目的　探讨儿童丙戊酸相关性高血氨脑病（VHE）的临床特点和诊治。方法　回顾分析1例丙戊酸钠治疗 Dravet综合征后并发VHE患儿的临床资料。结果　女性患儿， 6岁2个月。10月龄诊断Dravet综合征，长期口服丙戊酸钠 及托吡酯治疗。因抽搐发作就诊，意识障碍，血氨明显升高，肝功能异常，脑功能受损严重；脑电图示低电压明显，头颅磁 共振示大脑半球弥漫性水肿，双侧基底节信号异常。患儿经停用丙戊酸钠及降血氨治疗后，血氨下降，意识状态较前好转。 结论　对使用丙戊酸钠的癫痫患儿，特别是联合托吡酯治疗时，需警惕高氨血症及VHE，及时治疗可改善预后。

　Objective　To explore the clinical characteristics, diagnosis and treatment of valproate inducedhyperammonemic encephalopathy (VHE) in children. Method　The clinical data of VHE developed in a child with Dravet syndrome treated by valproate were analyzed retrospectively. Results　A 6-year- and 2-month-old girl was diagnosed with Dravet syndrome at the age of 10 months. She received long-term oral administration of valproate and topiramate. The child visited for convulsive seizures this time, accompanied by consciousness disorders, significantly increased blood ammonia, abnormal liver function and severe brain impairment. Electroencephalogram showed low voltage. Cerebral magnetic resonance imaging showed diffuse edema in the cerebral hemisphere and abnormal signals in bilateral basal ganglia. After the discontinuation of sodium valproate and the treatment for lowering blood ammonia, the blood ammonia decreased, and the state of consciousness was better than before. Conclusion　Clinician should be vigilant of existence of hyperaminemia and VHE in epileptic children treated with valproate, especially in combination with topiramate, and timely treatment is needed to improve the prognosis.