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Genetic and Rare Diseases Information Center (GARD)

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Susac syndrome


Other Names for this Disease

  • Retinocochleocerebral vasculopathy
  • SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Susac syndrome is an autoimmune condition that affects very fine blood vessels in the brain, retina, and cochlea (inner ear).[1][2] It is characterized by three main symptoms: encephalopathy (which can cause headache, mild memory loss, 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 used to treat Susac syndrome.[3] However, the extent to which individuals respond to treatment is not clear.[1] Improvement may occur spontaneously although in some cases there may be residual dysfunction.[1]
Last updated: 7/21/2014

References

  1. Susac's Syndrome. National Organization for Rare Disorders (NORD). December 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Susac%27s%20Syndrome. Accessed 4/25/2011.
  2. 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 4/25/2011.
  3. Aubart-Cohen, et.al.. Long-Term Outcome in Susac Syndrome. Medicine. March 2007;
Your Questions Answered
by the Genetic and Rare Diseases Information Center

5 question(s) from the public on Susac syndrome have been answered. See questions and answers. You can also submit a new question.

Basic Information

  • The University of Calgary provides general information on Susac syndrome for¬†patients, families, their friends, and health care professionals.
  • 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.

In Depth Information

  • Orphanet is a European reference portal for¬†information on rare diseases and orphan drugs.¬† Access to this database is free of charge.
  • 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.
Other Names for this Disease
  • Retinocochleocerebral vasculopathy
  • SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.