Abstract
The West syndrome, known since 1841 is one of the most therapeutically resistant and unfavourable epilepsies of the first months of life. First line drug for treatment is vitamine B6. A response is really a 'lucky shot'. Long term follow up of neuropsychological outcome in these children is not available up to now. We report the course over 12 years on a boy with a vitamine B6 sensible West syndrome. He is the 2nd child of healthy parents, pregnancy and birth were normal. At the age of 6 months West syndrome of idiopathic origin started with typical seizures and hypsarrhythmia in the EEG. CSF GABA concentration before vitamine B6 (300mg/kg/body weight) was 8 pmol/ml (normal range 54–184 pmol/ml).
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