PDF(Pubmed)
Abstract：
Valproate-induced hyperammonemic encephalopathy (VHE) is a rare and severe side effect that can occur with valproic acid (VPA) therapy, despite therapeutic doses and normal serum levels of valproate. The typical signs of this condition include a sudden onset of impaired consciousness, focal neurologic symptoms, and an increase in seizure frequency. The exact cause of VHE is unknown, but it is believed to be related to the accumulation of toxic VPA metabolites and increased levels of ammonia that can cause swelling of the astrocytes and cerebral edema. We present a case of a 19-year-old male patient with a history of bipolar disorder on valproic acid 250 mg daily, admitted to the hospital after a new-onset seizure. He was found to have elevated levels of ammonia in his blood, despite having therapeutic levels of valproate and no liver dysfunction. His symptoms improved with discontinuation of the medication and his ammonia levels decreased. We discuss possible mechanisms and risk factors leading to encephalopathy while on valproate therapy. VHE should be considered a possibility when patients treated with valproate show signs of impaired consciousness.
摘要：
丙戊酸诱导的高氨血症性脑病（VHE）是一种罕见且严重的副作用，可与丙戊酸（VPA）治疗一起发生。尽管治疗剂量和丙戊酸盐血清水平正常。这种情况的典型迹象包括意识障碍的突然发作，局灶性神经症状,以及癫痫发作频率的增加.VHE的确切原因未知,但它被认为与有毒的VPA代谢物的积累和氨水平的增加有关，这可能导致星形胶质细胞肿胀和脑水肿。我们介绍了一个19岁的男性患者，有双相情感障碍病史，每天服用丙戊酸250毫克，在新发作的癫痫发作后入院。他被发现血液中的氨含量升高，尽管有治疗水平的丙戊酸盐和无肝功能障碍。他的症状随着停药而改善，氨水平降低。我们讨论了丙戊酸盐治疗时导致脑病的可能机制和危险因素。当丙戊酸盐治疗的患者表现出意识障碍的迹象时，应考虑VHE的可能性。
