We report on a 31-year-old female with vitamin Bs dependent seizures whose
seizure onset was in the neonatal period. Rer elder brother had the same di
sorder and died in infancy. Administration of vitamin B-6 was initiated in
the postnatal period. At the age of 12 years 1 month, 2 months after withdr
awal of vitamin Be, visual seizures began to occur frequently, Myoclonic se
izures and occasional generalized convulsive seizures were also observed. A
t the same time, photoparoxysmal response and spontaneous diffuse spike-wav
e bursts were seen on her EEG;, Myoclonic seizures were provoked by intermi
ttent photic stimulation during the EEG, It is distinctive that visual seiz
ures were one of the main seizure types in this patient, that her clinical
course was relatively benign, and that she has normal intellectual outcome.