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Other Names for this Disease
- Retinocochleocerebral vasculopathy
- SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
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Susac syndrome is an autoimmune condition that affects very fine blood vessels in the brain, retina, and the cochlea (inner ear) . It is characterized by three main symptoms: encephalopathy (which can include headache, mild memory losses, personality changes, and confusion); sensorineural hearing loss; and vision loss. Most people do not have all the symptoms of Susac syndrome at the beginning of their illness but develop symptoms over the course of several years. Susac syndrome affects women more than men and the age of onset is usually between 20 and 40 years. The cause of this condition is still unknown. Corticosteroids, antiplatelets, anticoagulation, and cyclophosphamide have been frequently used to treat Susac syndrome. However, the extent to which individuals respond to treatment is not clear. Improvement may occur spontaneously although in some cases there may be residual dysfunction.
- Susac's Syndrome. National Organization for Rare Disorders (NORD). http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Susac%27s%20Syndrome. Accessed April 25, 2011.
- Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO . Pediatr Rheumatol Online J. January 2008. http://www.ped-rheum.com/content/pdf/1546-0096-6-3.pdf. Accessed April 25, 2011.
- Aubart-Cohen, et.al.. Long-Term Outcome in Susac Syndrome. Medicine. March 2007.
On this page
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Susac syndrome. Click on the link to view a sample search on this topic.
- The University of Calgary provides general information on Susac syndrome for patients, families, their friends, and health care professionals.