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Susac-Syndrom: Eine interdisziplinaere Herausforderung [Susac syndrome: an interdisciplinary challenge]

Item Type:Review
Title:Susac-Syndrom: Eine interdisziplinaere Herausforderung [Susac syndrome: an interdisciplinary challenge]
Creators Name:Doerr, J. and Jarius, S. and Wildemann, B. and Ringelstein, E.B. and Schwindt, W. and Deppe, M. and Wandinger, K.P. and Promesberger, J. and Paul, F. and Kleffner, I.
Abstract:Susac syndrome, named after John Susac, the first to describe this condition, is characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Although certainly a rare disease, Susac syndrome needs to be considered in the differential diagnosis of a broad variety of diseases. The pathogenesis is not yet clear. Autoimmune processes leading to damage and inflammation-related occlusion of the microvessels in brain, retina, and inner ear are thought to play a causal role. The diagnosis is based primarily on the clinical presentation, the documentation of branch retinal artery occlusion by fluorescence angiography, and characteristic findings on cerebral MRI. Usually, immunosuppressive therapy is required, though controlled therapy trials are missing so far. The intention of this review article is to raise awareness of this disease among neurologists, psychiatrists, ophthalmologists, and ENT specialists as a high number of unreported cases probably exists. Accordingly, the focus is on the clinical presentation and the diagnostic approach.
Keywords:Susac Syndrome, Encephalopathy, Branch Retinal Artery Occlusion, Sensorineural Deafness, Microangiopathy
Page Range:1250-1263
Date:October 2011
Official Publication:https://doi.org/10.1007/s00115-011-3280-0
PubMed:View item in PubMed

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