Suppression-burst patterns in intractable epilepsy with focal cortical dysplasia.
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.
Ohtsuka
Y
Y
Department of Child Neurology, Okayama University Medical School, 2-5-1, Shikatacho, Okayama, Japan.
Sato
M
M
Sanada
S
S
Yoshinaga
H
H
Oka
E
E
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