Index 555

....................................................................................................

A • • • • • • • • • • • • • • • • • • • • • • • • A1542 marker, in Alzheimer's disease, 221 Abscess

brain, 282, 421-422 epidural

intracranial, 417-418 spinal, 418-419

fungal, 455-456 Acanthocytosis, 47 Acetyl ethyl tetramethyl tetralin neurotoxicity,

523 Achondroplasia, 5 Achromatopsia, 251 Acoustic neuroma, 63, 292-293 Acquired immunodeficiency syndrome (AIDS),

439-443 brain abscess in, 422 cryptococcal meningitis in, 452 cytomegalovirus polyradiculitis in, 437 dementia in, 440-442 lymphoma in, 294 neurologic syndromes in, 439-440 toxoplasmosis in, 464-466 treatment of, 443 tuberculous meningitis in, 415

Acromegaly, 70, 80 Acrylamide neurotoxicity, 522 Acute inflammatory demyelinating polyneuropa­

thy, 386, 488, 489 Acyclovir, in herpes simplex virus infection, 426-

427 Adenocarcinoma, in Von Hippel-Lindau disease,

67 Adenoma

eosinophilic, 80-81 growth hormone-secreting, 80-81 pituitary, 74, 78-80, 294

relative frequency of, 271 prolactin-secreting, 78, 79

Adrenocorticotropic hormone, 70 in Cushing's syndrome, 82

Adrenoleukodystrophy, 46 Adrenomyeloneuropathy, 46 Aging

cognitive changes with. See also Dementia Aging, cognitive changes with, 203-204 Agnosia, 244-250 AIDS; See Acquired immunodeficiency syndrome Akathisia, drug-induced, 349 Akinetic mutism, 89, 264 Alcohol

fetal exposure to, 512 overdose of, 90

Alcoholism, 512 Alexia, 252 Alfentanil, 87 Alopecia, in thallotoxicosis, 519 Alzheimer's disease, 38-40, 211-221, 260

AM2 marker in, 221

amyloid cascade hypothesis of, 215 15-amyloid in, 213 amyloid precursor protein in, 214-215 apolipoprotein E and, 212 biomarkers of, 221 cerebral amyloid angiopathy in, 225 cholinergic hypothesis of, 218 cortical disconnection hypothesis of, 218 diagnostic criteria for, 211 in Down syndrome, 41 dystrophic neurites in, 213 electroencephalography in, 209 entorhinal cortex in, 218 granulovacuolar degeneration in, 213 histopathology of, 213 historical perspective on, 211 Lewy body pathology in, 233-234 magnetic resonance imaging in, 219, 220 memory impairment in, 218 neurofibrillary tangles in, 213, 216 neuropil threads in, 213 pathogenesis of, 40 pathology of, 216 pedigree in, 38 plaques in, 213, 216 positron-emission tomography in, 210,219 progression of, 217 risk factors for, 212 staging of, 217 tau marker in, 221 vascular dementia with, 223 Amanita muscaria, 504 Amanita phalloides, 504

Amebiasis, 464, 469-470 Amitriptyline, in migraine headache prophylaxis,

537 Amnesia, 244, 258-263

acquired personality disorder and, 265 in Alzheimer's disease, 218 anoxia and, 263 basal forebrain lesions and; 262 cerebral anoxia and, 263 diencephalic lesions and, 262 temporal lobe lesions and, 260, 261

Amphetamine, in experimental cerebrovascular disease, 17 4

Amusia, 244, 249 Amygdala, calcification of, 265 15-Amyloid, in Alzheimer's disease, 213 Amyloid angiopathy, 176-180 Amyloid neuropathy, 398 Amyloid precursor protein, 38

in Alzheimer's disease, 214-215 Amyotrophic lateral sclerosis

familial, 51-52 sporadic, 487

Anderson-Fabry's disease, 64 Anencephaly, 2 Aneurysm

fusiform, 126-127 ventricular, cerebral embolism and, 146

Angelman syndrome, 14 Angiitis, granulomatous, 158-159, 224 Angiofibroma, in tuberous sclerosis, 63 Angiography

in brain tumors, 277 of cerebral artery, 104 in granulomatous angiitis, 224 in vasculitis, 550

Angiokeratoma, diffuse, 64 Angioma

cavernous epilepsy and, 373 familial, 6 7

in Sturge-Weber syndrome, 27 Angiomatosis, neuroretinal, 26 Angiopathy, amyloid, 176-180 Angiostrongylus cantonensis infection, 470 Anomie aphasia, 253, 257 Anosognosia, 244, 250 Anterograde memory, 258 Antibody, 478 Antidepressanroverdose, 90 Antigen-antibody complex, 478 Antigen-presenting cells, 479 Antihypertensive drugs, tardive syndromes with,

350 Anti-MAG-associated neuropathy, 490 Antiphospholipid antibody, 164 Antipsychotics, tardive syndromes with, 349, 350 Aortic valve, bioprosthetic, cerebral embolism

and, 146 Aphasia, 253-258

nonfiuent, progressive, 229-233 progressive, primary, 231

Apolipoprotein E, 39 Alzheimer's disease and, 212 lobar hemorrhage and, 180

APP gene, 38,39 Apperceptive agnosia, 244 Apperceptive prosopagnosia, 24 7 Apraxia, progressive, 232 Aprosodia, 253, 258 Arachnid neurotoxin, 508 Arachnoid cyst, 14 Arbovirus encephalitis, 431 Argopecten irradians neurotoxin, 506 Arnold-Chiari malformation, 547 Arsenic poisoning, 90, 519 Arteriovenous malformation

dural, 192-193 hemispheric, 190

Arteritis syphilitic, 152 Takayasu's, 155, 156-158 temporal, 155, 158 tuberculous, 153

Aryl sulfatase deficiency, 45 Ascending aortic atheromatous disease, cerebral

embolism and, 149 Aspartylglycosaminuria, dysostosis multiplex in,

31

556 Atlas of Clinical Neurology

Aspergillosis, 154, 455, 456 Associative agnosia, 244 Associative prosopagnosia, 247 Asterixis, 348 Astrocytoma

computed tomography in, 279 grading systems for, 286 histology of, 287 magnetic resonance imaging in, 279, 281 pediatric, 308 survival with, 272

Ataxia autosomal recessive, 50-51 dominant, 49-50 episodic, 354 Friedr~ich's, 51

Ataxia telangiectasia, 51 Ataxic hemiparesis, in lacunar infarction, 138 Atherosclerosis. See also Cerebrovascular disease,

atherothrombotic of basilar artery, 125-127 of middle cerebral artery, 124 pathology of, 119, 120 plaque of, 119, 120 risk factors for, 118 of vertebral artery, 124-125

Atlanto-axial subluxation, in Down syndrome, 30 Atrial fibrillation, cerebral embolism and, 147 Atrial myxoma, cerebral embolism and, 148-149 Atropine-scopolamine overdose, 90 Auditory agnosia, 244, 248, 249 Aura, in migraine headache, 531 Autism, in tuberous sclerosis, 25 Autoimmunity, 480

B • • • • • • • • • • • • • • • • • • • • • • • • Bacterial infection, 404-423. See also specific infec­

tions Balint's syndrome, 250-251 Barbiturates

dosage ranges for, 87 neurologic effects of, 86 neurophysiologic effects of, 87 overdose of, 90 pharmacokinetics of, 87

Barbiturate coma, for status epilepticus, 114, 115 Basal ganglia

calcification of, 49 lacunar infarction of, 136-139

Basal ganglia aphasia, 256 Basilar artery

anatomy of, 125 atherosclerosis of, 125-127 dolichoectasia of, 126-127 embolism of, 126, 142

Batrachotoxin, 510 Behs;et's disease, 155 Benedikt syndrome, 344 Benign intracranial hypertension, 544 Benzodiazepines

dosage ranges for, 87 neurologic effects of, 86 neurophysiologic effects of, 87

overdose of, 90 pharmacokinetics of, 87

Bereitschaftspotential, in tics, 339 Bickerstaff's encephalitis, 487 Binswanger's encephalopathy, 224 Bioprosthetic aortic valve, cerebral embolism and,

146 Biopsy

muscle in dermatomyositis, 395 in neuromuscular disease, 392-401 normal, 392

nerve in Charcot-Marie-Tooth disease, 56,401 in chronic inflammatory demyelinating

polyneuropathy, 400 in congenital hypomyelinating neuropathy,

400 in Dejerine-Sottas syndrome, 400

sural nerve, 56, 397 Black widow spider neurotoxin, 508 Blood patch, in low-CSF pressure headache, 543 Blue rubber bleb nevus syndrome, 66 Botulinum toxin, 518

in hemifacial spasm, 352 Botulism, repetitive stimulation studies in, 392 Bovine spongiform encephalopathy, 238 Braak staging system, in Alzheimer's disease, 217 Brain abscess, 282, 421-422 Brain death, 89 Brain stem

cavernous malformation of, 191 glioma of, 286-287 in migraine headache, 532

Brain tumors age-related incidence of, 271, 272 angiography in, 277 calcification in, 278, 295 vs. cerebral abscess, 282 cerebrospinal fluid examination in, 276 chemotherapy in, 299-301 computed tomography in, 278 diagnosis of, 274-283 electroencephalography in, 276 hemorrhage with, 282 Humphrey visual field examination in, 275 imaging studies in, 277-283 incidence of, 270, 271, 272 location of, 274 magnetic resonance imaging in, 283 vs. multiple sclerosis, 282 neurologic examination in, 275 papilledema in, 275 pediatric, 307-309

computed tomography in, 308, 309 distribution of, 307 hydrocephalus with, 308 magnetic resonance imaging in, 308, 309

radiation therapy in, 297-299 seizures and, 3 71-3 73 survival with, 272, 273, 296, 297 symptoms of, 274 treatment of, 296-301 visual field loss in, 275 WHO histologic classification of, 284-285

Broca's aphasia, 253, 254

Bromocriptine, in prolactinoma, 79 Brown recluse spider neurotoxin, 508 Butyrophenone, tardive syndromes with, 350

c • • • • • • • • • • • • • • • • • • • • • • • • Cafe-au-lait spots, in neurofibromatosis, 1, 62 Caffeine, in low-CSF pressure headache, 543 CAG triplet repeats, in Huntington's disease, 337 Calcification

of basal ganglia, 49 in brain tumors, 278, 295

Calcium channel blockers, tardive syndromes with, 350

Calcium channel disorders, 59 Carbohydrate-deficient glycoprotein syndrome,

dysostosis multiplex in, 31 Carbon monoxide poisoning, 90 Cardiac arrest, neurologic syndromes after, 92 Cardiomyopathy, dilated, cerebral embolism and,

145 Carotid artery

asymptomatic occlusion of, 122 computed tomographic angiography of, 132 conventional angiography of, 132 dissection of, 159-160, 548 embolism from, 121-122 magnetic resonance angiography of, 132 siphon portion of, atherothrombotic disease

of, 123 transcranial Doppler examination of, 130, 131 ultrasonography of, 129

Carotid artery disease, 119, 120 angioplasty in, 135 extracranial-intracranial bypass for, 136 management of, 133-136

Carotid endarterectomy, 133-135 Carpal tunnel syndrome, nerve conduction stud-

ies in, 389 Cassava neurotoxin, 505 Caudal regression sequence, 3 Cavernous angioma

epilepsy and, 373 familial, 6 7

Cavernous malformation, 190-191 Central neurocytoma, 291 Centronuclear myopathy, 394 Centruroides spp. envenomation, 508 Cerebellar degeneration, 485

paraneoplastic, 306 Cerebellar hemorrhage, 176 Cerebral amyloid angiopathy, 176-180, 225

algorithm for, 179 computed tomography in, 177, 178 hemorrhage in, 178-180 immunostain in, 177 magnetic resonance imaging in, 179 pathology of, 177

Cerebral artery angiography of, 104 anterior

embolism of, 141 transcranial Doppler examination of, 130

middle

atherosclerosis of, 124 embolism of, 121-122, 140 magnetic resonance imaging of, 131 transcranial Doppler examination of, 130

posterior embolism of, 142 transcranial Doppler examination of, 130

Cerebral atrophy, in dementia, 208, 219, 220 Cerebral autosomal dominant arteriopathy with

subcortical infarcts and leukoen-cephalopathy (CADASIL), 226, 548

Cerebral embolism, 139-149 algorithm for, 167 of anterior cerebral artery, 141 of anterior choroidal artery, 141 ascending aortic atheromatous disease and,

149 atrial fibrillation and, 147 atrial myxoma and, 148-149 ofbasilar artery, 142 bioprosthetic aortic valve and, 146 diagnosis of, 143 dilated cardiomyopathy and, 145 infective endocarditis and, 14 7-148 of middle cerebral artery, 140 mitral valve prolapse and, 144 myocardial infarction and, 145, 146 nonbacterial thrombotic endocarditis and, 145 patent foramen ovale and, 144 of posterior cerebral artery, 142 recanalization in, 139 recovery after, 171-17 4 rheumatic valvular disease and, 146 risk for, 149 sources of, 143-149 spontaneous echo contract in, 144 treatment of, 167-171 ventricular septal aneurysm and, 146

Cerebral herniation, 547 Cerebrospinal fluid

in bacterial meningitis, 407, 409, 413 in brain tumor, 276 in fungal meningitis, 453 low pressure of, headache with, 543-544 in Lyme disease, 462 in multiple sclerosis, 483 in neurosyphilis, 460

Cerebrotendinous xanthomatosis, 65 Cerebrovascular disease

atherothrombotic, 117-136. See also Stroke, ischemic

algorithm for, 167 computed tomography in, 128 conventional angiography in, 127, 132 extracranial-intracranial bypass for, 136 imaging of, 127-132 lacunar, 136-139 magnetic resonance angiography in, 127,

132 magnetic resonance imaging in, 128, 131 management of, 133-136 positron-emission tomography in, 128 prevention of, 135 recovery after, 171-17 4 spiral computed tomography angiography

in, 127,132

treatment of, 167-171 ultrasonography in, 127, 129, 130, 131, 134

coagulation defects and, 162-163 dendritic arborization pattern after, 17 4 embolic, 139-149. See also Cerebral embolism epilepsy and, 373 evaluation of, 165-168 experimental amphetamine for, 17 4 hypertensive, 17 5-17 6 imaging of, 166 magnetic resonance imaging in, 166, 173 pathophysiology of, 165 positron-emission tomography in, 173 recovery after, 171-17 4 treatment of, 169-171

Cervical spine, in Down syndrome, 30 Cervicogenic headache, 545 Cestode infection, 4 72 Charcot-Marie-Tooth disease

classification of, 54 gene mutation in, 399 median nerve conduction velocity in, 55 nerve biopsy in, 56, 401 pedigree for, 54, 56 physical manifestations of, 55

Chemotherapy, in brain tumors, 299-301 Cherry red spot, 42 Chiari malformation, type II, 19 Chickling pea neurotoxin, 505 Childhood spinal muscular atrophy, 53 Children, brain tumors in, 307-309 Chironex jieckeri neurotoxin, 507 Chlordecone neurotoxin, 519 Chloride channel disorders, 59 Chlorpromazine, 87 Chorea, 335-338

etiology of, 335-336 pathophysiology of, 338

Chorea-acanthocytosis syndrome, 47 Choroid plexus, papilloma of, 20, 290 Choroidal artery, anterior, embolism of, 141 CHRNA4 gene, 44 Chromosomal abnormalities

in Angelman syndrome, 14 in Charcot-Marie-Tooth disease, 54 in Down syndrome, 40 in fragile X mental retardation syndrome, 43 in hereditary ataxia, 49, 50 in holoprosencephaly sequences, 4 in hypermelanosis oflto, 26 in incontinentia pigmenti, 28 in Miller-Dieker lissencephaly, 8 in Prader-Willi syndrome, 13 in spastic paraplegia, 53

Chronic inflammatory demyelinating polyneu­ropathy, 488

evaluation of, 386 nerve biopsy in, 400

Chronic paroxysmal hemicrania, 541 Churg-Strauss syndrome, 155, 397 Ciguatoxin, 506 Circle ofWillis, 118

in internal carotid artery occlusion, 122 Claude syndrome, 344 Clinical dementia rating scale, 201 Clonidine, 87

Index 557

neurologic effects of, 86 Clostridium tetani neurotoxin, 514 Cluster headache, 539-541

treatment of, 541 Coagulation defects

cerebrovascular disease and, 162-163 experimental studies of, 165

Cobblestone complex, 9 Cocaine

overdose of, 90 vasculitis with, 159

Coccidioidal meningitis, 453-454 Coelenterate neurotoxin, 507 Cognition, age-related changes in, 203-204. See

also Dementia Colloid cyst, 295 Color anomia, 251 Coma, 88-95

barbiturate, 114, 115 after cardiac arrest, 92 clinical signs of, 88 computed tomography in, 93 in critically ill patient, 91 drug overdose and, 90 head injury and, 93-95 intracranial mass and, 91 metabolic, 89 postanoxic, 92

Compound muscle action potential, 389 Computed tomography

in aspergilloma, 455 in astrocytoma, 279 in bacterial meningitis, 408 in brain abscess, 422, 423 in brain tumors, 278, 308, 309 in cerebral amyloid angiopathy, 177, 178 in cerebral autosomal dominant arteriopathy

with subcortical infarcts and leukoen­cephalopathy, 226

in cerebrovascular disease, 128 in coma, 93 in dementia, 207 in dystonia, 329 in echinococcosis, 473 in epidural abscess, 417 in glioblastoma multiforme, 277 in glioma, 277, 299 in herpes simplex virus encephalitis, 430 in intracerebral hemorrhage, 100 in intracranial metastases, 304 in medulloblastoma, 278 in normal-pressure hydrocephalus, 207 in oligodendroglioma, 277 in stroke, 98-99 in subarachnoid hemorrhage, 181, 182, 549 in subdural empyema, 420 in torticollis, 329 in tuberculous meningitis, 551 in vascular dementia, 222, 223, 224

Conduction aphasia, 253, 255 Congenital hypomyelinating neuropathy, nerve

biopsy in, 400 Congenital myopathy, 394 Connexin, 399 Contingent negative variation, in migraine

headache, 533

558 Atlas of Clinical Neurology

Conus geographus envenomation, 509 Conus tulipa envenomation, 509 Corpus callosum, agenesis of, 4 Cortex, in migraine headache, 533 Cortical deafness, 248 Cortical dysplasia

epilepsy and, 370 focal, 12

Cortical plate, formation of, 5-6 Cortical-basal ganglionic degeneration, 323-324 Corticobasal body, 232 Corticotropin releasing hormone, 70 Cranial-cervical dystonia, 330 Craniopharyngioma, 7 4

histology of, 294-295 relative frequency of, 271

Creutzfeldt-Jakob disease, 41, 235, 446-447 clinical features of, 237, 448, 449 electroencephalography in, 348, 448 magnetic resonance imaging in, 235, 236 model of, 23 7 myoclonus in, 348 new variant, 238, 449 positron-emission tomography in, 210

Crossed aphasia, 253, 257 Crotalus durissus terrijicus bite, 510 Cryptococcal meningitis, 452 Cushing's disease, 70 Cushing's syndrome, 70, 81-82 Cyad seed neurotoxin, 515 Cyclopia, 4 Cycloserine, seizures with, 517 Cyclosporine, overdose of, 90 Cyst

arachnoid, 14 hydatid, 473 neurenteric, 15, 16-17

Cystatin B gene, 44 Cysticercosis, 154

epilepsy and, 374 Cytomegalovirus infection

microcephaly with, 29 polyradiculitis with, 437

D • • • • • • • • • • • • • • • • • • • • • • • • Dandy-Walker malformation, 18-19 Dawson's finger, in multiple sclerosis, 483 Declarative memory, 258 Deep brain stimulation, in Parkinson's disease,

320 Dejerine-Sottas syndrome, nerve biopsy in, 400 Dementia. See also Alzheimer's disease

AIDS, 440-442 causes of, 204-206, 226 cerebral atrophy in, 208 clinical classification of, 203 computed tomography in, 207 drug-induced, 206 electroencephalography in, 209 evaluation of, 200, 202, 207 frontotemporal, 227-229 grading of, 201 ICD-10 criteria for, 200

Lewy body, 233-234 limbic encephalitis and, 238-239 neuropsychologic testing in, 208 parkinsonism and, 325 positron-emission tomography in, 210 reversibility of, 206 secondary, 206 semantic, 231 spongiform encephalopathy and, 235-238 subcortical, 205 vascular, 221-226. See also Vascular dementia

Dementia lacking distinctive histopathology, 230 Dermal sinus tract, 15 Dermancentor andersoni neurotoxin, 508 Dermatomyositis

clinical features of, 395, 496 diagnosis of, 495 Gottron's sign in, 498 histologic changes in, 497 membrane attack complex in, 497 muscle biopsy in, 395 treatment of, 498

Dermoid, 15 Diabetes insipidus, 70-71, 77 Diabetes mellitus

caudal regression sequence and, 3 neuropathy in, 488

Diabetic ketoacidosis, 89 Diastematomyelia, 16

split notochord theory of, 16-17 Diazepam, 87 Dibenzazepine, tardive syndromes with, 350 Dilated cardiomyopathy, cerebral embolism and,

145 Dinoflagellate neurotoxins, 506 Diodon hystrix neurotoxin, 510 DMD gene, 57 Dolichoectasia, of basilar artery, 126-127 Doll's eye reflexes, in progressive supranuclear

palsy, 321 Dopamine, 70

in Parkinson's disease, 317 Double cortex, 9 Doublecortin, 9 Down syndrome (trisomy 21)

Alzheimer's disease in, 41 atlanto-axial subluxation in, 30 chromosomal abnormality of, 40 physical features of, 40

Doxorubicin neurotoxicity, animal model of, 521 Droperidol, 87 Drug overdose, 90 Drug-induced movement disorders, 349-351 Duchenne muscular dystrophy, 474

gene mutation in, 399 Duchenne-Becker muscular dystrophy, 56-57 Dysarthria-clumsy hand syndrome, in lacunar

infarction, 138 Dysequilibrium syndrome, 89 Dyskinesia paroxysmal, 354 tardive, 349, 351 Dysostosis multiplex, in lysosomal storage dis­

ease, 31-32 Dysplasia, cortical, 12 Dystonia, 326-330

age of onset of, 327 classification of, 328 diagnosis of, 329 differential diagnosis of, 333 drug-induced, 349, 351 electromyography in, 328 primary, 331 psychogenic, 353 secondary, 331, 332 segmental, 330 tardive, 349, 351 treatment of, 330

Dystrophin, 57

E • • • • • • • • • • • • • • • • • • • • • • • • Echinococcosis, 473 EJM1 gene, 44 Electroencephalography

in Alzheimer's disease, 209 in benign rolandic epilepsy, 360 in brain tumors, 276 in Creutzfeldt-Jakob disease, 348, 448 in dementia, 209 in encephalopathy, 235 in frontal lobe seizures, 361 in hepatic encephalopathy, 209 in juvenile absence epilepsy, 365 in Lennox-Gastaut syndrome, 367 in myoclonic epilepsy, 365, 366 in myoclonic jerks, 366 in myoclonus, 347 in status epilepticus, 111, 115 in temporal lobe epilepsy, 362, 363, 377 in tics, 339 in tonic-clonic seizure, 366

Electromyography in asterixis, 348 in dystonia, 328 in neuromuscular disease, 387-388 in tension-type headache, 539

Electrooculography, in Creutzfeldt-Jakob disease, 348

Embolism, cerebral, 139-149. See also Cerebral embolism

Emotional disorders, acquired, 263, 265 Empyema, subdural, 419-420 EMX2 gene, 11 Encephalitis, 105-106

algorithm for, 106 arbovirus, 431 herpes simplex virus, 107-110,429-431. See

also Herpes simplex virus encephalitis in immunodeficient patient, 106 limbic, 238-239 postinfectious, 434-435 Rasmussen's, 374 toxoplasma, 464-466 viral, 105, 424, 427-431

causes of, 428 clinical manifestations of, 427 differential diagnosis of, 428

Encephalocele, 2 Encephalomalacia, epilepsy and, 375

Encephalopathy Binswanger's, 224 drug-induced, 206 hepatic, 89 HIV, 440-442 in immunodeficient patient, 106 Leigh, 62 spongiform, 235-238

Endocarditis, cerebral embolism and, 145, 147-148

Endocrine system, 70 Enterovirus infection, neurologic syndromes

with, 424-425 Entorhinal cortex, in Alzheimer's disease, 218 Environmental sound agnosia, 244 Eosinophilic adenoma, 80-81 Eosinophilic inclusion bodies, in herpes simplex

virus encephalitis, 430 Ependymoma

histology of, 289 magnetic resonance imaging of, 296 pediatric, 309 survival with, 272

Epidermoid tumor, seizures and, 372 Epidural abscess

intracranial, 417-418 spinal, 418-419

Epidural hematoma, 102 Epilepsy, 357-380. See also Status epilepticus

absence, juvenile, 365 causes of, 368-369

cavernous angioma and, 373 cerebrovascular disease and, 373 classification of, 358, 359 cortical dysplasia and, 370 cryptogenic, 366-36 7 cysticercosis and, 374 diagnosis of, 376-377 differential diagnosis of, 376 drug treatment of, 377-379 epidemiology of, 367 familial, 44 generalized, idiopathic, 365 genetic factors in, 368-369 head injury and, 375 infection and, 3 7 4 localization-related

idiopathic, 359 symptomatic, 360

middle cranial meningocele and, 371 myoclonic, 365, 366 nodular heterotopia and, 370-371 Rasmussen's encephalitis and, 374 rolandic, benign, 360 status, 380 surgical treatment of, 379 symptomatic, 366-367 temporal lobe, 362, 363, 364, 377 treatment of, 377-380 tumor-related, 371-373 unexplained death in, 380

Exertional dyskinesia, 354 Eye of the tiger sign, 334

• • • • • • • • • • • • • • • • • • • • • • • • Fwaves, 390 Fabry's disease, 64 Facies

in Angelman syndrome, 14 in lissencephaly, 8 in Williams syndrome, 12, 13

Fahr's disease, 49 Familial amyotrophic lateral sclerosis, 51-52 Familial calcification of basal ganglia, 49 Familial cavernous angioma, 67 Familial epilepsy, 44 Familial multiple sclerosis, 67 Fasciculations, grouped, 388 Fatal familial insomnia, 41, 446-447, 450 FEB genes, 44 Fentanyl, 87 Fetal alcohol syndrome, 512 FGFR3 gene, 5 Fibrillation potentials, 388 Fibroma, in tuberous sclerosis, 63 Figure 8 appearance, in lissencephaly, 8 Fillipin, 43 FMR1 gene, 43 Focal cortical dysplasia, 12 Follicle-stimulating hormone, 70 Fracture, odontoid, 547 Fragile X mental retardation syndrome, 43 Freckling, in neurofibromatosis 1, 62 Friedreich's ataxia, 51 Frontotemporal dementia, 42, 227-229

diagnostic criteria for, 228 magnetic resonance imaging in, 227, 228 positron-emission tomography in, 228

FTDP-17 syndrome, 232-233 Fucosidosis, dysostosis multiplex in, 31 Fungal infection, 451-454, 455-456

clinical features of, 451 meningeal, 452-454 predisposing factors in, 451

Fungal neurotoxins, 514 Fusiform aneurysm, 126-127

G • • • • • • • • • • • • • • • • • • • • • • • • Gabapentin, in migraine headache prophylaxis,

537 Gambierdiscus toxicus neurotoxin, 506 Ganglioglioma

histology of, 291 seizures and, 372

Gerstmann-S triiussler-Scheinker syndrome, 41, 236,446-447,449

Geste antagoniste, in dystonia, 326 Giant cell arteritis, 549 Giant cell astrocytoma, subependymal, 288-289 Glasgow coma scale, 88-95 Glaucoma, in Sturge-Weber syndrome, 27 Glioblastoma multiforme

chemotherapy in, 300 computed tomography in, 277 histology of, 287-288

Index 559

magnetic resonance imaging in, 279 survival with, 272

Glioma age-related survival with, 273 bleeding with, 282 brain stem, 286-287 chemotherapy in, 300-301 computed tomography in, 277, 299 location of, 27 4 magnetic resonance imaging in, 279, 280 pediatric, 309 recurrent, 301 relative frequency of, 271 survival with, 272, 273, 296, 297 treatment of, 296-301 visual field loss in, 2 7 5

Global aphasia, 253, 255 Glycoconjugates, 490 Glycol overdose, 90 Gnathostoma infection, 470 Gonadotropin releasing factor, 70 Gonyaulax catenella neurotoxin, 506 Gottron's sign, in dermatomyositis, 498 Gower's maneuver, 56 Granular cortical atrophy, 225 Granulomatous angiitis, 158-159, 224 Granulovacuolar degeneration, in Alzheimer's

disease, 213 Grass pea neurotoxin, 505 Grouped discharged, 388 Growth hormone, 70 Growth hormone releasing factor, 70 Growth hormone-secreting adenoma, 80-81 GTP cyclohydrolase gene, 48 Guam parkinsonism dementia-amyotrophic lat­

eral sclerosis complex, 513, 515 Guillain-Barre syndrome, 386, 488, 489

H • • • • • • • • • • • • • • • • • • • • • • • • H reflex, 391 Hachinski Ischemic Score, in vascular dementia,

222 Hallervorden-Spatz disease, 334 Haloperidol, 87 Hamartin, 64 Head injury

algorithm for, 93 amnesia and, 260 diagnostic categories for, 94 epilepsy and, 375 headache with, 547 intracranial pressure management in, 94 outcome of, 95

Headache in Arnold-Chiari malformation, 547 in carotid dissection, 548 in cerebral herniation, 547 cervicogenic, 545 classification of, 526 cluster, 539-541 in giant cell arteritis, 549 infectious, 551-552 low-CSF pressure, 543-544

560 J Atlas of Clinical Neurology

metabolic, 552 migraine, 527-537. See also Migraine headache in neurofibromatosis II, 546 post-spinal tap, 543 referred skin areas in, 546 secondary, 546-552 in sinusitis, 551 in subarachnoid hemorrhage, 549 tension-type, 538-539 in Tolosa-Hunt syndrome, 550 toxic, 552 in toxoplasmosis, 552 traumatic, 547 in tuberculous meningitis, 551 unilateral, 541 vascular, 548-550 in vasculitis, 550

Heliotrope rash, 497 Helminthic infection, 470-473 Hemangioblastoma

capillary, 293-294 magnetic resonance imaging in, 281 in Von Hippel-Lindau disease, 66

Hemangioma, relative frequency of, 271 Hematoma, 102 Hemicrania continua, 541 Hemifacial spasm, 352 Hemiparesis, in lacunar infarction, 138 Hemiparkinsonism-hemiatrophy syndrome, 324 Hemiplegia, in lacunar infarction, 138 Hemophilus injluenzae type b vaccine, 411 Hemorrhage

in brain tumors, 282 cerebellar, 176 in cerebral amyloid angiopathy, 178-180 in herpes simplex virus encephalitis, 430 intracerebral, 100-104, 175-176 intraventricular, 17-18 subarachnoid, 102, 103, 104, 181-191,549.

See also Subarachnoid hemorrhage thalamic, 175

Hepatic encephalopathy, 89 electroencephalography in, 209 Hepatolenticular degeneration, 47 Hereditary bilateral acoustic neuroma, 63 Hereditary neuropathy with predisposition to

pressure palsies, 400 gene mutation in, 399 Heroin overdose, 90 Herpes simplex virus encephalitis, 107-110

algorithm for, 108 amnesia and, 260 brain edema in, 108, 109 clinical features of, 107, 429 computed tomography in, 430 differential diagnosis of, 107 eosinophilic inclusion bodies in, 430 intracranial hypertension in, 109 magnetic resonance imaging in, 431 neuropathology of, 109-110 pathogenesis of, 429 pathology of, 430 prognosis for, 110 temporal lucency in, 109

Herpes simplex virus meningitis, 426-427 Herpes zoster,438

Hexachlorophene neurotoxicity, animal model of, 520

n-Hexane neurotoxicity, 522 Hippocampal system, 259 HIV infection. See Acquired immunodeficiency

syndrome Holoprosencephaly sequence, 4 HTLV-1 associated myelopathy, 444 Humphrey visual field examination, in brain

tumors, 275 Hunter syndrome, dysostosis multiplex in, 31 Huntingtin, 46 Huntington's disease, 46-47

CAG triplet repeats in, 337 clinical features of, 336 magnetic resonance imaging in, 338 pathophysiology of, 338 positron-emission tomography in, 338

Hurler syndrome, dysostosis multiplex in, 31, 32 Hydatid cyst, 473 Hydranencephaly, 12 Hydrocephalus

in choroid plexus papilloma, 20 normal-pressure, 207, 325 posthemorrhagic, 17-18 subarachnoid hemorrhage with, 184-185 in tuberculous meningitis, 415

Hypercalcemia, 89 Hyperkeratosis, in arsenic poisoning, 519 Hypermelanosis oflto, 26 Hypernatremia, 89 Hyperosmolar diabetic coma, 89 Hypersensitivity vasculitis, 155 Hypertension, intracranial, benign, 544 Hypertensive intracerebral hemorrhage, 175-176 Hypocalcemia, 89 Hypoglycemia, 89 Hypomelonatic macule, in tuberous sclerosis, 64 Hyponatremia, 89 Hypopituitarism, 70

hypothalamic, 75 pituitary, 77

Hypothalamic-pituitary unit, 69, 71 Hypothalamus, 69, 72

lesions of, 7 4 Hypoxia, 89

I • • • • • • • • • • • • • • • • • • • • • • • • I cell disease, dysostosis multiplex in, 31 Idiopathic stabbing headache, 541 Iminodiropionitrile neurotoxicity, 522-523 Imipramine, oral-buccal dyskinesia with, 517 Immune system

anatomy of, 478 function of, 478, 479

Impaired emotional processing, 265 Inclusion body myositis, 499

clinical features of, 496, 499 diagnosis of, 495 histologic changes in, 497

Incontinentia pigmenti, 28 Incontinentia pigmenti achromians, 26 Indolone, tardive syndromes with, 350

Infantile spasms, in tuberous sclerosis, 25 Infarction. See also Stroke, ischemic

lacunar, 136-139 myocardial, 14 5, 146 paramedian, 344

Infection, 403-473. See also Encephalitis; Meningitis

bacterial, 404-423. See also specific infections fungal, 451-454 parasitic, 463-473. See also specific infections prion, 446-450 spirochete, 456-463. See also Lyme disease;

Neurosyphilis viral, 423-450. See also specific infections

Infective endocarditis, cerebral embolism and, 145,147-148

Inflammatory demyelinating polyradiculoneu­ropathy, 488, 489

Inflammatory myopathy, 495-499 clinical features of, 496, 497, 498, 499 diagnosis of, 495 treatment of, 498

Insect envenomation, 507 Insecticide neurotoxicity, 519 Insulin-like growth factor, 70 Intracerebral hemorrhage, 100-104

algorithm for, 101 computed tomography in, 100 hypertensive, 175-176

Intracranial epidural abscess, 417-418 Intracranial mass, 91 Intraventricular hemorrhage, 17-18 Ion channel disorders, 59 Iris, Lisch nodules of, 22, 23 Isaac's syndrome, 490 Isoniazid, neuropathy with, 517 Ito, hypermelanosis of, 26

J • • • • • • • • • • • • • • • • • • • • • • • • Janetta procedure, in trigeminal neuralgia, 543 Jelly fish neurotoxin, 507

K • • • • • • • • • • • • • • • • • • • • • • • • Kayser-Fleischer ring, 47,333,519 KCNQgenes, 44 Kennedy's spinobulbar muscular atrophy, 52 Kepone neurotoxicity, 519 Ketamine, 87

neurologic effects of, 86 Ketoacidosis, diabetic, 89 Kidneys, adenocarcinoma of, in Von Rippel-

Lindau disease, 6 7 Kinesigenic dyskinesia, 354 Klippel-Feil anomaly, 21 Kugel berg-Welander disease, 53 Kuru, 41, 446-447

L • • • • • • • • • • • • • • • • • • • • • • • • Lacunar infarction, 136-139

clinical findings in, 137, 138 multiple, 139

Lambert-Eaton myasthenic syndrome, 491 paraneoplastic, 307 repetitive stimulation studies in, 392 treatment of, 494

Lathyrism, 505 Latrodectus mactans neurotoxin, 508 Laxosceles reclusa neurotoxin, 508 Lead poisoning, 90,519 Leber's optic neuropathy, 61 Leigh encephalopathy, 62 Lennox-Gastaut syndrome, 367 Leptomeningeal carcinomatosis, 306 Leukodystrophy, 45-46 Leukoencephalopathy, radiation-induced, 298 Levodopa, in Parkinson's disease, 318, 319 Lewy body, 316, 325 Lewy body dementia, 233-234 Limbic encephalitis, 238-239 Lipohyalinosis, 137 Lisch nodules, in type 1 neurofibromatosis, 22,

23 Lissencephaly, 7

cobblestone, 9 Miller-Dieker, 7-8 X-linked, 9

Lithium overdose, 90 Loa loa, 470 Locked-in syndrome, 89 Long-term memory, 258-259 Lorazepam, 87 Luteinizing hormone, 70 Lyme disease, 461-463

cerebrospinal fluid in, 462 differential diagnosis of, 462 epidemiology of, 462 serologic tests for, 462 stages of, 461 treatment of, 463

Lymphocytes, function of, 479 Lymphoma, CNS, 294 Lymphomatoid granulomatosis, 155

M • • • • • • • • • • • • • • • • • • • • • • • • Machado-Joseph disease, 50 Macula, cherry red spot of, 42 Magnetic resonance imaging

in Alzheimer's disease, 219, 220 in astrocytoma, 279, 281 in brain abscess, 282 in brain tumors, 283, 308, 309 in carotid dissection, 548 in cerebral amyloid angiopathy, 225 in cerebral autosomal dominant arteriopathy

with subcortical infarcts and leukoen­cephalopathy, 226, 548

in cerebral herniation, 547 in cerebrovascular disease, 128, 131, 166, 173 in cluster headache, 540 in creutzfeldt-Jakob disease, 235, 236 in ependymoma, 296 in frontotemporal dementia, 227, 228

in glioblastoma multiforme, 279 in glioma, 279, 280 in H allervorden-Spatz disease, 334 in hemangioblastoma, 281 in herpes simplex virus encephalitis, 431 in HIV encephalopathy, 442 in Huntington's disease, 338 in intracranial metastases, 303, 304 in intracranial venous thrombosis, 550 in meningioma, 280 of middle cerebral artery, 131 in neurofibromatosis II, 546 in odontoid fracture, 547 in oligoastrocytoma, 279 in oligodendroglioma, 280 in olivopontocerebellar atrophy, 323 in postinfectious encephalitis, 435 in primary progressive aphasia, 231 in progressive multifocalleukoencephalopathy,

445 in radiation necrosis, 283, 298 in Schistosoma infection, 471 in schwannoma, 281 in sinusitis, 551 in spinal epidural abscess, 419 in spongiform encephalopathy, 235, 236 in subdural empyema, 420 in temporal lobe epilepsy, 364 in toxoplasmosis, 465, 552 in vascular dementia, 223

Maitotoxin, 506 Malaria, 464, 466-46 7 Manganese poisoning, 513 Mannosidosis, dysostosis multiplex in, 31 Maroteaux-Lamy syndrome, dysostosis multiplex

in, 31 Medullary infarction, 125 Medulloblastoma, 292

cerebrospinal fluid examination in, 276 computed tomography in, 278 pediatric, 309 relative frequency of, 271 survival with, 272

Mees' lines, in arsenic poisoning, 519 Meige syndrome, 330 MELAS (mitochondrial myopathy, encephalopa­

thy, lactic acidosis, and stroke-like episodes), 61

Membrane attack complex, in dermatomyositis, 497

Memory disorders of, 218, 258-263

in Alzheimer's disease, 218 hippocampal system and, 259 types of, 258

Meningioma, 293 locations of, 280 magnetic resonance imaging in, 280 radiation-induced, 299 relative frequency of, 271 survival with, 272

Meningitis bacterial, 151

acute, 404-413 adjunctive treatment of, 410 causative organisms in, 406, 407

Index 561

cerebrospinal fluid in, 407, .409 clinical manifestations of, 404 computed tomography in, 408 differential diagnosis of, 408, 409 mortality rates in, 410 pathogenesis of, 405 pathology of, 405 predisposing factors in, 406 rash of, 404 treatment of, 409, 410 vaccination against, 411

chronic, 411-413 cerebrospinal fluid in, 413 diagnosis of, 10, 413 differential diagnosis of, 411

coccidioidal, 453-454 cryptococcal, 452 fungal, 452-454

cerebrospinal fluid in, 453 differential diagnosis of, 453 treatment of, 453

tuberculous, 153, 414-416 viral, 424

acute, 425-426 clinical manifestations of, 425 differential diagnosis of, 424 tests for, 424 herpes simplex virus type 2 in, 426-427 neurologic syndromes with, 424-425

Meningocele, 371 Meningococcal rash, 404 Meningoencephalitis, amebic, 469-470 Meperidine, 87 Mercury poisoning, prenatal, 511 Metabolic headache, 552 Metachromatic leukodystrophy, 45 Metastases

intracranial, 302-304 computed tomography of, 304 histology of, 303 magnetic resonance imaging of, 303, 304 signs and symptoms of, 303 sources of, 302 treatment of, 304

intraspinal, 304-306 ambulation with, 305 signs and symptoms of, 305 sources of, 304 treatment of, 305

Methanol overdose, 90 Methylsergide, in migraine headache prophylaxis,

537 Microatheroma, of penetrating artery, 137 Microcephaly, 29 Midazolam, 87 Middle cranial meningocele, epilepsy and, 371 Migraine headache, 527-537

aura in, 531 biochemistry of, 534 brainstem in, 532 components of, 528 contingent negative variation in, 533 cortical activity in, 533 diagnostic criteria for, 527 pain referral in, 530 periaqueductal gray matter in, 532

562 Atlas of Clinical Neurology

phases of, 530 positron-emission tomography in, 531, 532 prophylactic treatment of, 537 symptoms of, 529 treatment of, 534-537 trigeminovascular system in, 532 triptans in, 534-537 vascular tone in, 531

Miller-Dieker lissencephaly, 7-8 Minamata disease, 511 Mitochondrial diseases, 60-62 Mitochondrial myopathy, encephalopathy, lactic

acidosis, and stroke-like episodes (MELAS), 61, 394

Mitral valve prolapse, cerebral embolism and, 144 Moersch-Woltman syndrome, 487 Mollusc envenomation, 509 Morphine, 87 Morquio syndrome, dysostosis multiplex in, 31 Motor unit, 387 Motor unit potential, 387 Mount-Reback syndrome, 354 Moya-Moya configuration, 299 Mucolipidoses, dysostosis multiplex in, 31-32 Mucor infection, 456 Multifocal motor neuropathy, 490 Multiple sclerosis, 481-484

vs. brain tumors, 282 cerebrospinal fluid abnormalities in, 483 familial, 6 7 optic neuritis in, 481, 482, 483 pathogenesis of, 481 periventricular plaques in, 483 transverse myelitis in, 484 treatment of, 484

Multiple system atrophy, 322-323 Muscle biopsy

in dermatomyositis, 395 in neuromuscular disease, 393-401 normal, 392

Muscle regeneration, 393 Muscular dystrophy

Duchenne,399,474 Duchenne-Becker, 56-57 facioscapulohumeral, 60

Mushroom poisoning, 90, 504 Musician's cramp, 330 Mussel poisoning, 90 Mutism, akinetic, 89, 264 Myasthenia gravis, 491-493

nerve conduction testing in, 492-493 repetitive stimulation studies in, 391 treatment of, 493

Myelin, 399 Myelin sheath, 396, 397

proteins of, 399 Myelin-associated glycoprotein, antibodies

against, 490 Myelitis, transverse, acute, 436 Myelomeningocele, 2 Myocardial infarction, cerebral embolism and,

145, 146 Myoclonic epilepsy with ragged red fibers, 60-61 Myoclonic jerks, 366 Myoclonus, 345-348

classification of, 345

cortical, 34 7 in Creutzfeldt-Jakob disease, 348 drug treatment of, 347 electroencephalography in, 347 etiology of, 346 localization of, 345, 346 somatosensory-evoked potentials in, 347

Myokymia, 354 Myokymic discharges, 388 Myonecrosis, 393 Myotonic discharges, 388 Myotonic dystrophy, 58, 394 Myotonin kinase gene, 58 Myotubular myopathy, 394 Myxoma, atrial, cerebral embolism and, 148-149

• • • • • • • • • • • • • • • • • • • • • • • • Naming, disorders of, 247 Naratriptan, in migraine headache, 534-537 Negri body, 433 Neiman-Pick type C disease, 43 Neisseria meningitides vaccine, 411 Nemaline rod myopathy, 59 Nematode infection, 470 Nerve conduction studies, in neuromuscular dis­

ease, 389-391,492-493 Neurenteric cyst, 15, 16-17

split notochord theory of, 16-17 Neurilemmoma, 292-293 Neurinoma, relative frequency of, 271 Neurites, dystrophic, in Alzheimer's disease, 213 Neuroacanthocytosis, 334-335 N eurocysticercosis, 4 72-4 73 Neurocytoma, central, 291 Neurofibrillary tangles

in Alzheimer's disease, 213, 216 in progressive supranuclear palsy, 232, 321

Neurofibromatosis 1, 22-23, 62, 293 Neurofibromatosis 2, 63, 292-293

headache in, 546 Neurofibromin, 22, 62 Neuroleptics

dosage ranges for, 87 neurologic effects of, 86 neurophysiologic effects of, 87 pharmacokinetics of, 87 tardive syndromes with, 351

Neuroleptic malignant syndrome, 349 Neuroma, acoustic, 63 Neuromuscular disease

congenital, 394 diagnostic studies in, 386-392 electromyography in, 387-388 muscle biopsy in, 392-401 nerve conduction studies in, 389-391 neurogenic changes in, 396-401 repetitive stimulation studies in, 391-392

Neuromuscular junction disorders, 491-494 Neuromyotonia, 354

acquired, 490 Neuropathy, hereditary, 54-56 Neuropil threads, in Alzheimer's disease, 213 Neuropsychologic testing, in dementia, 208

Neurosyphilis, 152, 456-461 angiography in, 458 cerebrospinal fluid in, 460 classification of, 457 forms of, 459 palsies in, 457 pathology of, 458 serologic tests for, 460 tabetic, 458-459 time course of, 456 treatment of, 461

Neurotoxins, 90 alcohol, 512 arachnid, 508 arsenic, 90,519 botulinum, 518 carbon monoxide, 90 cassava, 505 coelenterate, 507 cyad seed, 515 dinoflagellate, 506 distal axonopathy with, 521-522 drug-derived, 517-518 experimental animal exposures to, 520-523 fetal exposure to, 511 fish, 510 frog, 510 fungal, 514 grass pea, 505 insect, 507 manganese, 513 mercury, 511 mollusc, 509 mushroom, 90, 504 mussel, 90 myelinopathy with, 520 plant, 90 proximal axonopathy with, 522-523 rapeseed oil, 515-516 scorpion, 508 sensory neuronopathy with, 521 signs of, 519 snake, 510 stingray, 509 subcellular targets of, 504 tick, 508 toluene, 513 vacuolar demyelination with, 523

Nevi blue, 66 in Sturge-Weber syndrome, 27

Newborn, posthemorrhagic hydrocephalus of, 17-18

NF1 gene, 22, 62 3-Nitropropionic acid neurotoxin, 514 Nodular heterotopia, epilepsy and, 370-371 Nondeclarative memory, 258 Nonfluent aphasia, progressive, 229-233 Nonunique memory, 259 Nonverbal memory, 258 Normal-pressure hydroc~phalus, 325

computed tomography in, 207 NPC1 gene, 43

0 • • • • • • • • • • • • • • • • • • • • • • • • Ocular apraxia, 251 Ocular ataxia, 354 Oculocephalic reflexes, in progressive supranu­

clear palsy, 321 Odontoid, fracture of, 547 Oligoastrocytoma, magnetic resonance imaging

in,279 0 ligodendroglioma

chemotherapy in, 300 computed tomography in, 277 histology of, 289 magnetic resonance imaging in, 280 seizures and, 373 survival with, 272

Olivopontocerebellar atrophy, 50, 323 Onchocerca volvulus infection, 470 Onion bulb, in Charcot-Marie-Tooth disease,

401 Opioids

dosage ranges for, 87 neurologic effects of, 86 neurophysiologic effects of, 87 pharmacokinetics of, 87

Opsoclonus, paraneoplastic, 485 Optic ataxia, 251 Optic glioma, 7 4 Optic neuritis, in multiple sclerosis, 481, 482,

483 Oxytocin, 70, 72

p • • • • • • • • • • • • • • • • • • • • • • • • Pachygyria, 10 Pallidotomy, in Parkinson's disease, 319 Papilledema

in brain tumors, 275 toxin-induced, 519

Papilloma, choroid plexus, 20, 290 Paraneoplastic syndromes, 306-307, 485-487

algorithm for, 486 Parasitic infection, 463-473. See also specific injec­

tions Parkin gene, 48 Parkinsonism, 314-320

characteristics of, 314 classification of, 314-315 dementia and, 233-234, 325 drug-induced, 349

Parkinsonism-dementia syndromes, 325 Parkinson's disease, 513

clinical manifestations of, 315 dementia in, 233-234 vs. essential tremor, 343 familial, 48 handwriting sample in, 316

Lewy bodies in, 316 motor symptoms of, 315 pathophysiology of, 317 pedigree for, 48 positron-emission tomography in, 322 pull test in, 316

treatment of, 318-320, 344 tremor of, 343, 344

Paroxysmal dyskinesias, 354 Patent foramen ovale, cerebral embolism and,

144 Pelizaeus-Merzbacher disease, 46 Pentobarbital, 87 Periaqueductal gray matter, in migraine

headache, 532 Peripheral myelin protein 22, 399 Periventricular plaques, in multiple sclerosis, 483 Perphenazine, extrapyramidal dysfunction with,

517 Persistent vegetative state, 89 Personality disorder, acquired, 265 Pesticide overdose, 90 Phakoma, 24 Phakomatoses, 24 Phenobarbital, 87 Phenothiazine, tardive syndromes with, 350 Phenylbutazone, neuropathy with, 517 Phenytoin overdose, 90 Phyllobates spp. neurotoxin, 510 Pianist's cramp, 330 Pick bodies, 229 Pick cells, 229 Pick's disease, 229 Pilocybe mexicana, 504 Pineal gland, tumor of, 291 Pituitary gland, tumor of, 70, 73-83 Plant poisoning, 90 Plaques, in Alzheimer's disease, 213, 216 Plasminogen activator, tissue-type, in cerebrovas-

cular disease, 169, 170 Plumbism, 519 Poison-dart frog, 510 Poisoning. See Neurotoxins Poliomyelitis, 435-436 differential diagnosis of, 436 Polyarteritis nodosa, 155 Polymicrogyria, 10 Polymyositis, 496

clinical features of, 496 diagnosis of, 495 histologic changes in, 497 treatment of, 498

Polyradiculitis, cytomegalovirus, 437 Porcupine fish neurotoxin, 510 Porencephaly, 11 Positron-emission tomography

in Alzheimer's disease, 210, 219 in cerebrovascular disease, 128, 173 in Creutzfeldt-Jakob disease, 210 in dementia, 210 in frontotemporal dementia, 228 in Gerstmann-S traussler-Scheinker disease,

236 in Huntington's disease, 338 in migraine headache, 531, 532 in multiple system atrophy, 323 in Parkinson's disease, 322 in primary progressive aphasia, 231 in progressive supranuclear palsy, 322 in radiation necrosis, 281

Posterior cortical atrophy syndrome, 220. See also Alzheimer's disease

Index 563

Posthemorrhagic hydrocephalus of newborn, 17-18

Postinfectious encephalitis, 434-435 Potassium channel disorders, 59 Prader-Willi syndrome, 13 Presenilin 1, 39 Primary progressive aphasia, 231 Prion diseases, 41, 235-238, 446-450 Progressive multifocalleukoencephalopathy, 445 Progressive nonfluent aphasia, 229-233

diagnostic criteria for, 230 Progressive supranuclear palsy, 232, 320-322 Prolactin, 70 Prolactinoma, 70, 78, 79 Promazine, extrapyramidal dysfunction with, 516 Propofol, 87

neurologic effects of, 86 Propranolol, in migraine headache prophylaxis,

537 Prosopagnosia, 244 Protein C deficiency, ischemic infarction in, 163 Protein zero, 399 Pseudallescheria boydii infection, 456 Pseudo-Hurler polydystrophy, dysostosis multi-

plex in, 31 Pseudonitzcshia americana neurotoxin, 506 Pseudotumor cerebri, 544 Psychic gaze paralysis, 251 Psychogenic movement disorders, 353-354 Puffer fish neurotoxin, 510 Puff-of-smoke configuration, 299 Pull test, 316 Pure sensory stroke syndrome, in lacunar infarc­

tion, 138 Pyrimidinone, tardive syndromes with, 350

R • • • • • • • • • • • • • • • • • • • • • • • • Rabies, 432-434

clinical manifestations of, 432 diagnosis of, 433 Negri body in, 433 postexposure prophylaxis for, 433, 434

Radiation necrosis, 297-298 magnetic resonance imaging in, 283, 298 positron-emission tomography in, 281

Radiation therapy in glioma, 297-299 meningioma after, 299 stroke after, 299

Ragged red fibers, 394 Ramsey sedation scale, 86 Rapeseed oil neurotoxin, 515-516 Rash, meningococcal, 404 Rasmussen's encephalitis, 374, 487 Repetitive stimulation studies, in neuromuscular

disease, 391-392 Reteplase, in cerebrovascular disease, 169., 170 Retina

paraneoplastic degeneration of, 485 in Von Hippel-Lindau disease, 66

Retinocephalic vascular malformation, 26 Retrograde memory, 258 Rey-Ostrrieth complex figure, 208

564 Atlas of Clinical Neurology

Rheumatic valvular disease, embolism and, 146 Rheumatoid arthritis, 155 Riboflavin, in migraine headache prophylaxis,

537 Rizatriptan, in migraine headache, 534-537 Roundworm infection, 470

s • • • • • • • • • • • • • • • • • • • • • • • • S182 gene, 39 Salicylate overdose, 90 Sanfilippo syndrome, dysostosis multiplex in, 31 SBMA gene, 52 SCA genes, 50 Schistosoma infection, 471 Schizencephaly, 11 Schwann cell, 398 Schwannoma, 281, 292-293 Scleroderma, 155 SCNlB gene, 44 Scoliosis, dystonia and, 326 Scorpion envenomation, 508 Scrapie, 446-447 Secobarbital, 87 Sedation, 86-87 Seizures. See also Epilepsy

classification of, 358, 359 frontal lobe, 361 ganglioglioma and, 372 in incontinentia pigmenti, 28 oligodendroglioma and, 373 in Sturge-Weber syndrome, 27 tonic-clonic, 366 in tuberous sclerosis, 25 tumor-related, 3 71-3 73

Self-tolerance, 479 Sella, in pituitary adenoma, 79 Semantic dementia, 231 Sensorimotor stroke, 138 Shagreen patches, in tuberous sclerosis, 64 Sharp waves, positive, 388 Short-lasting unilateral neuralgiform headache

attacks with conjunctival injection and tearing (SUNCT), 541

Short-term memory, 258-259 Sialidosis, dysostosis multiplex in, 31 Sickle cell anemia, ischemic infarction in, 163 Simultanagnosia, 250-251 Sinusitis, 551 Sirenomelia, 3 Sjogren-Larsson syndrome, 65 Sly syndrome, dysostosis multiplex in, 31 Snake bite, 510 Sneddon's disease, 155 Social conduct disorders, 263-265 Sociopathy, 263, 264 SOD gene, 51 Sodium channel disorders, 59 Somatosensory-evoked potentials, in myoclonus,

347 Somatotrophic hormone, 70 Source amnesia, 262 South American rattlesnake bite, 510 Spasm, hemifacial, 352

Spastic paraplegia, hereditary, 53 Spinal cord, abscess of, 418-419 Spinal muscular atrophy, 53 Spinobulbar muscular atrophy of Kennedy, 52 Spirochete infections, 456-463 Split notochord theory, 16-17 Spongiform encephalopathy, 235-238

clinical features of, 23 7 magnetic resonance imaging in, 235, 236 model of, 237

Status epilepticus, 110-115, 380 barbiturate coma for, 114, 115 brain metabolism in, 111 classification of, 110 differential diagnosis of, 111 electroencephalography in, 111, 115 etiology of, 110, 113 outcome of, 115 pathophysiology of, 112 treatment of, 112-115

Status migrainosus, 537 Stiff-man syndrome, 487 Stingray neurotoxin, 509 STM2 gene, 39 Streptococcus pneumoniae vaccine, 411 Stroke, 95-99, 117. See also Cerebral embolism;

Cerebrovascular disease, atherothrom­botic

diagnostic studies for, 98 embolic, 96-97 evolution of, 98-99 hemorrhagic, 117 ischemic

atherothrombotic, 117-136 embolic, 117,139-149 pathophysiology of, 121-122 thrombus propagation in, 120

neurologic examination in, 96 nonembolic, 97 radiation-associated, 299 stable, 95 subtypes of, 95 treatment of, 99

Sturge-Weber syndrome, 27 Subacute sclerosing panencephalitis, 446 Subarachnoid hemorrhage, 102, 103, 181-191,

549 algorithm for, 181, 182, 183 angioplasty in, 187 computed tomography in, 181, 182, 549 giant aneurysm in, 189 grades 1 and 2, 181 grade 3, 182, 188 grades 4 and 5, 183 in herpes simplex virus encephalitis, 430 hydrocephalus with, 184-185 infectious aneurysm in, 189 transcranial Doppler examination in, 186 vs. unruptured aneurysm, 184 vasospasm with, 104, 185-187

Subcortical aphasia, 253, 256 Subcortical band heterotopia, 9 Subdural empyema, intracranial, 419-420 Subdural hematoma, 102 Subependymal giant cell astrocytoma, 288-289 Subependymoma, histology of, 290

Sumatriptan, in migraine headache, 534-537 Supranuclear palsy, progressive, 320-322 Sural nerve biopsy, 397 Sympodia, 3 Syndrome of inappropriate secretion of vaso-

pressin (SIADH), 70, 76 a-Synuclein gene, 48 Syphilis, 456-461. See also Neurosyphilis Syphilitic arteritis, 152 Systemic lupus erythematosus, 155

T • • • • • • • • • • • • • • • • • • • • • • • • Tables dorsalis, 458-459 Tactile agnosia, 244, 249 Takayasu's arteritis, 155, 156-158 Tapeworm infection, 472 Tardive syndromes, drug-induced, 349, 350 Target fibers, 396 Tau gene, 42, 233

in parkinsonism, 48 Tau marker, in Alzheimer's disease, 221 Tauopathy, 232-233 Tay-Sachs disease, 42 Temporal arteritis, 155, 158 Temporal lobe lesions, amnesia and, 260, 261 Tension-type headache, 538-539 Tetanus, 514 Tetrodotoxin, 510 Thalamic hemorrhage, 175 Thalamotomy, in Parkinson's disease, 344 Thalidomide, neuropathy with, 518 Thallium overdose, 90 Thallotoxicosis, 519 Thiamine deficiency, 89 Thienobenzodiazepine, tardive syndromes with,

350 Thiopental, 87 Thioxanthene, tardive syndromes with, 350 Thrombolytic therapy

in cerebrovascular disease, 169-171 in stroke, 99

Thrombosis, venous, 550 Thunderclap headache, 549 Thyroid-stimulating hormone, 70 Thyrotropin-releasing hormone, 70 Thyroxine, 70 Tick neurotoxin, 508 Tics, 339-340

classification of, 339 drugs for, 340 electroencephalography in, 339

Tolosa-Hunt syndrome, 550 Toluene abuse, 513 Tonic-clonic seizures, 366 Top of basilar syndrome, 126 Torticollis, computed tomography in, 329 Toxic headache, 552 Toxic oil syndrome, 515-516 Toxins. See Neurotoxins Toxocara infection, 470 Toxoplasmosis, 464-466, 552 Transcortical aphasia, 253, 256 Transient ischemic attack, 121-122

Transmissible spongiform encephalopathy, 446-447

Transverse myelitis acute, 436 in multiple sclerosis, 484

Trauma. See Head injury Trematode infection, 471 Tremor, 340-344

differential diagnosis of, 341 divalproex sodium-induced, 378 essential, 342, 343 midbrain, 344 Parkinson's, 343, 344 physiologic, 341, 343 psychogenic, 353 types of, 340

Trichinosis, 470 Tricyclic antidepressants, tardive syndromes with,

350 Trifluoperazine, oral-buccal dyskinesia with, 517

Trigeminal neuralgia, 541, 542-543 Trigeminovascular system, in migraine headache,

532 Trihexyphenidyl, oral-buccal dyskinesia with, 517

Triiodothyronine, 70 Triptans, in migraine headache, 534-537

Trisomy 21 (Down syndrome) Alzheimer's disease in, 41 atlanto-axial subluxation in, 30 chromosomal abnormality of, 40 physical features of, 40

Tropical spastic paraparesis, 444 Trousseau's syndrome, 164 Trypanosomiasis, 464, 467-468 TSC1 gene, 25 Tuberculous arteritis, 153 Tuberculous meningitis, 153, 414-416, 551

in AIDS, 415 basilar, 415 clinical staging of, 414 hydrocephalus in, 415 mortality rates for, 416 symptoms and signs of, 414 treatment of, 416

Tuberin, 64 Tuberous sclerosis, 24-25, 63-64

subependymal giant cell astrocytoma in, 288-289

Tumors of brain. See Brain tumors of pituitary gland, 70, 73-83

u • • • • • • • • • • • • • • • • • • • • • • • • Ultrasonography, in cerebrovascular disease, 127,

129, 130, 131, 134 Unique memory, 259 Urbach-Wiethe disease, 265 Uremia, 89

v • • • • • • • • • • • • • • • • • • • • • • • • Vaccine, against bacterial meningitis, 411

Valproate sodium, in migraine headache prophy-laxis, 537

Vascular dementia, 221-226 Alzheimer's disease with, 223

computed tomography in, 222, 223, 224 diagnostic criteria for, 221 Hachinski Ischemic Score in, 222 magnetic resonance imaging in, 223 subtypes of, 222

Vascular malformations, 190-193 Vascular tone, in migraine headache, 531 Vasculitic neuropathy, 488 Vasculitis, 150-159, 550

cerebral, 224 cocaine-related, 159 infectious, 150, 151-154 mechanisms of, 151 noninfectious, 150, 155-158 Wallerian degeneration with, 397

Vasopressin, 70, 72-73 Vasospasm, with subarachnoid hemorrhage, 104,

185-187 Venous thrombosis, intracranial, 550 Ventricular septal aneurysm, cerebral embolism

and, 146 Ventriculomegaly, posthemorrhagic, 17-18 Verapamil, in migraine headache prophylaxis, 537

Index 565

Verbal memory, 258 Vertebral artery

atherosclerosis of, 124-125 dissection of, 161-162

Vespula spp. envenomation, 507

Vestibular ataxia, 354 Viral infection, 423-450. See also specific infections

chronic, 439 Viral syndromes, antibody-associated, 487

Visual agnosia, 244, 248 Visual fields, in brain tumor, 275

Visual neglect, 253 Visual object agnosia, 244, 245, 246

Von Hippel-Lindau disease, 66-67

w • • • • • • • • • • • • • • • • • • • • • • • • Walker-Warburg syndrome, 9 Wallerian degeneration, 397

Wegener's granulomatosis, 155

Werdnig-Hoffman disease, 53 Wernicke's aphasia, 253, 254 Williams syndrome, 12-13 Wilson's disease, 47,333,519 Withdrawal-emergence syndrome, 349

Working memory, 259 Wyburn-Mason syndrome, 26

X • • • • • • • • • • • • • • • • • • • • • • • • Xanthoastrocytoma, pleomorphic, 288

X-linked adrenoleukodystrophy, 46

X-linked lissencephaly, 9

y • • • • • • • • • • • • • • • • • • • • • • • • Yellow jacket envenomation, 507

z • • • • • • • • • • • • • • • • • • • • • • • • Zolmitriptan, in migraine headache, 534-537