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. (C) 2000 Elsevier Science B.V.
- Early-infantile epileptic encephalopathy with suppression bursts
- Focal cortical dysplasia
- Ohtahara syndrome
- Suppression-burst pattern
ASJC Scopus subject areas
- Clinical Neurology
- Pediatrics, Perinatology, and Child Health