神经病学 Neurology （英文原版改编版）(留学生与双语教学用)

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Neurological diseases are some of the most frequently encountered diseases in medicine. Neurology has always been independently and widely learned by medical students across the globe. In recent years, an increasing number of foreign students have arrived in China and enrolled in the Bachelor of Medicine programs. Unfortunately, it is often difficult for medical students to learn the complexities of neurologic anatomy and physiology. Thus, it is imperative and highly beneficial to tailor an English text book of Neurology for an undergraduate program that is designed for international students. Recently, supported and organized by Tsinghua University Press and Tianjin Medical University, many Neurology experts and scholars have made contributions to this textbook, which is based entirely on the structure and profile of ¡°Netter¡¯s Neurology,¡± written by Professor H. Royden Jones, Jr., M.D., et al. and published by Elsevier Saunders. The contents of ¡°Netter¡¯s Neurology¡± are presented using vivid pictures that enable us to better understand the details of neurological

 

Neurological diseases are some of the most frequently encountered diseases in medicine. Neurology has always been independently and widely learned by medical students across the globe. In recent years, an increasing number of foreign students have arrived in China and enrolled in the Bachelor of Medicine programs. Unfortunately, it is often difficult for medical students to learn the complexities of neurologic anatomy and physiology. Thus, it is imperative and highly beneficial to tailor an English text book of Neurology for an undergraduate program that is designed for international students. Recently, supported and organized by Tsinghua University Press and Tianjin Medical University, many Neurology experts and scholars have made contributions to this textbook, which is based entirely on the structure and profile of ？°Netter？ˉs Neurology,？± written by Professor H. Royden Jones, Jr., M.D., et al. and published by Elsevier Saunders. The contents of ？°Netter？ˉs Neurology？± are presented using vivid pictures that enable us to better understand the details of neurological

Contents
目.录
SectionⅠ　Initial Clinical Evaluation
Chapter1 Clinical Neurologic Evaluation.2
CRANIAL NERVES: AN INTRODUCTION .3
CRANIAL NERVE TESTING.4
HORNER SYNDROME.7
CEREBELLAR DYSFUNCTION12
GAIT EVALUATION.12
ABNORMAL ADVENTITIOUS MOVEMENTS14
MUSCLE STRENGTH EVALUATION.15
MOTOR TONE .18
MUSCLE STRETCH REFLEXES, CLONUS, AND THE BABINSKI SIGN19
SENSORY EXAMINATION .20
SPINAL CORD SYNDROMES.21
THALAMIC INVOLVEMENT.22
CORTICAL SENSORY INVOLVEMENT.23

SectionⅡ　Headache and Facial Pain
Chapter2 Primary and Secondary Headache26
PRIMARY HEADACHE DISORDERS .26
MIGRAINE26
CLUSTER HEADACHE30
OTHER TRIGEMINAL AUTONOMIC CEPHALGIAS.32
TENSION-TYPE HEADACHE.32
CHRONIC DAILY HEADACHES. 33
SECONDARY HEADACHE DISORDERS 34
PRIMARY HEADACHE SYNDROMES WITH DEFINED TRIGGERS . 34
SECONDARY HEADACHE DISORDERS . 34
GIANT CELL (TEMPORAL) ARTERITIS. 35
BRAIN HEMORRHAGE, INFECTIONS, AND TUMORS.36
IDIOPATHIC INTRACRANIAL HYPERTENSION.36
LOW CSF PRESSURE HEADACHE .38

CRANIAL NEURALGIAS 39
OBSTRUCTIVE SLEEP APNEA.41
INFECTIOUS MECHANISMS.41
CONTIGUOUS STRUCTURE HEADACHES42

SectionⅢ　Epilepsy
Chapter3 Epilepsy .46
DIFFERENTIAL DIAGNOSIS  47
PARTIAL SEIZURES .48
GENERALIZED SEIZURES51
EPILEPTIC SYNDROMES  53
STATUS EPILEPTICUS . 53
ANTIEPILEPTIC THERAPY 55
SURGICAL TREATMENTS FOR EPILEPSY58
FUTURE DIRECTIONS.61

SectionⅣ　Cognitive and Behavioral Disorders
Chapter4　Delirium and Acute Encephalopathies.64
DEFINITION.65
EPIDEMIOLOGY65
DIAGNOSIS 66
NEUROANATOMIC CONSIDERATIONS67
NEUROCHEMICAL FOUNDATIONS.67
EVALUATION67
TREATMENT68
SUMMARY69

Chapter5　    Dementia:Mild Cognitive Impairment, Alzheimer Disease, Lewy Body Dementia,
Frontotemporal Lobar Dementia, Vascular Dementia.71
MILD COGNITIVE IMPAIRMENT.71
DEMENTIA.72
ALZHEIMER DISEASE. 73
TREATMENT84
DEMENTIA WITH LEWY BODIES.87
FRONTOTEMPORAL LOBAR DEMENTIA91
VASCULAR COGNITIVE IMPAIRMENT.94

Chapter6　Transmissible Spongiform Encephalopathy (CreutzfeldtJakob Disease)99
EPIDEMIOLOGY99
PATHOGENESIS100
CLINICAL PRESENTATION.100
DIAGNOSIS 101
TREATMENT102

SectionⅤ　Gait and Movement Disorders

Chapter7 Gait Disorders .104
ANATOMY AND PATHOPHYSIOLOGY.104
ETIOLOGY AND CLASSIFICATION.104
CORTICAL GAIT DISORDERS .106
SUBCORTICAL GAIT DISORDERS108
PERIPHERAL GAIT DISORDERS.110

Chapter8　Parkinson Disease.112
ETIOLOGY 114
GENES FOR PARKINSON DISEASE .115
PATHOLOGY/PATHOPHYSIOLOGY115
CLINICAL PRESENTATION.116
DIFFERENTIAL DIAGNOSES.118
DIAGNOSTIC EVALUATION.121
TREATMENT121

Chapter9 Atypical ParkinsonianSyndromes.125
PROGRESSIVE SUPRANUCLEAR PALSY126
PATHOPHYSIOLOGY 127
CLINICAL PRESENTATION.128
DIAGNOSIS 128
TREATMENT128
CORTICOBASAL DEGENERATION.129
PATHOPHYSIOLOGY 129
CLINICAL PRESENTATION.129
DIAGNOSIS 130
TREATMENT130
FRONTOTEMPORAL DEMENTIA PARKINSONISM–CHROMOSOME 17130
PATHOPHYSIOLOGY AND CLINICAL PRESENTATION.130
DIAGNOSIS AND TREATMENT131
MULTIPLE SYSTEM ATROPHY131
PATHOPHYSIOLOGY 132
CLINICAL PRESENTATION.132
DIAGNOSIS 132
TREATMENT133

Chapter10 Chorea .134
ETIOLOGY 134
PATHOPHYSIOLOGY 135
CLINICAL PRESENTATION.135
DIFFERENTIAL DIAGNOSES.138
DIAGNOSTIC EVALUATION.138
TREATMENT139

FUTURE DIRECTIONS.139

Chapter11　Wilson Disease.141
HISTORY .141
GENETICS.141
HEPATIC COPPER METABOLISM142
CLINICAL PRESENTATION.142
DIAGNOSIS 144
TREATMENT AND PROGNOSIS.145

Chapter12　Dystonia.147
CLASSIFICATION OF DYSTONIA.148
PRIMARY DYSTONIA148
SECONDARY DYSTONIA .149
PATHOPHYSIOLOGY 151
TREATMENT151

SectionⅥ Spinal Cord Disorders
Chapter 13 Anatomic Aspects of Spinal Cord Disorders .154
ANATOMIC CORRELATIONS.154
PATHOANATOMY.161
INTRA-AXIAL SPINAL CORD PATHOLOGIES .163

Chapter 14 Spinal Cord Myelopathies.166
ACUTE MYELOPATHIES 166
ACUTE EXTRADURAL SPINAL LESIONS.166
ACUTE INTRADURAL INTRAMEDULLARY SPINAL LESIONS.168
CHRONIC MYELOPATHIES173
EXTRADURAL MYELOPATHIES173
INTRADURAL INTRAMEDULLARY SPINAL CORD LESIONS .178

SectionⅦ Multiple Sclerosis and Other Demyelination Disorders
Chapter 15 Multiple Sclerosis 188
GENETIC FACTORS .188
PATHOLOGY .189
CLINICAL SUBTYPES 190
DIFFERENTIAL DIAGNOSIS 192
DIAGNOSTIC APPROACH.196
MANAGEMENT AND THERAPY .201
RELATED MS MANAGEMENT PROBLEMS.202
PROGNOSIS204

Chapter 16 Other Autoimmune CNS Demyelinating Disorders205
NEUROMYELITIS OPTICA/DEVIC DISEASE205
DIAGNOSIS 205

PROGNOSIS206
TREATMENT206
ACUTE DISSEMINATED ENCEPHALOMYELITIS.207
CLINICAL PRESENTATION.207
DIAGNOSTIC APPROACH.207
DIFFERENTIAL DIAGNOSIS 208
THERAPY AND PROGNOSIS.208
ACUTE HEMORRHAGIC LEUKOENCEPHALOPATHY.208
CLINICAL PRESENTATION.208
DIAGNOSIS 208
PATHOLOGY .208
TREATMENT208

SectionⅧ Infectious Disease
Chapter 17 Bacterial Diseases212

COMMON SYNDROMES 212

BACTERIAL MENINGITIS 212

PARAMENINGEAL INFECTIONS.217

SPECIFIC PATHOGENS .217
LYME DISEASE (BORRELIA BURGDORFERI)217
TUBERCULOSIS: BRAIN AND SPINE (MYCOBACTERIUM TUBERCULOSIS).220
NEUROSYPHILIS (TREPONEMA PALLIDUM)221

Chapter 18 Viral Diseases225
HERPES SIMPLEX ENCEPHALITIS 225
ETIOLOGY 225
CLINICAL PRESENTATION.225
DIAGNOSIS 226
THERAPY227
PROGNOSIS227
EASTERN EQUINE ENCEPHALITIS 228
EPIDEMIOLOGY228
CLINICAL PRESENTATION AND TREATMENT 228
DIAGNOSIS 228
THERAPY/PROGNOSIS228
WESTNILE VIRUS.229
ETIOLOGY/EPIDEMIOLOGY229
CLINICAL PRESENTATION.229
DIAGNOSIS 229
THERAPY229
HUMANIMMUNODEFICIENCY VIRUS (HIV)229
PRIMARY NEUROLOGIC HIV INFECTION (PNHI)230
HIV DEMENTIA 231

HIV PRIMARY CNS ANGIITIS.231
HIV MYELOPATHY231
HIV PERIPHERAL NEUROPATHY .231
HIV MYOPATHY.232
SHINGLES (HERPES ZOSTER)232
ETIOLOGY AND EPIDEMIOLOGY .232
PATHOPHYSIOLOGY 232
CLINICAL PRESENTATION.232
DIAGNOSIS 233
TREATMENT233
RABIES.234
ETIOLOGY 234
CLINICAL PRESENTATION.234
DIAGNOSIS 234
THERAPY235
POLIOMYELITIS235
EPIDEMIOLOGY AND ETIOLOGY .235
PATHOGENESIS235
CLINICAL PRESENTATION.236
POSTPOLIO SYNDROME237
DIAGNOSTIC APPROACH.238
PROGNOSIS238

SectionⅨ Neuro-Oncology
Chapter 19 Brain Tumors242
MALIGNANT BRAIN TUMORS .243
GLIOMAS243
GLIOBLASTOMA 244
LOW-GRADE GLIOMA245
ANAPLASTIC GLIOMA.247
PRIMARY CNS LYMPHOMA .247
OTHER PRIMARY BRAIN TUMORS.248
METASTATIC BRAIN TUMORS.250
BENIGN BRAIN TUMORS.251
MENINGIOMAS251
PITUITARY ADENOMA 253
CRANIOPHARYNGIOMA 254
ACOUSTIC NEUROMAS/ VESTIBULAR SCHWANNOMA255
OTHER BENIGN INTRACRANIAL TUMORS.257
FUTURE DIRECTIONS.258

Chapter 20 Spinal Cord Tumors .260
EXTRADURAL SPINAL TUMORS .261
CLINICAL PRESENTATION.261

DIAGNOSTIC APPROACH.261
TREATMENT AND PROGNOSIS.262
INTRADURAL EXTRAMEDULLARY TUMORS 263
CLINICAL PRESENTATION.264
TREATMENT264
INTRADURAL INTRA-AXIAL TUMORS264
CLINICAL PRESENTATION.264
TREATMENT265
FUTURE DIRECTIONS.265

SectionⅩ Cerebrovascular Diseases
Chapter 21 Anatomic Aspects of Cerebral Circulation 268
THE CAROTID ARTERY SYSTEM .268
VERTEBROBASILAR ARTERIES269
CEREBRAL SINUSES AND VEINS.272

Chapter 22 Ischemic Stroke273
ETIOLOGY AND PATHOPHYSIOLOGY273
CLINICAL PRESENTATION.277
DIAGNOSTIC APPROACH.286
TREATMENT289
FUTURE DIRECTIONS.293

Chapter23 Cerebral Venous Thrombosis295
ANATOMY .295
CLINICAL PRESENTATION.299
DIAGNOSTIC APPROACH.300
TREATMENT301
PROGNOSIS AND LONG-TERM COMPLICATIONS301

Chapter24 Subarachnoid Hemorrhage.303
CLINICAL PRESENTATION.305
DIFFERENTIAL DIAGNOSIS 307
DIAGNOSTIC APPROACH.307
PATHOPHYSIOLOGY 308
MANAGEMENT310

Chapter25 Intracerebral Hemorrhage314
PATHOPHYSIOLOGY OF HYPERTENSISVE PRIMARY ICH314
CLINICAL PRESENTATION.315
SECONDARY INTRACEREBRAL HEMORRHAGE319
MANAGEMENT AND PROGNOSIS .323
SUMMARY324

SectionⅪ　Trauma
Chapter26 Trauma to the Brain 328

GENERAL PRINCIPLES OF HEAD INJURY CARE328

SKULL FRACTURES .329
EXTRA-AXIAL TRAUMATIC BRAIN INJURIES.329
INTRA-AXIAL TRAUMATIC INJURIES .332
POSTERIOR FOSSA LESIONS.333
TRAUMATIC BRAIN INJURY IN MILITARY COMBAT SETTINGS.334
OVERALL TREATMENT PROTOCOLS 334
LONG-TERM PROGNOSIS OF TRAUMATIC BRAIN INJURY 335

SectionⅫ　Motor Neuron Disorders
Chapter27 Amyotrophic Lateral Sclerosis .338
ETIOLOGY, GENETICS, AND PATHOGENESIS 338
CLINICAL PRESENTATIONS.339
DIFFERENTIAL DIAGNOSIS 343
DIAGNOSTIC APPROACH.344
MANAGEMENT AND THERAPY .346
FUTURE DIRECTIONS.348

Chapter28 Other Motor Neuron Diseases and Motor Neuropathies349
CLINICAL PRESENTATION.349
DIFFERENTIAL DIAGNOSIS 356
DIAGNOSTIC APPROACH.357
MANAGEMENT AND THERAPY .358
FUTURE DIRECTIONS.358

Section Ⅻ
　Neuromuscular Hyperactivity Disorders
Chapter29 Stiff Person Syndrome 362
ETIOLOGY 363
CLINICAL PRESENTATION.363
DIFFERENTIAL DIAGNOSIS 364
DIAGNOSTIC APPROACH.364
TREATMENT AND PROGNOSIS.365

Section
　Polyneuropathies
Chapter30 Hereditary Polyneuropathies368
ETIOLOGY AND PATHOGENESIS .370
CLINICAL PRESENTATION.370
DIFFERENTIAL DIAGNOSIS 371
DIAGNOSTIC APPROACH.371
CLASSIFICATION OF CMT.372
MANAGEMENT AND THERAPY .374
FUTURE DIRECTIONS.374

Chapter31 Acquired Polyneuropathies .376

DIAGNOSTIC APPROACH.376

IDIOPATHIC LENGTH-DEPENDENT POLYNEUROPATHIES 381
TREATMENT384
GUILLAIN–BARRé SYNDROME .384
DIFFERENTIAL DIAGNOSIS OF DEMYELINATING POLYNEUROPATHIES.387
AIDP AND CIDP VARIANTS389
TREATMENT390
SENSORY NEURONOPATHIES390
TREATMENT AND PROGNOSIS.393

Section
　Neuromuscular Transmission Disorders
Chapter32 Myasthenia Gravis.396
ETIOLOGY AND PATHOGENESIS .397
CLINICAL PRESENTATION.399
DIFFERENTIAL DIAGNOSIS 399
DIAGNOSTIC APPROACH.400
MANAGEMENT AND PROGNOSIS .400

Chapter33 Other Neuromuscular Transmission Disorders.403
LAMBERT–EATON MYASTHENIC SYNDROME .403
ETIOLOGY AND PATHOPHYSIOLOGY404
CLINICAL PRESENTATION.405
DIAGNOSTIC APPROACH.405
DIFFERENTIAL DIAGNOSIS 407
TREATMENT AND PROGNOSIS.407

Section
　Myopathies
Chapter34 Hereditary Myopathies.410
CHANNELOPATHIES 412
PERIODIC PARALYSIS AND CONGENITAL MYOTONIC DISORDERS412
CLINICAL PRESENTATION.413
DIFFERENTIAL DIAGNOSIS 414
DIAGNOSTIC APPROACH.414
TREATMENT AND PROGNOSIS.415
GLYCOGEN AND LIPID STORAGE DISORDERS.415
PATHOPHYSIOLOGY 417
GENETICS.418
CLINICAL PRESENTATION.418
DIAGNOSTIC APPROACH.418
IDIOPATHIC HYPERCKEMIA419
MUSCLE BIOPSY.419
TREATMENT AND PROGNOSIS.420
MUSCULAR DYSTROPHIES 420
MYOTONIC MUSCULAR DYSTROPHY, TYPE 1 (DM1).421

PROXIMAL MYOTONIC MYOPATHY (DM2) 422
LIMB-GIRDLE MUSCULAR DYSTROPHIES422
DYSTROPHINOPATHIES422
FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY 425
EMERY–DREIFUSS MUSCULAR DYSTROPHY425
BETHLEM MYOPATHY.426
OCULOPHARYNGEAL MUSCULAR DYSTROPHY426
MYOFIBRILLAR (DESMIN) MYOPATHY 426
CONGENITAL MUSCULAR DYSTROPHIES 426
DISTAL MYOPATHIES OR MUSCULAR DYSTROPHIES.426
CONGENITAL MYOPATHIES427
HEREDITARY INCLUSION BODY MYOPATHY428
TREATMENT428
PROGNOSIS428

Chapter35 Acquired Myopathies 430

CLASSIFICATION.430

DIAGNOSTIC APPROACH.431
LABORATORY EVALUATION.431
ELECTROMYOGRAPHY433
IMAGING STUDIES 433
MUSCLE BIOPSY.434

SPECIFIC INFLAMMATORY MYOPATHIC DISORDERS .436
POLYMYOSITIS .436
DERMATOMYOSITIS .436
TREATMENT OF POLYMYOSITIS AND DERMATOMYOSITIS437
INCLUSION BODY MYOSITIS437

OTHER ACQUIRED MYOPATHIES438
TOXIC MYOPATHIES.438
CRITICAL ILLNESS MYOPATHY439
ENDOCRINE MYOPATHIES 439
INFECTIOUS MYOPATHIES.441
PARANEOPLASTIC NECROTIZING MYOPATHY441

TREATMENT OF MYOPATHIES442
PROGNOSIS442

Preface
前.言
Neurological diseases are some of the most frequently encountered diseases in medicine. Neurology has always been independently and widely learned by medical students across the globe. In recent years, an increasing number of foreign students have arrived in China and enrolled in the Bachelor of Medicine programs. Unfortunately, it is often difficult for medical students to learn the complexities of neurologic anatomy and physiology. Thus, it is imperative and highly beneficial to tailor an English text book of Neurology for an undergraduate program that is designed for international students. Recently, supported and organized by Tsinghua University Press and Tianjin Medical University, many Neurology experts and scholars have made contributions to this textbook, which is based entirely on the structure and profile of “Netter’s Neurology,” written by Professor H. Royden Jones, Jr., M.D., et al. and published by Elsevier Saunders. The contents of “Netter’s Neurology” are presented using vivid pictures that enable us to better understand the details of neurological diseases.
Matching the depth of the leading Chinese undergraduate textbooks for Neurology and written in a suitable format for undergraduate programs for international students, this adaptation is the carefully nurtured descendant of the original book; importantly, it retains most content and chapters of the original version. We have reorganized some chapters to facilitate complete comprehension of systemic knowledge. The text covers 16 sections, including clinical evaluation and common neurological diseases, comprising a total of 35 chapters. The main contents of this textbook provide the main symptoms and clinical features alongside vivid figures. The goal of this adaptation is to provide a comprehensive summary of the specialty of Neurology, enabling readers to gain clear and current knowledge of this field.
We gratefully acknowledge many individuals who have contributed to this book. We hope that this book will be instructive for neurology trainees, including medical students and residents, and will facilitate effective assessment in clinical practice. Suggestions and opinions are welcomed from teachers, undergraduates, and residents who utilize the book, to ensure that its subsequent editions are increasingly optimized for the success of future students of neurology.
YANG Li
Jan19,2018

Initial Clinical Evaluation

Chapter
1

Clinical Neurologic Evaluation

Overview and Basic Tenets
The neurologic examination begins the moment the patients get out of their seat to be greeted, the character of their smile or lack thereof, and subsequently as they walk to enter the neurologist’s office. An excellent opportunity to judge the patient’s language function and cognitive abilities occurs during the acquisition of the patient’s history. Concurrently, the neurologist is always attuned to carefully making observations in order to identify various clinical signs. Some are overt movements (tremors, restlessness, dystonia or dyskinesia); others are subtler, e.g., vitiligo, implying a potential for a neurologic autoimmune disorder. Equally important may be the lack of normal movements, as seen in patients with Parkinson disease. By the time the neurologist completes the examination, she or he must be able to categorize and organize these historical and examination findings into a carefully structured diagnostic formulation.
The subsequent definition of the formal examination may be subdivided into a few major sections. Speech and language are assessed during the history taking. The cognitive part of the examination is often clearly defined with the initial history and often does not require formal mental status testing. However there are a number of clinical neurologic settings where this evaluation is very time consuming and complicated; Chapter 2 is dedicated to this aspect of the patient evaluation. However, when there is no clinical suspicion of either a cognitive or language dysfunction, these
more formal testing modalities are not specifically
required.
Here the multisystem neurologic examination provides a careful basis for most essential clinical evaluations. Neurologists in training and their colleagues in practice cannot expect to test all possible cognitive elements in each patient that they evaluate. Certain basic elements are required; most of these are readily observable or elicited during initial clinical evaluation. These include documentation of language function, affect, concentration, orientation, and memory. When concerned about the patient’s cognitive abilities, the neurologist must elicit evidence of an apraxia or agnosia and test organizational skills. Once language and cognitive functions are assessed, the neurologist dedicates the remaining portion of the exam to the examination of many functions. These include visual fields, cranial nerves (CNs) (Fig. 1-1), muscle strength, muscle stretch reflexes (MSRs), plantar stimulation, coordination, gait and equilibrium, as well as sensory modalities. These should routinely be examined in an organized rote fashion in order not to overlook an important part of the examination. The patient’s general health, nutritional status, and cardiac function, including the presence or absence of significant arrhythmia, heart murmur, hypertension, or signs of congestive failure, should be noted. If the patient is encephalopathic, it is important to search for subtle signs of infectious, hepatic, renal, or pulmonary disease.