丙戊酸致高氨血症脑病二例并文献复习

Valproate-induced hyperammonemic encephalopathy:report of two cases and literature review

目的了解丙戊酸致高氨血症脑病(Valproate-induced hyperammonemic encephalopathy,VHE)的临床特点、发病机制和治疗措施。方法分析2例丙戊酸脑病患者的临床资料并复习文献。结果例1为52岁男性患者,发病初期出现反应迟钝、行为异常,按照非惊厥持续状态治疗后症状加重,发现血氨增高并撤用丙戊酸钠,予降氨治疗,2d后恢复;例2为22岁女性患者,诊断为青少年肌阵挛,近4年一直服用丙戊酸钠,加用左乙拉西坦后出现共济失调、扑翼样震颤,发现血氨升高,停用左乙拉西坦后恢复正常。结论丙戊酸致高氨血症脑病的临床症状不特异,临床医生对丙戊酸的不良反应及药物相互作用应提高警惕,避免漏诊、误诊。

Objective To discuss the clinical manifestation, pathophysiological mechanism and treatment of valproate-induced hyperammonemic encephalopathy(VHE). Methods The special clinical manifestations of two VHE patients were reported and the literature reviewed. Results A 52-year-old man presented with mental obtundation and dystropy after taking valproate. He was misdiagnosed with nonconvulsive status epilepticus and his state of consciousness worsened. The level of serum ammonia was found to increase, then diagnosis of VHE was made. The patient’s condition was controlled and recovered by discontinuation of valproate and administration of lactulose. The second patient was a 22-year-old female,who was taking valproate for the last four years. After levetiracetam was added,the patient suffered from drowsy and ataxia and higher ammonia. Then levetiracetam was discontinued, the patient showed completely recovery. Conclusion Patients with VHE may present with nonspecific symptoms. Clinicians should be aware of rare adverse drug reactions and drug interactions to conclusively reach the correct diagnosis.