Dravet syndrome

Dravet syndrome, also known as severe myoclonic epilepsy of infancy, is an autosomal dominant genetic disorder causing a catastrophic form of epilepsy with prolonged seizures often triggered by hot temperatures or fever. It usually begins before one year of age, most often around six months, with prolonged febrile convulsions as its most indicative sign. It is difficult to treat with anticonvulsant medications, is diagnosed clinically with genetic testing recommended in doubtful cases, and its drug-refractory nature has led to exploration of other therapies to extend patient life expectancy.