Pyridoxine-Dependent Epilepsy as a Cause of Neonatal Seizures.

Pediatric seizures are a common reason for emergency department visits. The highest risk of seizures in children is during the neonatal period. A low index of suspicion is important to facilitate the early assessment, workup, and treatment of inborn errors of metabolism to optimize developmental outcomes. We present the rare case of a 9-day-old with seizures refractory to multiple anticonvulsant medications who was diagnosed with pyridoxine-dependent epilepsy. We review differences in the management of neonatal seizures from older patients, the utility of a trial of pyridoxine in refractory neonatal seizures, and the importance of preparing for emergent airway management given pyridoxine's ability to cause apnea and central nervous system depression.