Topics in Neuroscience Managing Editor: GIANCARLO COMI Co-Editor: JACOPO MELDOLESI Associate Editors: UGO ECARI MASSIMO FILIPPI GIANVITO MARTINO
Springer-V erlag Italia Srl.
A. Vincent G. Martino (Eds) Autoantibodies in Neurological Diseases Springer
ANGELA VINCENT Neurosciences Group Department of Clinical Neurology Institute of Molecular Medicine John Radcliffe Hospital Oxford, United Kingdom GIANVITO MARTINO Neuroimmunology Unit Department of Neuroscience San Raffaele Scientific Institute Milan, The Editors and Authors wish to thank FARMADES-SCHERING GROUP () for the support and help in the realization and promotion of this volume Springer-Verlag Italia 2002 Originally published by Springer-Verlag Italia, Milano in 2002 Softcover reprint ofthe hardcover Ist edition 2002 ISBN 978-88-470-2163-1 ISBN 978-88-470-2097-9 (ebook) DOI 10.1007/978-88-470-2097-9 Library of Congress Cataloging-in-Publication Data: applied for This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, re-use of illustrations, recitation, broadcasting, reproduction on microfilms or in other ways, and storage in data banks. Duplication of this publication or parts thereof is only permitted under the provisions of the Italian Copyright Law in its current version, and permission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the Italian Copyright Law. The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: the publisher cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Typesetting: Photo Life-Ideanet (Milan) Cover design: Simona Colombo SPIN: 10831851
To Enzo who taught me the art of reasoning and everything else worthwhile (G. MARTINO)
Foreword Immune-mediated neurological disorders present an increasing challenge to neurologists. Recent advances have identified new antibodies, new effector mechanisms and new diagnostic tests; these have greatly enhanced our understanding of disease aetiology and opened up therapeutic opportunities, but a critical understanding of this fast -developing field is not easy to find. This volume was conceived following a very successful course on neuroimmuno logy in Bergamo in 2000, and was written mainly by clinicians who are active in the laboratory. It will help to keep practising neurologists and neuroimmunologists abreast of the most recent developments, and to assess new information more critically. Angela Vincent and Gianvito Martino should be congratulated on master-minding this timely and practical text. July 2001 J. Newsom-Davis Professor Emeritus University of Oxford
Table of Contents Introduction - Approaches to Understanding Immune-Mediated Neurological Disorders: Measuring and Evaluating Autoantibodies to Neuronal Antigens G. MARTINO, A. VINCENT........................ Chapter 1 - New Antibodies to Neuronal and Muscle Antigens A. VINCENT, C. BUCKLEY, P. DALTON, L. CLOVER, R. LIGUORI, P. MONTAGNA, J. MCCONVILLE, W. HOCH..................... 15 Chapter 2 - Pregnancy and Myasthenia Gravis A.P. BATOCCHI........................ 28 Chapter 3 - Immunological Mechanisms in the Lambert-Eaton Myasthenic Syndrome A. EVOLl, P.A. TONALI........................... 40 Chapter 4 - Immunotherapy of Myasthenia Gravis C. ANTOZZI, F. BAGGI, F. ANDREETTA, M. MILANI, A. ANNONI, P. BERNASCONI, R. MANTEGAZZA, F. CORNELIO....................... 52 Chapter S - Ontogeny of Skeletal Muscle Cells G. COSSU................................ 63 Chapter 6 - Idiopathic Inflammatory Myopathies: Immunological Aspects R. MANTEGAZZA, P. BERNASCONI, F. CORNELIO.............. 76 Chapter 7 - Immunological Mechanisms of Paraneoplastic Nervous System Diseases B. GIOMETTO, P. NICOLAO, T. SCARAVILLI, M. VIANELLO, B. VITALIANI, A.M. FERRARINI, B.TAVOLATO.................... 93 Chapter 8 - Clinical Presentation and Mechanisms of Immune-Mediated Cerebellar Ataxia J. HONNORAT................................ 104 Chapter 9 - Stiff-Man Syndrome: Pathogenetic, Nosological and Therapeutic Considerations F. FOLLI, G. PICCOLO................. 124
x Table of Contents Chapter 10 - Paraneoplastic Neuropathies: a Neuropathological Overview A. QUATTRINI..................... 136 Chapter 11 - Opsoclonus-Myoclonus Syndrome in Childhood F. BLAES, B. LANG................... 144 Chapter 12 - Epilepsy and Autoantibodies P. BERNASCONI, T. GRANATA, F. BAGGI, L. PASSERINI, R. MANTEGAZZA....... 152 Chapter 13 - Primary and Secondary Vasculitis of the Central Nervous System V. MARTINELLI, A. MANFREDI, L. MOIOLA, M.G. SABBADINI, G. COMI....... 162 Subject Index.................................................... 175
List of Contributors F. Andreetta A. Annoni C. Antozzi F. Baggi A.P. Batocchi Institute of Neurology, Catholic University, Rome, P. Bernasconi F. Blaes Department of Neurology, Justus-Liebig University, Giessen, Germany C. Buckley Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom L. Clover Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital,Oxford, United Kingdom G.Comi Department of Neuroscience, San Raffaele Scientific Institute, Milan, F. Cornelio G. Cossu Stem Cell Research Institute, San Raffaele Scientific Institute, DIBIT, Milan; Department of Histology and Medical Embryology, "La Sapienza" University, Rome, P. Dalton Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital,Oxford, United Kingdom A. Evoli Institute of Neurology, Catholic University, Rome, A.M. Ferrarini Psychiatric Sciences, Second Neurological Clinic, University of Padua, F. Folli Unit for Metabolic Diseases and Department of Medicine, San Raffaele Scientific Institute, Milan,
XII List of Contributors B. Giometto T. Granata Department of Child Neurology, W. Hoch Max Planck Institute for Developmental Biology, Tiibingen, Germany J. Honnorat Neurologie B, H6pital Neurologique, Lyon, France B. Lang Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom R. Liguori Institute of Neurology, University of Bologna, A. Manfredi Department of Medicine, San Raffaele Scientific Institute, Milan, R. Mantegazza V. Martinelli Department of Neuroscience, San Raffaele Scientific Institute, Milan, G. Martino Neuroimmunology Unit, Department of Neuroscience, San Raffaele Scientific Institute, DIBIT, Milan, J. McConville Department of Clinical Neurology, Neurosciences Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom M. Milani L. Moiola Department of Neuroscience, San Raffaele Scientific Institute, Milan, P. Montagna Institute of Neurology, University of Bologna, P. Nicolao L. Passerini G. Piccolo Department of Neurology, Fondazione Mondino, University of Pavia, A. Quattrini Department of Neuroscience, San Raffaele Scientific Institute, Milan, M.G. Sabbadini Department of Medicine, San Raffaele Scientific Institute, Milan, T. Scaravilli B. Tavolato P.A. Tonali Institute of Neurology, Catholic University, Rome,
List of Contributors M. Vianello XIII B. Vitaliani A. Vincent Neurosciences Group, Department of Clinical Neurology, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom