Suppression-burst patterns in intractable epilepsy with focal cortical dysplasia

We report on a patient with early-onset spasms in series and partial seizur es associated with focal cortical dysplasia whose EEGs showed suppression-b urst patterns during early infancy. These electroclinical characteristics s uggested a diagnosis of Ohtahara syndrome, but the EEG findings were atypic al because of the lack of suppression-burst patterns during wakefulness. In addition, the patient did not have severe psychomotor retardation. With hi gh-dose pyridoxal phosphate therapy, seizures were suppressed and suppressi on-burst patterns disappeared at 2 months of age. Focal motor seizures recu rred later and they often evolved into epilepsia partialis continua. Patien ts with early-onset intractable seizures associated with suppression-burst patterns on EEGs have several different etiologies, and these patients shou ld be categorized according to their etiology in addition to their syndromi c diagnosis. (C) 2000 Elsevier Science B.V. All rights reserved.