Nakazato Yoshihiko, Ando Satsuki, Yamamoto Toshimasa, Tamura Naotoshi, Shimazu Kunio
Department of Neurology, Saitama Medical School.
Rinsho Shinkeigaku. 2004 Oct;44(10):682-5.
We describe a 56-year-old woman with hyperammonemic encephalopathy caused by side effect of valproic acid (VPA). Ten months before the admission to our hospital, she had the first attack of convulsive seizure. Diagnosis of epilepsy was made, and the oral administration of VPA (800mg/day) was started at another hospital. Seizure was well controlled by VPA, until the recurrence of attack forced her to visit our hospital. Convulsive seizure disappeared immediately after intravenous administration of diazepam, but consciousness disturbance was prolonged for a few days. Since laboratory examinations revealed hyperammonemia without liver dysfunction, VPA was discontinued. Subsequently, her consciousness completely recovered. Other laboratory findings, including positive antinuclear antibody, antibodies to Sjögren's syndrome A, reduced lacrimal secretion in Schirmer's test, and cell infiltration in the salivary gland on lip biopsy specimen, suggested the presence of Sjögren's syndrome. The hyperammonemia occurs by the side effect of VPA, often has basal disease or drug interactions. It was, however, especially in patients with basal disease or other drugs, obscure whether (and how) Sjögren's syndrome contributed to the development of hyperammonemic encephalopathy in this case, since she took only VPA.
我们描述了一名56岁患有由丙戊酸（VPA）副作用引起的高氨血症性脑病的女性。在我院入院前十个月，她首次出现惊厥发作。癫痫被确诊，在另一家医院开始口服VPA（800mg/天）。VPA很好地控制了癫痫发作，直到发作复发迫使她前来我院就诊。静脉注射地西泮后惊厥发作立即消失，但意识障碍持续了几天。由于实验室检查显示高氨血症且无肝功能障碍，停用了VPA。随后，她的意识完全恢复。其他实验室检查结果，包括抗核抗体阳性、抗干燥综合征A抗体、Schirmer试验中泪液分泌减少以及唇活检标本中唾液腺细胞浸润，提示存在干燥综合征。高氨血症由VPA副作用引起，常伴有基础疾病或药物相互作用。然而，特别是在有基础疾病或使用其他药物的患者中，由于她仅服用了VPA，干燥综合征是否（以及如何）导致了该病例中高氨血症性脑病的发生尚不清楚。
