van der Kooij S M, van Buchem M A, Overbeek O M, Dijkman G, Huizinga T W J
Department of Rheumatology, Haga Hospital, The Hague, the Netherlands, Departments of Radiology, Neurology, Ophthalmology, Rheumatology, Leiden University Medical Centre, Leiden, the Netherlands, *corresponding author: email:
Neth J Med. 2015 Jan;73(1):10-6.
In Susac syndrome, occlusions of pre-capillary arterioles of the brain, retina, and cochlea lead to the classical clinical triad of subacute encephalopathy, visual disturbances due to branch retinal artery occlusions and sensorineural hearing impairment. Its pathogenesis is still obscure, but it is presumed to be mediated by an autoimmune response to an as yet unknown antigen. The syndrome is considered a rare but important differential diagnosis in various neurological, psychiatric, ophthalmological, and ear-nose-throat disorders. Brain magnetic resonance imaging, retinal fluorescein angiography, and audiometry findings enable diagnosis. Early therapy may reduce relapses and improve recovery. The features of four cases of this syndrome are presented, illustrating that cooperation among different medical specialists is essential, and that treatment may be best guided by an immunologist or rheumatologist as a case manager.
在Susac综合征中，脑、视网膜和耳蜗的毛细血管前小动脉闭塞导致典型的临床三联征，即亚急性脑病、视网膜分支动脉闭塞引起的视觉障碍和感音神经性听力损害。其发病机制仍不清楚，但推测是由针对一种未知抗原的自身免疫反应介导的。该综合征在各种神经、精神、眼科和耳鼻喉疾病中被认为是一种罕见但重要的鉴别诊断。脑磁共振成像、视网膜荧光血管造影和听力测定结果有助于诊断。早期治疗可能会减少复发并促进恢复。本文介绍了4例该综合征的病例特点，说明不同医学专科之间的合作至关重要，且治疗最好由免疫学家或风湿病学家作为病例管理者来指导。
