| Names |
- Retinocochleocerebral vasculopathy
- Retinopathy-encephalopathy-deafness associated microangiopathy
- Small infarctions of cochlear, retinal and encephalic tissue
Category: Vascular system |
| Prevalence |
Extremely rare. Fewer than 100 cases in the US (Dörr 2013). Female/Male ratio: 11.0x. |
| Age of Onset |
Onset typically occurs between ages 20 and 40. |
| 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.
Evidence of autoimmunity: Antibody Suspected
|
| Patient Groups |
|
| Bloggers |
|
| Symptoms |
|
| Resources |
Link to Clinical Trials
Link to PubMed |
| References |
Link to Mayo Clinic
Link to Cleveland Clinic
Link to Healthline
|
|
Profile by: Fabliha HussainProfile updated September 2024Charts generated Oct 12, 2024 at 12:54 PM ET |
|
Retinocochleocerebral vasculopathy sex breakdown
Please wait while the Retinocochleocerebral vasculopathy sex breakdown results are loaded
Retinocochleocerebral vasculopathy age breakdown
Please wait while the Retinocochleocerebral vasculopathy age breakdown results are loaded
|
