Hemiconvulsion-Hemiplegia-Epilepsy Syndrome

Objective

Hemiconvulsion-Hemiplegia-Epilepsy syndrome (HHE) is an initial phase of unilateral or predominantly unilateral convulsive seizures usually of long duration, with a second phase of hemiplegia (usually permanent), immediately following the hemiconvulsions;and then a third stage, characterized by the appearance of partial epileptic seizures. The causes of the initial convulsions in HHE syndrome are multiple but in many patients no cause in obvious. Neuroimaging studies demonstrate an acquired atrophy, that often is preceded by swelling and edema of the hemisphere. Here we report a case of 18 monthold boy, with an initial phase of repeated episodes of partial seizures, followed by hemiparesis and epileptic seizures, with one episode of generalized convulsive status epilepticus . Minor head trauma was the trigger factor of the initial attack. Brain neuroimaging showed generalized atrophy , that was more prominent in the left hemisphere.