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8A61.11 Dravet syndrome

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

A refractory epileptic encephalopathy occurring in otherwise healthy infants during the first year of life with clonic/tonic-clonic, generalised and unilateral seizures, hemiclonic or generalised status epilepticus. The interictal EEG may initially be normal but with time background activity deteriorates and bilateral asymmetric, focal or multifocal paroxysms of polyspike and slow-waves appear. Mutations in the voltage-gated sodium channel gene SCN1A are commonly found.

postcoordination

synonyms

• Dravet syndrome
• Severe myoclonic epilepsy in infancy

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