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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
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Tests & Diagnosis

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How is Susac syndrome diagnosed?

The diagnosis of Susac syndrome may be suspected based on a thorough clinical evaluation, a detailed medical history, and a variety of specialized tests including magnetic resonance imaging (MRI) and fluorescein angiography.

In people with Susac syndrome, an MRI can show characteristic changes in the brain, especially the corpus callosum. A complete eye exam, including a flourescein angiography, usually also shows specific abnormalities. Other tests include a hearing exam (which may show hearing loss) and analysis of cerebrospinal fluid.[1][2]    
Susac syndrome can mimic several diseases, including multiple sclerosis, acute disseminated encephalomyelitis, chronic encephalitis, aseptic meningitis, Lyme disease, cardioembolic disorder, complicated migraine, systemic lupus erythematosus, Bechet syndrome, sarcoidosis, tuberculosis, syphilis, lymphomas, and Creutzfeldt-Jakob disease.[3][4][5]
Last updated: 7/21/2014

References
  1. Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441.
  2. 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.
  3. Do TH, Fisch C, Evoy F. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/382. Accessed 12/18/2008.
  4. Susac JO. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/351. Accessed 12/18/2008.
  5. Sandhya V, Anand N. Eye. 2002 Nov; http://www.nature.com/eye/journal/v16/n6/pdf/6700170a.pdf. Accessed 12/18/2008.


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.