Electroclinical characteristics of hemimegalencephaly

Abstract

Presented here are two long-term follow-up patients with hemimegalencephaly. Patient 1 had Ohtahara's syndrome, which evolved into West's syndrome. Patient 2 had localization-related epilepsy, which demonstrated epilepsia partialis continua throughout the clinical course. The patients' interictal electroencephalograms revealed asymmetric suppression-burst patterns sometime during the clinical course: only during early infancy in patient 1 and until the last follow-up (at 30 years of age) in patient 2. Both patients had moderate mental and motor disturbances with persistence of seizures. Hemiplegia was progressive during early childhood. Aggravation of hemiplegia might be related to frequent seizures and persistent electroencephalographic abnormalities during early childhood. Although asymmetric suppression-burst patterns are considered characteristic electroencephalographic findings in these cases, the duration of their appearance did not have definite prognostic significance.