Papo T, Biousse V, Lehoang P, Fardeau C, N'Guyen N, Huong D L, Aumaitre O, Bousser M G, Godeau P, Piette J C
Department of Internal Medicine, Hôpital Pitié-Salpêtrière, Paris, France.
Medicine (Baltimore). 1998 Jan;77(1):3-11. doi: 10.1097/00005792-199801000-00002.
Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some infarcts may be asymptomatic and only revealed by ancillary investigation: 1) audiogram that shows bilateral sensorineural hearing loss predominating on low frequencies, 2) funduscopy and fluorescein retinal angiography demonstrating bilateral distal branch retinal artery occlusions, and 3) brain MRI T2-weighted images disclosing small multifocal hyperintensities in white and gray matter. Treatment options are not codified, ranging from antithrombotic drugs to immunomodulatory therapy. Course is self-limited after an active fluctuating phase. Dementia, blindness, and deafness are rare late sequelae, and half of patients return to normal life.
Susac综合征是一种闭塞性小动脉疾病，可导致年轻患者（主要为女性）的耳蜗、视网膜和脑部发生梗死。其病因不明。一些梗死可能无症状，仅通过辅助检查发现：1）听力图显示以低频为主的双侧感音神经性听力损失；2）眼底镜检查和荧光素视网膜血管造影显示双侧视网膜远端分支动脉闭塞；3）脑部MRI T2加权图像显示白质和灰质中有小的多灶性高信号。治疗方案尚无统一标准，从抗血栓药物到免疫调节治疗不等。在活跃的波动期后病程呈自限性。痴呆、失明和失聪是罕见的晚期后遗症，一半的患者可恢复正常生活。
