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“桶中人”综合征作为脑桥外和脑桥中央髓鞘溶解症的延迟表现:静脉注射免疫球蛋白的有益作用

"Man-in-the-barrel" syndrome as delayed manifestation of extrapontine and central pontine myelinolysis: beneficial effect of intravenous immunoglobulin.

作者信息

Deleu Dirk, Salim Khalid, Mesraoua Boulenouar, El Siddig Abbas, Al Hail Hassan, Hanssens Yolande

机构信息

Department of Medicine (Neurology), Hamad Medical Corporation, P.O. Box 3050, Doha, State of Qatar.

出版信息

J Neurol Sci. 2005 Oct 15;237(1-2):103-6. doi: 10.1016/j.jns.2005.05.012.

Abstract

"Man-in-the-barrel" syndrome has been rarely described following osmotic myelinolysis. We report a case of a 45-year-old woman admitted with septicemia and severe hyponatremia. She presented with a "man-in-the-barrel" syndrome which developed more than 10 days after rapid correction of the hyponatremia. There was radiological evidence of central pontine and extrapontine myelinolysis. Three days after completing a course of intravenous immunoglobulin therapy (0.4 g/kg body weight/day for 5 days) there was considerable improvement (Expanded Disability Status Scale score improved 30%). This case, reported for its peculiar mode of development, unusual presentation and challenging therapeutic response to intravenous immunoglobulin, highlights the enigmatic and unpredictable aspects of osmotic myelinolysis.

摘要

“桶人”综合征在渗透性脱髓鞘后鲜有报道。我们报告一例45岁女性,因败血症和严重低钠血症入院。她出现了“桶人”综合征,在低钠血症快速纠正10多天后发生。有脑桥中央和脑桥外脱髓鞘的影像学证据。在完成一个疗程的静脉注射免疫球蛋白治疗(0.4 g/kg体重/天,共5天)后三天,病情有显著改善(扩展残疾状态量表评分提高了30%)。该病例因其独特的发展模式、不寻常的表现以及对静脉注射免疫球蛋白具有挑战性的治疗反应而被报道,凸显了渗透性脱髓鞘的神秘和不可预测性。

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