Kaneko Masahiko, Shikata Hisaharu, Kihara Hisafumi
Department of Internal Medicine and Hematology, Uwajima City Hospital, Uwajima, JPN.
Cureus. 2024 Dec 30;16(12):e76603. doi: 10.7759/cureus.76603. eCollection 2024 Dec.
An 87-year-old male with a history of seizure disorder on long-term prophylaxis with valproate and concomitant levetiracetam presented with impaired consciousness and anorexia. The evaluation revealed a markedly elevated blood ammonia level of 518 μmol/L and decreased serum carnitine levels, leading to a diagnosis of valproate-induced hyperammonemic encephalopathy in the absence of liver dysfunction. Discontinuation of valproate, continuation of levetiracetam, and initiation of levocarnitine supplementation and branched-chain amino acid infusion resulted in a durable resolution of symptoms. This case underscores the importance of promptly considering valproate-induced hyperammonemic encephalopathy due to carnitine depletion in patients on long-term valproate therapy who develop acute encephalopathy and initiating levocarnitine treatment for effective management.
一名87岁男性，有癫痫病史，长期服用丙戊酸盐并同时服用左乙拉西坦进行预防，出现意识障碍和厌食。评估发现血氨水平显著升高至518 μmol/L，血清肉碱水平降低，导致在无肝功能障碍的情况下诊断为丙戊酸盐诱导的高氨血症性脑病。停用丙戊酸盐，继续使用左乙拉西坦，并开始补充左卡尼汀和输注支链氨基酸，症状得到持久缓解。该病例强调了对于长期接受丙戊酸盐治疗且出现急性脑病的患者，应及时考虑因肉碱缺乏导致的丙戊酸盐诱导的高氨血症性脑病，并启动左卡尼汀治疗以进行有效管理的重要性。
