Neglected Atypical Pyridoxine Dependent Seizures

Vitamin b6 (pyridoxine) dependent seizure (pds) is an autosomal-recessively inherited disorder which starts within a few hours of birth or even earlier, and can cause intrauterine seizures[12]. it occurs while the serum level of b6 vitamin is normal. it is the result of a defect in pyridoxine binding to its apoenzyme glutamate decarboxylase which finally leads to reduced concentrations of gamma-aminobutyric acid (gaba). low concentration of gaba is related to decreased seizure threshold[3]. the frequency of pds is unknown and limited cases have been reported worldwide[4]. some cases from iran have already been reported too[5].