Retinocochleocerebral vasculopathy

Evidence: Unconfirmed

Names

Susac syndrome
Susac’s syndrome
Retinocochleocerebral vasculopathy
RED-M (retinopathy-encephalopathy-deafness associated microangiopathy)
SICRET (small infarctions of cochlear, retinal and encephalic tissue)

Description

Susac’s syndrome affects three parts of the body: the brain, the retina of the eye, and the inner ear. The cause is uncertain, but the disease is believed to be autoimmune.  Swelling in the lining of the blood vessels in these three areas blocks and decreases blood flow, which results in impaired brain function and vision/ hearing loss.  Symptoms are typically unstable as the disease progresses, and patients often experience disturbance in only 2 of the 3 areas at any given time. 

The severity of cases may vary, with mild cases resolving in under a year and severe cases having longer duration and causing irreparable damage.  During the course of the disease, some patients may experience symptoms in a flare-remission pattern, while others are consistently symptomatic until time of recovery.  Treatment is available to prevent permanent damage, and most patients under medical care can expect to experience complete or near complete recovery.

Patient Groups

Facebook: Susac Syndrome Support Group
Creaky Joints

Bloggers

None found.