index [assets.cambridge.org]assets.cambridge.org/97805211/14479/index/9780521114479... · 2011. 3....

Index

abetalipoproteinemia 212abnormal spindle-like

microcephaly related(ASPM) protein334, 338

abscessespyogenic intracranial

see intracranial abscesses,pyogenic

tuberculous 512absence epilepsies 92–95complex inheritance 49distinction between

subsyndromes 99of early childhood 95non-epileptic paroxysmal

disorders 95see also childhood absence

epilepsy, juvenileabsence epilepsy

absence seizuresadult-onset idiopathic

generalized epilepsy 96with ataxia 30childhood absence epilepsy

92–93Dravet syndrome 78eyelid myoclonia with 95,

687, 753with generalized spike-

and-wave discharges(GSW) 29–30

juvenile myoclonic epilepsy 96other possible syndromes

with 94–95perioral myoclonus with

95, 753in symptomatic epilepsies 95

absence status (AS) 752–753,754, 755

acanthocytes 212acetylcholine (ACh) receptors

25, 27acquired epilepsies 35, 113early and late seizures 116latent period 116Lennox on 14–15, 17temporal characteristics 116use of term 113–114vs. genetic epilepsies 44see also organic epilepsy

action myoclonus–renal failuresyndrome (AMRF) 136,169–171

action programming-inducedseizures 720–722

acute disseminatedencephalomyelitis(ADEM) 610

acute intermittent porphyria(AIP) 232–233, 234, 235

acute neonatal encephalopathy376

acute on remote symptomaticconvulsive statusepilepticus 732

acute postoperative seizures(APOS) 418

acute symptomatic seizures3, 626–627

bacterial meningitis 483–484,489–490

HIV infection 520metabolic and endocrine

causes 650–651neurocysticercosis 513–514suppurative intracranial

disorders 485tuberculosis 514–515vs. febrile seizures 731see also early seizures,

immediate post-traumatic seizures

acute symptomatic statusepilepticus 731–732, 736

acyclovir 469, 471acyl-CoA oxidase deficiency

219–220, 221adenosine deaminase activity

(ADA) 512–513adenylate kinase 5 antibodies

588–589adrenocorticotropic hormone

(ACTH)infantile spasms 127tuberous sclerosis 180

adrenoleukodystrophyneonatal 216–217, 220, 226X-linked 261

aganglionic megacolon,congenital (Hirschsprungdisease) 307

agenesis of corpus callosum(ACC) 305–309

with abnormal genitalia(Proud syndrome) 121,301, 307

epilepsy and 305–306familial 124peripheral neuropathy with 307related syndromes 306–309schizencephaly and 316

agyria–pachygyria-bandspectrum 298–303

genetics and diagnosis 298–299infantile spasms 124–125pathogenesis 300phenotypes 300–303

Aicardi syndromeagenesis of corpus callosum

305–306, 306–307epilepsy in 123–124, 125, 317polymicrogyria 311, 315, 316

Aicardi–Goutières syndrome(AGS)

with cerebral folate deficiency255

microcephaly 333, 336AIDS see HIV infectionAKT3 gene 309, 332, 335, 337albendazole 498–499, 504albumin 395alcohol-induced seizures 675–676alcohol-related status epilepticus

738–739ALDH7A1 (antiquitin) gene

mutations 52, 238,239, 652

allopregnanolone (AP) 637–638Alpers(–Huttenlocher) syndrome

150, 154, 746, 764aluminum 174Alzheimer disease 618–623diagnosis 620Down syndrome 267, 269early onset, withmyoclonus 173epilepsy in 619–620pathophysiology and clinical

features 618–619treatment of epilepsy 620–623

amino acid disorders, inherited216–229

clinical features 217–224

definition and epidemiology216–217

diagnostic tests and therapy224–229

pathogenesis of epilepsy 217a-amino adipic semialdehyde

(AASA) 238, 239a-amino adipic semialdehyde

(AASA) dehydrogenase52, 652

g-aminobutyric acid see GABAd-aminolevulinic acid (ALA)

234–235amitriptyline 666ammonia 246, 247Ammon’s horn sclerosis

see hippocampal sclerosisamoxapine 599, 666AMPA receptors 726–727amphetamines 597, 669amphiphysin-associated limbic

encephalitis 587, 588b-amyloid 618, 619amyloid angiopathy, cerebral

(CAA) 537, 538, 568amyloid precursor protein (APP)

gene 619mutations 173, 618, 619

Andermann syndrome 307anesthesia 407, 669Angelman syndrome (AS)

201–204genetics 53–56, 201, 281interictal EEG 201, 202management 203–204microcephaly 333, 337pathophysiology 203seizure disorder 201–203, 337status epilepticus 203, 204, 749

angiocentric gliomas 442, 446–447angiostrongyliasis 502anoxic status epilepticus see post-

anoxic status epilepticusanti-amphiphysin antibodies

587, 588anti-b2-glycoprotein I (b2GPI)

antibodies 580antibioticsantiepileptic drug interactions

489meningitis prophylaxis 478

771

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antibiotics (cont.)meningitis therapy 477, 488seizures induced by 667–668status epilepticus induced

by 749suppurative intracranial

infections 489anti-cardiolipin antibodies (aCL)

579, 580, 581anticonvulsants see antiepileptic

drugsanti-CRMP-5 antibodies 587, 588antidepressants 594–595, 596,

598–599, 666–667antiepileptic drugs (AEDs)absence status caused by

752–753antibiotic interactions 489antiretroviral drug interactions

525–526channelopathies 32chemotherapeutic drug

interactions 463–464elderly with dementia 620–623HIV infection 525–526movement disorders induced

by 214seizure aggravation after

670–671status epilepticus and 670,

736, 749anti-glutamic acid decarboxylase

(GAD) antibodies 587, 588anti-GM1 antibodies 589anti-Hu antibodies 587, 588anti-Ma-2 antibodies 587, 588, 589antimalarial drugs 494anti-neuronal antibodiesencephalopathies associated

with 585–589epilepsy syndromes without

encephalopathy 589extracellular antigens 587,

588, 589intracellular antigens 587, 589systemic lupus erythematosus

580see also limbic encephalitis

anti-NMDA-receptor antibodies580, 588, 745

anti-NR2-glutamate-receptorantibodies 580

anti-nuclear antibodies (ANA) 579anti-phospholipid antibodies

(aPL) 580, 581anti-phospholipid syndrome

(APS) 567–568, 579,580, 581–582

antipsychotic drugs 599, 667antiquitin (ALDH7A1) gene

mutations 52, 238,239, 652

antiretroviral treatment (ART)antiepileptic drug interactions

525–526

tuberculosis co-infection514–515, 516

anti-ribosomal P antibodies 580anti-thyroid antibodies 590anti-thyroid peroxidase (TPO)

antibodies 589, 590antituberculous drugs 516, 517apolipoproteinE-4 allele (APOE-4)

619apoptosis, microcephaly and 336aquaporin-4 antibodies 610arachnoid cysts (AC) 341–344clinical and imaging features

341–342epidemiology 341epilepsy and 342–344location 341, 342management 344

arenaviruses 472–473ARFGEF2 gene mutations 323,

324, 332, 335, 337arginase deficiency 246–247arginine : glycine

aminotransferase(AGAT) deficiency 259

arginosuccinate lyase (ASL)deficiency 246–247

arginosuccinate synthetase (ASS)deficiency 246–247

aromatic L-aminoaciddecarboxylase deficiency223–224

array comparative genomichybridization (array-CGH) 51, 57

arteriovenous malformations(AVMs) 551–556

clinical features 551, 552diagnostic tests 553, 554epilepsy and 551–553management of epilepsy

553–554, 556pathology 551treatment 554–556

ARX-related disorders 50, 121,299, 301, 307

microcephaly 301, 336, 337arylsulfatase A deficiency 209, 210Asperger syndrome 601ASPM protein 334, 338association studies, genome-wide

48, 64astrocytes 39, 435–436astrocytomas 433–434asymmetric bilateral tonic

seizures, nocturnalfrontal lobe epilepsy 71

atavism 6, 8ataxiadentato-rubro-pallido-luysian

atrophy 139, 140, 141mitochondrial spinocerebellar

ataxia and epilepsy 151progressive myoclonic 174

ataxia telangiectasia 174

atovaquone/proguanil 494ATP7B gene 249, 250ATR gene 334, 336atropine 679attention deficit hyperactivity

disorder (ADHD)602–603

auditory-induced epilepsy704–707

more complex types 704,706–707

startle epilepsy 704–706see also musicogenic epilepsy

auras 367, 416, 689autistic regression 602autistic spectrum disorder (ASD)

600–602autoantibodies 37auto-induced seizures see self-

induced seizuresautointoxication 9, 10–11automatisms 367autosomal dominant cortical

myoclonus and epilepsy(ADCME) 85

autosomal dominant nocturnalfrontal lobe epilepsy(ADNFLE) 32, 70–72

clinical features 70–71diagnostic tests 71–72management 72molecular genetics 70

autosomal dominant temporallobe epilepsy (ADTLE) 707

Averrhoa carambola poisoning749

axonal injury, traumatic 37, 426axonal sprouting, post-traumatic

402

baclofen 610, 669, 753bacterial meningitis (acute

bacterial meningitis; ABM)adults 482–490children 475–478differential diagnosis731–732

epidemiology 475epilepsy after 475–477, 480pathogenesis of epilepsy 477prevention 478status epilepticus 476,731–732

treatment 477–478complications 479definition and epidemiology

482diagnostic tests 486–487epilepsy after 483–485HIV infection 521management 488, 489–490neonatal 475, 476, 478pathology and clinical features

482–483balloon cells 293, 348–349, 350

Baltic myoclonus; Balticmyoclonic epilepsysee Unverricht–Lundborgdisease

band heterotopia 322subcortical see subcortical

band heterotopiaundulating 350, 352

Baraitser–Winter syndrome 302barbiturates 597, 669Bartonella henselae 746Bassen–Kornzweig syndrome

212bathing epilepsy see hot-water

epilepsyBatten disease see neuronal

ceroid lipofuscinosesBayesian theory 64BCG vaccination 517Beck Depression Inventory

(BDI-II) 596benign adult familial myoclonic

epilepsy (BAFME)85–89, 96, 173–174

clinical and EEG features85–88

differential diagnosis 88–89genetic studies 85, 86somatosensory evoked

potentials 87–88benign childhood epilepsy with

centrotemporal spikes(BCECTS) 104, 105–107

associated epilepsy syndromes107, 108, 110

clinical features 105differential diagnosis 129–130EEG features 105etiology 106, 110fragile X syndrome and 273sleep and 644, 646–647,

647–648benign familial neonatal seizures

(BFNS) 67–68, 91,378–379

BCECTS and 107clinical presentation 67–68molecular genetics 28, 29, 67outcome 68treatment 32, 68

benign familial neonatal–infantileseizures (BFNIS) 28–29

benign focal (partial) epilepsiesof childhood 104–110

benign infantile seizures,BCECTS and 107

benign myoclonic epilepsy ofinfancy (BMEI) 91–92, 120

benign neonatal convulsions91, 378

benign rolandic epilepsysee benign childhoodepilepsy withcentrotemporal spikes

benign tonic upward gaze 120

Index

772






benzodiazepines 597, 669hot-water epilepsy 718seizure aggravation 671status epilepticus 724–725,

740–741Berger, Hans 13BH4 defects

see tetrahydrobiopterindefects

Binswanger, O 6bioinformatics 44biotinpathway deficits 260responsive infantile

encephalopathy 173supplements 221, 229, 260,

262, 652biotinidase deficiency 216,

221–222, 227, 229,260, 377

Bloch–Sulzberger syndrome 196blood–brain barrier, breakdown

37, 395, 435bottom of the sulcus focal

cortical dysplasia (BOSD)294, 295

BR serine/threonine kinase 2antibodies 588–589

brain abscesschildren 478–479, 480diagnostic tests 487–488epidemiology 482epilepsy after 485–486management 489pathology and clinical features

483brain circuitry, alterations in

38–39brain damageprimary mechanisms 394–395secondarymechanisms 394–395see also traumatic brain injury

brain-derived neurotropic factor(BDNF) 243, 637

brain tumorsadjuvant therapy 410–411antiepileptic drug therapy 410de novo postoperative

epilepsy 408presenting with epilepsy

434, 441secondary see metastatic brain

diseasesee also specific types

breast cancer 587brefeldin-inhibited GEF2 (BIG2)

323, 335bupropion 599, 666, 667Burton, Robert 6

cachexia 3CACNA1A gene 30CACNA1G gene 29–30CACNA1H gene 30CACNB4 gene 30, 308

CADASIL (cerebral autosomaldominant arteriopathywith subcortical infarctsand leukoencephalopathy)566–567

calcification, microcephaly withintracranial 333

calciumbalance, disturbances 656,

659–661intracellular 37, 395, 401

calcium channels see voltage-gated Ca2þ channels

calculation-triggered seizures720, 721

Canavan disease 216cancer see malignant diseasecannabis (marijuana) 597, 598CAP see cyclic alternating

patterncarbamazepineHIV infection 526seizure aggravation 670–671,

752–753carbamyl-phosphate synthetase

deficiency 1 (CPS 1)246–247

carbon monoxide poisoning676–677

cardiac arrest, status epilepticusafter see postanoxic statusepilepticus

cardiac rhabdomyomas 178, 179carmustine wafers 437case control studies 115, 116CASK gene 332, 335, 336, 337catabolic states 651catamenial epilepsy 635–641, 651hormonal treatment 638–641pathophysiology 636–638patterns 635, 636, 637

cataplexy, gelastic 208cat-scratch disease 746causal mechanisms of epilepsyJackson’s approach 4, 17vs. causes of epilepsy 21,

114–115, 767causes of epilepsy see etiology

of epilepsycavernous malformations (CMs)

559–563clinical features 560–561demographic data 559diagnostic tools 561–562genetic aspects 560mechanismsof epilepsy 559–560pathology 559treatment 562–563

CCM1 gene 560CCM2 gene 560CCM3 gene 560CDK5 regulatory subunit

associated protein 2(CDK5RAP2) 334

CDKL5 gene 50, 121–122, 244

celiac disease 590–591diagnosis 591epilepsy in 173, 591secondary folate deficiency 255treatment 592

cell death 36–37central nervous system (CNS)

infections 475convulsive status epilepticus

in children 731–732neonatal seizures 377

central pontine myelinolysissee osmoticdemyelination syndrome

central precocious puberty(CPP) 449

centromere protein J (CENPJ) 334centrotemporal spikes (CTS)

105, 273see also benign childhood

epilepsy withcentrotemporal spikes

cerebellar hemorrhage 376–377,537

cerebral abscess see brain abscesscerebral amyloid angiopathy

(CAA) 537, 538, 568cerebral aneurysms, ruptured

541–542cerebral angiitis, isolated 569–570cerebral autosomal dominant

arteriopathy withsubcortical infarcts andleukoencephalopathy(CADASIL) 566–567

cerebral dysgenesissee malformations ofcortical development

cerebral edema 408hyponatremia 656neurocysticercosis 497

cerebral folate deficiency 255, 256cerebral hemorrhage

see intracerebralhemorrhage, intracranialhemorrhage

cerebral infarction 544, 545cerebral palsy (CP) 382–386with centrotemporal spikes 106classification of subtypes

382–383convulsive status epilepticus

732diagnostic tests 384–386epilepsy in 383–384etiologies 382, 383, 385management 386

cerebral vasoconstrictionsyndrome, reversible568–569

cerebral (sino)venous thrombosis376, 567, 632

cerebritis 483, 487cerebrohepatorenal syndrome

see Zellweger syndrome

cerebromicroangiopathy withcalcifications and cysts(CRMCC) 333

cerebrotendinous xanthomatosis261

cerebrovascular disease (CVD)hemorrhagic 537–540ischemic 544status epilepticus 736

ceruloplasmin 250, 251Chandipura virus 533–534chemotherapy 411, 437, 438antiepileptic drug interactions

463–464cherry-red spot, myoclonus and

epilepsy syndromesee sialidoses, type I

cherry-red spots, macular 164, 165chickenpox 471childhood absence epilepsy

(CAE) 92–93BCECTS and 107childhood occipital epilepsies

and 108, 110complex inheritance 49genetic etiology 99, 100pathophysiologic mechanisms

29–30as separate entity 99treatment 100

childhood occipital epilepsy(COE) of Gastaut 104,108–110

associated epileptic syndromes108, 110

clinical features 109EEG features 109etiology 109–110

childhood occipital epilepsy ofPanayiotopoulos typesee Panayiotopoulossyndrome

childrenbacterial meningitis 475–478benign focal (partial)

epilepsies 104–110focal suppurative intracranial

infections 478–480photoparoxysmal EEG

response (PPR) 687status epilepticus 724, 725,

730–733chlorambucil 669chlorpromazine 667chorea-acanthocytosis (ChAc)

212–213diagnosis 213–214epilepsy in 213management and prognosis 214pathophysiology 213

chorein 213choroidal hemangioma,

Sturge–Weber syndrome190, 192

CHRNA2 gene 70

Index

773






CHRNA4 genedeletion 285mutations 32, 70

CHRNA7 gene 32CHRNB2 gene 32, 70chromosomal abnormalities

47, 50–51periventricular nodular

heterotopia 323chromosome analysis,

high-resolution 57ciprofloxacin 668circadian rhythms 643–644citalopram 599citron kinase 334classification of epilepsy 768etiological 21–23, 114, 768historical schemes 18Reynolds’ scheme 3, 18role of genetics 44–47

ClC-2 channel variants 30–31CLN gene mutations 158–163,

208–209clobazam, hot-water epilepsy 718clomifene 639clomipramine 599, 666, 667clonazepam, reading epilepsy 701clonic status epilepticus 737clozapine 599, 667coagulopathies, stroke 544, 546cobalamin (vitamin B12)

deficiency 252–254inborn errors of metabolism

252, 253, 254metabolism 252, 253supplementation 253–254

cobalamin C/D (cblC/cblD)deficiencies 222, 227,252, 254

cobblestone malformations314–315, 318, 352–353,354, 355

cocaine 524, 597, 598, 669,679–680

cognitive–behavioral therapy(CBT) 596, 598

cognitive declineafter status epilepticus 740see also dementia

COL18A1 gene 311, 312colectomy 9, 10–11collagen vascular diseases

579–583“common disease – common

variant” model 48, 63, 64common gene variants 48,

63, 64comorbidities 18comparative genomic

hybridization (CGH) 57complex inheritance, epilepsies

with 47–49common gene variants 48,

63, 64rare gene variants 48, 63, 64–65

complex partial seizures (CPS)malaria 493mesial temporal lobe

epilepsy 367neurofibromatoses 185, 186, 187

complex partial status epilepticus(CPSE) 752, 754–756

compulsive self-gratificationbehavior 120

congenital disorders ofglycosylation (CDG)syndrome 260–261

congenital heart diseasebrain abscesses 479, 482neonatal seizures 376

congenital infections, neonatalseizures 378

connective tissue diseases579–583

continuous spikes and wavesduring sleep (CSWS orelectrical status epilepticusduring sleep; ESES)

cerebral palsy 384hydrocephalus 613–614polymicrogyria 317unilateral, porencephaly with

614–615vs. Lennox–Gastaut syndrome

129–130contrast media, intravenous

669, 753copper 250, 251coproporphyria, hereditary

(HCP) 232, 234, 235copy-number variations (CNVs)

47, 51corpus callosum, agenesis of

see agenesis of corpuscallosum

cortical dysgenesis,neurofibromatosis 1184, 185

cortical dysplasias see focalcortical dysplasia,malformations of corticaldevelopment

cortical myoclonic tremor withepilepsy, familial 173–174

cortical tremor 759cortical tubers 177–178, 179corticosteroidsbacterial meningitis 477–478,

488Hashimoto encephalopathy

590metastatic brain disease 463multiple sclerosis 610neurocysticercosis 498suppurative intracranial

infections 489systemic lupus erythematosus

583tuberculosis 516–517

Costello syndrome 199

Cotton, Henry 10–11coxsackievirus meningitis 472craniotelencephalic dysplasia 302craniotomy, de novo epilepsy

after 408creatine synthesis or transport

defects 259creatine transporter (CRTR)

deficiency, X-linked 259Creutzfeldt–Jakob disease (CJD)

174, 619–620, 749criminality, Lombroso’s theory

of 6–8CRMP-5-associated limbic

encephalitis 587, 588Crohn disease 590–592cryptococcal meningitis 523cryptogenic epilepsy 19, 22, 44definition 23origin of term 11vs. symptomatic epilepsy

113, 114CTSD (CLN10) gene 161, 162cutaneous stimulation-induced

seizures 697–698, 709cyclic alternating pattern (CAP)

645–648ictal manifestations and

647, 648interictal discharges and

646–648rate 646time 646

cyclin D1 334, 335cyclin D2 334–335, 337cyclophosphamide 583cyclosporine 669Cystatin B (CSTB) gene

135, 136cysticercosis 12, 495, 502control measures 497see also neurocysticercosis

cytogenetic techniques 58cytokines, inflammatory 436cytomegalovirus (CMV)encephalitis 470–471polymicrogyria 357

cytoskeletal regulation defects335

cytotoxic T lymphocytes 574

Dandy, Walter 11, 17D-bifunctional protein deficiency

219–220, 221, 227DCX (XLIS) gene 298–299, 300de novo mutations 53Dean, Geoffrey 231–232degeneracy theories 2, 5–6, 10,

17–18dehydration 652, 653Dejerine, J.J. 6dementia 618–623diagnosis 620Down syndrome 267, 269epilepsy in 619–620

pathophysiology and clinicalfeatures 618–619

treatment of epilepsy 620–623vascular (VaD) 619

dementia with Lewy bodies(DLB or LBD) 174,618, 619

demyelinating diseases, acquired607–610

DEND syndrome 260denervation 37dengue 532–533dentato-rubro-pallido-luysian

atrophy (DRPLA)139–142

case studies 140–141, 142clinical features 139differential diagnosis 88–89epidemiology 139genetics 139–140, 141, 142neurophysiology 140, 141

depression 595–596diagnosis 596link with stress 595prevalence 596treatment 596

determining causessee predisposing causes

developmental conditionsdifficulties in categorizing 40epileptogenic mechanisms 37Lennox on 14–15as symptomatic epilepsies 113

developmental delaysee intellectual disability

developmental guidance factors,loss of 37

Devic disease 610dexamethasone

see corticosteroidsdextromethorphan 217diabetes mellitushyperglycemia 659hypoglycemia 650–651, 658

diabetic ketoacidosis 659dialysis disequilibrium

syndrome 652diathesis, epileptic 1diathetic epilepsy 3diazepam, status epilepticus

724–725, 740–741dicarboxylic aciduria 223Dicer 336DiGeorge syndrome (22q11.2

deletion) 312, 313diphtheria, pertussis, and tetanus

(DPT) vaccine 388–389disintegrative disorder 601domoic acid poisoning 676, 749double cortex syndrome

see subcortical bandheterotopia

doublecortin gene see DCX geneDown syndrome (DS) 265–270,

619

Index

774






clinical features 265–267cognitive function 267epilepsy in 126, 267–269genetics and pathophysiology

269–270senile/late-onset myoclonic

epilepsy 269startle seizures 704treatment and course 270

doxycycline 494Dravet syndrome (severe

myoclonic epilepsyof infancy; SMEI)75, 78–83

brain imaging 82cognitive development 79definition 78diagnostic tests 76differential diagnosis 82epidemiology 78etiology/genetics 28, 79–82genetics 49, 51post-vaccination onset 389seizure types and EEG features

78, 79, 80, 81status epilepticus 78, 732treatment and outcome 82–83

drawing-induced seizures 721drop attacks 128, 129drug abuse 596–598, 669–670HIV infection and 524indirect mechanisms 598treatment 598

drug withdrawal 669drug-induced absence status

752–753drug-induced hyperthermia 631drug-induced seizures 664–671drug-induced status epilepticus

664, 669, 724, 748, 749duloxetine 599Durck granulomata 492dysembryoplastic neuroepithelial

tumors (DNT) 441–444diagnosis 443–444pathology 441–443

ear infections 479, 482early infantile epileptic

encephalopathysyndrome withsuppression burstssee Ohtahara syndrome

early myoclonic epilepsy(EME) 379

early seizures 116bacterial meningitis 476,

483–484epilepsy surgery 418head injury 395–396, 400,

401, 402neurosurgery 407–408, 409non-accidental brain injury

427–428stroke 547

suppurative intracranialdisorders 485

vs. acute symptomatic seizures626, 627

viral encephalitis 473Eastern equine encephalitis 528,

535–536eating epilepsy 627, 684, 710eccentric epilepsy 3Echeverria 6Echinococcus 502eclampsia 566ecstasy 597Ehlers–Danlos syndrome 323, 569elderly patientsabsence status 753, 755dementia 618, 620new-onset epilepsy 619, 620status epilepticus 725stroke 544, 547

electrical status epilepticusduring sleep (ESES)see continuous spikesand waves during sleep

electroconvulsive therapy(ECT) 596

electroencephalography (EEG),history 13–14

electrolyte disturbances655–661

embolization, arteriovenousmalformations 555

EMX2 gene 316, 318en coup de sabre 197encephalitisfever and 632HIV-associated 525Rasmussen see Rasmussen

encephalitistuberculous 512viral see viral encephalitis

endocrine-induced seizures650–653

endophenotyping 48endoscopic arachnoid cyst

marsupialization 344endoscopic resection/

disconnection,hypothalamichamartomas 452

enteroviruses 71, 472, 532enzyme replacement therapy

(ERT), Gaucher disease167, 207

ependymomas 434epidermal nevus syndrome 289epidural abscess 482children 479–480diagnostic tests 487–488epilepsy after 485–486management 489pathology and clinical

features 483epidural (extradural)

hemorrhage 541

epilepsia arithmetices 720epilepsia partialis continua

(EPC) 737, 759–765causes 738, 760, 761clinical features 759–760diagnostic tests 760, 763,

764–765EEG features 739, 760–762HIV infection 525management 742, 765meningioma 456non-ketotic hyperosmolar

hyperglycemia 659pathophysiology 760–762recurrence after surgery

419–420epilepsia verminosa 501epilepsy, as disease or symptom

116epilepsy and mental retardation

limited to females(EFMR) 49

epilepsy surgeryarachnoid cysts 344cerebral palsy 386epilepsy after 413–422antiepileptic drug therapyand 420–422

biological and psychosocialissues 413–414

de novo 417–420dynamics of recurrences414–415

extratemporal epilepsy416–417

temporal lobe epilepsy415–416, 419, 421

focal cortical dysplasia 296hemimegalencephaly 291–292infantile spasms 127mesial temporal lobe

epilepsy 370periventricular nodular

heterotopia 327–328polymicrogyria 318–319postmeningitic epilepsy

489–490post-traumatic epilepsy 397,

404–405schizencephaly 319Sturge–Weber syndrome

191, 193tuberous sclerosis 180

epilepsy syndromes 116epilepsy with myoclonic absences

(EMA) 91, 93–94epilepsy with myoclonic astatic

seizures (EMAS) 91epileptic encephalopathy 733epileptic spasms 119late-onset 126see also infantile spasms

epileptic wanderings 71epileptogenesisconcepts 35, 40, 401

in idiopathic epilepsy 24–32secondary (SE) 434, 450,

552, 560in symptomatic epilepsy

35–40, 116epileptogenic zones (EZ)created by epilepsy surgery

417–419incomplete resection 414, 415,

416–417postoperative disinhibition

of potential 414, 415,416, 417, 419–420

problems in delineating413–414

surgical resection see epilepsysurgery

temporal dynamics 414epitempin (LGI1) mutations 707EPM2A mutations 143–144Epstein–Barr virus (EBV) 470Esquirol, Etienne Dominique 6estradiol/estrogen 651neuroactive properties 637seizure frequency and 635

estradiol/progesterone ratio,serum 635

ether convulsions 12ethnic differences, convulsive

status epilepticus 732, 735etiological classification of

epilepsy 21–23, 114, 768etiology of epilepsyin clinical practice 767vs. causal mechanisms of

epilepsy 21, 114–115, 767eugenics 11, 12–13, 18eukaryotic initiation factor (eIF2B)

gene mutations 173exciting causes 1–2, 4, 5, 10, 625excitotoxic brain damage 36–37,

395, 401extensor spasms 119extracellular space, changes in 39extracorporeal membrane

oxygenation (ECMO) 376extradural hemorrhage 541eye deviation, childhood occipital

epilepsies 107–108, 109eyelid myoclonia with absences

(EMA or EMEA) 95,687, 753

eyestrain 9

facial angiofibromas 178, 179Falret, Jules 6familial adult myoclonic epilepsy

(FAME) 85, 96see also benign adult familial

myoclonic epilepsyfamilial essential myoclonus and

epilepsy (FEME) 85Far Eastern encephalitis 534fatigue 626FBN1 gene 569

Index

775






febrile seizures (FS) 75, 632–633definition 632–633differential diagnosis 633,

731–732Dravet syndrome 78genetic predisposition 633, 731hippocampal sclerosis and

365, 632, 730–731influenza 472malaria 493management 76, 633pathogenesis 631–632plus (FSþ) 75, 76plus (FSþ) with other seizure

types 75prolonged 730–731simple and complex 633vs. acute symptomatic

seizures 731vs. hot-water epilepsy 716vaccine-related 388, 389,

390, 472see also fever

febrile status epilepticus (FSE)633, 730–731

felbamate, Lennox–Gastautsyndrome 130

Féré, Charles 6fetal brainexamination issues 346traumatic injury 346–348, 349

fever 631–634convulsive status epilepticus

632, 731–732epilepsies related to 632–633management 633–634physiology 632see also febrile seizures

filamin A (FLNA) gene 309, 323,324, 325

5p chromosome deletions 323flexor spasms 119fluorescent in situ hybridization

(FISH) 57fluoxetine 595, 599, 666, 667FMR1 gene 272, 273, 274–275focal cortical dysplasia (FCD)

293–296clinical and EEG features 294development 348–350dysembryoplastic

neuroepithelial tumorsand 443

epilepsia partialis continua 764infantile spasms 125–126neuroimaging 294–296pathophysiology 293–294postoperative seizure

recurrence 414, 416–417,419–420

reflex seizures 709focal epilepsiesbenign, of childhood 104–110classification 21, 44complex partial status

epilepticus 754

GEFSþ spectrum 76hippocampal sclerosis 363reflex seizures 684–685,

709–711sleep and 646, 647, 648

focal seizuresDravet syndrome 78focal cortical dysplasia 294

folatecerebral (CNS) deficiency

255, 256congenital (hereditary)

malabsorption 254deficiency 254–256disorders of intracellular

metabolism 255metabolism 254, 255supplementation 254

folate receptor 1 proteindefects 255

folate receptor a (FRa) 254,255, 256

folinic acid supplements 240,256, 262, 652

folinic acid-responsive seizures238, 377, 652

F�lling, Ivar Asbj�rn 12foreign bodies, retained 394,

395, 397fosphenytoin, status epilepticus

724, 7414p syndrome 277–279FOXG1 gene 333, 337fragile X mental retardation

protein (FMRP) 272,273, 274

fragile X syndrome 272–275clinical features and pathology

272–273diagnostic tests 274–275epilepsy in 273–274genetics 272mechanisms of seizures

273, 274seizure management 275

fragile-X-associated tremor/ataxia syndrome(FXTAS) 272

Friedreich ataxia 174frontal lobe epilepsy (FLE)nocturnal see nocturnal frontal

lobe epilepsysleep and 646, 647–648status epilepticus 754, 756

Fukuyama congenital musculardystrophy (FCMD) 302,314–315, 318

GABAepileptogenic mechanisms 38estradiol interactions 637inborn errors of metabolism

222–223mechanisms in status

epilepticus 725–726GABA receptors 25, 26

GABA transaminase deficiency223

GABAA receptors 27Angelman syndrome 203genetic defects 74mutations 30–32progesterone actions 638status epilepticus 725–726

GABRB3 gene 203, 204GABRD gene 74GABRG2 gene 74, 81, 82galactosialidosis 164, 208b-galactosidase deficiency 206, 209galactosphingosine 206Galen 1, 8gamma knife radiosurgery (GK),

hypothalamichamartomas 452–453

gangliocytomas 445–446gangliogliomas 441, 442, 443,

445–446gangliosidoses 206–207GM1 206GM2 173, 206–207

Gastaut, Henri 13–14, 625Gastaut-type childhood occipital

epilepsy see childhoodoccipital epilepsy (COE)of Gastaut

gastrointestinal surgery 9, 10–11Gaucher disease (GD) 164,

165–167, 207diagnostic tests 167epilepsy in 166management 167, 207Norrbottnian type 166types 1–3 165

GBA gene 165, 166, 167GEFSþ see genetic epilepsy with

febrile seizures plusgelastic cataplexy 208gelastic seizures 449–450gene(s), epilepsy 51, 56definitions 62detection methods 62–63see also common gene

variants, rare genevariants, single geneepilepsies

gene–environment interactions 56gene expression, changes

in 37, 38gene polymorphisms 48generalized clonic seizures,

Dravet syndrome 78generalized convulsive status

epilepticus 737generalized epilepsy,

classification 21, 44generalized epilepsy with febrile

seizures plus 74see also genetic epilepsy

with febrile seizures plusgeneralized spike-and-wave

discharges (GSW)29–30, 93

generalized tonic–clonic seizures(TCS)

adult-onset idiopathicgeneralized epilepsy 96

childhood absence epilepsy 93idiopathic generalized

epilepsies with(IGE-TCS) 96, 99

MERRF 149Panayiotopoulos syndrome 108Unverricht–Lundborg

disease 135genetic absence epilepsy rats from

Strasbourg (GAERS) 29genetic counseling 56genetic epilepsies 767classification issues 44–47de novo mutations 53Lennox on 14–15modes of inheritance 47–51as symptomatic epilepsies

113, 114vs. acquired epilepsies 44see also heredity as cause of

epilepsy, idiopathicepilepsy

genetic epilepsy with febrileseizures plus (GEFSþ)74–76

clinical features 75definition 74diagnostic tests 76epidemiology 74epilepsy phenotypes 75–76management 76pathology and physiology

74–75genetic testing 49, 56genetics of epilepsy 58current concepts 18cytogenetic and molecular

diagnostics 58future prospects 56–57genetic changes causing

disease 48genetic vs. acquired

epilepsies 44idiopathic epilepsies 24, 58known and missing

heritability 62–65mechanisms of genetic effects

51–56problems and approaches

to studying 43–44reasons for increased

awareness 44–45role in classification 44–47symptomatic epilepsy 40, 43

genius 6, 8genome-wide association studies

48, 64genomic imprinting 53–56, 201genotypic heterogeneity 43, 47germ cell tumors, intratubal 587giant cell granulomatous angiitis

569–570

Index

776






Gibbs, F.A. 13, 14Gilles de la Tourette syndrome

(GTS) 599–600Glasgow Coma Scale (GCS)

396, 427Glasgow Outcome Scale (GOS)

396, 398glaucoma, Sturge–Weber

syndrome 189, 190, 192glia 39, 435–436gliomas 433, 438angiocentric 442, 446–447clinical features 434diagnostic tests 436epilepsy in 434management 436–438mechanisms of epileptogenesis

435–436NF2 187pathology 433–434

gliosis 395, 409, 560globoid cell leukodystrophy

see Krabbe diseaseglucocerebrosidasedeficiency 166, 207lysosomal targeting 170replacement therapy 167, 207

glucose disturbances 655–661glucose transporter type 1

deficiency syndrome(GLUT1-DS) 24,52–53, 258–259,384, 652

glutamateexcitotoxic damage 36impaired mitochondrial

transport 217, 219, 226mechanisms in status

epilepticus 726–727glutamate dehydrogenase (GDH)

gene mutations 259glutamate formimino transferase

(GFT) deficiency 255glutamate receptor subunit 3

(GluR3) antibodies573–574, 589

glutamate receptors 25glutamic acid decarboxylase

(GAD)-associated limbicencephalitis 587, 588

glutamine 246glutamine synthetase deficiency

217, 219, 226glutaric acidemia type I 217, 652glycerol 478glycine receptors 25, 26antibodies 588–589

GM1 ganglioside antibodies 589GM1-gangliosidosis 206GM2-gangliosidosis 173, 206–207Goldberg–Shprintzen syndrome

312, 318gonadotropin-releasing hormone

(GnRH) analogs 639,640–641

goserelin 640–641Gowers, William 4–5, 9GPR56 gene 312, 314–315, 318, 353grand mal epilepsy (IGE-TCS)

96, 99grand mal on awakening 89, 96group B streptococcus (GBS)meningitis 478vaccine 478

guanidinoacetatemethyltransferase(GAMT) deficiency 259

gunshot wounds 393–394, 395

Haemophilus influenzae type B(HiB) meningitis 475

treatment 477, 478vaccination 475, 478, 488

Hallermann–Streiff syndrome 199Hallervorden–Spatz disease

(neurodegeneration withbrain iron accumulation)172–173, 212

hallucinogens 598hamartin 177, 178HARD þ/�E syndrome 302Hashimoto encephalopathy

589–590, 745HCCS gene 308HCN channels 27inherited defects 29–30

head circumference (OFC)hemimegalencephaly 289inborn errors of metabolism 225microcephaly 330, 333, 337

head injuriesabusive see non-accidental

brain injuryclosed 400–405historical theories 11–12intracranial hemorrhage

540, 541open (OHI) 393–398epilepsy after 395–396management 396–397pathophysiology 394–395pediatric case series 398

see also post-traumaticepilepsy, post-traumaticseizures, traumatic braininjury

hearing-induced epilepsies706–707

heat stroke 631helminths 501–508hemimegalencephaly 289–292diagnosis 290–291epilepsy in 125, 290management 291–292

hemiplegia/hemiparesiscerebral palsy 383, 386hemimegalencephaly 289polymicrogyria with 315, 319porencephaly 614Rasmussen encephalitis 575

startle epilepsy with infantile695–697, 711

Sturge–Weber syndrome190–191, 192, 193

hemispherectomyhemimegalencephaly 291–292Rasmussen encephalitis 575, 577Sturge–Weber syndrome 193

hemispherotomy, Sturge–Webersyndrome 193

hemosiderin depositscavernous malformations 559role in epileptogenesis 462,

552, 559–560surgical removal 562–563

Hendra virus 528, 532Henoch–Schönlein purpura 580hepatitis B vaccination 390hereditary coproporphyria

(HCP) 232, 234, 235hereditary hemorrhagic

telangiectasia (HHT) 551heredity as cause of epilepsycurrent concepts 18early-twentieth-century views

11, 12–13historical concepts 17–18nineteenth-century concepts

1, 3, 5–6, 8, 9–10see also genetic epilepsies

heritability of epilepsy 44, 62–65heroin use 597herpes simplex (HSV) encephalitis

467, 468–470, 528diagnostic tests 468–469HIV infection 521treatment 469

herpesvirus infections 468–471heteroplasmy 149, 152heterotopia, neuronal 322see also periventricular

nodular heterotopia,subcortical bandheterotopia

hexosaminidase A and Bdeficiencies 206–207

HHH (hyperammonemia,hyperornithemia,homocitrullinemia)syndrome 247

hidden epilepsy 8higher-level processing, reflex

epilepsy with 720–722hippocampal sclerosis (HS;

mesial temporal sclerosis)363–370

after bacterial meningitis477, 489–490

diagnostic tests 367–370Dravet syndrome 82epidemiology 363epilepsy and 367febrile seizures and 365, 632,

730–731GEFSþ spectrum 76

gliomas associated with 438historical aspects 13–14management 370neurofibromatosis 185pathogenesis 363–367periventricular nodular

heterotopia 322–323porencephaly 615–616progression 366–367risk factors 367surgical resection 370, 414,

415–416, 418–419hippocampusdomoic-acid-induced

damage 676sex hormone actions 637, 638

Hippocrates 1Hirschsprung disease 307historical concepts of epilepsy

causation 1–191860–1907 1–101907–1960 10–16

HIV encephalopathy 524–525HIV infection 520–526antiepileptic drug therapy

525–526EEG features 524etiology of seizures 521–524frequency of seizures 520, 521magnitude and mechanisms of

seizures 524–525seizure types 520–521status epilepticus 746–749tuberculosis co-infection 511,

514–515, 516, 517, 523holocarboxylase synthetase

(HCS) deficiency 216,221–222, 227, 260

holocytochrome c-type synthase(HCCS) 308

homocarnosinosis 223homocysteine 253homocystinuria 228, 229, 254homoplasmy 152hormonal treatment, catamenial

epilepsy 638–641hormone replacement therapy

(HRT) 651hot-water epilepsy (HWE) 626,

627, 713–718clinical features 713–714EEG features 714functional imaging 714–715genetics 715–717history 713management 717–718pathogenesis 714prevalence 713

Hu/ANNA-1-associated limbicencephalitis 587, 588

human herpesvirus 6 (HHV-6)470, 528, 533, 633

human immunodeficiency virusinfection see HIVinfection

Index

777






Human Variome Project 56–57Hunter syndrome (MPS II)

206, 210Huntington-disease 618Huntington-disease-like-2

(HDL2) 212hydatid disease 502hydrocephalus 612–614epilepsy and 612–614neurocysticercosis 495–496

4-hydroxybutyric aciduriasee succinic semialdehydedehydrogenase deficiency

D-2-hydroxyglutaric aciduria216, 222, 227

L-2-hydroxyglutaric aciduria 222,228

hyperammonemia 246, 247,248, 259

hypercalcemia 660–661hyperglycemia 659hyperglycinemia, non-ketotic

see non-ketotichyperglycinemia

hyperinsulinemia/hyperammonemiasyndrome (HI/HA)259–260

hyperkalemia 661hypermagnesemia 661hypermotor seizures, nocturnal

frontal lobe epilepsy 71hypernatremia 657–658hyperphenylalaninemias 216, 223hypertensive encephalopathy

568–569hyperthermiadrug-induced 631mechanisms of seizures

631–632hyperthermic kindling 714, 718hypertonic saline 657hypnagogic jerks see sleep startshypnotics, sedative 597hypobetalipoproteinemia 212hypocalcemia 652, 653, 659–660hypoglycemia 650–651, 658–659diagnosis 652, 658hyperinsulinism/

hyperammonemiasyndrome 259

neonatal 377hypokalemia 661hypomagnesemia 661hypomelanosis of Ito 196,

289, 378hyponatremia 652, 655–657hypothalamic hamartoma (HH)

449–453epilepsy in 449–450management 451–453pathology and symptoms 449

hypothalamuscircadian oscillator 643thermoregulatory function 631

hypothermia, induced, statusepilepticus 634, 742

hypothyroidism 651hypoxic–ischemic encephalopathy

(HIE) 376hypsarrhythmia 119, 120, 127

idiopathic epilepsychanged use of term 113channelopathies 27–32classification 22, 44, 627definition 23epileptogenesis 24–32genetics 24, 58heritability 62–65historical concepts 3, 4–5,

11, 12, 17vs. symptomatic epilepsy

40, 44, 113, 114idiopathic focal (partial)

epilepsies of childhood104–110

idiopathic generalized epilepsies(IGE) 91–101

absence status 752–753adult-onset 96antiepileptic drugs aggravating

670–671brain architectural changes 100epidemiology 91with generalized spikes and

waves 29–30with generalized tonic–clonic

seizures (IGE-TCS)96, 99

genetic etiology 30–31, 32,48–49, 99, 100

myoclonic status epilepticus737, 738

overlaps between subsyndromes99

with phantom absences 753phenotypes/subsyndromes

91, 92photosensitivity 690, 691provoked seizures 627reflex seizures 684, 720–721sex-related effects 99–100sleep and 645, 646, 647–648treatment 100

idiopathic localization-relatedepilepsy, age-related 700

imipenem-cilastatin 668imipramine 666immediate post-traumatic

seizures 395, 400, 427immune processes, hippocampal

sclerosis 365immune reconstitution

inflammatory syndrome(IRIS) 514–515, 517

immunization 388–390see also vaccination

immunological nervous systemdiseases 585–592

immunologically mediatedcauses of statusepilepticus 745, 746

imprinting, genomic 53–56, 201inborn errors of metabolism

(IEM) 12, 52–53, 258–262,650, 651

classification 258, 261diagnostic approach 261–262infantile spasms 126metabolic treatment 262neonatal seizures 377see also specific disorders

incontinentia pigmenti (IP) 196,197, 378

infantile spasms (IS) 119–127clinical and EEG features

119–120clinical presentation vs.

etiology 126cryptogenic 122differential diagnosis 120, 129Down syndrome 126, 267–269etiology 50, 121–126hemimegalencephaly 125, 290idiopathic 123microcephaly 337neurofibromatosis 123, 184, 185prognostic factors 126–127symptomatic 122treatment 127tuberous sclerosis 123, 178, 180X-linked 121, 307

infectionsuncommon, status epilepticus

746–749see also central nervous system

(CNS) infections, specificinfections

infectious mononucleosis 470inflammation 37, 365inflammatory bowel disease

(IBD) 590–592inflammatory nervous system

diseases 585–592inflicted traumatic brain injury

see non-accidental braininjury

influenza encephalitis 472inheritance, modes of 47–51inhibitory constraints, loss of 38inhibitory interneurons, selective

loss of 36, 38, 334–335INI1 gene 187insulinoma 658insulinoma-associated 1 protein

(Insm1) 334intellectual disability (mental

retardation)arachnoid cysts 342autism with 601cerebral palsy 384Down syndrome 267with epilepsy, genetics 50,

51, 53

fragile X syndrome 272hemimegalencephaly 289infantile spasms and 119Lennox–Gastaut syndrome 127Lennox’s views on causes

15, 17microcephaly 330neurofibromatosis 184, 186pyridoxine-dependent

epilepsy 237–238,238–239, 240

ring chromosome 20syndrome 287

Sturge–Weber syndrome 191tuberous sclerosis 178X-linked 121

interferon-a 669interferon-b 610interferon-g-releasing assays

(IGRAs) 513interictal epileptiform discharges

(IEDs)cyclic alternating pattern

and 646–648effects of sleep 644

intermediate petit malepilepsy 95

intermittent photic stimulation(IPS) 687, 690–693

intracerebral hemorrhage (ICH)537–540

diagnostic tests 538–539epilepsy and 537–538fever 632management 539–540

intracranial abscesses, pyogenicchildren 478–480diagnostic tests 487–488epidemiology 482epilepsy after 407, 485–486management 489pathology and clinical

features 483intracranial hemorrhage 537–542arteriovenous malformations

551, 552cavernous malformations 560cerebral amyloid angiopathy

568metastatic brain lesions 462neonatal seizures 376–377see also extradural

hemorrhage,intracerebralhemorrhage, subduralhematoma

intracranial pressure, open headinjuries 394–395

intractable childhood epilepsywith generalized tonic–clonic seizures (ICEGTC)81, 82

intrauterine growth retardation,microcephaly with (MIC-IUGR) 332, 334

Index

778






intraventricular hemorrhage,neonatal seizures 376–377

intrinsic factor (IF) 252inverted duplicated chromosome

15 (inv dup[15]) 281–283ion channelopathies 24, 27–32anticonvulsant therapy 32genetics 45

ion channels 24–25ligand-gated 25, 26, 27subunit composition and

distribution 25–27voltage-gated 25, 26, 27

iron, epileptogenic effects 395,462, 559–560

ischemic brain damageintrauterine 346–348, 357,

358, 359, 360neonatal and perinatal 376

isodicentric chromosome 15 (idic[15]) 281–283

isolated cerebral angiitis 569–570isoniazid 517, 668isovaleric acidemia 216, 218ivermectin 505

Jackson, John Hughlings 3–4,6, 8–9, 17

Japanese encephalitis virus528–529

Jasper, H.H. 13, 14jaw jerks, reading epilepsy 700, 721juvenile absence epilepsy (JAE) 93genetic etiology 49, 99, 100as separate entity 99treatment 100

juvenile myoclonic epilepsy(JME) 95–96

antiepileptic drug-inducedaggravation 670

differential diagnosis 89, 144genetic etiology 49, 99, 100reading epilepsy and 701reflex seizures 684, 720–721as separate entity 99sleep and 646treatment 100, 721

juvenile neuroaxonaldystrophy 172

kainic acid 676karyotypingmolecular 57standard 57

Kawasaki disease 580Kayser–Fleischer rings 249, 250KCC3 gene 307KCNQ2 genedeletion 285mutations 29, 67, 68, 378

KCNQ3 gene 29, 67, 378ketamine 597–598ketogenic dietcerebral palsy 386GLUT-1 deficiency 259, 652

mitochondrial cytopathies153–154

tuberous sclerosis 180KIAA1279 gene 311, 312, 318kindling 552, 560hyperthermic 714, 718

kinesiogenic paroxysmal choreo-athetosis, familial 698

kinky hair disease see Menkesdisease

Kir6.2 gene 260Klippel–Trénaunay–Weber

syndrome 289Knobloch syndrome (COL18A1

gene defects) 311, 312Kozhevnikov epilepsy

(Kojewnikov syndrome)534, 759

Krabbe disease 206, 209Krit1 protein 560

LaCrosse encephalitis 534–535Lafora bodies 143, 144Lafora disease 143–145clinical features 143diagnosis 143, 145diagnostic tests 144differential diagnosis 137,

140, 144epidemiology 143etiology 143–144features of epilepsy 144management 144–145prognosis 145

laforin 143, 144Lambert–Eaton myasthenic

syndrome 589lamotrigineLennox–Gastaut syndrome 130neuronal ceroid lipofuscinoses

162seizure aggravation 670,

671, 753Landau–Kleffner syndrome 602language-related epilepsies

700, 721see also reading epilepsy

large neutral aminoacid (LNAA)hypothesis 217

larva migrans 502, 504Lassa virus 472, 473late seizures 116, 626bacterial meningitis 476,

484–485head injury 396, 400, 401, 402neurosurgery 408, 409non-accidental brain injury 428post-stroke 547suppurative intracranial

disorders 485–486viral encephalitis 473

latent period, symptomaticepilepsy 40, 116

late-onset epilepsy, strokeand 547

lead poisoning 677–679Legius syndrome 183Leigh syndrome 764Lennox, William 13, 14–16,

17, 44Lennox–Gastaut syndrome

(LGS) 127–130clinical and EEG features 128differential diagnosis 120,

129–130Down syndrome 269etiology 121, 130status epilepticus 128, 732treatment 130

leptomeningeal angiomatosis189, 190, 191, 192

EEG features 191pathogenesis 192Sturge–Weber syndrome

subtypes 189surgical resection 191, 193

leptomeningeal cystssee arachnoid cysts

lesional cerebral disease 11–12LETM1 gene 277leukoencephalopathy with

vanishingwhitematter 173levetiracetamseizure aggravation 670,

671, 753status epilepticus 724, 741

Lewy body dementia (LBD orDLB) 174, 618, 619

LGI1 gene 707ligand-gated ion channels 25,

26, 27limbic encephalitis (LE) 585–589clinical features 585–586epilepsy in 586extracellular-antigen-

associated 587, 588, 589intracellular-antigen-

associated 587, 589investigations 586–587other antibodies 588–589status epilepticus 745treatment 589voltage-gatedpotassiumchannel

antibody-associated 574,587, 588, 589

LIMP2 mutations see SCARB2/LIMP2 mutations

linear scleroderma 197LIS1 gene 298–299, 300lissencephaly (LIS) 298–303

and bone dysplasia 302with cerebellar hypoplasia

(RELN-related) 37, 299,301–303

with cleft palate and cerebellarhypoplasia 302

phenotypes 300–303syndrome, isolated (ILS) 299TUBA1A-related 299,

300–301, 332, 335, 337

type 1 (classical) 37, 298–299,300, 350, 351

type 2 (cobblestonemalformations) 314–315,318, 352–353, 354, 355

X-linked, with ambiguousgenitalia (XLAG) 121,124, 299, 301, 307

X-linked (XLIS) 298–299see also agyria–pachygyria-

band spectrumListeria 488lithium 667Lombroso, Cesare 6–8lorazepam, status epilepticus

724–725, 740–741lupus anticoagulant 579, 580lymphangioleiomyomatosis

(LAM), pulmonary178, 179, 180

lymphocytic choriomeningitisvirus (LCMV) 472–473

lymphoma, CNS 524lysergic acid diethylamide

(LSD) 598lysosomal storage disorders

206–210

Ma-2-associated limbicencephalitis 587, 588, 589

MAG12 gene deletions 122magic 1Magnan, Valentine 6magnesium balance,

disturbances 656, 661magnesium sulfate 566, 661malaria 492–494, 732cerebral 492–493clinical features 492–493epilepsy and 493epileptogenic mechanisms 493management of epilepsy

493–494pathogenesis 492, 493prophylaxis in epilepsy 494

Malcavernin protein 560malformations of cortical

development (MCD) 293case study 346–348, 349development 346–358genetics 46, 53infantile spasms 123–126, 127neonatal seizures 377–378reflex seizures 709

malignant diseaseinvestigations 587limbic encephalitis 585, 587

malin 143, 144malonic aciduria 216maple-syrup urine disease 216,

218, 225maprotiline 599, 666Marchiafava–Bignami disease 610MARCKS deficiency 354, 356Marfan’s syndrome 569

Index

779






marijuana 597, 598marsupialization, arachnoid

cyst 344mastoiditis 479, 483masturbationcompulsive 120historical ideas 2, 3

McLeod syndrome 212, 213diagnosis 214epilepsy in 213management and prognosis 214pathophysiology 213

MCPH1–6 genes 330, 334MDMA (methylenedioxy-

methamphetamine;ecstasy) 597

measles, mumps, and rubella(MMR) vaccine 390,471–472

measles encephalitis 471–472MECP2 gene 242–243, 336, 337Mediterranean myoclonus

see Unverricht–Lundborgdisease

medroxyprogesterone, depot639, 640

mefloquine 494melanosis, neurocutaneous 198MELAS see mitochondrial

encephalomyopathy,lactic acidosis, andstroke-like episodes

memory dysfunctionhippocampal sclerosis

369–370limbic encephalitis 585viral encephalitis 469–470

Mendelian disorders see single-gene epilepsies

meningioangiomatosis 187meningiomas 454–458

epilepsy and 454–455management 455, 457NF2-associated 187, 454surgical decision-making

455–458meningitisbacterial see bacterial

meningitiscryptococcal 523neoplastic 460tuberculous 512–513, 514,

515–516meningococcal conjugate vaccine

478, 488meningococcus see Neisseria

meningitidisMenkes disease 206, 210–211menopause 651menstrual cycle 626, 635see also catamenial epilepsy

mental arithmetic, seizurestriggered by 720

mental retardationsee intellectual disability

merlin 186–187MERRF see myoclonus epilepsy

and ragged-red fibersmescaline 598mesial temporal complex partial

status epilepticus (CPSE)752, 754, 755

mesial temporal lobe epilepsy(MTLE) 363

after malaria 494clinical features 367diagnosis 367–370familial 365–366management 370pathogenesis 363–367recurrence after surgery 414,

415–416, 418–419see also temporal lobe epilepsy

mesial temporal sclerosissee hippocampal sclerosis

metabolic changesepileptogenesis 37fever 632

metabolic disorders 650–653absence status 753diagnostic tests 651–652, 653inherited see inborn errors of

metabolismmanagement 652–653neonatal seizures 377

metachromatic leukodystrophy(MLD) 209–210, 261

metastatic brain disease 459–465antiepileptic drug therapy

464–465drug interactions 463–464epileptogenesis 461–462frequency of epilepsy 460genetics 460incidence 459treatment guidelines 462–463

methamphetamine 597a-methylacyl CoA racemase

deficiency 261methylcobalamin 253methyl-CpG-binding protein 2

(MECP2) gene mutations242–243, 336, 337

3-methylcrotonyl-CoAcarboxylase deficiency,isolated 222, 227

methylene tetrahydrofolatereductase (MTHFR)deficiency 217, 229, 255

methylenedioxymethamphet-amine (MDMA;ecstasy) 597

methylmalonic aciduria 216, 218,253, 254

vitamin B12 responsive 222, 227methylphenidate 597, 603metrizamide 753mevalonic aciduria 222, 227micro syndrome see Warburg

micro syndrome

microcephalic osteodysplasticprimordial dwarfism

type I (MOPDI) 332type II (MOPDII) 332, 334

microcephaly (MIC) 330–338with agenesis of corpus

callosum and cerebellarvermis hypoplasia 309,332, 335

Amish type (MCPHA) 336with asymmetric

polymicrogyria 332with calcifications (CALC-

MIC) 333clinical features 330–333congenital 330–332definitions and epidemiology

330diagnostic tests 337–338epilepsy in 336–337genes 331, 333–334inborn errors of metabolism 225with intrauterine growth

retardation (MIC-IUGR)332, 334

management 338with other brain malformation

types 332pathophysiology 333–336with polymicrogyria

(MIC-PMG) 332, 337postnatal 332–333with simplified gyri (MSG)

330–332,334,336–337,338X-linked postnatal, with

mental retardationand pontocerebellarhypoplasia 332

microdeletions 51microophthalmia with linear

skin defects (MLS) 308microtubule-associated proteins

37, 300microtubules 335midazolam, status epilepticus

724–725, 741middle cerebral artery infarction,

intrauterine 346–348,358, 359, 360

miglustat 167, 208migraine 107, 689migraine-like symptoms 109Miller–Dieker syndrome (MDS)

299, 300mirtazepine 599missile wounds 393–395mitochondrial cytopathies147–156causal disease 147–148diagnostic tests 150, 151–152,

155epilepsia partialis continua 764epilepsy in 148–151management 153–154POLG1-related 148, 150–151,

152, 153, 154

status epilepticus 150, 151,152, 154, 745–746

mitochondrial DNA (mtDNA)147, 148, 149

mutations 53, 147–148, 149nuclear gene defects

affecting 150testing 152

mitochondrialencephalomyopathy,lactic acidosis, andstroke-like episodes(MELAS) 148,149–150, 764

diagnostic tests 150, 151differential diagnosis 140management 153–154

mitochondrial glutamatetransport, impaired 217,219, 226

mitochondrial respiratory chain(MRC) 147, 148

dysfunction 147nuclear gene mutations

affecting 148, 150mitochondrial spinocerebellar

ataxia and epilepsy(MSCAE) 150–151

diagnostic tests 150, 151treatment 154

MMR vaccine 390, 471–472molecular diagnostic techniques

58molybdenum cofactor deficiency

218, 225monkeypox virus 534Moreau, Jacques Joseph 6Morel, Bénédict 2, 5, 6morphea 197Morvan syndrome 588mosaic mutations 53motor seizures, childhood

occipital epilepsy ofGastaut 109

motor vehicle accidents 394, 400movement disordersparoxysmal, absence epilepsy

and 95Rasmussen encephalitis 574

movement-evoked seizures 697,698, 711

Mowat–Wilson syndrome307–308

moyamoya syndrome/disease565–566

mTOR inhibitors, pharmacologic180–181

mTOR signaling pathway 177,178–179, 183

mucopolysaccharidoses (MPS) 210multicystic encephalomalacia

(or encephalopathy) 614,615, 616–617

multifactorial causation ofepilepsy 21, 114, 115

Index

780






multifactorial inheritance,epilepsies withsee complex inheritance,epilepsies with

multiple carboxylase deficiency221–222

multiple rare variant, commondisease hypothesis 63,64–65

multiple sclerosis (MS) 607–610clinical patterns 607, 608diagnosis 607–608, 609, 610epilepsy in 608–609treatment 610

multiple sulfatase deficiency 210multiplex ligation probe

amplification (MLPA)57–58

mumps encephalitis 472Munc 18-1 antibodies 589Murray Valley encephalitis 536muscle–eye brain disease 302vs. polymicrogyria 314–315, 318

muscular dystrophy with severecentral nervous systematrophy and absenceof large myelinatedfibers 302

musicogenic epilepsy 706, 711history 9, 626mechanisms 627, 684, 706,

707, 711Mycobacterium tuberculosis 511myoclonic absences 93–94myoclonic–astatic epilepsy

(MAE) 75, 94, 129myoclonic encephalopathy,

early 217myoclonic epilepsybenign, of infancy (BMEI)

91–92, 120benign adult familial

see benign adult familialmyoclonic epilepsy

early (EME) 379juvenile see juvenile myoclonic

epilepsyprogressive see progressive

myoclonus epilepsiessenile/late-onset, Down

syndrome 269severe, of infancy see Dravet

syndromesevere borderline, of infancy

(SMEB) 78, 81, 82myoclonic seizuresbenign adult familial

myoclonic epilepsy86, 88

benign myoclonic epilepsy ofinfancy 91

childhood absence epilepsy 93Dravet syndrome 78, 79,

80, 82juvenile myoclonic epilepsy 95Lafora disease 144

Lennox–Gastaut syndrome128, 130

mitochondrial cytopathies149, 150, 151

myoclonic status epilepticus737–738, 739

myoclonic tremor with epilepsy,familial cortical 173–174

myoclonusaction 169, 171Angelman syndrome 203drug-induced 174essential 89perioral, with absences 95, 753periorofacial reflex 701reading epilepsy 700, 721spinal segmental 759symptomatic causes 174toxic causes 174Unverricht–Lundborg

disease 135visually-evoked 688–689

myoclonus epilepsy with ragged-red fibers (MERRF)148, 149, 764

diagnostic tests 151–152, 155differential diagnosis 137, 140management 153–154

N-acetylaspartate (NAA),hippocampal sclerosis 369

N-acetylcysteine (NAC),Unverricht–Lundborgdisease 136

N-acetylglutamate synthetase(NAGS) deficiency246–247

natalizumab 610National Childhood

Encephalopathy Study(NCES) 388–389

Nazi Germany 13Neisseria meningitidis

(meningococcus)meningitis 475, 478, 482, 488vaccine 478, 488

neonatal encephalopathy, acute376neonatal screening programs

216, 223–224neonatal seizures 373–380acute causes 376–377chronic causes 377–378classification 373–374diagnosis of cause 374–379epilepsy syndromes of early

infantile onset 378–379etiologies 375management 380

neonatal stroke 376neonatesbrain abscess 479meningitis 475, 476, 478

neoplastic meningitis 460nerve agents, liquid 678–679network organization, altered

38–39

Neu–Laxova syndrome 302neural networks, altered

organization 38–39neural progenitor cellsdefects in cytoskeletal

regulation 335impaired signaling and protein

trafficking 335reduced proliferation, growth

and differentiation334–335, 348–350

neuraminidase (NEU1) gene164, 208

neuritogenesis, microcephalyand 336

neuroacanthocytosis 212–214definition and history 212diagnostic tests 213–214epilepsy in 213management and prognosis 214

neuroblasts see neural progenitorcells

neurocutaneous melanosis 198neurocutaneous syndromes

196–199hemimegalencephaly 289neonatal seizures 378

neurocysticercosis (NCC)495–499, 502

diagnostic tests 497–498epilepsy and 496–497, 508extraparenchymal 495–496intraparenchymal 495, 496management 498–499vs. tuberculosis 497–498,

513–514neurodegeneration with brain iron

accumulation (NBIA1;Hallervorden–Spatzdisease) 172–173, 212

neurodegenerative diseases618–623

see also dementianeurofibromatosis 183–1871 (NF1) 183–186, 196diagnosis 186epilepsy in 183–185infantile spasms 123, 184, 185management of epilepsy 186

2 (NF2) 183, 186–187meningiomas 187, 454

neurofibromin 183, 184, 185Neurological Disorders

Depression Inventory forEpilepsy (NDDI-E) 596

neurological taint (neuropathictrait) 1, 5–6, 9–10, 17–18

neuromyelitis optica 610neuronal antibodies see anti-

neuronal antibodiesneuronal ceroid lipofuscinoses

(NCLs) 158–163,208–209, 652

adult 161classic infantile, PPT1 deficiency

(CLN1) 161, 208

classic late infantile, TPP1deficiency (CLN2)158–161, 208–209

congenital, late infantile andearly juvenile variants(CLN10) 161

diagnosis 161–162epilepsy in 158–161genetics 158, 159juvenile (CLN3, or CLN1 and

CLN2) 158, 209management 162–163variant late infantile and early

juvenile (CLN5–8 andothers) 161, 209

neuronal death/injurycerebrovascular disease 544–545HIV-related 525traumatic brain injury 36–37,

401–402neuronal heterotopia 322see also periventricular

nodular heterotopia,subcortical bandheterotopia

neuronal migration defectscytoskeletal regulation

and 335hemimegalencephaly 289, 290intrauterine brain damage 347periventricular nodular

heterotopia 322neuronal migration disorders

350–357infantile spasms 123–125overmigration syndromes

352–357undermigration syndromes

350–352see also agyria–pachygyria-

band spectrum;lissencephaly;polymicrogyria;schizencephaly

neuronsaltered properties 38post-traumatic sprouting 402reversion to immature

state 39neuron-specific enolase

739, 756neuropathic family 6neuropathic trait see neurological

taintneuroserpin inclusion body

disease 173neurosurgery, de novo epilepsy

after 407, 417–418frequency 407–408management 409–411pathophysiology 408–409risk factors 409seizure types 409

neurotuberculosissee tuberculosis

nevus of Ota 199

Index

781






nevus sebaceous syndrome196–197, 378

NF1 gene 183, 185NF2 gene 186–187NHLRC1 gene 143–144nicotine therapy 72nicotinic acetylcholine receptors

(nAChR) 27inherited defects 32, 70

Niemann–Pick disease type C206, 207–208

nimodipine 410Nipah virus 528, 530–532N-methyl-D-aspartate (NMDA)

receptorsantibodies 580, 588, 745status epilepticus 726, 737

Nocardia 488nocturnal frontal lobe epilepsy

(NFLE) 70autosomal dominant

(ADNFLE) 32, 70–72diagnostic tests 71–72NREM sleep 647, 648sporadic 70–71

nocturnal paroxysmal dystonia(NPD) 70

non-accidental brain injury(NABI) 425–430

clinical features andpathological mechanisms425–426, 428

diagnostic tests 426, 427,428, 429–430

seizures and epilepsy after427–428

treatment 430non-cyclic alternating pattern

(NCAP) 645non-ketotic hyperglycinemia

(NKH) 216, 218, 225,260, 652

clinical features 217–218, 219pathogenesis of epilepsy 217

non-ketotic hyperosmolarhyperglycemia(NKHH) 659

noradrenergic and specificserotonergicantidepressants(NaSSA) 599

North London status epilepticusin childhood surveillancestudy (NLSTEPSS) 724,725, 730, 732

Northern epilepsy 161NOTCH3 gene 566NPC1 gene 207NREM (non-rapid eye movement)

sleep 645–648NREM–REM (non-rapid eye

movement – rapid eyemovement) sleep cycle644–645

nutritive changes, nervoussystem 2–3, 4

obtundation status, Dravetsyndrome 78

occipital lobe epilepsiesidiopathic photosensitive 711overlap with migraine 689see also childhood occipital

epilepsy of Gastaut,Panayiotopoulossyndrome

occipital (visual) seizureschildhood occipital epilepsies

108, 109Lafora disease 144mitochondrial spinocerebellar

ataxia and epilepsy 151photosensitive 689

occipitofrontal circumference(OFC) see headcircumference

ocular disordersinborn errors of metabolism 225Sturge–Weber syndrome

189, 190oculocerebrocutaneous

syndrome 198Ohtahara syndrome (early

infantile epilepticencephalopathy withsuppression bursts) 379

chromosomal abnormalities 51complex inheritance 50hemimegalencephaly 290

olanzapine 599, 667oligoastrocytomas 434oligodendrogliomas 434onchocerciasis 502, 504–505, 5081q44 deletion syndrome 309opioid misuse 596–597, 598organic acidurias 216–229clinical features 217–224definition and epidemiology

216–217diagnostic tests and therapy

224–229pathogenesis of epilepsy and

CNS involvement 217organic epilepsyhereditary (Lennox) 14–15historical concepts 5, 9,

11–12, 17see also acquired epilepsies;

symptomatic epilepsyornithine transcarbamylase

(OTC) deficiency 246–247orofacial motor signs BCECTS

105oscillatory activity, subclinical 39Osler–Weber–Rendu

syndrome 551osmotic demyelination

syndrome 656, 657otitis media (ear infections)

479, 482ovarian teratoma 588overmigration 347, 352–357oxcarbazepine 671

oxidative stress 401–402, 462,559–560

palmitoyl protein thioesterase 1(PPT1) deficiency 161,162, 208

Panayiotopoulos syndrome (PS)104, 107–108

associated epilepsy syndromes107, 108

clinical features 107–108EEG features 108etiology 108, 110

pantothenate-kinase-associatedneurodegeneration172–173

paragonimiasis 502, 506–507parahereditary theories 15paraneoplastic syndromes 585,

587, 745see also limbic encephalitis

parasitic diseases 501–508associations with epilepsy

507–508see also malaria;

neurocysticercosis;toxocariasis;toxoplasmosis

parasomnias 70, 72Parkinson disease 618paroxysmal arousals, nocturnal

frontal lobe epilepsy71, 72

paroxysmal movement disorders,absence epilepsy and 95

Parry–Romberg syndrome 197,580–581

partial epilepsies see focalepilepsies

Partington syndrome 121, 307Pascual–Castroviejo type II

syndrome (PCIIS)197–198

paternal uniparental disomy 201pattern-sensitive epilepsy 683,

684, 720, 721PAX6-related polymicrogyria

311, 312PCDH19 gene 49–50, 81, 82PDCD10/TFAR15/CCM3

gene 560PEHO (progressive

encephalopathy withedema, hypsarrhythmias,and optic atrophy)syndrome 122

penicillamine 250, 251penicillins 668pericentrin (PCNT) 334perinatal stroke 376perioral myoclonus with

absences 95, 753periorofacial reflexmyoclonia 701periventricular nodular

heterotopia (PVNH)308–309, 322–328

classification 325–326diagnostic tests 326–327epilepsy in 323–325management 327–328microcephaly with 332pathophysiology 322–323,

324, 352, 353pernicious anemia 252peroxisomal disorders 216–229clinical features 217–224definition and epidemiology

216–217diagnostic tests and therapy

224–229pathogenesis of epilepsy 217

pertussis vaccination 388–389pervasive developmental

disorders (PDD) 600, 601petechial hemorrhages, cerebral

malaria 492petit mal epilepsyimpulsive see juvenile

myoclonic epilepsyintermediate 95true see absence epilepsies

PEX genes 221PHACE (posterior fossa

malformations,hemangiomas, arterialanomalies, cardiacdefects, and eyeabnormalities)association 197–198

phakomatoses 189phencyclidine (PCP) 597–598,

669, 670phenobarbitalHIV infection 526status epilepticus 724–725,

740, 741phenocopies 47phenotypic heterogeneity 43, 47phenylketonuria 12, 126, 216, 228pathogenesis of epilepsy 217untreated 223, 224

phenytoin (PHT)HIV infection 525, 526metastatic brain lesions 465postoperative seizure

prophylaxis 410seizure aggravation 670–671,

752–753status epilepticus 724–725,

740, 741traumatic brain injury 396,

404, 430tuberculosis 516Unverricht–Lundborg

disease 136phosphomannomutase

deficiency 260–261photic stimulation, intermittent

(IPS) 687, 690–692,692–693

photoparoxysmal EEG response(PPR) 687, 688

Index

782






epilepsy types and 690, 691frequency of epilepsy 689–690genetics 689healthy children and aircrew

candidates 687migraine and 689photic-evoked clinical signs

and 690prevalence in epilepsy

patients 687photosensitive epilepsy 626,

689–692clinical features 688–689focal 711mechanisms 627, 683–684treatment and prognosis

627–628, 692see also visually induced

seizuresphotosensitive occipital lobe

epilepsy, idiopathic 711photosensitivity 687–693baboon model 627diagnostic tests 692–693Dravet syndrome 78epidemiology 687–688genetics 49, 627, 628,

683, 689migraine and 689pathophysiology 689visual stimuli provoking

690–692see also photosensitive

epilepsy; visually inducedseizures

physical stigmata 6, 7, 8, 10physiognomy 6, 7, 8pigmentary mosaicism 196pipecolic acid (PA) 238, 239pituitary surgery, de novo

epilepsy after 408Plasmodium falciparum malaria

492–494platelet-activating factor

acetylhydrolase (PAF-AH) regulatory subunit(PAFAH1B1) 300

pleural epilepsy 12pneumococcal conjugate vaccine

(PCV) 475, 478, 488pneumococcal meningitis

see Streptococcuspneumoniae meningitis

poison control centers 675POLG1-related mitochondrial

cytopathies 148, 150–151diagnostic tests 150, 152management 153, 154

polydipsia, psychogenic 655polygyria 318polymicrogyria (PMG) 311–319Aicardi syndrome 311, 315, 316bilateral frontal 313–314bilateral frontoparietal 314–315,

317, 353

bilateral parasagittal parieto-occipital 313

bilateral perisylvian 311,313, 317

cytomegalovirus infection 357development 347, 348, 349,

355–357diagnostic tests 317–318epilepsy in 317generalized 315genetics 311–312, 318management 318–319microcephaly with (MIC-

PMG) 332, 337parieto-occipital cortex 313, 314TBR2-related 311, 312, 332,

334, 337TUBB2B-related 311, 318, 332,

335, 336, 354–355unilateral perisylvian or

hemispheric 315, 317pontine hemorrhage 537porencephaly 614–617

startle seizures 695, 696porphobilinogen (PBG) 234–235porphobilinogen deaminase

(PBGD) deficiency233, 234

porphyria 231–235, 652, 749acute (hepatic) 231–232,

232–234diagnostic tests 234–235epilepsy and 232–234safe and unsafe drugs 234treatment and prognosis 235

port-wine stain (PWS) 189–190,192

possession by evil spirits/thedevil 1

postanoxic status epilepticus736–738, 742

posterior fossa surgery 407posterior leukoencephalopathy

syndrome, reversible568–569

postnatal stress, early 593–594postneonatal epilepsy 374post-stroke epilepsy (PSE)

545, 546post-traumatic epilepsy (PTE)

400, 403extradural hemorrhage 541historical views 11–12management 396–397open head injuries 395–396pathogenesis 36–37, 394–395,

401–402pediatric case series 398risk factors 396, 398, 402subdural hematoma 540

post-traumatic seizures (PTS)400–401

categories 395, 400, 427clinical types 401diagnosis 403–404

early 395–396, 400, 401, 402,427–428

epidemiology 401immediate 395, 400, 427late 396, 400, 401, 402, 428natural history 402–403non-accidental brain injury

427–428prophylaxis 396, 397, 404, 430treatment 404–405

potassium (Kþ)balance, disturbances 656, 661extracellular 36, 401

potassium channels see voltage-gated Kþ channels

Pott’s puffy tumor 483PPT1 deficiency 161, 162, 208P/Q-type calcium channel

defects 30Prader–Willi syndrome (PWS)

56, 201, 281praxis-induced seizures 684,

720–722praziquantel (PZQ) 498–499, 503precipitants, seizure 625, 768precocious puberty, central

(CPP) 449predisposing causes 1–2, 4,

5, 625vs. precipitating causes 625

pre-eclampsia 566pregabalin 670pregnancy 651prenatal stress 593–594presenilin 1/2 mutations

618, 619PRICKLE1 gene 136, 174primary generalized epilepsies

(PGE) see idiopathicgeneralized epilepsies

prion diseases 619–620probabilityposterior 64prior 64

progesteroneneuroactive properties 637–638seizure frequency and 635therapy 638–640

progestogen therapy 638–640progressive epilepsy with mental

retardation (EPMR) 161progressive facial hemiatrophy

197progressive multifocal

leukencephalopathy(PML) 522, 523, 524

progressive myoclonic ataxia 174progressive myoclonus epilepsies

(PMEs) 51–52, 169,172–174

diagnostic tools 52differential diagnosis 136, 137,

140, 174Gaucher disease 165, 166molecular genetics 52

Unverricht–Lundborg type(EPM1) see Unverricht–Lundborg disease

see also specific typesprogressive supranuclear

palsy 618Propionibacterium acnes

487–488propionic aciduria 216, 218propofol 669, 725proprioceptive-induced

seizures 711see also movement-evoked

seizuresprotective protein cathepsin A

(PPCA) deficiency164, 208

Proteus syndrome 199, 289proton-coupled folate

transporter (PCFT)254–255

Proud syndrome 121, 301, 307provoked epilepsy 117, 625–628definition 23, 117etiological classification 22mechanisms 627–628vs. symptomatic epilepsy 113

provoking factors 768see also exciting causes

pseudo-status epilepticus 740pseudo-TORCH 333psilocybin 598psychiatric disorders 593–603,

740psychoactive substance misuse

596–598psychological treatments 595, 598psychosine 206psychotropic drugs 598–599,

666–667, 753pure epilepsiesdefinition 62heritability 62–65

putamenal hemorrhage 537pyrazinamide 517pyridoxal-5’-phosphate (PLP)

238, 262, 652pyridoxine (vitamin B6)

diagnostic testing 239, 262in utero and postnatal

prophylaxis 240isoniazid actions 668therapy 237, 238, 240, 652, 668

pyridox(am)ine 5’-phosphateoxidase (PNPO) 238

pyridoxine-dependent epilepsy(PDE) 52, 237–240, 377

causal disease 237–238diagnosis 239–240, 652epilepsy in 238–239management 240

pyridoxine-responsive seizures(PRS) 239

quetiapine 667

Index

783






RAB3GAP1 gene 311, 312, 332,335, 337

rabies 528, 534Rac1 336radiosurgery, stereotacticarteriovenous malformations

555gliomas 438hypothalamic hamartomas

452–453metastatic brain lesions 463

radiotherapy 410–411gliomas 437, 438metastatic brain lesions 462, 463

rapamycin and analogs 180–181rare gene variants 48, 63, 64–65RAS pathway 183, 186RASA1 mutations 551Rasmussen encephalitis (RE)

573–577, 589clinical features 574diagnostic tests 575–577differential diagnosis 574, 759epilepsia partialis continua

762–764, 765epilepsy in 574–575management 577pathology 573pathophysiology 573–574

reading epilepsy (RE) 626,700–702, 721

clinical features 700diagnostic tests 701–702management 701, 722mechanisms 683, 684, 700–701primary (specific) 700secondary 700specific characteristics 701treatment and prognosis 702

recapitulation, theory of 6reelin 37reflex epilepsy 625–626

complex 626definition 626, 720with higher-level processing

720–722history 8–9, 625–626management 721–722simple 626

reflex seizures 720cerebral palsy 384Down syndrome 269focal 684–685, 709–711mechanisms 627–628,

683–685, 709reflex theories of causation 5, 8–9Refsum disease, infantile

216–217, 220, 226RELN gene 37, 299, 301–303REM (rapid eye movement)

sleep 645remote symptomatic seizures 627bacterial meningitis 483,

484–485see also late seizures

renal angiomyolipomas 178, 179renal failure 169, 171repetitive sleep starts 120respiratory alkalosis, fever-

related 632retigabine 32retinal hemorrhages,

non-accidental braininjury 426

Rett syndrome (RTT) 242–244,333, 601

CDKL5 genotype 244EEG and video-EEG

monitoring 244management of seizures 244MECP2 mutations and

epilepsy 242–243,336, 337

natural history of epilepsy 242pathogenesis of seizures 243prognosis 243–244seizure character 243status epilepticus 749

Rett-like featureswith cerebral folate

deficiency 255microcephaly with 333, 337

reversible cerebralvasoconstrictionsyndrome 568–569

reversible posteriorleukoencephalopathysyndrome 568–569

Reynolds, J. Russell 2–3,8–9, 18

classification of epilepsy 3rhizomelic chondrodysplasia

punctata type I 216–217,220, 221, 226

ribavirin 473ring chromosome 20 (RC20)

syndrome 285–287, 749,753, 754

ring hemorrhages, cerebralmalaria 492

river blindness (onchocerciasis)502, 504–505, 508

RNA viruses 471–472RNASEH2 gene 336road traffic accidents 394, 400rolandic spikes 105roseola infantum 470, 533, 633rufinamide, Lennox–Gastaut

syndrome 130running-down phenomena

415, 450running-up phenomena 415, 417Russian spring–summer

encephalitis 534, 573, 759

S100B protein 436St. Louis encephalitis 535Sakoda complex 308Sandhoff disease 206–207Sandifer syndrome 386

Sanfilippo B syndrome(MPS III) 210

saposin B deficiency 209, 210sarin 678–679SCARB2/LIMP2 gene 136,

170–171SCARB2/LIMP2 protein 170scarringafter epilepsy surgery 418–419post-traumatic 395

Schimmelpenning/nevussebaceous syndrome196–197

schistosomiasis 502, 505–506schizencephaly 312, 313,

315–317clinical features 316–317closed-lip 315–316development 347, 349, 358diagnostic tests 318epilepsy in 317management 319open-lip 315–316

schwannomas 186schwannomatosis 186, 187SCL25A19 gene 330scleroderma 579linear 197, 580–581localized 580–581

SCN1A mutations 28childhood focal epilepsies

and 109–110Dravet syndrome (SME1)

28, 75, 76, 79–81GEFSþ 74genotype-phenotype

correlations 81other early onset epileptic

encephalopathies 82Panayiotopoulos syndrome 108

SCN1B mutations 28, 74SCN2A mutations 28–29Seckel syndrome 332, 334, 336secondary epileptogenesis (SE)

434, 450, 552, 560sedative hypnotics 597seizures, epilepticJackson’s theory 3–4precipitants 625, 768

seizures beget seizures concept 5selective serotonin reuptake

inhibitors (SSRIs)594–595, 599, 666

self-gratification behavior,compulsive 120

self-induced seizures 687,690, 692

hot-water epilepsy 714, 718sensory-induced epilepsy 695–698septo-optic dysplasia,

schizencephaly and 316septum pellicidum, agenesis

of 316serine deficiency, congenital 217,

218–219, 225, 652

serotonin and norepinephrinereuptake inhibitors(SNRI) 599

serotonin transporter (5-HT)T promoter genepolymorphism 595

sertraline 599severe infantile multifocal

epilepsy (SIMFE) 81, 82severe myoclonic epilepsy of

infancy, borderline(SMEB) 78, 81, 82

severe myoclonic epilepsy ofinfancy (SMEI)see Dravet syndrome

severe polymorphic epilepsy ofinfants 82

sex hormones 636–638, 651sexual disturbances 1–2shagreen patches 178, 179shaken baby syndrome see non-

accidental brain injuryshingles 471shunts, cerebrospinal fluiddysfunction 613post-insertion epilepsy 407,

612, 613sialidoses 164–165differential diagnosis 137management 167type I 164, 165, 208type II 164, 165, 208

sialysis syndrome 174Siberian (Russian spring–

summer) encephalitis534, 573, 759

sickle-cell disease 544, 546, 548Sieveking, Edward 1–2single-gene disorders, with

epilepsy as prominentfeature 51–52, 54

single-gene epilepsies 43, 45,49–50, 63

diagnosis 51–53genotypic and phenotypic

complexity 43heritability 63–64see also ion channelopathies

single-nucleotide polymorphism(SNP) arrays 58

sinusitis 479, 482, 483situation-related epileptic

seizures 650SIX3 gene 335–336skull fracturesgrowing 397non-accidental brain injury 426open head injuries 394, 397, 398

SLC2A1 (GLUT-1) gene 24,52–53

SLC25A22 gene 217, 219sleep 643–648homeostatic processes 644mechanisms of regulation 644NREM–REM cycle 644–645

Index

784






propensity, circadian rhythm643

sleep deprivation 644sleep starts 120, 386small-cell lung cancer (SCLC) 587smallpox vaccine 390Sneddon syndrome 567socioeconomic differences,

convulsive statusepilepticus in childhood732

sodium balance, disturbances in655–658

sodium channels see voltage-gated Naþ channels

somatosensory-evoked seizures627, 684, 695–698, 709

sparganosis 501–503, 508spatial-task-induced seizures 684speech dyspraxia, familial

rolandic epilepsywith 106

Spielmeyer–Vogt syndrome(juvenile neuronal ceroidlipofuscinosis) 158, 209

spinal segmental myoclonus 759spinocerebellar degenerations 174Spirometra 501–503, 508Spratling, W. 6SPRED1 gene 183SRPX2 gene 311, 312, 313star fruit poisoning 749startle, non-epileptic 386startle epilepsy 711auditory-evoked seizures

704–706with infantile hemiplegia

695–697, 711startle reaction 695–696startle seizures 695–697, 698

in epileptic syndromes 697localization 627, 695, 697

status epilepticus (SE) 723–727absence 752–753, 754, 755Angelman syndrome 203,

204, 749antiepileptic-drug-induced

670, 749children 724, 725, 730–733classification 723complex partial (CPSE) 752,

754–756convulsive (CSE) 735–743acute bacterial meningitis476, 731–732

children 730–732clinical features 737–738definition 730, 735epidemiology 735–737etiologies 730–732, 735–736fever 632, 731–732incidence 730, 735intracerebral hemorrhage538, 539

management 740–743

pathophysiology 737social/geographic factors738–739

socioeconomic/ethnicdifferences 732

definition 723diagnostic tests 739–740, 749Dravet syndrome 78, 732drug-induced 664, 669, 724,

748, 749electrical, during sleep

see continuous spikesand waves during sleep

epidemiology 723–724etiologies 724, 736adults 735–743children 730–733uncommon 745–750

febrile 633, 730–731genetic disorders 747, 749genetic factors 738hypothermia treatment 634, 742immunologically-mediated

causes 745, 746as initial manifestation of

epilepsy 738Lennox–Gastaut syndrome

128, 732mechanistic causes 725–727mitochondrial cytopathies

150, 151, 152, 154,745–746

non-accidental brain injury427, 430

non-convulsive (NCSE) 723,752–756

children 732–733etiologies 733, 752–756intracerebral hemorrhage 538raised body temperature 632

outcome/mortality 725, 730,736–737, 740

Panayiotopoulos syndrome 108in previously diagnosed

epilepsy 738refractory 634, 725, 741toxin-induced 748, 749treatment 724–725, 750tuberculosis 514uncommon infectious causes

746–749stereotactic radiosurgery

see radiosurgery,stereotactic

sterilization, enforced 13steroid hormones, reproductive

636–638, 651steroid responsive

encephalopathyassociated withautoimmune thyroiditis(SREAT) 589–590

steroid therapysee corticosteroids

stiff person syndrome 587

stimulant drugs 597, 598, 631,632, 669

Streptococcus pneumoniae(pneumococcal)meningitis 475, 482

epilepsy risk 484treatment 478, 488vaccine 475, 478, 488

stress 593–595in adult life (in humans) 594evaluation 594link with depression 595prenatal/early postnatal

(in rats) 593–594as seizure precipitant

593, 594treatment 594–595

strokeantiphospholipid syndrome 567clinical features 545definition 544diagnostic tests 547epilepsy risk factors 545–547hemorrhagic 537–540, 565ischemic 544, 545, 565management of epilepsy 548neonatal and perinatal 376older adults 544, 547pathophysiology 544, 545pediatric 544, 545, 548status epilepticus 736vascular dementia 619

stroke-like episodesMELAS 149–150Sturge–Weber syndrome

189, 192strongyloidiasis 502structural causes of epilepsy

767–768structural–metabolic epilepsies

44–47Sturge–Weber syndrome (SWS)

189–193, 196clinical features 189–191,

193, 378CNS abnormalities 190,

191, 192epilepsy in 190–191etiology 191–192subtypes 189treatment 192–193

subacute sclerosingpanencephalitis (SSPE)471, 472

subarachnoid hemorrhage (SAH)541–542

de novo epilepsy aftertreatment 408

fever 632neonatal seizures 376–377non-accidental brain injury 426

subclinical electrical activity 39subcortical band heterotopia

(SBH) (double cortexsyndrome) 298

clinical features 300genetic basis and diagnosis

298–299pathogenesis 300, 350, 352undulating band heterotopia

350subcortical vascular

encephalopathy(SVE) 548

subdural empyema 482children 479, 480diagnostic tests 487–488epilepsy after 485–486management 489pathology and clinical

features 483subdural hematoma (SDH)

540, 541diagnostic tests 540epilepsy and 540neonatal seizures 376–377non-accidental brain injury

426, 429, 430seizures after surgery for

407–408subependymal giant cell tumors

(SGCT) 179subependymal nodules 178, 179subgaleal abscess 483substance misuse, psychoactive

596–598, 669–670substrate reduction therapy (SRT)Gaucher disease 167Niemann–Pick disease

type C 208succinic semialdehyde

dehydrogenase (SSADH)deficiency 216, 223,228, 259

sugar disturbances 655–661suicide, attempted 664sulfite oxidase deficiency 218,

225, 652SUMF1 gene 210supernatural theories 1supernumerary marker

chromosomes (SMCs) 281suppurative intracranial

infections, focaladults 482–490children 478–480definition and epidemiology

482diagnostic tests 487–488epilepsy after 485–486management 488–490

surgeryarteriovenous malformations

555cavernous malformations

562–563de novo epilepsy after 407dysembryoplastic

neuroepithelial tumors 444epilepsy see epilepsy surgery

Index

785






surgery (cont.)gangliogliomas and

gangliocytomas 446gliomas 436–438hypothalamic hamartomas

451–452intracerebral hemorrhage 540meningiomas 455–458metastatic brain lesions 462open head injuries 397, 398porencephaly 614–615Rasmussen encephalitis

575, 577startle epilepsy with infantile

hemiplegia 696–697suppurative intracranial

infections 489sylvian fissure cystsclinical and imaging features

341–342epilepsy and 342–343

sylvian seizures 104sympathetic epilepsy 8–9symptomatic epilepsy 117absence seizures in 95categories 114classification issues 44, 114definitions 23, 35, 113–114epileptogenic mechanisms

35–40, 116etiological classification 22frequency of different

underlying etiologies 115genetic predisposition 43historical concepts 3, 11–12, 17mechanism vs. etiology 114–115risk factors 115–116vs. cryptogenic epilepsy 113,

114vs. idiopathic epilepsy 40, 44,

113, 114vs. provoked epilepsy 113see also acquired epilepsies;

developmentalconditions; organicepilepsy

synaptic contactsaltered organization 38–39reversion to immature state 39

syntaxin binding protein 1(STXBP1) gene 50, 51

systemic lupus erythematosus(SLE) 579–583

diagnostic tests 582–583frequency of epilepsy 580–581management 583pathophysiology and clinical

features 579–580risk factors for epilepsy 581–582seizure types and treatment 582

Taenia solium 495see also neurocysticercosis

Takayasu arteritis 570tau protein 618–619

Tay–Sachs disease 206–207TB see tuberculosisTBR2-related polymicrogyria

311, 312, 332, 334, 337telephone-induced epilepsy

706–707television (TV) viewing 683,

687–688, 692temperature, bodycircadian rhythm 643measurement 631methods of lowering 633–634regulation 631see also fever

temperature-sensitive Drosophilamutants 716–717

temporal lobe epilepsy (TLE)after bacterial meningitis 477,

489–490autosomal dominant

(ADLTE) 707chorea-acanthocytosis 213focal cortical dysplasia 294gliomas 438hippocampal sclerosis 363historical aspects 13–14postoperative recurrence 414,

415–416, 419, 420, 421sleep and 646, 648vs. Alzheimer disease 619see also mesial temporal

lobe epilepsytemporal lobectomy 370, 416Temtamy syndrome 309tetracyclic antidepressants

599, 666tetrahydrobiopterin (BH4)

defects 223–224, 228tetraplegia, non-epileptic

startle 386tetraplegic cerebral palsy 383TFAR15/PDCD10/CCM3 gene

560thalamic hemorrhage 537thalamocortical loops 29–30theophylline 668theta pointu alternant 378–379thinking-induced seizures 684,

720–722thyroid hormones 651thyrotoxicosis 651tiagabine 670, 749, 753tick-borne encephalitis 528, 534tonic seizuresDravet syndrome 78Lennox–Gastaut syndrome 128

tonic status epilepticus 737, 739tonic upward gaze, benign 120tonic–clonic seizures (TCS)juvenile myoclonic epilepsy 96see also generalized tonic-

clonic seizures, unilateraltonic-clonic seizures

topiramate 670Lennox–Gastaut syndrome 130

neuronal ceroid lipofuscinoses162

TORCH (toxoplasmosis, otherinfections, rubella,cytomegalovirus, andherpes simplex)infections, neonatalseizures 378

touch-induced seizures 697–698Tourette syndrome 599–600toxin-induced absence status 753toxin-induced seizures 665,

666, 680diagnosis 674treatment 674–675see also drug-induced seizures

toxin-induced status epilepticus748, 749

toxocariasis 502, 503–504,507–508

toxoplasmosis 521, 522–523, 524TPP1 deficiency 158–161, 162,

208–209tramadol 668transcobalamin II (TCII; TC)

252, 253transcription factors,

microcephaly-related335–336

transmantle cortical dysplasia(TCD) 294–295,347–348, 349

traumatic brain injury (TBI) 400epidemiology 400epileptogenic mechanisms

36–37, 394–395, 401–402fetal 346–348, 349, 357inflicted see non-accidental

brain injuryopen head injuries 394–395see also post-traumatic

epilepsy, post-traumaticseizures

tremoraction myoclonus-renal failure

syndrome 169Angelman syndrome 203benign adult familial

myoclonic epilepsy 86, 88cortical 759familial cortical myoclonic,

with epilepsy 173–174Trenton State Hospital 10–11TREX1 gene 336Trichinella 502trichinosis 502tricyclic antidepressants 599,

665, 666trientine 251trigger zone, cutaneous 697–698,

709trinucleotide repeat expansions 56dentato-rubro-pallido-luysian

atrophy 139–140, 141, 142fragile X syndrome 272

tripeptidyl-peptidase (TPP1)deficiency 158–161,162, 208–209

triptorelin 640trisomy 21 see Down syndromeTSC1 gene 177TSC1–TSC2 complex 177TSC2 gene 177T-type calcium channel defects

29–30TUBA1A-related disorders

299, 300–301, 311, 332,335, 337

TUBB2B gene mutations 311,318, 332, 335, 336,354–355

tuberculin skin test 513tuberculomas 511–512, 513–514tuberculosis (TB) 511–517clinical features 512diagnosis 514epidemiology 511–512epilepsy and 514–515HIV co-infection 511, 514–515,

516, 517, 523investigations 512–514management 515–517multi-drug resistant (MDR-

TB) 517vs. neurocysticercosis 497–498,

513–514tuberin 177tuberous sclerosis complex (TSC)

177–181, 196diagnosis 179epilepsy in 178–179epileptogenic mechanisms

39, 178–179genetics and molecular

physiology 177infantile spasms 123, 178, 180management 179–181neonatal seizures 378pathology 177–178, 348–350,

351tubulin 335tumor necrosis factor-alpha

(TNF-alpha) 436Turner, William Aldren 6, 8,

9–1022q11.2 deletion syndrome

(DiGeorge syndrome)312, 313

twin studies 44twin-to-twin transfusion

syndrome 313tyrosine hydroxylase deficiency

223–224

UBE3A gene 55–56, 201, 203, 337ulcerative colitis (UC) 590–592ulegyria 318underlying causes

see predisposing causesundermigration 347, 350–352

Index

786






undulating band heterotopia350, 352

unilateral clonic seizuresDravet syndrome 78Panayiotopoulos syndrome 108

unilateral somatic intracranialhypoplasia 198–199

unilateral tonic–clonic seizures,Panayiotopoulossyndrome 108

Unverricht–Lundborg disease(EPM1) 135–136

clinical features 135diagnostic tests 135–136differential diagnosis 136, 137,

140, 144management 136type B (EPM1B) 136, 137

urea cycle disorders (UCD) 229,246–248

clinical features 247diagnostic tests 247–248management 248seizures and EEG features 247

urokinase-type plasminogenactivator receptor(uPAR) gene 334–335

vaccination 388–390, 472epilepsy after 388–390infantile spasms after 126

vaccine-related encephalopathy388–389, 390

vagal nerve stimulationeating epilepsy 710ring chromosome 20

syndrome 287valproate/valproic acidcavernous malformations 562glioma-related seizures 438HIV infection 526induction of

hyperammonemia 248photosensitive epilepsy 692POLG1-related mitochondrial

cytopathies 151, 153reading epilepsy 701seizure aggravation 670, 671

status epilepticus 724, 741tuberculosis 516

varicella zoster virus (VZV)encephalitis 471

variegate porphyria (VP) 232,233–234, 235

vascular congestion 4vascular dementia (VaD) 619vascular disorders, uncommon

570vascular malformations

see arteriovenousmalformations,cavernous malformations

vasculitis 579, 580Venezuelan equine encephalitis

528venlafaxine 599very-long-chain fatty acids

(VLCFA) 221vestibular schwannomas 186video games 687–688vigabatrininfantile spasms 127seizure aggravation 671, 753tuberous sclerosis 180

viral encephalitis 467–473clinical features 467–468emerging and less common

causes 528–536epidemiology 467, 528investigations 468

visual seizures see occipitalseizures

visual sensitivitysee photosensitivity

visual stimuli, provocative 683,690–692

avoidance 692focal onset seizures 711

visually induced seizures 687general population prevalence

687–688self-induction 687, 690, 692visual stimuli provoking

690–692see also photosensitive

epilepsy

vitamin B6 see pyridoxinevitamin B12 see cobalaminvitamin B12-responsive

methylmalonic aciduria222, 227

voltage-gated Ca2þ channels(Cav channels)

antibodies 589genetic defects 29–30structure and function 25, 26subunit composition and

localization 27voltage-gated ion channels

25, 26, 27voltage-gated Kþ channels

(Kv channels)antibodiesdrug-resistant epilepsywithout encephalopathy589

limbic encephalitis and 574,587, 588, 589

genetic defects 28, 29structure and function 25, 26subunit composition and

localization 27voltage-gated Naþ channels

(Nav channels)genetic defects 27–29, 74structure and function 25, 26subunit composition and

localization 26, 27vomiting, ictal 107, 109von Recklinghausen disease

see neurofibromatosis 1VPS13A gene 213, 214

Walker–Warburg syndrome 302Walshe, F.M.R. 12Warburg micro syndrome

(RAB3GAP1 defects) 311,312, 332, 335

warfarin-related intracerebralhemorrhage 539

water-immersion epilepsysee hot-water epilepsy

watershed zone damage,intrauterine 347, 357, 359

Wegener granulomatosis 580West Nile virus 530West syndrome 119–127clinical and EEG features

119–120differential diagnosis 120etiology 121–126hemimegalencephaly 290microcephaly 337X-linked (IS) 121, 307see also infantile spasms

Western equine encephalitis535

Western European encephalitis534

Wilson, S.A. Kinnier 11–12Wilson disease 249–251clinical features and pathology

249–250diagnostic tests 249, 250, 251epilepsy in 250management 250, 251

Wistar Albino Glaxo ratsbred in Rijswijk(WAG/Rij) 29

Wolf–Hirschhorn syndrome(4p-syndrome)277–279

worms, helminth 501–508writing-induced seizures 721

X-inactivation 53XK gene 213, 214X-linked lissencephaly (XLIS)

298–299X-linked lissencephaly with

ambiguous genitalia(XLAG) 121, 124, 299,301, 307

XLIS gene defects see DCX genedefects

Zellweger spectrum disorders216–217, 219–220, 221

Zellweger syndrome 216–217,220, 221, 226

ZFHX1B (ZEB2) gene 308zinc sulfate 251

Index

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