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[年轻患者的视网膜、耳蜗及脑小梗死,Susac综合征的“SICRET”综合征]

[Small retinal, cochlear, and cerebral infarctions in the young patient, "SICRET" syndrome of Susac syndrome].

作者信息

Mala L, Bazard M C, Berrod J P, Wahl D, Raspiller A

机构信息

Service d'Ophtalmologie du Pr. A. Raspiller, Hôpital Central, Nancy.

出版信息

J Fr Ophtalmol. 1998 May;21(5):375-80.

PMID:9759431
Abstract

A 22-year-old-lady presented with multiple occlusions of the branches of the central retinal artery, accompanied by neuro-encephalic disorders and deafness. This triad is known as SICRET Syndrome (Small Infarction of Cochlear, Retinal and Encephalic Tissue). This rare syndrome, as well referred to as Susac syndrome, affects only the women and the three tissues mentioned above: eye, ear, brain. The course was characterised by a series of partially regressive evolutive steps. A remission had been obtained since two years with immuno-supressor and anti-coagulant therapy. The neuro-encephalic and cochlear disorder regressed in contrast to the severe sequel on the right eye.

摘要

一名22岁女性出现视网膜中央动脉分支多处闭塞,并伴有神经脑病和耳聋。这一三联征被称为SICRET综合征(耳蜗、视网膜和脑组织小梗死)。这种罕见综合征,也被称为苏萨克综合征,仅影响女性以及上述三种组织:眼睛、耳朵、大脑。病程的特点是一系列部分可逆的进展阶段。经过两年的免疫抑制和抗凝治疗后病情缓解。神经脑病和耳蜗疾病有所好转,但右眼留下了严重后遗症。

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