Hemiconvulsion-hemiplegia-epilepsy syndrome

Definition

A rare childhood-onset epilepsy syndrome characterized by hemiplegia occurring in children (typically under the age of 4) following prolonged focal seizures, often febrile in nature. The condition progresses through three stages: initial prolonged seizures, subsequent hemiplegia (weakness or paralysis on one side of the body), and eventual epilepsy, following a latent period. The condition may be idiopathic or associated with structural, infectious, traumatic, or degenerative brain abnormalities. Patients present with acute unilateral brain swelling, followed by cerebral hemiatrophy. Inflammation, genetic predisposition, and prolonged seizure activity may play a role in the disease development/progression.