Severe myoclonic epilepsy of infancy. This very rare syndrome is delimitated from benign myoclonic epilepsy by its severity and is closely related to Lennox-Gastaut syndrome and Doose’s myoclonic-astatic epilepsy. Onset in first year of life. Symptoms peak at about 5 months of age with febrile hemiclonic or generalized status epilepticus. Boys twice as often affected as girls. Prognosis is poor. Only about 300 cases described. Most cases are idiopathic, about 25 percent may be familial.
- C. Dravet:
Les epilepsies graves de l’enfant. Vie Médicale 1978, 8: 543–548.
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