Cerebral Sinus Thrombosis. Experimental and Clinical Aspects
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1 Cerebral Sinus Thrombosis Experimental and Clinical Aspects
2 Cerebral Sinus Thrombosis Experimental and Clinica! Aspects Edited by Karl Einhăupl Ludwig-Maximilians-University Klinikum Grosshadern Munich, Germany Oliver Kempski Johannes Gutenberg-University Institute for Neurosurgical Pathophysiology Mainz, Germany and Alexander Baethmann Ludwig-Maximilians-University Klinikum Grosshadern Munich, Germany Springer Science+Business Media, LLC
3 L1brary of Congress Catalog1ng-1n-Pub11cat1on Data Cerebral sinus thrombosis : experimental and clinical aspects 1 ~dited by Karl Einhaupl, Oliver Kempski. and Alexander Baethmann. p. cm. Proceedings based on an international symposium held Sept , 1987, in Nymphenburg, Germany. Includes bib11ographical references and index. ISBN ISBN (ebook) DOI / Sinus thrombosis--congresses. I. Einhăupl, Karl. II. Kempski, Oliver. III. Baethmann, A. [DNLM: 1. Sinus Thrombosis--diagnosis--congresses. 2. Sinus Thrombosis--physiopathology--congresses. WL 355 C ] RC388.5.C '12--dc20 DNLM/DLC for Library of Congress CIP Proceedings based on an international symposium on Experimental and Clinica! Aspects of Cerebral Sinus Thrombosis, held September 20-22, 1987, in Nymphenburg, Germany ISBN Springer Science+Business Media New York Originally published by Plenum Press, New York in 1990 Softcover reprint ofthe hardcover lst edition 1990 AII 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
4 PREFACE Contrary to the neurological manifestations of arterial cerebral blood flow disturbances, respective conditions resulting fram obstruction of the cerebro-venous system are far less well understood. Hence, cerebral sinus vein thrombosis (CSVT) ranks prominently among the group of neglected diseases of the brain. This might be attributable (a) to the diagnostic difficulties of the disorder and (b) to the fact that CSVT is associated with a host of heterogeneous neurological symptoms which often are not specific for the underlying venous flow disorder. Another complicating aspect is that CSVT is a consequence of other diseases as disparate as focal infection, trauma, neoplasia, or a thrombosis disposition caused by oral contraceptive use. Although progress has been made in establishing the correct diagnosis of the syndrome, many problems remain as the discussions contained within this volume vividly demonstrate. The same is true for the present understanding of the pathophysiological basis of the disease, eg, concerning the cerebro-venous circulation, the hemodynamic and neuropathological consequences in particular. Part of these deficits may be attributed to a scarcity of solid experimental data due to the limited availability of animal models. However, relevant experimental models are required for an in-depth analysis of the pathophysiological mechanisms, eg, causing brain tissue damage in relationship with the topographical distribution of the venous flow obstruction and, most importantly, for the testing of specific methods of treatment. This volume is based on an international symposium which covered all the important aspects of SVT. These are, among others, the structural and functional basis of the cerebra-venous system, and the neuropathology of SVT, particularly emphasizing specificities of the resulting brain tissue damage. Further topics concern novel experimental models for induction of an SVT in animals, together with a comprehensive morphological and functional analysis. The physiology and pathophysiology of the venous circulation of the brain is reviewed as well as recent findings of cerebral blood flow studies in patients. General discussions on the mechanisms of brain damage are included, eg, concerning smooth muscle reactivity under abnormal conditions, the nature of ischemic brain injury, fluid translocation between the extra- and intracellular space as the cause of v
5 cytotoxic cell swelling in ischemia and, finally, the role of vasoactive mediator compounds. The basic science issues are balanced by detailed analyses of the clinical aspects reaching from historical perspectives to the most recent experiences with modern methods of diagnosis. Yet, recent technical progress notwithstanding, the conclusion appears unavoidable that a prompt diagnosis of svr is still difficult to achieve. Therefore, in spite of modern technologies such as contrast enhanced computertomography, subtraction angiography, magnetic resonance imaging or ultrasound sonography, assessment of the disorder is still based on a careful clinical analysis of the patient. An update of the subject would be incomplete without discussions of treatment. Both neurosurgical interventions as well as conservative methods using heparin are competently covered. A variety of neurological disorders require neurosurgery of the cerebral sinus and of the cerebrovenous system. On the other hand, thrombotic occlusion of the sinus or of the cerebral veins is a domain of medical treatment. High-dose heparin appears currently to be the method of choice. Since heparin is not expected to dissolve the thrombotic obstruction but rather to prevent further spread of thrombosis, the effectivity of heparin provides additional evidence for svr as a gradual process. This is well reflected by the clinical course. This volume will fulfil its purpose if it raises the level of attention of clinicians to consider svr in patients with a subacute, often insidious onset of neurological symptoms and progressing deterioration as opposed to the acute symptomatology of an arterial occlusion. Since effective therapeutical methods are available, prompt establishment of the diagnosis is important. The editors would like to express their gratitude to the sponsors who have provided the basis for publication of this volume. We gratefully acknowledge support by Drs 1. Bauer and P. Hansen, UCB-Chemie GmbH, Kerpen/Sindorf, FRG, and by Mr Bartusch, Carl-Friedrich von Siemens Stiftung. We would also like to acknowledge the valuable secretarial support provided by Isolde Juna and Helga Kleylein and the editorial assistance offered by Ms M. Carter and J. Curtis of Plenum Publishing Co., London, United Kingdom. Munich, 1990 K. Einhaupl O. Kempski A. Baethmann vi
6 CONTENTS STRUCTURAL AND FUNCTIONAL BASIS Histomorphology and Ultrastructure of the Cerebro-Venous System w. Roggendorf 3 Neuropathological Findings in the Thrombosis of Cerebral Veins and Sinuses: Vascular Aspects J. Cervos-Navarro and S. Kannuki 15 Thrombosis of Cranial Veins and Sinuses: Brain Parenchymal Effects J.H. Garcia 27 A New Experimental Model of Sinus Vein Thrombosis M. Deckert, K. Frerichs, P. Mehraein, O. Kempski, A. Baethmann and K. Einhaupl 39 Experimental Model in the Cat for Cerebral Sino-Venous Occlusion S. Kannuki, J. Cervos-Navarro, K. Matsumoto and Y. Nakagawa 43 Brain Function, Pathophysiology and Heparin Therapy in Experimental Cerebral Sinus Vein Thrombosis K. Frerichs, M. Deckert, O. Kempski, K. Einhaupl and A. Baethmann 53 MECHANISMS OF BRAIN TISSUE DAMAGE Physiology and Pathophysiology of the Cerebro-Venous Circulation L.M. Auer 61 Cerebral Blood Flow in Patients with Sinus Vein Thrombosis P. Schmiedek, K. Einhaupl, E. Moser, U. Bull, G. Leinsinger and T. Kreisig 75 Vascular Smooth Muscle Reactivity in Hypoxia J. Grote and G. Siegel 85 Mechanisms of Ischemic Brain Damage O. Kempski, F. Staub, G.-H. Schneider, M. Zimmer and A.Baethmann 93 vii
7 Cellular Mechanisms Involved in Generation of Brain Edema U. Heinemann and J. Sarvey 105 Eicosanoids, Peptides and Amines and the Cerebral Blood Vessels G. Feuerstein 123 CLINICAL ASPECTS OF SINUS VEIN THROMBOSIS Historical Perspectives on the Pathogenesis of Thrombosis in the Cerebral Veins and Sinuses H.J.M. Barnett 143 Clinical Spectrum of Sinus Venous Thrombosis K.M. Einhaupl, A. Villringer, R.L. Haberl, W. Pfister, M. Deckert, H. Steinhoff and P. Schmiedek 149 Aseptic Cerebral Venous Thrombosis M. Jacewicz and F. Plum 157 Clinical Findings and Differential Diagnosis of Cerebral Vein Thrombosis A. Hartmann, J. Wappenschmidt, L. Solymosi and F. Brassel 171 Diagnostic Strategies in Cerebral Sinus Vein Thrombosis M.-G. Bousser, C. Goujon, V. Ribeiro and J. Chiras 187 Ultrasound Diagnosis of Cerebro-Venous Flow Disturbances M. Hennerici 201 Delta (Empty Triangle) Sign in Patients Without Thrombosis of the Superior Sagittal Sinus C. Hamburger, A. Villringer, M. Bauer and T. Lorz 211 Neurosurgery of the Cerebral Venous and Sinus System O. Gratzl 2 19 Treatment of Patients with Cerebral Sinus and Vein Thrombosis with Heparin W. Meister, K.M. Einhaupl, A. Villringer, P. Schmiedek, R. Haberl, H.W. Pfister, M. Pellkofer, H. Steinhoff, M. Deckert and F. Anneser 225 Index 231 viii
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