Susac综合征的临床研究进展

Clinical progress of Susac syndrome

Susac综合征(SS)目前认为是由免疫介导的以脑部、内耳和视网膜相关小血管的闭塞为主要表现的一种罕见病。SS以脑病、视网膜分支动脉闭塞和感觉神经性耳聋三联征为主要表现,但是由于临床症状相继出现,易导致漏诊、误诊。头颅MRI、荧光素眼底血管造影和听力检查有助于SS的早期诊断。SS的治疗较为复杂,需采用联合糖皮质激素、静脉注射Ig、免疫抑制治疗的综合治疗方案。

Susac syndrome(SS)is a rare immune-mediated disorder characterized by occlusion of arterioles associated with the brain,inner ear and retina.The main clinical manifestations of SS are encephalopathy,branch retinal artery occlusion and sensorineural hearing loss.However,they always appear one after another,and always lead to missed diagnosis and misdiagnosis.Brain magnetic resonance imaging,fundus fluorescein angiography and audiometry are helpful for the early diagnosis of SS.Glucocorticoid,intravenous injection of immunoglobulins and immunosuppression are suggested for the comprehensive treatment of SS.