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Diseases

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.

Treatment

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How might Susac syndrome be treated?

Early, aggressive, and sustained treatment of Susac syndrome is recommended to avoid or minimize neurological damage, hearing loss, and/or vision loss. Treatment includes medications that suppress the activity of the immune system (immunosuppressive therapy) in the acute period. This may include high doses of steroids, cyclophosphamide, methotrexate, azathioprine and/or mycophenolate mofetil, as well as intravenous immunoglobulins.[1][2][3] After the acute period, treatment is continued with a slow tapering of corticosteroids. Alternative drugs such as rituximab, etanercept or cyclosporine A may be helpful in cases that don't improve with other treatments. Affected people with severe hearing loss may benefit from cochlear implants.[3]

The disease is usually self-limiting, and it often stabilizes in 2-4 years. However, it often results in various cognitive, visual and/or hearing problems.[3]
Last updated: 7/21/2014

References
  1. Susac JO, Egan RA, Rennebohm RN, Lubow M. J Neurol Sci. June 15, 2007; http://tinyurl.com/yph8kg. Accessed 12/18/2008.
  2. Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441.
  3. Jan-Markus Dorr. Susac syndrome. Orphanet. September, 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=838. Accessed 7/21/2014.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Susac syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research.  Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. Currently there is a research study titled Endothelial-Reactive Antibodies: A diagnostic test for Susac's syndrome. Click on the study title for further information on the study.
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.