Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

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.

Tests & Diagnosis


Newline Maker

How is Susac syndrome diagnosed?

The diagnosis of Susac syndrome is 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 individuals with Susac syndrome, an MRI can show characteristic changes to the brain, especially the corpus callosum. A complete eye exam, including a flourescein angiography (angiogram) is necessary.  Individuals suspected to have Susac syndrome should also have a hearing exam to detect any hearling loss.[1]    

Susac syndrome can mimic several diseases. These conditions may include multiple sclerosis,[2][3][4] acute disseminated encephalomyelitis,[3] chronic encephalitis,[3][4] aseptic meningitis,[4] Lyme disease,[3] cardioembolic disorder,[3] complicated migraine,[3][4] systemic lupus erythematosus,[4] Bechet syndrome,[4] sarcoidosis,[4] tuberculosis,[4] syphilis,[4] lymphomas,[4] and Creutzfeldt-Jakob disease.[3]
Last updated: 4/25/2011

References
  1. Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441. Accessed 1/1/1900.
  2. 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.
  3. Susac JO. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/351. Accessed 12/18/2008.
  4. Sandhya V, Anand N. Eye. 2002 Nov; http://www.nature.com/eye/journal/v16/n6/pdf/6700170a.pdf. Accessed 12/18/2008.