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Neuropathology Review Richard A. Prayson, MD Humana Press Contents NEUROPATHOLOGY REVIEW 2 Contents Contents NEUROPATHOLOGY REVIEW By RICHARD A. PRAYSON, MD Department of Anatomic Pathology Cleveland Clinic Foundation, Cleveland, OH HUMANA PRESS TOTOWA, NEW JERSEY 4 Contents © 2001 Humana Press Inc. 999 Riverview Drive, Suite 208 Totowa, New Jersey 07512 For additional copies, pricing for bulk purchases, and/or information about other Humana titles, contact Humana at the above address or at any of the following numbers: Tel.: 973-256-1699; Fax: 973-256-8341; E-mail:[email protected]; Website: http://humanapress.com All rights reserved. No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording, or otherwise without written permission from the Publisher. Due diligence has been taken by the publishers, editors, and authors of this book to assure the accuracy of the information published and to describe generally accepted practices. The contributors herein have carefully checked to ensure that the drug selections and dosages set forth in this text are accurate and in accord with the standards accepted at the time of publication. Notwithstanding, as new research, changes in government regulations, and knowledge from clinical experience relating to drug therapy and drug reactions constantly occurs, the reader is advised to check the product information provided by the manufacturer of each drug for any change in dosages or for additional warnings and contraindications. This is of utmost importance when the recommended drug herein is a new or infrequently used drug. It is the responsibility of the treating physician to determine dosages and treatment strategies for individual patients. Further it is the responsibility of the health care provider to ascertain the Food and Drug Administration status of each drug or device used in their clinical practice. The publisher, editors, and authors are not responsible for errors or omissions or for any consequences from the application of the information presented in this book and make no warranty, express or implied, with respect to the contents in this publication. Cover illustrations: Inset gross picture depicts a brain with lissencephaly. The background microscopic picture is of subacute sclerosing panencephalitis. Artwork courtesy of Richard A. Prayson, MD. Cover design by Patricia F. Cleary. This publication is printed on acid-free paper. ∞ ANSI Z39.48-1984 (American National Standards Institute) Permanence of Paper for Printed Library Materials. Photocopy Authorization Policy: Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by Humana Press Inc., provided that the base fee of US $10.00 per copy, plus US $00.25 per page, is paid directly to the Copyright Clearance Center at 222 Rosewood Drive, Danvers, MA 01923. For those organizations that have been granted a photocopy license from the CCC, a separate system of payment has been arranged and is acceptable to Humana Press Inc. The fee code for users of the Transactional Reporting Service is: [1-58829-024-7/01 $10.00 + $00.25]. Printed in the United States of America. 10 9 8 7 6 5 4 3 2 1 Library of Congress Cataloging-in-Publication Data Prayson, Richard A. Neuropathology review / by Richard A. Prayson. p. ;cm. Includes bibliographical references and index. ISBN 1-58829-024-7 (alk. paper) 1. Nervous system—Diseases—Outline, syllabi, etc. 2. Nervous system—Diseases—Examinations, questions, etc. I. Title. [DNLM: 1. Nervous System Diseases—Outlines. WL 18.2 P921n 2001] RC347.P724 2001 616.8’0076—dc21 2001016673 PREFACEContents The scope of neuropathology continues to expand, as evidenced by increasing numbers of multivolume and specialty texts that have been published in recent years. For those in the neuroscience disciplines, the ever increasing amount of information one needs to assimilate and master can be challeng- ing and even at times daunting. Neuropathology Review attempts to summarize, in outline form, the essentials of neuropathology. The objective is twofold: (1) to provide an overview of neuropathology, for those initially encountering the discipline and, (2) to provide a framework for review for those preparing for in-service and board examinations in the disciplines of neurology, neurosurgery, and pathology that require some knowledge of neuropathology. The text is divided into three main sections. The first part (Chapters 1–11) presents, in outline form, basic information on the spectrum of neurologic-related disease. The second part (Chapters 12 and 13) will present pertinent pictorial examples of a spectrum of neuropathologic conditions in a question format with answers and brief explanation provided. The final section (Chapters 14 and 15) will include a series of written questions provided with answers and explanations in order to review the material presented in the first section of the text. The author would like to thank Ms. Denise Egleton for her help in preparing this manuscript and to acknowledge Dr. Caroline Abramovich for her review of the text. Richard A. Prayson, MD v 6 Contents CONTENTSContents Preface .................................................................................................................................... v 1 Normal Histology .............................................................................................................1 2 Vascular Lesions .............................................................................................................. 7 3 Tumors............................................................................................................................ 15 4 Trauma............................................................................................................................ 35 5 Congenital Malformations, Perinatal Disease, and Phacomatoses ............................... 41 6 Demyelinating and Dysmyelinating Diseases ............................................................... 51 7 Metabolic and Toxic Diseases ....................................................................................... 55 8 Degenerative Diseases ................................................................................................... 59 9 CNS Infection................................................................................................................. 65 10 Skeletal Muscle .............................................................................................................. 75 11 Peripheral Nerve............................................................................................................. 85 12 Figures with Questions................................................................................................... 91 13 Answers to Figures with Questions ............................................................................. 185 14 Written Self-Assessment Questions............................................................................. 193 15 Answers to Written Self-Assessment Questions ......................................................... 211 Bibliography ....................................................................................................................... 217 Index ................................................................................................................................... 219 vii 8 Contents 1 Normal Histology I. Central Nervous System • Synaptophysin immunostain positive—mem- brane protein of synaptic vesicles A. Neurons • Lipofuscin (aging pigment, lipochrome) • 5–100 µminsize ◦ • Variety of shapes: stellate, oval, round, pyra- Increases with age midal, bipolar and unipolar ◦ Intracytoplasmic • Nucleus ◦ Light yellow-brown color on routine ◦ Vesicular with prominent nucleolus hematoxylin and eosin staining • ◦ Scalloped—Purkinje cells and pyramidal Hyaline (colloid inclusion) cells in cortical layer V ◦ Cytoplasmic • Cytoplasm ◦ Endoplasmic reticulum dilated cisternae ◦ Mitochondria prominent • Marinesco bodies ◦ Nissl substance—granular basophilic mate- ◦ Eosinophilic intranuclear inclusions rial, rough endoplasmic reticulum ◦ May be multiple within one nucleus ◦ Neurofilaments (60–100 A˚ , transport ◦ Prominently seen in pigmented neurons function) ◦ Increases with age ◦ May have pigments such as lipofuscin • and melanin A variety of other inclusions within neurons may be seen in association with certain dis- • Cell processes ease processes (e.g., Lewy bodies, tangles, ◦ Axon—conducts away from cell body, Hirano bodies, Pick bodies, granulovacuolar arises from axon hillock which contains degeneration, viral inclusions, etc.) no Nissl substance; axoplasm with mito- • Ischemic changes—“red and dead”— chondria, neurotubules, and neurofila- shrunken cell body with dark nucleus, indis- ments tinguishable nucleolus, and eosinophilic cyto- ◦ Dendrite—conducts toward cell body, con- plasm. tains Nissl substance • Central chromatolysis—related to axonal or • Pigmented (neuromelanin) neurons—coarse, retrograde degeneration, cytoplasmic swell- dark brown granules ing with loss of Nissl substance, and periph- eralization/flattening of the nucleus, may be ◦ Substantia nigra (midbrain) reversible to a point ◦ Locus ceruleus (pons) • Ferruginization of neurons (fossilized neu- ◦ Dorsal motor nucleus of the vagus nerve rons)—encrusted with calcium and/or iron, (medulla)