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Susac’s syndrome is microangiopathy defined by a triad of encephalopathy, sensorineural hearing loss, and multiple branch retinal artery occlusions. First case reports appeared in the literature in 1973 (Weidauer and Tenner 1973; Pfaffenbach and Hollenhorst 1973) and in 1979, Dr. John O. Susac published reports of microangiopathy of the brain and retina in two young women (Susac et al. 1979). Since first reported in the 1970s, approximately 300 case reports have been published on the disease. Susac’s syndrome most commonly affects young women and was initially only thought to affect females, however, it has been reported in males also. A recent systematic case review of all reported cases found a 3.5 to 1 female to male predominance and an average age of onset between 21 and 35 years (Dörr et al. 2013).
- Magro CM. Susac’s syndrome: an autoimmune endotheliopathy. In: 1st Susac symposium held at Ohio State University, Columbus; 2005.Google Scholar