Valproate-induced hyperammonaemic encephalopathy in a neonate: Treatment with carglumic acid

Abstract Valproate-induced hyperammonaemic encephalopathy (VHE) is an unusual and serious complication of valproate (VA) treatment. When an early diagnosis is made, it can be reversed with VA withdrawal and early treatment for hyperammonaemia. We describe the case of a 20-day-old male, who developed a serious VHE after receiving VA for refractory neonatal seizures. The VHE was resolved with VA withdrawal in association with carglumic acid and other measures for hyperammonaemia treatment.