Cerebellar Degenerations: Clinical Neurobiology
This book encompasses basic and clinical reports on the cerebellum and its primary atrophic disorders, the cerebellar degenerations. Rapid progress has been made in undestanding the organization and function of the cerebellum at the neuronal, synaptic, an
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FOUNDATIONS OF NEUROLOGY
SERIES EDITORS: Louis R. Caplan Jack Antel David Dawson Porter R.J., Schoenberg B.S. (eds): Controlled Clinical Trials in Neurological Disease 1990. ISBN: 0-7923-0613-9. Plaitakis, A. (ed): Cerebellar Degenerations: Clinical Neurobiology. 1992.
CEREBELLAR DEGENERATIONS: CLINICAL NEUROBIOLOGY
Edited by ANDREAS PLAITAKIS Department of Neurology Mount Sinai School of Medicine ofthe City of New York
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SPRINGER SCIENCE+BUSINESS MEDIA, LLC
Library of Congress Cataloging-in-Publication Data Cerebellar degenerations : clinical neurobiology / edited by Andreas Plaitakis. p. cm. - (Foundations of neurobiology) Includes index. ISBN 978-1-4613-6557-0 ISBN 978-1-4615-3510-2 (eBook) DOI 10.1007/978-1-4615-3510-2 1. Cerebellum-Degeneration. 2. Excitatory aminoacids-Pathophysiology. 3. Ataxia-Pathophysiology. 4. Ataxia telangiectasia-Pathophysiology. 5. Olivopontocerebellar atrophies-Pathophysiology. 1. Plaitakis, Andreas. II. Series. [DNLM: 1. Cerebellar Ataxia-etiology. 2. Cerebellar Ataxia-physiopathology. WI F099I v. 2/ WL 320 C4113] RC394.C47C47 1992 616.8-dc20 DNLM/DLC for Library of Congress 91-35348 CIP
Copyright © 1992 by Springer Science+Business Media New York Originally published by Kluwer Academic Publishers in 1992 Softcover reprint ofthe hardcover Ist edition 1992 Ali rights reserved. No part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher, Kluwer Academic Publishers, 101 Philip Drive, Assinippi Park, Norwell, Massachusetts 02061.
DEDICATION
To my wife, Ingrid, whose love, understanding, and patience have contributed to a family environment supportive of creative work; my daughters, Ariadne and Iris, whose artistic creativity and belief in the power of knowledge and imagination has provided intellectual stimulation; and my parents, Joannis and Aristea, who first suggested the dream of medicine to me.
CONTENTS
Contributing authors Preface
ix xv
INTRODUCTION
1. The cerebellum and its disorders in the dawn of the molecular age
1
A. PLAITAXIS
I. BASIC NEUROSCIENCES OF THE CEREBELLUM
2. Anatomy and neurochemical anatomy of the cerebellum J.
11
HAMORI
3. Physiology of the cerebellum
59
M.ITO
4. Amino acid transmitters in the adult and developing cerebellum
89
R. BALAZS
5. Glutamate receptors in mammalian cerebellum: Alterations in human ataxic disorders and cerebellar mutant mice
123
E.D. KOUVELAS, A. MlTSACOS, F. ANGELATOU, A. HATZIEFTHIMIOU, P. TSIOTOS, AND G. VOUKELATOU
6. Regional and cellular distribution of glutamate dehydrogenase and pyruvate dehydrogenase complex in brain: Implications for neurodegenerative disorders C. AOKl, T.A. MILNER, AND V.M. PICKEL
139
viii Contents
7. The Purkinje cell degeneration mutant: a model to study the consequences of neuronal degeneration
159
B. GHETTI AND L. TRIARHOU
II. CLINICAL NEUROSCIENCES OF THE CEREBELLUM
8. Classification and epidemiology
