Lennox–Gastaut syndrome

Also known as: Childhood epileptic encephalopathy with diffuse slow spike-wave

Childhood-onset developmental and epileptic encephalopathy defined by multiple seizure types, slow spike-wave on EEG and cognitive impairment.

Medical specialty: Neurology
Subspecialty: Neurology / Epilepsy; Pediatric neurology
Sex & age distribution: Male: slightly more common; Female: affected; Children: yes, onset typically 1–8 years
Gene(s) involved: ALG13, CHD2, DNM1, GABRB3, GRIN2A, GRIN2B, KCNA2, SCN8A, STXBP1, SYNGAP1

Etiology & mechanism

Heterogeneous: structural injury or malformation, prior infantile spasms, genetic causes, or unknown.

Gene(s) involved (10)

ALG13CHD2DNM1GABRB3GRIN2AGRIN2BKCNA2SCN8ASTXBP1SYNGAP1

Symptoms & signs (8)

atonic seizures (drop attacks)atypical absencesbehavioral problemsintellectual disabilitymyoclonic seizuresnonconvulsive statusslow spike-wave eegtonic seizures

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