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Epilepsy and seizure disorders

90 articles

Absence seizure

Absence seizure (petit mal): a generalized-onset epileptic seizure with sudden brief loss of awareness, blank stare, and 3 Hz spike-wave EEG, occurring mainly in children 4 to 12, treated with ethosuximide or valproic acid.

Absence seizures, atypical

Atypical absence seizures are generalized absence seizures with gradual onset and offset, pronounced tone changes, and slow irregular spike-and-wave EEG activity, typically in Lennox–Gastaut syndrome.

Absence status epilepticus

Absence status epilepticus (ASE, petit mal status) is a nonconvulsive status epilepticus: a generalized absence seizure over 30 minutes to days causing confusion, with 2.5-4 Hz spike-wave EEG, treated with low-dose benzodiazepines.

Action myoclonus-renal failure syndrome

Action myoclonus-renal failure syndrome (AMRF, EPM4, OMIM 254900) is a rare autosomal recessive progressive myoclonic epilepsy with proteinuria and kidney failure, caused by biallelic SCARB2/LIMP-2 loss-of-function mutations, onset in teens or twenties, death within 7-15 years.

Acute repetitive seizures

Acute repetitive seizures (seizure clusters, cluster or serial seizures) are closely grouped seizures in epilepsy, increased from the patient's baseline, with recovery between seizures; ICD-11 8A67, treated with benzodiazepine rescue medication.

Alcohol intoxication

Alcohol intoxication, or drunkenness and alcohol poisoning, is CNS depression from ethanol accumulating faster than the liver clears it; GABA-A potentiation, NMDA inhibition, zero-order elimination, BAC 0.08% binge threshold, coma risk above 50 mM.

Asterixis

Asterixis (flapping tremor, liver flap) is negative myoclonus: brief lapses of posture from sudden muscle relaxation, first described by Adams and Foley in 1949, seen mainly in toxic-metabolic encephalopathies such as hepatic encephalopathy.

Audiogenic epilepsy

Audiogenic epilepsy (ORPHA:166415, ICD-11 8A61.40) is a rare reflex epilepsy in which acoustic stimuli, from sudden noises to specific music or voices, trigger seizures; inferior colliculus-driven in rodent models, extremely rare as simple sound-triggered seizures in humans.

Aura (symptom)

Aura (symptom): subjective symptoms at the start of an epileptic seizure, equivalent to a focal aware seizure in ILAE terms; distinguished from migraine aura, syncope, and panic disorder; term from Latin for breeze, dating to Galen.

Benign focal seizures of adolescence

Benign focal seizures of adolescence (BFSA, adolescent benign focal crisis): rare idiopathic seizure-susceptibility condition peaking at 13-15 years, with one or 2-5 focal seizures within 36 hours, normal EEG and MRI, no recurrence, no treatment needed.

Childhood absence epilepsy

Childhood absence epilepsy (CAE, formerly pyknolepsy) is an idiopathic generalized epilepsy of school-aged children with daily absence seizures and 2.5–4-Hz spike-wave on EEG; ethosuximide is first-line, remission about 60%.

Childhood occipital visual epilepsy

Childhood occipital visual epilepsy (COVE), formerly Gastaut-type idiopathic childhood occipital epilepsy, is a self-limited childhood epilepsy with brief visual seizures, occipital EEG spikes, normal MRI, and remission in most children.

Complex febrile seizures

A complex febrile seizure is a fever-related seizure in a young child that is focal, lasts 15 minutes or more, recurs within 24 hours, or causes lasting weakness.

Convulsion

Convulsion: sudden, violent involuntary muscle contractions causing rapid rhythmic shaking, also called a fit or attack; seen in tonic-clonic epileptic seizures, childhood fever, syncope, and drug or alcohol abuse.

Convulsive status epilepticus

Convulsive status epilepticus (CSE, ICD-11 8A66.0) is generalized tonic-clonic seizure activity lasting 5 minutes or more or recurring without recovery, a neurological emergency treated first with benzodiazepines, with mortality up to 30% in adults.

Cryptogenic late-onset epileptic spasms

Cryptogenic late-onset epileptic spasms (ORPHA:163708, late-onset infantile spasms) is a rare epilepsy syndrome with spasms after age 1 year in clusters, tonic seizures, atypical absences, cognitive deterioration, and temporal/temporofrontal EEG focus without hypsarrhythmia.

Developmental and epileptic encephalopathy with spike-wave activation in sleep

DEE-SWAS, also called EE-SWAS and formerly ESES or CSWS, is a rare childhood epilepsy syndrome with regression of cognition, language, behavior, or motor skills tied to marked sleep-activated 1.5–2 Hz spike-and-wave EEG activity, onset at 2–12 years.

Dravet syndrome

Dravet syndrome, formerly severe myoclonic epilepsy of infancy (SMEI), is a rare genetic epileptic encephalopathy starting in infancy, usually from de novo SCN1A mutations causing Nav1.1 loss in inhibitory interneurons, with drug-resistant seizures, intellectual disability, and elevated SUDEP risk.

Early infantile developmental and epileptic encephalopathy

Early infantile developmental and epileptic encephalopathy (EIDEE), formerly Ohtahara syndrome and early myoclonic encephalopathy, is a severe epilepsy syndrome with drug-resistant tonic or myoclonic seizures, burst-suppression EEG, and onset from birth to 3 months.

Eating reflex epilepsy

Eating reflex epilepsy (eating epilepsy, ORPHA:166418) is a rare reflex epilepsy in which seizures are triggered by eating, chewing, or food-related stimuli. Onset typically in the second decade, male predominance, focal impaired-awareness seizures, temporolimbic or perisylvian onset.

Ecstatic seizures

Ecstatic seizures are rare focal epilepsy auras of bliss, physical well-being, and heightened self-awareness with preserved consciousness. About 50-52 cases reported; linked to the dorsal anterior insula; first described by Dostoevsky.

Epilepsy

Epilepsy, a chronic noncommunicable brain disorder also called a seizure disorder, is defined by recurrent unprovoked seizures. About 50 million people are affected worldwide; up to 70% could become seizure-free with antiseizure medicines, and surgery helps drug-resistant focal cases.

Epilepsy of infancy with migrating focal seizures

EIMFS, also called malignant migrating partial seizures of infancy, is a rare genetic epileptic encephalopathy with drug-resistant focal seizures migrating between hemispheres, onset before 6 months, KCNT1 mutations in many cases, and severe developmental impairment.

Epilepsy with auditory features

Epilepsy with auditory features (EAF), formerly ADLTE or ADPEAF, is a focal epilepsy syndrome with auditory seizures or receptive aphasia from lateral temporal onset, onset typically age 10-30, linked to LGI1 and other genes.

Epilepsy with eyelid myoclonia

Epilepsy with eyelid myoclonia (EEM), formerly Jeavons syndrome, a rare childhood-onset genetic generalized reflex epilepsy with eyelid myoclonia, eye closure and light triggers, photosensitivity, female predominance, and frequent drug resistance.

Epilepsy with generalized tonic-clonic seizures alone

Epilepsy with generalized tonic-clonic seizures alone (EGTCS, GTCA), formerly GTCS on awakening, is a genetic generalized epilepsy syndrome with tonic-clonic seizures as the only seizure type, onset at 5–40 years, and mandatory 3–5.5 Hz spike-wave on EEG.

Epilepsy with myoclonic absences

Epilepsy with myoclonic absences (EMA, ORPHA:86911, ICD-11 8A61.23) is a rare childhood-onset generalized epilepsy with rhythmic 3-Hz myoclonic absence seizures, male predominance, and variable prognosis.

Epilepsy with myoclonic-atonic seizures

Epilepsy with myoclonic-atonic seizures (EMAtS, Doose syndrome, myoclonic-astatic epilepsy) is a rare childhood epilepsy syndrome with myoclonic-atonic drop seizures, 2-6 Hz spike-wave EEG, male predominance, and remission in about two thirds of children.

Epilepsy-mild cortical malformation-oligodendroglial hyperplasia

MOGHE (mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy) is an ILAE-recognized cause of childhood drug-resistant epilepsy, defined by white-matter heterotopic neurons, Olig2-positive oligodendroglial hyperplasia, and somatic SLC35A2 variants.

Epileptic encephalopathies

Epileptic encephalopathies, now often termed developmental and epileptic encephalopathies (DEE), are severe epilepsies in which seizures and EEG epileptiform activity themselves worsen cognition and development; classic syndromes include West, Dravet, Ohtahara, and Lennox-Gastaut.