pyridoxine dependent epilepsy  The typical manifestations are uncontrollable seizures in the neonatal period and early infancy, and status epilepticus often occurs. Various anti-epileptic drugs cannot control the seizures. High-dose vitamin B6 (pyridoxine) treatment responds well. The clinical manifestations of 1/3 of patients may be atypical, such as initial response to commonly used antiepileptic drugs, especially phenobarbital, and late onset of convulsions until 3 years old.