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PLUM AND POSNER’S DIAGNOSIS OF STUPOR AND COMA
Fourth Edition
SERIES EDITOR
Sid Gilman, MD, FRCP
William J. Herdman Distinguished University Professor of Neurology
University of Michigan
Contemporary Neurology Series
53SLEEP MEDICINE Michael S. Aldrich, MD
54BRAIN TUMORS
Harry S. Greenberg, MD, William F. Chandler, MD, and Howard M. Sandler, MD
56MYASTHENIA GRAVIS AND MYASTHENIC DISORDERS Andrew G. Engel, MD, Editor
57NEUROGENETICS
Stefan-M. Pulst, MD, Dr. Med., Editor
58DISEASES OF THE SPINE AND SPINAL CORD
Thomas N. Byrne, MD, Edward C. Benzel, MD, and Stephen G. Waxman, MD, PhD
59DIAGNOSIS AND MANAGEMENT OF PERIPHERAL NERVE DISORDERS Jerry R. Mendell, MD, John T. Kissel, MD, and David R. Cornblath, MD
60THE NEUROLOGY OF VISION Jonathan D. Trobe, MD
61HIV NEUROLOGY
Bruce James Brew, MBBS, MD, FRACP
62ISCHEMIC CEREBROVASCULAR DISEASE
Harold P. Adams, Jr., MD, Vladimir Hachinski, MD, and John W. Norris, MD
63CLINICAL NEUROPHYSIOLOGY OF THE VESTIBULAR SYSTEM, Third Edition Robert W. Baloh, MD, and Vicente Honrubia, MD
64NEUROLOGICAL COMPLICATIONS OF CRITICAL ILLNESS, Second Edition Eelco F.M. Wijdicks, MD, PhD, FACP
65MIGRAINE: MANIFESTATIONS, PATHOGENESIS, AND MANAGEMENT, Second Edition
Robert A. Davidoff, MD
66CLINICAL NEUROPHYSIOLOGY, Second Edition
Jasper R. Daube, MD, Editor
67THE CLINICAL SCIENCE OF NEUROLOGIC REHABILITATION, Second Edition
Bruce H. Dobkin, MD
68NEUROLOGY OF COGNITIVE AND BEHAVIORAL DISORDERS
Orrin Devinsky, MD, and Mark D’Esposito, MD
69PALLIATIVE CARE IN NEUROLOGY Raymond Voltz, MD, James L. Bernat, MD, Gian Domenico Borasio, MD, DipPallMed, Ian Maddocks, MD, David Oliver, FRCGP, and Russell K. Portenoy, MD
70THE NEUROLOGY OF EYE MOVEMENTS, Fourth Edition
R. John Leigh, MD, FRCP, and
David S. Zee, MD
PLUM AND POSNER’S DIAGNOSIS OF STUPOR AND COMA
Fourth Edition
Jerome B. Posner, MD
George C. Cotzias Chair of Neuro-oncology
Evelyn Frew American Cancer Society Clinical Research Professor
Memorial Sloan-Kettering Cancer Center
New York, NY
Clifford B. Saper, MD, PhD
James Jackson Putnam Professor of Neurology and Neuroscience,
Harvard Medical School
Chairman, Department of Neurology
Beth Israel Deaconess Medical Center
Boston, MA
Nicholas D. Schiff, MD
Associate Professor of Neurology and Neuroscience
Department of Neurology and Neuroscience
Weill Cornell Medical College
New York, NY
Fred Plum, MD
University Professor Emeritus
Department of Neurology and Neuroscience
Weill Cornell Medical College
New York, NY
1
2007
1
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Copyright # 2007 by Oxford University Press, Inc.
Published by Oxford University Press, Inc.
198 Madison Avenue, New York, New York 10016
www.oup.com
Oxford is a registered trademark of Oxford University Press
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise,
without the prior permission of Oxford University Press.
Library of Congress Cataloging-in-Publication Data
Plum and Posner’s diagnosis of stupor and coma / Jerome B. Posner . . . [et al.]. — 4th ed. p. ; cm.—(Contemporary neurology series ; 71)
Rev. ed. of: The diagnosis of stupor and coma / Fred Plum, Jerome B. Posner. 3rd ed. c1980. Includes bibliographical references and index.
ISBN 978-0-19-532131-9
1. Coma—Diagnosis. 2. Stupor—Diagnosis. I. Posner, Jerome B., 1932– II. Plum, Fred, 1924– Diagnosis of stupor and coma. III. Title: Diagnosis of stupor and coma. IV. Series.
[DNLM: 1. Coma—diagnosis. 2. Stupor—diagnosis. 3. Brain Diseases—diagnosis. 4. Brain Injuries—diagnosis.
W1 CO769N v.71 2007 / WB 182 P7335 2007] RB150.C6P55 2007
616.8'49—dc22 2006103219
9 8 7 6 5 4 3 2 1
Printed in the United States of America on acid-free paper
Jerome Posner, Clifford Saper and Nicholas Schiff dedicate this book to Fred Plum, our mentor. His pioneering studies into coma and its pathophysiology made the first edition of this book possible and
have contributed to all of the subsequent editions, including this one. His insistence on excellence, although often hard to attain, has been an inspiration and a guide for our careers.
The authors also dedicate this book to our wives, whose encouragement and support make our work not only possible but also pleasant.
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Preface to the Fourth Edition
Fred Plum came to the University of Washington in 1952 to head up the Division of Neurology (in the Department of Medicine) that consisted of one person, Fred. The University had no hospital but instead used the county hospital (King County Hospital), now called Harborview. The only emergency room in the entire county was at that hospital, and thus it received all of the comatose patients in the area. The only noninvasive imaging available was primitive ultrasound that could identify, sometimes, whether the pineal gland was in the midline. Thus, Fred and his residents (August Swanson, Jerome Posner, and Donald McNealy, in that order) searched for clinical ways to differentiate those lesions that required neurosurgical intervention from those that required medical treatment. The result was the first edition of The Diagnosis of Stupor and Coma.
Times have changed. Computed tomography (CT) and magnetic resonance imaging (MRI) have revolutionized the approach to the patient with an altered level of consciousness. The physician confronted with such a patient usually first images the brain and then if the image does not show a mass or destructive lesion, pursues a careful metabolic workup. Even the laboratory evaluation has changed. In the 1950s the only pH meter in the hospital was in our experimental laboratory and many of the metabolic tests that we now consider routine were time consuming and not available in a timely fashion. Yet the clinical approach taught in The Diagnosis of Stupor and Coma remains the cornerstone of medical care for comatose patients in virtually every hospital, and the need for a modern updating of the text has been clear for some time.
The appearance of a fourth edition now called Plum and Posner’s Diagnosis of Stupor and Coma more than 25 years after the third edition is deserving of comment. There were several reasons for this delay. First, the introduction and rapid development of MRI scanning almost immediately after the publication of the last edition both stimulated the authors to prepare a new edition and also delayed the efforts, as new information using the new MRI methods accumulated at a rapid pace and dramatically changed the field over the next decade. At the same time, there was substantial progress in theory on the neural basis of consciousness, and the senior author wanted to incorporate as much of that new material as possible into the new edition. A second obstacle to the early completion of a fourth edition was the retirement of the senior author, who also developed some difficulty with expressive language. It became apparent that the senior author was not going to be able to complete the new edition with the eloquence for which he had been known. Ultimately, the two original authors asked two of their proteges, CBS and NDS, to help with the preparation of the new edition. Fred participated in the initial drafts of this edition, but not fully in the final product. Thus, the mistakes and wrongheaded opinions you might find in this edition are ours and not his. We as his students feel privileged to be able to continue and update his classic work.
One of our most important goals was to retain the clear and authoritative voice of the senior author in the current revision. Even though much of the text has been rewritten, we worked from the original organizational and conceptual context of the third edition. Fred Plum’s description of how one examines an unconscious patient was, and is, classic. Accordingly, we’ve tried whenever possible to use his words from the first three
viii Preface to the Fourth Edition
editions. Because the clinical examination remains largely unchanged, we could use some of the case reports and many of the figures describing the clinical examination from previous editions. Fred was present at each of the critical editorial meetings, and he continued to contribute to the overall structure and scientific and clinical content of the book. Most important, he instilled his ideas and views into each of the other authors, whom he taught and mentored over many years. The primary writing tasks for the first four chapters fell to CBS, Chapters 5 to 7 to JBP, and Chapters 8 and 9 to NDS. However, each of the chapters was passed back and forth and revised and edited by each of the authors, so that the responsibility for the content of the fourth edition remains joint and several.
Most important, although the technologic evaluation of patients in coma has changed in ways that were unimaginable at the time of publication of the earlier editions, the underlying principles of evaluation and management have not. The examination of the comatose patient remains the cornerstone to clinical judgment. It is much faster and more accurate than any imaging study, and accurate clinical assessment is necessary to determine what steps are required for further evaluation, to determine the tempo of the workup, and most important, to identify those patients in critical condition who need emergency intervention. Coma remains a classic problem in neurology, in which intervention within minutes can often make the difference between life and death for the patient. In this sense, the fourth edition of Plum and Posner’s Diagnosis of Stupor and Coma does not differ from its predecessors in offering a straightforward approach to diagnosis and management of these critically ill patients.
The authors owe a debt of gratitude to many colleagues who have helped us prepare this edition of the book. Dr. Joe Fins generously contributed a section on ethics to Chapter 8 that the other authors would not have otherwise been able to provide. Chapters were reviewed at various stages of preparation by Drs. George Richerson, Michael Ronthal, Jonathan Edlow, Richard Wolfe, Josef Parvizi, Matt Fink, Richard Lappin, Steven Laureys, Marcus Yountz, Veronique van der Horst, Amy Amick, Nicholas Silvestri, and John Whyte. These colleagues have helped us avoid innumerable missteps. The remaining errors, however, are our own. Drs. Jonathan Kleefield and Linda Heier have provided us with radiologic images and Dr. Jeffrey Joseph with pathological images. The clarity of their vision has contributed to our own, and illuminates many of the ideas in this book. We also thank Judy Lampron, who read the entire book correcting typos, spelling errors (better than spellcheck), and awkward sentences. We owe our gratitude to a series of patient editors at Oxford University Press who have worked with the authors as we have prepared this edition. Included among these are Fiona Stevens, who worked with us on restarting the project, and Craig Panner, who edited the final manuscript. Sid Gilman, the series editor, has provided continuous support and encouragement.
Finally, we want to thank the members of our families, who have put up with our intellectual reveries and physical absences as we have prepared the material in this book. It has taken much more time than any of us had expected, but it has been a labor of love.
Fred Plum, MD
Jerome B. Posner, MD
Clifford B. Saper, MD, PhD
Nicholas D. Schiff, MD
Contents
1.PATHOPHYSIOLOGY OF SIGNS AND SYMPTOMS OF COMA 3
ALTERED STATES OF CONSCIOUSNESS 3
DEFINITIONS 5
Consciousness Acutely Altered States of Consciousness Subacute or Chronic Alterations of Consciousness
APPROACH TO THE DIAGNOSIS OF THE COMATOSE PATIENT 9
PHYSIOLOGY AND PATHOPHYSIOLOGY OF CONSCIOUSNESS AND COMA 11
The Ascending Arousal System Behavioral State Switching Relationship of Coma to Sleep The Cerebral Hemispheres and Conscious Behavior Structural Lesions That Cause Altered Consciousness in Humans
2.EXAMINATION OF THE COMATOSE PATIENT 38
OVERVIEW 38 HISTORY 39
GENERAL PHYSICAL EXAMINATION 40 LEVEL OF CONSCIOUSNESS 40
ABC: AIRWAY, BREATHING, CIRCULATION 42
Circulation Respiration
PUPILLARY RESPONSES 54
Examine the Pupils and Their Responses Pathophysiology of Pupillary Responses: Peripheral Anatomy of the Pupillomotor System Pharmacology of the Peripheral Pupillomotor System Localizing Value of Abnormal Pupillary Responses
in Patients in Coma Metabolic and Pharmacologic Causes of Abnormal Pupillary Response
OCULOMOTOR RESPONSES 60
Functional Anatomy of the Peripheral Oculomotor System Functional Anatomy of the Central Oculomotor System The Ocular Motor Examination Interpretation of Abnormal Ocular Movements
MOTOR RESPONSES 72
Motor Tone Motor Reflexes Motor Responses
FALSE LOCALIZING SIGNS IN PATIENTS WITH METABOLIC COMA 75
Respiratory Responses Pupillary Responses Ocular Motor Responses Motor Responses
ix
xContents
MAJOR LABORATORY DIAGNOSTIC AIDS 77
Blood and Urine Testing Computed Tomography Imaging and Angiography Magnetic Resonance Imaging and Angiography Magnetic Resonance Spectroscopy Neurosonography Lumbar
Puncture Electroencephalography and Evoked Potentials
3.STRUCTURAL CAUSES OF STUPOR AND COMA 88
COMPRESSIVE LESIONS AS A CAUSE OF COMA 89
COMPRESSIVE LESIONS MAY DIRECTLY DISTORT THE AROUSAL SYSTEM 90
Compression at Different Levels of the Central Nervous System Presents in Distinct Ways The Role of Increased Intracranial Pressure in Coma The Role of Vascular Factors and Cerebral Edema in Mass Lesions
HERNIATION SYNDROMES: INTRACRANIAL SHIFTS IN THE PATHOGENESIS OF COMA 95
Anatomy of the Intracranial Compartments Patterns of Brain Shifts That Contribute to Coma Clinical Findings in Uncal Herniation Syndrome Clinical Findings in Central Herniation Syndrome Clinical Findings in Dorsal Midbrain Syndrome Safety of Lumbar Puncture in Comatose Patients False Localizing Signs in the Diagnosis
of Structural Coma
DESTRUCTIVE LESIONS AS A CAUSE OF COMA 114 DIFFUSE, BILATERAL CORTICAL DESTRUCTION 114 DESTRUCTIVE DISEASE OF THE DIENCEPHALON 114 DESTRUCTIVE LESIONS OF THE BRAINSTEM 115
4.SPECIFIC CAUSES OF STRUCTURAL COMA 119
INTRODUCTION 120
SUPRATENTORIAL COMPRESSIVE LESIONS 120
EPIDURAL, DURAL, AND SUBDURAL MASSES 120
Epidural Hematoma Subdural Hematoma Epidural Abscess/Empyema Dural and Subdural Tumors
SUBARACHNOID LESIONS 129
Subarachnoid Hemorrhage Subarachnoid Tumors Subarachnoid Infection
INTRACEREBRAL MASSES 135
Intracerebral Hemorrhage Intracerebral Tumors Brain Abscess and Granuloma
INFRATENTORIAL COMPRESSIVE LESIONS 142
EPIDURAL AND DURAL MASSES 143
Epidural Hematoma Epidural Abscess Dural and Epidural Tumors
SUBDURAL POSTERIOR FOSSA COMPRESSIVE LESIONS 144
Subdural Empyema Subdural Tumors
Contents xi
SUBARACHNOID POSTERIOR FOSSA LESIONS 145
INTRAPARENCHYMAL POSTERIOR FOSSA MASS LESIONS 145
Cerebellar Hemorrhage Cerebellar Infarction Cerebellar Abscess Cerebellar Tumor Pontine Hemorrhage
SUPRATENTORIAL DESTRUCTIVE LESIONS CAUSING COMA 151
VASCULAR CAUSES OF SUPRATENTORIAL
DESTRUCTIVE LESIONS 152
Carotid Ischemic Lesions Distal Basilar Occlusion Venous Sinus
Thrombosis Vasculitis
INFECTIONS AND INFLAMMATORY CAUSES OF SUPRATENTORIAL DESTRUCTIVE LESIONS 156
Viral Encephalitis Acute Disseminated Encephalomyelitis
CONCUSSION AND OTHER TRAUMATIC BRAIN INJURIES 159
Mechanism of Brain Injury During Closed Head Trauma Mechanism of Loss of Consciousness in Concussion Delayed Encephalopathy After Head Injury
INFRATENTORIAL DESTRUCTIVE LESIONS 162
BRAINSTEM VASCULAR DESTRUCTIVE DISORDERS 163
Brainstem Hemorrhage Basilar Migraine Posterior Reversible
Leukoencephalopathy Syndrome
INFRATENTORIAL INFLAMMATORY DISORDERS 169
INFRATENTORIAL TUMORS 170
CENTRAL PONTINE MYELINOLYSIS 171
5.MULTIFOCAL, DIFFUSE, AND METABOLIC BRAIN DISEASES CAUSING DELIRIUM, STUPOR, OR COMA 179
CLINICAL SIGNS OF METABOLIC ENCEPHALOPATHY 181
CONSCIOUSNESS: CLINICAL ASPECTS 181
Tests of Mental Status Pathogenesis of the Mental Changes
RESPIRATION 187
Neurologic Respiratory Changes Accompanying Metabolic Encephalopathy Acid-Base Changes Accompanying Hyperventilation During Metabolic Encephalopathy Acid-Base Changes Accompanying Hypoventilation During Metabolic Encephalopathy
PUPILS 192
OCULAR MOTILITY 193
MOTOR ACTIVITY 194
‘‘Nonspecific’’ Motor Abnormalities Motor Abnormalities Characteristic of Metabolic Coma
DIFFERENTIAL DIAGNOSIS 197
Distinction Between Metabolic and Psychogenic Unresponsiveness Distinction Between Coma of Metabolic and Structural Origin