Susac's syndrome
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Susac's syndrome (retinocochleocerebral vasculopathy) is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss.[1] The cause is unknown but it is theorized that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness. Despite this being an extremely rare disease, there are four registries collecting data on the illness; two are the United States, one in Germany, and one in Portugal.[2][3][4]
This article needs more reliable medical references for verification or relies too heavily on primary sources. (August 2021) |
Quick Facts Other names, Specialty ...
Susac's syndrome | |
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Other names | Retinopathy-encephalopathy-deafness associated with microangiopathy |
Sagittal T1 image in a 19-year-old woman with Susac's syndrome showing the pathognomonic central callosal "holes" (microinfarcts) of SS. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve. | |
Specialty | Neurology |
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