A case of vitamin B6-responsive West syndrome caused by severe traumatic brain injury

Abstract

High-dose vitamin B6 (VB6) treatment is one of the therapeutic options for West syndrome (WS), particularly for cases without cerebral lesions. The effects of VB6 in patients with brain pathologies, however, may shed light on its presently unknown mechanisms of action for WS. We herein report an infant with WS caused by a very severe brain injury mainly involving the left temporal lobe, who responded dramatically to pyridoxal-5′-phosphate (PLP), the active form of VB6. The patient achieved long-term freedom from seizure with marked EEG improvement. His successful treatment indicated that VB6 should not be precluded from the therapeutic options however extensive the causative brain pathology is. Since WS remains an intractable epilepsy syndrome with limited treatment options, high-dose VB6 therapy is thought to be worth considering.