以脑病和顽固性呃逆为特征的复发性Susac综合征

Reza Vosoughi , Nupura Bakshi , Prem A.H Nichani , James J Marriott , Elaheh Shahmiri
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引用次数: 0

摘要

Susac综合征是一种罕见的自身免疫性内皮病,其特征是大脑、视网膜和耳蜗的小动脉闭塞,表现为脑病、视网膜血管闭塞性疾病和听力损失。该疾病可以是单相或复发性的,症状多种多样,大多数患者没有典型的三联征,尤其是在最初的临床表现中。在中枢神经系统神经炎症性疾病中,顽固性呃逆是NMOSD区后遗症综合征的公认表现。据我们所知,这是第一例以脑病和顽固性呃逆为特征的Susac综合征报告。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Relapsing Susac syndrome presenting with encephalopathy and refractory hiccups

Susac syndrome is a rare autoimmune endotheliopathy characterized by occlusion of small arteries in brain, retina, and cochlea presenting with triad of encephalopathy, retinal vaso-occlusive disease and hearing loss. The disease can be monophasic or relapsing with variety of symptoms and the majority of the patients do not have the classic triad, especially at the initial clinical presentation. In CNS neuroinflammaory disorders, refractory hiccup is a recognized manifestation of NMOSD area postrema syndrome. To our knowledge, this is the first report of Susac syndrome presenting with encephalopathy and refractory hiccup.

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