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
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