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Fifteen (12.7%) among 118 cases of the West syndrome were effectively treated by high-dose
pyridoxal phosphate (PALPI. 1 ) Clinical seizures were completely suppressed in 12
cases with PAL-P alone, and in 3 cases by an addition of PAL-P to the previously poorly-effective
regimen. At the follow-up, 12 cases have continued to be free from seizures, while
two cases relapsed into the Lennox-Gastaut syndrome, and one died. 2) Electroencephalographically
hypsarhythmia disappeared by PAL-P in all 15 effective cases. 3) Effective daily dose
of PAL-P was 30 to 400 mg. 4) Notably, PAL-P was effective even in the cases with
obvious organic brain pathology, such as tuberous sclerosis, porencephaly, holoprosencephaly,
postmeningitis, besides 5 idiopathic cases. 5) Efficacy of PAL-P was significantly
higher in idiopathic cases than symptomatic cases; 35.7% vs 9.6%. 6) Response to PAL-P
was not predictable by any laboratory data nor clinical features. 7) Prognosis of
PAL-P responsive cases was favorable; as many as 6 cases developed normally among
14 cases followed-up. Treatment with a high-dose PAL-P should be tried in all cases
of the West syndrome at first.
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© 1987 Published by Elsevier Inc.