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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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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