PEDIATRICS Vol. 90 No. 2 August 1992, pp. 221-223
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Postneonatal Vitamin B6-Dependent Epilepsy

STEVEN B. COKER MD1

1 Department of Neurology, Loyola University Medical Center, Maywood, IL

Vitamin B6 acts as coenzyme in decarboxylation and transamination of amino acids. Pyridoxal phosphate is the coenzyme for a glutamic decarboxylase and ggr-aminobutyric acid transaminase, enzymes necessary in production and metabolism of brain ggr-aminobutyric acid. Vitamin B6 deficiency has been known to produce peripheral neuritis, dermatitis, anemia, and convulsions in infants.1 Administration of isonicotinic acid hydrazide may produce a vitamin B6-responsive neuropathy. Convulsions from vitamin B6 dependency occur despite normal vitamin B6 levels. These seizures, according to pediatric and neurological texts, occur immediately after birth up to 6 months.1-4

In 1985, Goutieres and Aicardi5 described three patients with atypical pyridoxine-dependent seizures with onset at 7 weeks, 5 days, and 4 months.

Submitted on October 28, 1991
Accepted on December 29, 1991