Preface

This textbook of neurology, a translation and revision of the original book in German, was written for students of medicine. Persons starting their careers in the other health professions may also find it useful.

We the authors are two Swiss academic neurologists with our own narrow subspecialties in the broad field of neurology. In writing this book, we have set our personal interests aside, presenting in detail only what seemed to us to be most important for the student. The book begins with a brief review of fundamentals, then deals with the syndromes affecting each major component of the nervous system. It also includes chapters on the clinical neurological examination and on common ancillary tests, with explanations of their underlying principles.

The main emphasis of the book is on clinical disease states. Most of its chapters are thus devoted to the diseases affecting a particular part of the nervous system.

Despite enormous progress in our understanding of pathogenesis, especially in microbiology and molecular genetics, and despite advances in neuroimaging and electrophysiology, diagnostic evaluation in neurology still crucially depends on the meticulous analysis of clinical information, i. e., the findings of the clinical his-

tory and neurological examination. The book includes many illustrations of abnormalities that can be seen either in radiological images or directly on examination of the patient.

The information in the book, and the degree of detail in which each item is discussed, reflect the subjective and, sometimes, arbitrary choices of the authors. In choosing what to include, we have tried to answer the questions that our students have asked us most often over the years. We hope that the book will also meet the needs of readers of this English edition.

An English translation of a European book for medical students in America, Britain, and elsewhere might seem superfluous. Yet, having both lived, studied, and worked in the United States for many years, we have happily discovered that, in the interconnected world of medicine, all national and linguistic borders tend to lose their significance.

Finally, we offer thanks in advance to any readers who care to give us their criticism, thoughts, or suggestions for future editions.

Mark Mumenthaler

Heinrich Mattle

“The tongue of the wise brings healing” (Proverbs 12:18). Once again, it has been my privilege to translate and revise an important work by Professors Mumenthaler and Mattle. The fourth English edition of their comprehensive text, Neurology, was published by Thieme in 2004 and has met with an appreciative

audience. This shorter but still highly informative book is a teaching text intended to give the medical student a plentiful fund of knowledge in this wide-ranging and challenging field. May it enjoy a similar success.

Ethan Taub

Contents

Microscopic Anatomy of the

Nervous System . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Elements of Neurophysiology . . . . . . . . . . . . . . . 4

Elements of Neurogenetics . . . . . . . . . . . . . . . . . 5

General Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . 5

Neurogenetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

Genetic Counseling . . . . . . . . . . . . . . . . . . . . . . . . 6

2The Clinical Interview in Neurology . . . 8

Special Aspects of History Taking . . . . . . . . . . . 9

VII

Ancillary Tests in Neurology . . . . . . . . . . . 45

Imaging Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 Conventional Skeletal Radiographs . . . . . . . . . . 45 Computed Tomography (CT) . . . . . . . . . . . . . . . . 46 Magnetic Resonance Imaging (MRI) . . . . . . . . . 48 Angiography with Radiological Contrast

Media . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50 Myelography and Radiculography . . . . . . . . . . . 52 Diagnostic Techniques of Nuclear Medicine . 52

Electrophysiological Studies . . . . . . . . . . . . . . . . 54 Fundamentals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54 Electroencephalography (EEG) . . . . . . . . . . . . . . 54 Evoked potentials . . . . . . . . . . . . . . . . . . . . . . . . . . 56 Electromyography . . . . . . . . . . . . . . . . . . . . . . . . . 58 Electroneurography . . . . . . . . . . . . . . . . . . . . . . . . 60 Other Electrophysiological Studies . . . . . . . . . . 61

Other Ancillary Studies . . . . . . . . . . . . . . . . . . . . . 63

Cerebrospinal Fluid Studies . . . . . . . . . . . . . . . . . 63

Tissue Biopsies . . . . . . . . . . . . . . . . . . . . . . . . . . . . 64

Perimetry . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65

Circulatory Disorders of the Brain and

Nontraumatic Intracranial Hemorrhage . . . . . . 98

Cerebral Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . 98

Nontraumatic Intracranial Hemorrhage . . . . . 106

Infectious Diseases of the Brain and

Meninges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111

Infections Mainly Involving the Meninges . . . 112

Infections Mainly Involving the Brain . . . . . . . 114

Intracranial Abscesses . . . . . . . . . . . . . . . . . . . . . . 118

Metabolic Disorders and Systemic Illnesses

Affecting the Nervous System . . . . . . . . . . . . . . 120

Congenital Metabolic Disorders . . . . . . . . . . . . . 120

Acquired Metabolic Disorders . . . . . . . . . . . . . . 122

Diseases of the Basal Ganglia . . . . . . . . . . . . . . . 127

Fundamentals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127

Diseases Causing Hypertonia and

Hypokinesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127

Degenerative Systemic Diseases Causing

Hypertonia and Hypokinesia . . . . . . . . . . . . . . . 130

Diseases Causing Hyperkinesia . . . . . . . . . . . . . 131

Other Types of Involuntary Movement . . . . . . 135

Cerebellar Diseases . . . . . . . . . . . . . . . . . . . . . . . . 135

The More Common Diseases of the

Cerebellum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135

Dementing Diseases . . . . . . . . . . . . . . . . . . . . . . . . 137

The Dementia Syndrome . . . . . . . . . . . . . . . . . . . 137

Degenerative Brain Diseases Causing

Dementia as Their Most Prominent

Manifestation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139

Vascular Dementia . . . . . . . . . . . . . . . . . . . . . . . . . 140

7 Diseases of the Spinal Cord . . . . . . . . . . . . 141

Anatomical Fundamentals . . . . . . . . . . . . . . . . . . 141

The Main Spinal Cord Syndromes and Their Anatomical Localization . . . . . . . . . . . . . . . . . . . . 142

Spinal Cord Compression . . . . . . . . . . . . . . . . . . . 146 Spinal Cord Tumors . . . . . . . . . . . . . . . . . . . . . . . . 146 Myelopathy Due to Cervical Spondylosis . . . . 147

Circulatory Disorders of the Spinal Cord . . . . . 148 Blood Supply of the Spinal Cord . . . . . . . . . . . . 148 Arterial Hypoperfusion . . . . . . . . . . . . . . . . . . . . . 149 Impaired Venous Drainage . . . . . . . . . . . . . . . . . 149 Hemorrhage in or Adjacent to the Spinal

Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150

8Multiple Sclerosis and Other

Myelinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . 156

Fundamentals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156

Myelin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156

Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . 156

Other Demyelinating Diseases of Unknown

Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160

9Epilepsy and Its Differential

Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

Types of Epilepsy . . . . . . . . . . . . . . . . . . . . . . . . . . 161

Classification of the Epilepsies . . . . . . . . . . . . . . 161

Practical Clinical Management of a

Suspected Epileptic Seizure . . . . . . . . . . . . . . . . . 162

Generalized Seizures . . . . . . . . . . . . . . . . . . . . . . . 164

Partial (Focal) Seizures . . . . . . . . . . . . . . . . . . . . . 166

Status Epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . 168

Episodic Neurological Disturbances of Nonepileptic Origin . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Episodic Disturbances with Transient Loss of Consciousness and Falling . . . . . . . . . . . . . . . . . . 169 Episodic Falling without Loss of

Consciousness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170 Episodic Loss of Consciousness without

Falling . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171 Episodic Movement Disorders without Loss

of Consciousness . . . . . . . . . . . . . . . . . . . . . . . . . . . 171

10 Polyradiculopathy and

Polyneuropathy . . . . . . . . . . . . . . . . . . . . . . . . . 173

Fundamentals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173

Polyradiculitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173

Classic Polyradiculitis (Landry−Guillain−

Barré−Strohl Syndrome) . . . . . . . . . . . . . . . . . . . . 173

Chronic Inflammatory Demyelinating

(Recurrent) Polyneuropathy (CIDP) . . . . . . . . . 175

Cranial Polyradiculitis . . . . . . . . . . . . . . . . . . . . . . 175

Polyradiculitis of the Cauda Equina . . . . . . . . . 175

Polyneuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

Fundamentals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

Particular Etiologic Types of

Polyneuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176