Research Article| Volume 9, ISSUE 4, P418-421, 1987

Treatment of the west syndrome with high-dose pyridoxal phosphate

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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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        • Yamatogi Y
        • Ohtahara S
        Age-dependent epileptic encephalopathy: a longitudinal study.
        Folia Psychiatr Neurol Jpn (Tokyo). 1981; 35: 321-331
        • Ohtahara S
        Clinical investigation on effects of gamma-aminobutyric acid and its related substances on various types of epilepsy in children (in Japanese).
        Acta Paediatr Jpn (Tokyo). 1960; 64: 2114-2136
        • Coursin DB
        Convulsive seizures in infants with pyridoxine-deficient diet.
        JAMA. 1954; 154: 406-408
        • Hunt AD
        • Stockes J
        • McCrory WW
        • et al.
        Pyridoxine dependency: report of a case of intractable convulsions in an infant controlled by pyridoxine.
        Pediatrics. 1954; 13: 140-145
        • Hansson O
        • Hagberg B
        Effect of pyridoxine treatment in children with epilepsy.
        Acta Soc Med Uppsala. 1968; 73: 35-43
        • Ekelund H
        • Gamstorp I
        • von Studnitz W
        Apparent response of impaired mental development, minor motor epilepsy and ataxia to pyridoxine.
        Acta Paediatr Scand. 1969; 58: 572-576
        • French JH
        • Crueter BB
        • Druckman R
        • et al.
        Pyridoxine and infantile myoclonic seizures.
        Neurology. 1965; 15: 101-113