Susac's Syndrome.
Susac's syndrome is Autoimmune Endotheliopathy characterized by three main problems:
impaired brain function (encephalopathy),
partial or complete blockage (occlusion) of the small arteries that supply blood to the retina (branch retinal artery occlusion, or BRAO),
inner ear disease (hearing loss, most notably).
impaired brain function (encephalopathy),
partial or complete blockage (occlusion) of the small arteries that supply blood to the retina (branch retinal artery occlusion, or BRAO),
inner ear disease (hearing loss, most notably).
(Cotton wool like spots seen in corpus callosum)
Three main forms of Susac's syndrome have become apparent.
1)In one form, encephalopathy is the main problem,resolves spontaneously (often going away within 1-3 years).
1)In one form, encephalopathy is the main problem,resolves spontaneously (often going away within 1-3 years).
2) In the second form, BRAO and hearing loss are the main problems, and there is little or no brain disease.
3) In the third form, encephalopathy is the main problem in the beginning, but recurrent episodes of BRAO and hearing loss become the main problem after the encephalopathy goes away.
The other forms tend to follow a more prolonged, more chronic course (3-10 years, or more).
All forms require immunosuppressive treatment while the disease is active.
Most often misdiagnosed with MS and ADEM.
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