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丙戊酸钠相关的高氨血症性脑病

Sodium valproate-related hyperammonaemic encephalopathy.

作者信息

Pegg Emily Jane, Zaman Fawad

机构信息

Lancashire Teaching Hospitals, Preston, Lancashire, UK.

出版信息

BMJ Case Rep. 2014 Apr 10;2014:bcr2014203899. doi: 10.1136/bcr-2014-203899.

Abstract

A 59-year-old man with a background of poststroke epilepsy, lung cancer, chronic obstructive pulmonary disease and hypertension, presented to the medical assessment unit with acute confusion and altered consciousness. Medications included sodium valproate, aspirin and antihypertensives. On examination he was confused, with his Glasgow Coma Scale fluctuating between 10 and 14. Routine blood tests, thyroid function tests, serum sodium valproate level, urine dip, CT of the brain and cerebrospinal fluid analysis were all normal. EEG revealed changes consistent with an encephalopathic process. Serum ammonia was elevated (75 µg/dL), consistent with a diagnosis of valproate-related hyperammonaemic encephalopathy. Sodium valproate was changed to a different antiepileptic drug and his confusion gradually resolved. Valproate-related hyperammonaemic encephalopathy is a treatable condition which should be considered as a diagnosis in anyone taking sodium valproate with new onset confusion, even in the presence of therapeutic sodium valproate levels and normal liver function tests.

摘要

一名59岁男性,有中风后癫痫、肺癌、慢性阻塞性肺疾病和高血压病史,因急性意识模糊和意识改变入住医疗评估单元。用药包括丙戊酸钠、阿司匹林和抗高血压药。检查时他意识模糊,格拉斯哥昏迷评分在10至14之间波动。常规血液检查、甲状腺功能检查、血清丙戊酸钠水平、尿液试纸检查、脑部CT和脑脊液分析均正常。脑电图显示与脑病过程一致的变化。血清氨升高(75μg/dL),符合丙戊酸盐相关高氨血症性脑病的诊断。丙戊酸钠换用另一种抗癫痫药物后,他的意识模糊逐渐缓解。丙戊酸盐相关高氨血症性脑病是一种可治疗的疾病,对于任何服用丙戊酸钠且出现新发意识模糊的患者,即使丙戊酸钠水平在治疗范围内且肝功能检查正常,也应考虑诊断为此病。

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