377 Munich,
London, Ontario, Canada N6A 4B8 Germany
LOUIS R. CAPLAN (303, 327, 373, 393, 403, 517,
U. BAUMANN (1199), ENT Department, Ludv^ig-
939), Department of Cerebrovascular Disease, Beth
Maximilians University, 81377 Munich, Germany
Israel Deaconess Medical Center and Department
MARTIN BEGEMANN (865), Memorial Hospital, of Neurology, Harvard Medical School, Boston,
New York, New York 10021 Massachusetts 02215
VALERIE BIOUSSE (115), Department of Ophthalmol J. S. CHOPRA (647), Department of Neurology, Post
ogy and Neurology, Emory University School of Med graduate Institute of Medical Education and
icine, Atlanta, Georgia 30322 Research, Chandigarh 160 012, India
XI
xu CONTRIBUTORS
DAVID GLENN CLARK (265), Department of Neu D. B. DRACHMAN (1341), Neurology-Neuromuscu-
rology, UCLA School of Medicine, Los Angeles, lar Unit, Johns Hopkins University School of Medi
California 90095 cine, Baltimore, Maryland 21205
N. COLMAN (1495), Department of Internal Medicine, JAMES ECKLUND [IGS), Neurosurgery Service,
Academic Medical Centre, 1105 AZ Amsterdam, National Capital Consortium, (Walter Reed-
The Netherlands Bethesda), Walter Reed Army Medical Center,
Washington, DC 20307
PATRICK COOPER (765), Neurosurgery Service,
National Capital Consortium (Walter Reed- KARL M. EINHAUPL (447), Department of Neurol
Bethesda), Washington, DC 20307 ogy, Charite Hospital, 10117 Berlin, Germany
JAMES J. CORBETT (475, 921), Department of Neu ILONKA EISENSEHR (191), Department of Neurol
rology, University of Mississipi Hospitals, Jackson, ogy, Ludwig-Maximilians University, 81377 Munich,
Mississippi 39216 Germany
STANLEY FAHN (1021), Neurological Institute,
JEFFREY L. CUMMINGS (265, 311), Reed Neurolog
Columbia University, New York, New York 10032
ical Research Center, UCLA School of Medicine, Los
Angeles, Cahfornia 90095 CHARLOTTE FEINMANN (59), Joint Department of
Maxillofacial Surgery, Eastman Dental Hospital,
MARINOS C. DALAKAS (1363), National Institute of
London WCIX 8LD, United Kingdom
Neurological Disorders and Stroke, National Insti
tutes of Health, Bethesda, Maryland 20892 MICHAEL FETTER (185), Department of Neurology,
Reha-Klinik, 76307 Karlsbad-Langensteinbach,
DAVID M. DAWSON (1303), VA Hospital, Boston,
Germany
Massachusetts 02115
MARSHAL FOLSTEIN (303), Department of Psychia
LISA M. DEANGELIS (865), Memorial Hospital, Nev^
try, New England Medical Center, Boston, Massa
York, New York 10021
chusetts 02111
JOHANNES DICHGANS (1011, 1123), Department of
C. J. FOWLER (1479), Department of Uro-Neurology,
Neurology, University of Tubingen, 72076 Tubingen,
The National Hospital, London WCIN 3BG, United
Germany
Kingdom
MARTIN DICHGANS (363), Department of Neurol
R. FREEMAN (1453, 1495), Harvard Medical School
ogy, Ludwig-Maximilians University, 81377 Munich,
and Center for Autonomic and Peripheral Nervous
Germany
System Disorders, Beth Israel Deaconess Medical
H. CHRISTOPH DIENER (1, 23, 31, 95, 349, 1507), Center, Boston, Massachusetts 02215
Department of Neurology, University of Essen, 45122
GERAINT FULLER (615), Department of Neurology,
Essen, Germany
Gloucestershire Royal Hospital, Gloucester GLl
MARIANNE DIETERICH (59, 11), Department of 3NN, United Kingdom
Neurology, Johannes Gutenberg University, 55131
THOMAS GASSER (1149, 1525), Department of Neu
Mainz, Germany
rology, University of Tubingen, 72076 Tubingen,
Germany
VOLKER DIETZ (925, 1247), ParaCare, Paraplegic
Center, University Hospital Balgrist, Zurich CH- PETER J. GOADSBY (1, 17), Institute of Neurology,
8008, Sv^itzerland University College London, The National Hospital
for Neurology and Neurosurgery, London WCIN
SALVATORE DIMAURO (1385), Department of Neu
3BG, United Kingdom
rology, Columbia University College of Physicians &
Surgeons, New York, New York 10032 JOAO A. GOMES (403), Department of Neurology,
Boston University School of Medicine, Boston, Mass
MICHAEL DONAGHY (1281), Department of Neu
achusetts 02215
rology, Radcliffe Infirmary, Oxford OX2 6HE, United
Kingdom GEOFFREY K. GOURLAY [^S), Pain Management
Unit, Flinders Medical Center, Bedford Park, South
RICHARD L. DOTY (171), Smell & Taste Center and
Australia 5042, Australia
Department of Otorhinolaryngology: Head & Neck
Surgery, School of Medicine, University of Pennsyl ROBERT C. GRIGGS (1369), Department of Neurol
vania, Philadelphia, Pennsylvania 19104 ogy, University of Rochester School of Medicine,
CONTRIBUTORS Xlll
Rochester, and Strong Memorial Hospital, Rochester, RIGMOR JENSEN (23), Department of Neurology,
New York 14642 Glostrup Hospital, Glostrup DK-2600, Denmark
CHRISTIAN GUILLEMINAULT (191), Sleep Disor KARIN JURKAT-ROTT (1413), Department of
ders Clinic, Stanford University Medical Center, Palo Applied Physiology, University of Ulm, 89069 Ulm,
Alto, California 94304 Germany
ROBERTO GUILOFF (623), Department of Neurology, HANS-OTTO KARNATH (277), Department of Cog
Charing Cross Hospital, London W6 8RF, United nitive Neurology, University of Tubingen, 72076
Kingdom Tubingen, Germany
CARLOS S. KASE (403), Department of Neurology,
AMPARO GUTIERREZ (135), Louisiana State Univer
Boston University School of Medicine, Boston, Mass
sity Health Science Center, New^ Orleans, Louisiana
achusetts 02118
70112
STEFAN KASTENBAUER (545), Department of Neu
WERNER HACKE (327, 373, 721), Department of
rology, Ludw^ig-Maximilians University, 81377
Neurology, University of Heidelberg, 69120
Munich, Germany
Heidelberg, Germany
OLIVER KASTRUP (1435, 1507), Department of Neu
RODOLFO HAKIM (805), Clinical Pediatric Neuro
rology, University of Essen, 45122 Essen, Germany
surgery, Children's Hospital Boston, Boston, Massa
chusetts 02115 MARK T. KEEGAN (749), Anesthesiology/Critical
Care, Mayo Clinic, Rochester, Minnesota 55905
MARK HALLETT (1221), Human Motor Control
MATTHIAS KEIDEL (83), Departments of Neurol
Section, National Institute of Neurological Disorders
ogy and Neurorehabilitation, District Hospital of
and Stroke, National Institutes of Health, Bethesda,
Bayreuth, 35445 Bayreuth, Germany
Maryland 20892
CHRISTOPHER KENNARD (185, 255), Unit of Neu
D. E HANLEY (721), Johns Hopkins, Division of Neu-
roscience, Charing Cross Hospital, London W6 8RF,
rosciences Critical Care, Baltimore, Maryland 21287
United Kingdom
KLAUS HANSEN (583), Department of Neurology,
KARL KIEBURTZ (1149), Department of Neurology,
The Neuroscience Center, Copenhagen University
University of Rochester, Nev^ York 14642
Hospital Rigshospitalet, Copenhagen 2100, Denmark
THOMAS KLOCKGETHER (1011), Department of
M. HARPER (1479) Department of Uro-Neurology,
Neurology, University of Bonn, 53105 Bonn, Germany
The National Hospital for Neurology and Neuro
THOMAS KLOPSTOCK (1385), Department of Neu
surgery, London WCIN 3BG, United Kingdom
rology, Ludv^ig-Maximilians University, 81377
S. HESSE (1185), Klinik Berlin, Department of Munich, Germany
Neurological Rehabilitation, Free University, 14089
RICHARD KNIGHT (707), National CJD Surveillance
Berhn, Germany
Unit, Western General Hospital, Edinburgh EH4
R. T. HIGASHIDA (481), University of California San 2XU, United Kingdom
Francisco Medical Center, Neuro Interventional Radi
R. KOLLMAR (721), Department of Neurology, Uni
ology, San Francisco, California 94143
versity of Heidelberg, 69120 Heidelberg, Germany
REINHARD HOHLFELD (677, 1341), Institute for
PAUL KRACK (1099), Service de Neurologie, Centre
Clinical Neuroimmunology, Ludw^ig-Maximilians
Hospitalier Universitaire de Grenoble, 38043
University, 81377 Munich, Germany
Grenoble, France
T A. T. HUGHES (245, 249), University Hospital of JOACHIM K. KRAUSS (1113), Department of Neuro
Wales, Heath Park, Cardiff CF14 4XN, United surgery, University Hospital, Klinikum Mannheim,
Kingdom 68167 Mannheim, Germany
R. J. JAEGER (1199), Research Service, Edv^ard Hines, FRANK LEHMANN-HORN (783, 1413), Institute of
Jr. Hospital; Department of Veterans Affairs, Hines, Applied Physiology, University of Ulm, 89069 Ulm,
Illinois 60141 Germany
J. JANKOVIC (1123), Parkinson's Disease Center and R. JOHN LEIGH (125), Department of Neurology,
Movement Disorder Clinic, Baylor College of Medi Case Western Reserve University and Veterans Affairs
cine, Houston, Texas 77030 Medical Center, Cleveland, Ohio 44106
XIV CONTRIBUTORS
SAMDEN D. LHATOO (207), Department of Neurol PATRICIA M. MOORE (461), Department of Neu
ogy, Institute of Clinical Neurosciences and Univer rology, University of Pittsburgh, Pittsburgh,
sity of Bristol, Frenchay Hospital, Bristol BS16 ILE, Pennyslvania 15260
United Kingdom
PRITHIVA NAVAN (1233), Division of Neuroscience
VOLKER LIMMROTH (37), Department of Neurol and Psychological Medicine, Imperial College,
ogy, University of Essen, 45122 Essen, Germany London W6 8RP, United Kingdom
ALAN H. LOCKWOOD (165), Center for PET (II5P), NANCY J. NEWMAN (115), Neuro-Ophthalmology
VA Western NY Healthcare System, Buffalo, New Unit, Emory University School of Medicine, Atlanta,
York 14215 Georgia 30322
E. LOGICIAN (1421), Department of Neurology, SOHEYL NOACHTAR (235), Department of Neurol
Neuromuscular Disease Unit, University of Rochester ogy, University of Munich, 81377 Munich, Germany
School of Medicine and Dentistry, Rochester, New
WOLFGANG H. OERTEL (1021), Department of
York 14642
Neurology, Philipps-University Marburg, 35033
HANS OTTO LUDERS (235), Department of Marburg, Germany
Neurology, Cleveland Clinic Foundation, Cleveland,
JES OLESEN (23), Department of Neurology, Glostrup
Ohio 44195
Hospital, Glostrup 2600, Denmark
JOSEPH R. MADSEN (805), Clinical Pediatric Neuro
RICHARD W. ORRELL (1369), University Department
surgery, Children's Hospital Boston, Boston, Massa
of Clinical Neurosciences, Royal Free and University
chusetts 02115
College Medical School, University College London
R. MALESSA (601), Department of Neurology, Sophen and Royal Free Hospital, London NW3 2PF United
and Hufeland Hospital Weimar, Teaching Hospital of Kingdom
the Friedrich Schiller University Jena, 99245 Weimar,
G. D. PERKIN (67, 77), Department of Neurology,
Germany
Charing Cross Hospital, London W6 8RF, United
HADI MANJI (623), Department of Neurology, Kingdom
National Hospital for Neurology and Neurosurgery,
HANS-WALTER PFISTER (529, 545, 559, 583),
London; and The Ipswich Hospital, Suffolk IP4 5PD,
Department of Neurology, Ludwig-Maximilians Uni
United Kingdom
versity, 81377 Munich, Germany
HANS J. MARKOWITSCH (287), Physiological Psy
chology, University of Bielefeld, 33615 Bielefeld, A. BERNARD PLEET*(1435), Division of Neurology,
Germany Baystate Medical Center, Springfield, Massachusetts
01199
ROLAND MARTIN (677), Cellular Immunology
Section; Neuroimmunology Branch, NINDS, National WERNER POEWE (1081), Department of Neurology,
Institutes of Health, Bethesda, Maryland 20892 University Hospital of Innsbruck, A-6020 Innsbruck,
Austria
HELENE MASSIOU (47), Department of Neurology,
Hopital Lariboisiere, Paris, France DIETER PONGRATZ (1363), Friedrich-Baur Foun
dation and Department of Neurology, Ludwig-
NINAN MATHEW (31), Houston Headache Clinic,
Maximilians University, 80366 Munich, Germany
Houston, Texas 77004
RUSSELL PORTENOY (813), Department of Pain
C. J. MATHIAS (1453), Pickering Unit, St. Mary's Hos
Medicine and Palliative Care, Beth Israel Medical
pital, London W2 INY, United Kingdom
Center, New York, New York 10003
H. R MCFARLAND (677), Neuroimmunology Branch,
JEROME B. POSNER (911), Cornell University
National Institute of Neurological Disorders and
Medical School, Memorial Sloan-Kettering Cancer
Stroke, National Institutes of Health, Bethesda,
Center, New York, New York 10021
Maryland 20892
H. PRANCE (575), Department of Neurology,
HANS-MICHAEL MEINCK (1429), Department of
University of Gottingen, 37075 Gottingen, Germany
Neurology, University of Heidelberg, 69120 Heidel
berg, Germany J. QUINTERN (1199), Neurological Hospital Bad
Aibling, 83043 Bad Aibling, Germany
A. MELMS (475, 1341), Department of Neurology,
University of Tubingen, 72076 Tubingen, Germany ^Deceased.
CONTRIBUTORS XV
KENNETH RICKER (1407), Department of Neurology, ANDREAS STRAUBE (125, 1157), Department of
University of Wiirzburg, 97070 Wlirzburg, Germany Neurology, Ludwig-Maximilians University, 81377
Munich, Germany
KAREN L. ROOS (559), Department of Neurology,
Indiana University School of Medicine, Indianapolis, MICHAEL STRUPP (447, 1113), Department of Neu
Indiana 46202 rology, Ludwig-Maximilians University, 81377
Munich, Germany
MARTIN ROSENER (595, 615), Department of Neu
rology, University of Tubingen, 72076 Tubingen, AUSTIN J. SUMNER (135), Neurology Department,
Germany LSU Medical University, New Orleans, Louisiana
70112
ANDRES M. SALAZAR^=- (765), Defense and Veterans
Head Injury Program, Washington, DC 20008 IAN SUTTON (1259), Department of Neurology,
Queen Elizabeth Hospital, Birmingham B15 2TH,
JOSEMIR W. A. S. SANDER (207), Epilepsy Research
United Kingdom
Group, University College London, Institute of Neu
rology, London WCIN 3BG, United Kingdom MARGARET M. SWANBERG (311), Department of
Neurology, UCLA School of Medicine, Los Angeles,
I. M. S. SAWHNEY (647), Morriston Hospital,
California 90095
Sv^ansea SA6 6NL, United Kingdom
PHILLIP D. SWANSON (1157), Department of
MARTIN SCHABET (865), Department of Neurology,
Neurology, University of Washington, Seattle,
Klinikum Ludwigsburg, 71640 Ludv^igsburg,
Washington 98195
Germany
MICHAEL SWASH (1289, 1303), Department of Neu
W. MICHAEL SCHELD (545), Division of Infectious
rology, Royal London Hospital, London El IBB,
Diseases, University of Virginia Health Science
United Kingdom
Center, Charlottesville, Virginia 22908
PETER THIER (971), Department of Neurology, Uni
B. L. SCHLAGGAR+ (947), St. Louis Children's Hospi
versity of Tubingen, 72076 Tubingen, Germany
tal, St. Louis, Missouri 63110
DAVID G. T. THOMAS (827), Department of Neuro
C. SCHNEIDER (1341), Department of Neurology, surgery, Insititute of Neurosurgery, University College
Heinrich-Heine University, 40225 Diisseldorf, London, London WCIN 3BG, United Kingdom
Germany
A. J. THOMPSON (1185), Department of Neuro-
JORG B. SCHULZ (991), Department of Neurology, Rehabilitation, Institute of Neurology, National
University of Tubingen, 72076 Tiibingen, Germany Hospital for Neurology and Neurosurgery, London
WCIN 3BG, United Kingdom
S. SCHWAB (721), Department of Neurology, Univer
sity of Heidelberg, 69120 Heidelberg, Germany PHILIP D. THOMPSON (1429), The University De
partment of Medicine, The Royal Adelaide Hospital,
R. MICHAEL SCOTT (805), Clinical Pediatric Neuro
Adelaide, South Australia 5000, Australia
surgery, Children's Hospital Boston, Boston, Massa
chusetts 02115 ARMIN K. THRON (517), Department of Neuro
radiology, University Hospital RWTH Aachen, 52074
RAAD A. SHAKIR (595), Charing Cross Hospital,
Aachen, Germany
London, London W6 8 RE, United Kingdom
HELGE TOPKA (1123, 1221, 1421), Department of
STEPHEN D. SILBERSTEIN (37, 47), Jefferson
Neurology and Clinical Neurophysiology, Academic
Headache Center, Philadelphia, Pennsylvania 19107
Hospital Munich Bogenhausen, 81925 Munich,
BARNEY J. STERN (567), Department of Neurology, Germany
The Emory Clinic, Atlanta, Georgia 30322
K. V. TOYKA (1341), Department of Neurology, Uni
MANFRED STOHR (1303,1337), Department of Neu versity of Wiirzburg, 97080 Wiirzburg, Germany
rology and Clinical Neurophysiology, Klinikum
CLAUDIA TRENKWALDER (1177), Department of
Augsburg, 86156 Augsberg, Germany
Clinical Neurophysiology, University of Gottingen,
37075 Gottingen, Germany
'^Current address: Ribopharm, Inc., Washington, DC 20008
KENNETH L. TYLER (601), Department of Neurol
^Current address: Departments of Neurology, Radiology, and
ogy, University of Colorado Health Science Center,
Pediatrics, Washington University School of Medicine, St. Louis,
Missouri Denver, Colorado 80262
XVI CONTRIBUTORS
J. VAN GIJN (421), Department of Neurology, Uni BOB WILL (707), National CJD Surveillance Unit,
versity of Utrecht, NL-3584 CX Utrecht, The Western General Hospital, Edinburgh EH4 2XU,
Netherlands United Kingdom
ARNO VILLRINGER (461), Department of Neurology, JOHN B. WINER (1259), Department of Neurology,
Charite Hospital, Berlin, Germany Queen Elizabeth Hospital, Birmingham B15 2TH,
United Kingdom
RAYMOND VOLTZ (813), Institute of Clinical Neuro-
immunology and Department of Neurology, Ludwig- PETER A. WINKLER (235), Department of Neuro
Maximilians University, 81366 Munich, Germany surgery, Ludwig-Maximilians University, 81377
Munich, Germany
FRIEDRICH VON ROSEN (791), Neurological Hospi
tal, Bad Aibling, 83043 Bad Aibling, Germany N. W. WOOD (1525), Institute of Neurology, Univer
sity College London, London WCIN 3BG, United
ARTHUR S. WALTERS (1177), John Fitzgerald
Kingdom
Kennedy Nev^ Jersey Neuroscience Institute, Edison,
New Jersey 08818
ULRICH WtJLLNER (921), Department of Neurol
MICHAEL WELLER (783, 827, 881, 897), Depart ogy, University of Tubingen, 72076 Tubingen,
ment of Neurology, University of Tubingen, 72076 Germany
Tubingen, Germany
ROBERT R. YOUNG (925,1247), Department of Neu
GREGOR K. WENNING (1081), Department of Neu rology, University of California, Irvine, California
rology, University of Innsbruck, Innsbruck A-6020, 92717
Austria
T YOUSRY (481), Departments of Radiology and Neu
W. WILLING (1495), Department of Internal Medicine, rology, University College London, WCIN 3BG
Academic Medical Centre, 1105 AZ Amsterdam, The London, United Kingdom
Netherlands
JOSEF ZIHL (255, 277), Department of Psychology,
EELCO E M. WIJDICKS (749), Department of Neurol Neuropsychology Unit, Ludv^ig-Maximilians Univer
ogy W8 A, Mayo Clinic, Rochester, Minnesota 55905 sity, 80802 Munich, Germany
PREFACE
The first edition of Neurological Disorders^ Course ties, since they are usually missing from popular text
and Treatment was so well received by the medical books. Unfortunately, in striving for a high standard of
community that the editors were encouraged to keep accuracy, we have not achieved our goal of concise for
the same uniform structure of the chapters: Clinical mulation, for the total pages in the book have increased
Aspects, Natural Course, Principles of Therapy, Practi from 1150 to over 1500. Nevertheless, we hope the
cal Management, and Treatments No Longer Recom uniform organization of the chapters, the numerous
mended. However, due to the numerous advances made tables, and the expanded index will still allow rapid ref
in neurology in the interim, almost all the previous erence and effective use.
chapters required extensive updating. Also, several Please note that in the sections "Principles of
new authors have participated in this edition, reflecting Therapy" and "Practical Management," the authors
our efforts to win more internationally recognized have indicated whenever possible the strength of the evi
experts. The number of chapters has increased from dence on which the recommended treatment is based.
95 to 107. The new chapters include Palliative care in The following system has been used: "' designates ret
the terminal stage of neurologic disease; Prion diseases; rospective studies, non-randomized studies, or empiri
Deep brain stimulation for movement disorders; Atyp cal recommendation without scientific proof; '''"* a
ical parkinsonism; Rehabilitation of motor function; randomized, prospective study; and ''''"''" more than one
Metabolic myopathies; Muscle stiffness; Microvascular prospective, randomized, placebo-controlled study or
diseases of the brain; Spinal cord ischemia; CNS para a meta-analysis. These symbols appear without any
sitic infestations-^ and also more specific conditions such further clarification in tables and the text next to the
as Hormone-sensitive headache in women; Anosmia, relevant study.
ageusia, and other disorders of chemosensation; as well Once again we thank all the authors, the editorial
as Disorders of spatial orientation. Because of the way staff at Academic Press, in particular Noelle Gracy, and
our colleagues use the book, we have continued to our own assistant Judy Benson, who again mastered
list the most important symptoms and diagnostic the awesome task of organizing and coordinating the
criteria under the section "Clinical Aspects," which we second edition, and finally, after many reminders, suc
consider to be an integral part of the management of ceeded in obtaining the very last manuscripts from the
individual disorders. In daily practice, diagnosis and over-committed experts. We would sincerely appreciate
therapy cannot be separated. It seemed especially impor hearing your suggestions or corrections to improve the
tant to include all therapeutic alternatives and special book's usefulness in any future edition.
exceptions under the heading of therapeutic possibili
Thomas Brandt
Louis R, Cap Ian
Johannes Dichgans
H. Christoph Diener
Christopher Kennard
xvii
PREFACE TO THE FIRST EDITION
Most neurological disorders pose an intellectual chal natural course, the frequency of symptoms, and com
lenge to the physician to make the correct neurological plications or causes of morbidity and death. Specific
diagnosis. The patient, on the other hand, is more con details are given on how the natural course is improved
cerned about the course and effective treatment of the by therapy.
condition. In the last analysis, it is only this effective Principles of Therapy, This section describes the
treatment of diseases or symptoms that justifies our very pathomechanism of the disorder on which the principles
expensive health care system and rewards the physician of therapy have been developed. At this stage the ration
for his day-to-day efforts. ale and results from controlled studies are presented,
Twenty years ago, neurology was considered a science along with hypothetical or experimental therapies not
of intriguing, but frequently untreatable, disorders, with yet tried or tested.
an "aesthetics of diagnosis" that exercised a unique fas Practical Management. In this "cookbook section"
cination. Fortunately, this has undergone a fundamen the reader can find immediate guidance concerning
tal change. Today the exciting prospect of new therapies situations when a prompt decision is required. Here
is at the center of the neurologist's work. Indeed, the specific guidelines are given, detailing which drug is
effectiveness of treatment in neurology corresponds used at which dosage, how the dosage should be
today to that of internal medicine. Striking examples of increased, the possible side effects and contraindications
this are the successful management of epilepsy, menin of the drug, if any, and alternative substances if the
gitis, Parkinson's disease, headache, vertigo, and myas drug of first choice cannot be prescribed or is ineffec
thenia gravis, the best elucidated model of the human tive. This section also includes various approaches
autoimmune disorders. to management of different stages and variants of a
The remarkable diversity of treatable diseases involv disorder (e.g., epilepsy, Parkinson's disease). Tables
ing the peripheral and central nervous systems requires and flow-charts are employed to illustrate the various
a clear and concise presentation, especially if this wealth stages of therapy as well as alternatives to the medical,
of information is packed into about 1000 pages. Our physical, and operative management. Indications for
aim is to present a comprehensive source of neurologi surgery are made clear. Also included are those special
cal therapy, applicable not only to the average patient situations in which routine therapy must be reconsid
but also to exceptional cases and covering alternative ered, such as pregnancy, surgery, anesthesia, allergy,
therapies and complications as well. In addition, we dependency, typical combinations of disorders, or
have included specific data on the expected success of simply differences of treatment in relation to age. It
therapy, prognostic factors, and limitations, as well as is this combination of precise guidelines for routine
a short comparison of natural course and course under management, alternative therapies, and variations of
therapy, if available. Organized for ease of use and therapy in exceptional situations that makes our book
quick reference, each chapter of the book presents a different.
neurological disorder or major symptom under the fol Treatments No Longer Recommended. This might be
lowing aspects: the most problematic section. Clear judgments are made
Clinical Aspects, Since therapy is based on the ap on forms of therapy that are still in use but have never
propriate diagnosis, a definition of the disease is given been shown to be effective or, even worse, have been
along with the relevant clinical and diagnostic features. shown to be ineffective or harmful. The benefit-to-risk
Also, the differential diagnosis relevant to therapy is ratio of such therapies is included, if known.
mentioned. The last chapter of the book summarizes most of the
Natural Course, Here we include facts and figures relevant side effects of drugs used in neurology. A final
on incidence and prevalence, age of onset, sex, genetics, table provides the reader with a selected list of trade
epidemiology, and provoking or aggravating factors. names for the generic drugs mentioned in the single
This section also contains a short description of the chapters. Since these were our aims, we hope it is under-
XX PREFACE TO THE FIRST EDITION
standable and excusable that the book in its current whereabouts of his manuscript perhaps best suggests the
version consists of 1122 pages. trials and tribulations of such a project:
Our special thanks to the authors, above all, for their
I received the chapter in question from Dr. X last March. I found
patience in the face of the editors' provoking criticism it unacceptable. I asked Dr. Y what to do, and he suggested that I
during the review process of the manuscripts. In the recommend extensive changes. I did that, and after a long time Dr.
ensuing discussions and confrontations they often X wrote back and suggested that I rewrite the chapter. I rewrote
the chapter and sent it to Dr. X in July. He found it unacceptable
shov^ed that they had been right in the beginning! Our
and wrote back asking for extensive changes. I made major
sincere thanks also to our assistant, Judy Benson, vs^ho
changes, trying to be fair to both points of view, and sent Drs. X,
has survived the difficult tasks of organizing and coor Y, and Z a final version in September. Since then. Dr. Y has told
dinating 95 chapters, helped resolve some difficult me the chapter is wonderful, Dr. Z has rejected it, and you act as
language-editing problems, and politely but relentlessly if you haven't even seen it! I suspect that it will not be possible to
come to an agreement on this chapter.
rounded up all delinquent manuscripts. Last but cer
tainly not least, we also express our appreciation to We are thankful that the prediction of the exasperated
Academic Press, above all Graham Lees, his assistant author has not come true. Nevertheless, our respect for
Karen Dempsey, and their coworkers, for their contin the difficulty of this project did not diminish over the
uing enthusiastic support of this project since its incep past four years. If anything, we have become even more
tion in 1992. aware of the remaining uncertainties and inadequacies
We have tried our best to produce a therapy book for (for example, on the border areas of neurosurgery, child
neurology that is as practical and specific as possible neurology, and psychiatry), but above all of the tran
while also advising the neurologist in the clinic and sience of concepts that are currently still valid. Thus, the
private practice on the value of different methods. Of editors would be thankful for any constructive sugges
course, nothing worthwhile is accomplished simply, and tions from critical readers that would allow us to elim
many nerves were at times quite frayed. The following inate any remaining weaknesses, correct errors, and in
excerpt from an author's response to a query about the general improve a later edition.
Thomas Brandt
Louis R. Caplan
Johannes Dichgans
H. Christoph Diener
Christopher Kennard
Acute and Chronic Pain
CHAPTER 1
Migraine
H. Christoph Diener and Peter J. Goadsby
The International Headache Society defines migraine an isolated aura without headache may occur, especially
as a disease characterized by intermittent attacks of in patients with the onset of migraine after the age of 20.
headache, often nausea, and associated features of Some of the subtypes of migraine currently recognized
sensory sensitivity, such as photophobia, phonophobia, are: migraine with prolonged aura (>60min), familial
and sensitivity to head movement (Headache Classifica hemiplegic migraine (with an idetified gene defect on
tion Committee of the International Headache Society, chromosome 19 in 50% of the cases) (Battistini et al.,
1988). The most frequent types of migraine are migraine 1999; Joutel et aL, 1993; Ophoff et aL, 1997; Terwindt
without aura and migraine with aura. et al., 1996)y basilar migraine, and retinal migraine
(monocular scotoma, monocular amaurosis).
CLINICAL ASPECTS
Complications of Migraine
Migraine without Aura
A complicated form of migraine attack is defined by
Migraine without aura (formerly common migraine) neurological deficits persisting for 7 or more days after
is characterized by repeated attacks of headache typi a migraine attack has ended or an ischemic lesion is
cally lasting from 4 to 72h. In 60% of the attacks the found in a CT or MRI scan while other reasons for a
headache is restricted to one side. It is pulsating, of cerebral infarction are excluded. Migraine is a risk
moderate to severe intensity, and aggravated by phy factor for stroke. Women who suffer from migraine
sical activity. In its typical form it is accompanied with aura, are overweight, smoke, and take the pill have
by nausea, vomiting, photophobia, and phonophobia. an increased stroke risk (Bousser, 1999; Buring et al.,
Migraine without aura should be diagnosed only after 1995; Chang et ai, 1999; Merikangas et aL, 1997).
five attacks. Focal dysrhythmia in the EEG may persist after the end
of a migraine attack even without any ischemic lesion
in the CT scan.
Migraine with Aura
Migraine with aura (formerly migraine accompanee Migraine in Children
or classic migraine) is characterized by neurological
symptoms and deficits, which are preferentially local Pediatric migraine is characterized by shorter attacks
ized in cortical areas (scotomas, hemianopsia, sensory and generalized headache. Nausea, vomiting, abdomi
deficits, aphasia) or in the brain stem (paraparesis, nal pain, or general impairment of well-being may pre
vertigo with nystagmus, ataxia, diplopia). These symp dominate (Gladstein et aL, 1993). After a short period
toms develop within a time period of 5 to 20min and of sleep the headache is often reduced. Some forms of
disappear completely within less than 60min. Con migraine in children are characterized by an isolated
comitant with or within 1 h after cessation of neurolog aura without headache (e.g., benign paroxysmal vertigo
ical symptoms the typical headache starts. It may be in children) (Abu-Arafeh and Russell, 1995; Lindskog
accompanied by autonomic disturbances. In some cases. et al, 1999).
Neurological Disorders: Course and Treatment, Second Edition
Copyright 2003, Elsevier Science (USA), All rights reserved.
