Saw V P, Canty P A, Green C M, Briggs R J, Cremer P D, Harrisberg B, McCluskey P, O'Day J, Paine M, Wakefield D, Watson J D
Department of Ophthalmology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Clin Exp Ophthalmol. 2000 Oct;28(5):373-81. doi: 10.1046/j.1442-9071.2000.00345.x.
Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea.
Analysis of two male and two female cases of Susac syndrome recognized in Australia.
In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis.
Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.
Susac综合征的特征为视网膜分支动脉阻塞、脑病和耳蜗微血管病三联征。其潜在过程被认为是一种小血管血管炎，导致视网膜、脑和耳蜗出现微梗死。
对在澳大利亚确诊的2例男性和2例女性Susac综合征病例进行分析。
本系列研究对Susac综合征的流行病学、临床表现方式、眼科特征、神经和耳蜗前庭特征、放射学特征、脑脊液检查结果、治疗干预、临床病程及转归进行了研究。主要的眼科鉴别诊断包括系统性红斑狼疮（SLE）、白塞病以及其他血管炎，如结节病、结核病、梅毒和淋巴瘤。神经耳科特征最常被误诊为多发性硬化症。
Susac综合征于1979年首次被描述，如今越来越被人们所认识。早期识别该综合征很重要，因为采用全身免疫抑制治疗可将永久性认知、听力和视觉后遗症降至最低。
