| A rare type of epilepsy that presents with seizures prior to 4 years of age with an episode of febrile, hemiclonic status epilepticus, which is immediately followed by permanent hemiparesis (acute phase). Within 3 years of status epilepticus, drug-resistant unilateral focal seizures develop (chronic phase). Previous development and neurological examination are normal. Immediately after status, patients develop hemiparesis, that usually leaves a permanent motor deficit. Aphasia may present acutely and typically resolves. Many individuals are left with various degrees of intellectual disability. EEG shows slowing of background activity over the affected hemisphere. Focal or multifocal epileptiform abnormalities over the affected hemisphere are evident after seizure onset. MRI shows unilateral edematous swelling of the affected hemisphere in the acute phase, followed by hemispheric atrophy at follow-up. |
