On a rare form of epilepsy in infants--Ohtahara syndrome.

We report 15 children with clinical and electroencephalographic evidence of Ohtahara syndrome. The onset of seizures in most of them was within the first week of life. The seizures were predominantly polymorphic and refractory to treatment with ACTH and anticonvulsants. Suppression-burst patterns were recorded on the EEG in all cases. The outcome in all children was grave--four died in the neonatal period, and three-in the infancy period. Five of the survivors evolved into the West syndrome and three-into the Lennox-Gastaut syndrome. It is concluded that early detection of Ohtahara syndrome is of essential importance for the prognosis of newborns and little infants with seizures.