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. (C) 2000 Elsevier Science B.V.
Original language | English |
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Pages (from-to) | 135-138 |
Number of pages | 4 |
Journal | Brain and Development |
Volume | 22 |
Issue number | 2 |
DOIs | |
Publication status | Published - Mar 14 2000 |
Keywords
- Early-infantile epileptic encephalopathy with suppression bursts
- Focal cortical dysplasia
- Ohtahara syndrome
- Spasms
- Suppression-burst pattern
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Developmental Neuroscience
- Clinical Neurology