Atlas of Clinical Neurology

1 Neurologic 1Investigation

The Cerebrospinal Fluid, 1

Plain Radiography, 1

Ultrasound: Noninvasive Scanning, 2

Computed Tomographic Scanning, 2

Magnetic Resonance Imaging, 2

Digital Subtraction Angiography, 13

Interventional Techniques, 13

Radioisotope Imaging, 15

Electrophysiology, 16

Electromyography, 20

Biopsy, 22

2 Motor Neuron 28and Peripheral Nerve Diseases

Pathogenesis of Motor Neuron Disease, 28

Clinical Variants of Classic Motor Neuron Disease, 28

Atypical Amyotrophic Lateral Sclerosis, 30

Monomelic Amyotrophy, 31

Acquired Sporadic Motor Neuron Diseases, 31

Genetic Motor Neuron Diseases, 31

Spinal Muscular Atrophies, 31

Other Forms of Spinal Muscular Atrophy, 33

Bulbar Syndromes, 33

Familial Motor Neuron Disease, 33

Peripheral Nerve Diseases, 33

Peripheral Neuropathy, 33

Genetically Determined Neuropathies, 34

Charcot-Marie-Tooth Disease, 34

Hereditary Neuropathy with Liability to Pressure Palsies, 36

Hereditary Sensory and Autonomic Neuropathy, 36

Hereditary Neuralgic Amyotrophy, 36

Neuropathies as Part of Genetic Systemic Neurologic Diseases, 37

Porphyria, 37

Disorders of Lipid Metabolism, 38

Familial Amyloid Polyneuropathies, 38

Metachromatic Leukodystrophy, 38

Polyneuropathies in Acquired Systemic Diseases, 39

Diabetic Neuropathy, 39

Nutritional and Deficiency Disorders, 41

Paraproteinemic Neuropathies, 41

Infectious and Postinfectious Causes, 42

Chronic Inflammatory Demyelinating Polyradiculoneuropathy, 43

Connective Tissue Disorders, 44

Toxic Neuropathies, 44

Paraneoplastic Neuropathies, 44

Neuropathy of Unknown Cause, 45

The Mononeuropathies, 45

3 Myopathies 57and Myasthenia

Genetic Myopathies, 57

Muscular Dystrophies and Related Disorders, 57

Myotonic Dystrophies and Non-dystrophic Myotonias, 65

Congenital Myopathies, 69

Mitochondrial Myopathies, 70

Metabolic Myopathies, 72

Acquired Myopathies, 74

Inflammatory Immune-Mediated Myopathies, 74

Myopathies in Systemic Diseases, 77

Toxin- and Drug-Induced Myopathies, 79

Myasthenia Gravis and Other Neuromuscular Transmission Disorders, 81

4 Cerebrovascular 99Disease: Cerebral Infarction

Anatomy, 99

Classification, 99

Cerebral Infarction, 99

Pathologic and Physiologic Consequences of Cerebral Infarction, 102

Investigation, 103

Clinical Syndromes, 109

Other Causes of Cerebral Infarction, 116

Hypertensive Encephalopathy, 121

Clinical Examination, 121

5 Cerebral Hemorrhage 143and Other Cerebrovascular Disorders

Cerebral Hemorrhage, 143

Clinical Features, 143

Investigation of Suspected Hematoma, 146

Nonhypertensive Intracerebral Hemorrhages, 147

Vascular Malformations, 147

Cerebral Amyloid Angiopathy, 153

Coagulation Disorders, 153

Aneurysms, 154

Cerebral Venous Thrombosis, 158

6 Dementia Syndromes 169

Alzheimers Disease, 169

Macroscopic Appearance, 169

Microscopic Appearance, 169

Clinical Features, 169

Investigation, 170

Treatment, 170

Dementia and Lewy Bodies, 170

Frontotemporal Dementia and Lobar Atrophies, 170

Frontotemporal Lobar Degeneration with Tau Pathology, 172

Picks Disease, 172

Familial Taupathies, 174

Frontotemporal Lobar Degeneration with Ubiquitin-Only Immunoreactive Changes, 174

Arteriosclerotic Dementia, 174

Imaging, 174

7 Extrapyramidal 176Disorders

Parkinsons Disease, 176

Clinical Features, 176

Investigation, 176

Treatment, 176

Postencephalitic Parkinsonism, 180

Atypical Parkinsonian Disorders, 182

Progressive Supranuclear Palsy, 182

Frontotemporal Dementia and Parkinsonism, 183

Corticobasal Degeneration, 184

Multisystem Atrophy, 185

Other Parkinsonian Syndromes, 186

Arteriosclerotic Parkinsonism, 186

Dystonia, 186

Primary Dystonia, 186

Secondary Dystonias, 189

Choreiform and Ballistic Movements, 191

Other Choreiform Disorders, 193

8 Intracranial Tumors: 201Paraneoplastic (Nonmetastatic) Neurologic Syndromes

Clinical Features of Intracranial Tumors, 201

The Investigation of Intracranial Tumors, 202

Treatment of Brain Tumors: General Considerations, 206

Specific Tumors, 207

Metastases, 207

Primary Intraaxial Brain Tumors, 207

Tumors in or Adjacent to the Ventricular System, 214

Embryonal CNS Tumors, 216

Nonneuroepithelial Intraaxial CNS Tumors, 217

Primary Extraaxial Brain Tumors, 219

The Nonmetastatic (Paraneoplastic) Cerebral and Cerebellar Syndromes, 224

9 Spinal Tumors 249and Paraneoplastic Disorders

Clinical Features of Spinal Tumors, 249

Investigation of Spinal Tumors, 249

Specific Tumors, 253

Intramedullary Tumors, 253

Tumors of the Spinal Meninges, 255

Tumors of the Epidural Space and Spine, 256

10 Developmental 266and Familial Disorders

Neural Tube Defects, 266

Spina Bifida Occulta, 266

Spina Bifida Cystica, 266

Chiari Malformations, 267

Dandy-Walker Syndrome, 268

Lhermitte-Duclos Syndrome, 268

Agenesis of the Corpus Callosum, 269

Anomalies of the Septum Pellucidum, 269

Neuronal Migration Defects, 270

Disorders of Diverticulation, 271

Neuronal Heterotopias in Cerebral White Matter, 272

Arachnoid Cysts, 272

Porencephaly, 272

Hydrocephalus, 273

Normal Pressure Hydrocephalus, 274

Tuberous Sclerosis (Bournevilles Disease), 275

Neurofibromatosis (Von Recklinghausens Disease), 276

Familial Disorders Affecting the Cerebellum, 278

11 Pain Syndromes 289and Trauma

Headache, 289

Primary Headaches, 289

Secondary Headaches, 295

Neuralgias, 298

Cranial Neuralgias, 298

Postherpetic Neuralgia, 300

Causalgia and Reflex Sympathetic Dystrophy, 300

Head Injury, 300

Severity of Head Injury, 300

Mechanisms of Brain Injury, 301

Chronic Complications of Head Injury, 304

Spinal Injury, 305

Injury to Both Bony and Neurologic Structures, 305

Spinal Cord Syndromes, 310

12 Infections 312

The Meningitides, 312

Viral Meningitis, 312

Bacterial Meningitis, 312

Tuberculous Meningitis, 313

Cryptococcal Meningitis, 314

Parenchymal Infections: Cerebritis, Encephalitis, Abscess, Subdural Empyema, 314

Fungal Encephalitis, 314

Parasitic Infection, 316

Spinal Cord Infarction, 376

Spinal Extradural Hematoma, 377

Amyloidosis, 378

Anterior Herniation of the Cord, 378

15 Cranial Neuropathies 386

The First Cranial Nerve (Olfactory), 386

The Second Cranial Nerve (Optic), 386

Orbital Tumors, 386

Pupillary Syndromes, 387

The Third Cranial Nerve (Oculomotor), 389

The Fourth Cranial Nerve (Trochlear), 391

The Sixth Cranial Nerve (Abducens), 391

Orbital Lesions, 391

Brainstem Oculomotor Syndromes, 396

Other Horizontal Gaze Disorders, 401

The Fifth Cranial Nerve (Trigeminal), 401

The Seventh Cranial Nerve (Facial), 402

The Eighth Cranial Nerve (Acoustic), 405

The Ninth, Tenth, Eleventh, and Twelfth Cranial Nerves, 408

Combined Cranial Nerve Palsies, 408

16 Neurologic Aspects 413Of Systemic Disease

Radiation Damage, 413

Radiation Damage to Extracranial Vessels, 413

Alcohol and the Nervous System, 413

Central Pontine Myelinolysis, 417

Marchiafava-Bignami Disease, 418

The Neurology of Endocrine Disease, 418

Pituitary Disorders, 418

Adrenal Disorders, 418

Thyroid Disorders, 418

Parathyroid Disorders and Abnormalities of Calcium Metabolism, 418

Pagets Disease, 418

Pancreatic Disease, 418

Neurologic Features of Vasculitis, 420

Systemic Necrotizing Vasculitis, 420

Hypersensitivity Vasculitides, 421

Systemic Granulomatous Vasculitides, 421

Connective Tissue Disorders, 422

Systemic Lupus Erythematosus, 422

Scleroderma, 422

Sjögrens Syndrome, 423

Behçets Disease, 423

Vasculitis Associated with Infection, 423

Sarcoidosis, 423

Conversion Syndromes, 424

17 Epilepsy 434

Partial Seizures, 434

Generalized Seizures, 434

Causes of Epilepsy, 434

Investigation, 435

Magnetic Resonance Imaging, 436

Single-Photon Emission Computed Tomography, 439

Positron Emission Tomography, 439

Therapy, 440

Adverse Drug Effects, 441

Index, 449

Bacterial Cerebritis, 318

Cerebral Bacterial Abscess, 319

Subdural Empyema, 321

Encephalitis, 322

Viral Encephalitis, 322

Acquired Immune Deficiency Syndrome, 328

Peripheral Nerve Complications, 329

Opportunistic Viral Encephalitides in AIDS, 329

Nonviral Opportunistic Infections in AIDS, 330

Cerebral Lymphoma in AIDS, 332

CNS Diseases Caused by Spirochetes, 332

Syphilis, 332

Lyme Disease, 334

Leptospirosis, 334

Prion Disease, 335

Creutzfeldt-Jakob Disease, 335

Variant Creutzfeldt-Jakob Disease, 336

13 Multiple Sclerosis 343and Other Demyelinating Disorders

Multiple Sclerosis, 343

Pathology, 343

Pathophysiology, 345

Clinical Features, 346

Investigation, 347

Devics Disease (Neuromyelitis Optica), 349

Balós Disease, 349

Schilders Disease, 349

Acute Disseminated Encephalomyelitis, 350

The Leukodystrophies, 350

Lysosomal Disorders, 350

Peroxisomal Disorders, 352

Other White Matter Disorders, 352

14 Spinal Disorders 360

Degenerative Disease of the Spine, 360

Cervical Disc Disease, 360

Investigation, 360

Management, 362

Thoracic Disc Disease, 362

Lumbosacral Disc Disease, 362

Investigation of Lumbosacral Disc Disease, 363

Spinal Stenosis, 364

Pagets Disease of the Spine, 364

Rheumatoid Arthritis and the Spine, 365

Infection, 366

Spinal Epidural Abscess, 366

Tuberculous Disease of the Spine, 367

Brucellosis, 368

Schistosomiasis, 368

HTLV-1-Associated Myelopathy, 369

Arachnoid Cysts, 369

Arachnoiditis, 369

Vascular Abnormalities of the Spine and Cord, 370

Spinal (Pial) Arteriovenous Malformations, 370

Spinal Dural Arteriovenous Fistulas, 373

Cavernous Hemangioma (Cavernoma), 373

Vertebral Body Hemangiomas, 373

Subacute Combined Degeneration of the Spinal Cord, 374

Radiation Myelopathy, 375

Syringomyelia, 375