Vitamin B(6) treatment of intractable seizures.

Vitamin B(6) (VB(6))-related seizures include clinical seizures associated with VB(6) deficiency and dependency. Both types of seizures are suppressed by VB(6). We proposed VB(6)-responsive seizures as the third category of VB(6)-related seizures in 1977. Vitamin B(6)-responsive seizures decrease or disappear in response to high-dose oral VB(6). Seizure onset in most of our cases occurred within the first year of life, although this varied between 3 months and 5 years. Etiologically, such cases were not only idiopathic or cryptogenic, but also symptomatic and associated with organic brain lesions. The tryptophan load test was usually negative. Vitamin VB(6)-responsive seizures or epilepsy were usually West syndrome (WS), however may also include Lennox-Gastaut syndrome, grand mal or partial motor seizures. High-dose VB(6) treatment administered to 216 consecutive WS cases had an overall response rate of 13.9%, being high not only in cryptogenic cases (32%), but also in symptomatic WS (11.5%) associated with identifiable brain pathologies. Notably, responsive patients had excellent long-term seizure and mental outcomes without the need for conventional antiepileptic medication. A gradual increase in clinical response to VB(6) was noted with increasing the VB(6) dose from 30 to 50-100mg/day, and a dramatic increase in clinical response with high-dose VB(6) (100-400mg). Little clinical response was noted with administration of low dose VB(6) (10-30 mg/day). Thus, high-dose oral VB(6) treatment is recommended in all WS patients at time of initial treatment for a minimum of 10 days, considering the safety and rapid onset of efficacy, usually within 1 week, of this treatment.

Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.