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[丙戊酸盐相关性高氨血症性脑病。1例报告]

[Valproate-associated hyperammonemic encephalopathy. Report of one case].

作者信息

Young Pablo, Finn Bárbara C, Alvarez Florencia, Bruetman Julio E, Trimarchi Hernán

机构信息

Servicios de Clínica Médica y Nefrología, Hospital Británico de Buenos Aires, Perdriel 74, Argentina.

出版信息

Rev Med Chil. 2007 Nov;135(11):1446-9. Epub 2008 Jan 16.

Abstract

Valproate can be associated to hyperammonemic encephalopathy, characterized by fluctuating sudden-onset alterations of sensorium, focal symptoms and an increase in the frequency of seizures. We report a 78 year-old female using valproate 1,000 mg/ day for 10 months for the treatment to tonic-clonic seizures. She was admitted on three occasions in the last fourth months for self limited clouding of sensorium. Laboratory, imaging and electroencephalographic studies were non-contributory Blood ammonia levels were 123 microg/dl (normal: 15-50 microg/dl). Due to the possibility of a hyperammonemic encephalopathy secondary to valproate, the drug was discontinued and she was treated with lactulose and intravenous L-carnitine, 1 g/day The patient showed a complete recovery within 48 hours. This drug-associated encephalopathy is a reversible but potentially fatal cause, probably underdiagnosed, that requires a high index of suspicion.

摘要

丙戊酸盐可与高氨血症性脑病相关,其特征为意识突然波动改变、局灶性症状以及癫痫发作频率增加。我们报告一例78岁女性,使用丙戊酸盐1000毫克/天治疗强直阵挛性癫痫10个月。在过去四个月内她三次因自限性意识模糊入院。实验室、影像学和脑电图检查均无异常发现。血氨水平为123微克/分升(正常:15 - 50微克/分升)。由于存在丙戊酸盐继发高氨血症性脑病的可能性,停用该药,给予乳果糖和静脉注射L - 肉碱,1克/天治疗。患者在48小时内完全康复。这种药物相关性脑病是一种可逆但可能致命的病因,可能诊断不足,需要高度怀疑。

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