THE EPILEPTIC SPECTRUM IN THE CONGENITAL BILATERAL PERISYLVIAN SYNDROME

We studied the frequency, clinical and EEG characteristics, and outcom e of the epileptic syndrome in 31 patients with a congenital neurologi c syndrome characterized by pseudobulbar palsy, cognitive deficits, an d bilateral perisylvian polymicrogyria. Seizures were present in 27 of 31 patients (87%) and usually began between the ages of 4 and 12 year s; they commonly consisted of atypical absence, atonic/tonic, and gene ralized tonic-clonic seizures. Partial attacks were present in 26%. EE G demonstrated generalized spike and wave abnormalities and, less freq uently, multifocal discharges, predominantly in centro-parietal region s. Seizures were poorly controlled in 65%, with the remaining patients well controlled. Seven patients underwent callosotomy, which resulted in seizure improvement. This study indicates that the epileptic spect rum in this syndrome is broad but follows predictable patterns. Callos otomy is a valuable treatment strategy in those with intractable drop attacks.