title: Suppression-burst patterns in intractable epilepsy with focal cortical dysplasia.
abstract: We report on a patient with early-onset spasms in series and partial seizures associated with focal cortical dysplasia whose EEGs showed suppression-burst patterns during early infancy. These electroclinical characteristics suggested a diagnosis of Ohtahara syndrome, but the EEG findings were atypical because of the lack of suppression-burst patterns during wakefulness. In addition, the patient did not have severe psychomotor retardation. With high-dose pyridoxal phosphate therapy, seizures were suppressed and suppression-burst patterns disappeared at 2 months of age. Focal motor seizures recurred later and they often evolved into epilepsia partialis continua. Patients with early-onset intractable seizures associated with suppression-burst patterns on EEGs have several different etiologies, and these patients should be categorized according to their etiology in addition to their syndromic diagnosis.
authors.affiliation: Department of Child Neurology, Okayama University Medical School, 2-5-1, Shikatacho, Okayama, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Ohtsuka
authors.forename: M
authors.initials: M
authors.lastname: Sato
authors.forename: S
authors.initials: S
authors.lastname: Sanada
authors.forename: H
authors.initials: H
authors.lastname: Yoshinaga
authors.forename: E
authors.initials: E
authors.lastname: Oka
mesh_terms: D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology*; D002648:Child; D004569:Electroencephalography; D004827:Epilepsy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007564:Japan; D008279:Magnetic Resonance Imaging; D008297:Male