Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Adult Hydrocephalus
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Adult Hydrocephalus Edited by
Daniele Rigamonti Professor of Neurosurgery, Radiology, and Radiation Oncology, Johns Hopkins University, Baltimore, MD, USA
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
University Printing House, Cambridge CB2 8BS, United Kingdom Published in the United States of America by Cambridge University Press, New York Cambridge University Press is part of the University of Cambridge. It furthers the University’s mission by disseminating knowledge in the pursuit of education, learning and research at the highest international levels of excellence. www.cambridge.org Information on this title: www.cambridge.org/9781107031777 © Cambridge University Press 2014 This publication is in copyright. Subject to statutory exception and to the provisions of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of Cambridge University Press. First published 2014 Printing in the United Kingdom by TJ International Ltd, Padstow, Cornwall A catalogue record for this publication is available from the British Library Library of Congress Cataloguing in Publication data Adult hydrocephalus / edited by Daniele Rigamonti. p. ; cm. Includes bibliographical references and index. ISBN 978-1-107-03177-7 (hardback) I. Rigamonti, Daniele, 1951– editor of compilation. [DNLM: 1. Hydrocephalus–etiology. 2. Adult. 3. Cerebrospinal Fluid Pressure. physiopathology. 5. Hydrocephalus–therapy. WL 203] RC391 616.85′8843–dc23 2013025680
4. Hydrocephalus –
ISBN 978-1-107-03177-7 Hardback Cambridge University Press has no responsibility for the persistence or accuracy of URLs for external or third-party internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate. Every effort has been made in preparing this book to provide accurate and up-to-date information which is in accord with accepted standards and practice at the time of publication. Although case histories are drawn from actual cases, every effort has been made to disguise the identities of the individuals involved. Nevertheless, the authors, editors and publishers can make no warranties that the information contained herein is totally free from error, not least because clinical standards are constantly changing through research and regulation. The authors, editors and publishers therefore disclaim all liability for direct or consequential damages resulting from the use of material contained in this book. Readers are strongly advised to pay careful attention to information provided by the manufacturer of any drugs or equipment that they plan to use.
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
I dedicate this book to my wife Karen, to my children Eva, Luigi and Alessandra, to my parents Luigi and Santa, to Bunny and Charles Salisbury who have endowed a chair for me, and to Carol Swenson-Tanzola and her family for their support of my work on hydrocephalus.
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
In memory of Professor Tony Marmarou: a scientist, a teacher, and a friend
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Contents List of contributors Prologue xii
ix
Section 1 – Basic sciences 1
Anatomy and physiology of the cerebrospinal fluid system 1 Thomas A. Kosztowski, Aristotelis S. Filippidis, C. Rory Goodwin, Benjamin D. Elder, and Daniele Rigamonti
2
Neuropathology of human hydrocephalus 14 Marc R. Del Bigio
3
Animal models of hydrocephalus 28 Marianne Juhler
4
Genetics of hydrocephalus 36 Jun Zhang
5
The epidemiology of hydrocephalus 57 Are Brean and Per Kristian Eide
Section 2 – Pathophysiology 6
7
8
Pathophysiology of gait dysfunction in normal pressure hydrocephalus 63 Jonathan Pindrik, Amy Bastian, and Daniele Rigamonti The pathophysiologic basis of cognitive dysfunction in idiopathic normal pressure hydrocephalus 70 Norman Relkin and Heather Katzen Incontinence and lower urinary tract symptoms in normal pressure hydrocephalus 80 Adam P. Klausner and Samuel P. Robinson
9 Normal pressure hydrocephalus grading scales 91 Masatsune Ishikawa
Section 3 – Diagnosis 10 The differential diagnosis of normal pressure hydrocephalus 99 Daniele Rigamonti, Marianne Juhler, and Carsten Wikkelsø 11 Core imaging in adult hydrocephalus 110 Benjamin D. Elder, Patrick Bankah, Ari M. Blitz, C. Rory Goodwin, Thomas A. Kosztowski, Ignacio Jusué-Torres, and Daniele Rigamonti 12 Imaging of the cerebrospinal fluid circulation 121 Olivier Balédent 13 Cerebrospinal fluid dynamics and infusion techniques 139 Cemil Kayis and Gunes A. Aygok 14 Monitoring of intracranial pressure and assessment of cerebrospinal fluid dynamics 150 Marek Czosnyka, Martin U. Schuhmann, Stefano Signoretti, Zofia Czosnyka, and John D. Pickard 15 Cerebrospinal fluid biomarkers in idiopathic normal pressure hydrocephalus 164 Andrew A. Tarnaris
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Contents
Section 4 – Treatment and outcomes 16 Hydrocephalus shunt procedures 175 Benjamin D. Elder, C. Rory Goodwin, Thomas A. Kosztowski, and Daniele Rigamonti 17 Hydrocephalus shunts: principles, hardware, shunt testing 190 Zofia Czosnyka, Krzysztof Cieslicki, Hugh K. Richards, John D. Pickard, and Marek Czosnyka 18 Management of shunts in normal pressure hydrocephalus: follow-up and late complications 207 Ahmed Mohyeldin, Ammar Shaikhouni, and John McGregor 19 Endoscopic third ventriculostomy: indications, technique, outcome, and complications 218 Steffen Fleck, Jörg Baldauf, and Henry W. S. Schroeder 20 Outcome of idiopathic normal pressure hydrocephalus 232 Gunes A. Aygok
22 Low-pressure syndromes and cerebrospinal fluid leaks: diagnosis and management 256 Debraj Mukherjee and Wouter I. Schievink 23 Management of the adult with congenital hydrocephalus 264 Jonathan Pindrik and Edward S. Ahn 24 Management of hydrocephalus with associated cerebrospinal fluid pathologies 275 Petra Margarete Klinge 25 Chiari malformation and hydrocephalus in adults 291 Aristotelis S. Filippidis and Harold L. Rekate 26 Pseudotumor cerebri syndrome 296 John D. Pickard and Nicholas Higgins
Epilogue 304 Index 305 Color plates appear between pages 132 and 133.
Section 5 – Associated conditions 21 Normal pressure hydrocephalus syndrome secondary to hemorrhage, infection, and malignancy 247 C. Rory Goodwin, Benjamin D. Elder, Thomas A. Kosztowski, and Daniele Rigamonti
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Contributors
Edward S. Ahn, MD Division of Pediatric Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA Gunes A. Aygok, MD, PhD Medical College of Virginia Commonwealth University, Department of Neurosurgery, Richmond, VA, and Department of Neurosurgery, North Shore Long Island Jewish Hospital School of Medicine, Manhasset, NY, USA Jörg Baldauf, MD Consultant, Department of Neurosurgery, University Medicine Greifswald, Germany Olivier Balédent, PhD Department of Magnetic Resonance Imaging Processing, University Hospital of Amiens, Amiens, France Patrick Bankah, MBChB, FWACS Neurosurgery Division, Department of Surgery, Korle Bu Teaching Hospital, Accra, Ghana Amy Bastian, PhD Professor, Department of Neuroscience, Johns Hopkins University School of Medicine, Kennedy Krieger Institute, Baltimore, MD, USA Marc R. Del Bigio, MD, PhD, FRCPC Department of Pathology, University of Manitoba, Diagnostic Services Manitoba, and Manitoba Institute of Child Health, Winnipeg, Canada Ari M. Blitz, MD Division of Neuroradiology, Department of Radiology and Radiologic Sciences, The Johns Hopkins Medical Institutions, Baltimore, MD, USA
Are Brean, MD, PhD Senior Consultant in Neurology, Department of Neurology, Oslo University Hospital – Rikshospitalet, Oslo, and Journal of the Norwegian Medical Association, Oslo, Norway Krzysztof Cieslicki, PhD Faculty of Mechatronics, Warsaw University of Technology, Warsaw, Poland Marek Czosnyka, PhD Department of Clinical Neurosciences, University of Cambridge, Addenbrooke’s Hospital, Cambridge, UK Zofia Czosnyka, PhD Department of Clinical Neurosciences, University of Cambridge, Addenbrooke’s Hospital, Cambridge, UK Per Kristian Eide, MD, PhD Professor of Neurosurgery, Department of Neurosurgery, Oslo University Hospital – Rikshospitalet, Oslo, and Faculty of Medicine, University of Oslo, Oslo, Norway Benjamin D. Elder, MD, PhD Department of Neurosurgery, The Johns Hopkins Medical Institutions, Baltimore, MD, USA Aristotelis S. Filippidis, MD, PhD Fellow, Department of Neurosurgery, Boston Medical Center, Boston, MA, USA Steffen Fleck, MD Consultant, Department of Neurosurgery, University Medicine Greifswald, Germany C. Rory Goodwin, MD, PhD Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, MD, USA Nicholas Higgins, FRCP, FRCR Department of Neuroradiology, Addenbrooke’s Hospital, Cambridge, UK
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
List of contributors
Masatsune Ishikawa, MD, PhD Normal Pressure Hydrocephalus Center, Otowa Hospital, Rakuwakai, Kyoto, Japan Marianne Juhler, MD, DMSc Professor of Experimental Neurosurgery, Copenhagen University and University Clinic of Neurosurgery at Rigshospitalet, Copenhagen, Denmark
Debraj Mukherjee, MD, MPH Maxine-Dunitz Neurosurgical Institute, Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA John D. Pickard, MD Professor, Academic Neurosurgery Unit, Department of Clinical Neurosciences, University of Cambridge, Addenbrooke’s Hospital, Cambridge, UK
Ignacio Jusué-Torres, MD, PhD Post-doctoral Fellow, Department of Neurosurgery, The Johns Hopkins Medical Institutions, Baltimore, MD, USA
Jonathan Pindrik, MD Assistant Resident/Housestaff, Department of Neurological Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
Heather Katzen, PhD Assistant Research Professor, Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, USA
Harold L. Rekate, MD Director, The Chiari Institute and Professor of Neurosurgery, Hofstra Northshore LIJ College of Medicine, Great Neck, NY, USA
Cemil Kayis, PhD Department of Neurosurgery, Medical College of Virginia Commonwealth University, Richmond, VA, USA
Norman Relkin, MD, PhD Department of Neurology, Weill Cornell Medical College, New York, NY, USA
Adam P. Klausner, MD Associate Professor, Division of Urology, Director, Neurology and Voiding Dysfunction, and Warren W. Koontz Professor of Urologic Research, Virginia Commonwealth University School of Medicine, Richmond, VA, USA
Hugh K. Richards, PhD Academic Neurosurgery, Addenbrooke’s Hospital, Cambridge, UK Daniele Rigamonti, MD, FACS, FAHA Professor of Neurosurgery, Radiology, and Radiation Oncology, Johns Hopkins University, Baltimore, MD, USA
Petra Margarete Klinge, MD Associate Professor of Neurosurgery, Warren Alpert Medical School of Brown University, Providence, RI, USA
Samuel P. Robinson, MD Virginia Commonwealth University School of Medicine, Richmond, VA, USA
Thomas A. Kosztowski, MD Department of Neurosurgery, The Johns Hopkins Medical Institutions, Baltimore, MD, USA
Wouter I. Schievink, MD Maxine-Dunitz Neurosurgical Institute, Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA
John McGregor, MD Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA Ahmed Mohyeldin, MD, PhD Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA
Henry W. S. Schroeder, MD, PhD Professor and Chairman, Department of Neurosurgery, University Medicine Greifswald, Germany Martin U. Schuhmann, MD, PhD Department of Neurosurgery, Eberhard-KarlsUniversität Tübingen, Germany
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
List of contributors
Ammar Shaikhouni, MD, PhD Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA Stefano Signoretti, MD, PhD Division of Neurosurgery, Department of Head & Neck Surgery, Azienda Ospedaliera S. CamilloForlanini, Rome, Italy Andrew A. Tarnaris, MD, MRCS Department of Neurosurgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Carsten Wikkelsø, MD, PhD Professor, Institute of Neuroscience and Physiology, Department of Clinical Neuroscience and Rehabilitation, University of Gothenburg, Gothenburg, Sweden Jun Zhang, ScD, PhD Associate Professor, Center of Excellence in Neurosciences, Texas Tech University Health Sciences Center, El Paso, TX, USA
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Cambridge University Press 978-1-107-03177-7 - Adult Hydrocephalus Edited by Daniele Rigamonti Frontmatter More information
Prologue
Solomon Hakim and colleagues, fifty years ago, described a new syndrome of reversible dementia, which they called normal pressure hydrocephalus (NPH). Prior to the advent of CT and MRI the diagnosis was limited and depended greatly on the clinical acumen of the clinician to recognize the presence of the triad of symptoms and to notice improvement following a lumbar puncture. CT and MRI have revolutionized the diagnostic process and document symptomatic and asymptomatic ventriculomegaly. The understanding of the pathophysiology has improved and we better understand the biology and the natural history of this syndrome. The late Tony Marmarou led an effort to develop, in the last decade, a very useful set of guidelines to improve the diagnosis and treatment of this condition. Currently, there is a general consensus that surgical intervention should be recommended when either a large volume spinal puncture or prolonged external lumbar drainage produces unequivocal improvement or an infusion test demonstrates an abnormal resistance to outflow. The recently published European multicenter study on hydrocephalus, however, confirmed that these diagnostic procedures have a significant false negativity. The standard surgical intervention for NPH has been a shunt. Endoscopic third ventriculostomy (ETV) has been very effective in those cases of NPH where a clear obstruction to the CSF pathway is present. Several early reports advocating the use of ETV in cases of “idiopathic” NPH have not been reproduced. Analysis of interventional outcomes in cases in which high definition MRI has ruled out any obstruction to CSF pathway confirms that a significant
percentage of patients diagnosed with hydrocephalus do not improve following ETV. The main purpose of this book is to provide an update on NPH, and more broadly on adult hydrocephalus, that will lead to better diagnosis and management for these patients. I, therefore, asked internationally recognized scholars in the field to contribute their knowledge and experience to critically summarize knowledge gained in recent years and to create a common foundation for future patient care. Section 1 discusses the pathology, epidemiology, anatomy, animal models, and genetics of hydrocephalus providing the foundation for the understanding of the nature of this condition. Section 2 discusses the pathophysiology of hydrocephalus in the human disease and the mechanisms underlying gait, memory, and urinary dysfunction. It also looks at the NPH grading scales that are critical to improving patient selection. Section 3 deals with the important elements of the differential diagnosis, imaging and physiological diagnostic modalities, as well as CSF biomarkers. Section 4 reviews the therapeutic options and the outcomes. Section 5 includes conditions that are frequently associated with or mentioned in the context of hydrocephalus. My heartfelt thanks go to all the contributors, whose effort made this book possible; to Jamie Hoffberger, my research assistant; and to the team at Cambridge University Press, led by Nicholas Dunton, that shepherded the project through.
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