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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
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Tests & Diagnosis

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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

  1. Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; Accessed 1/1/1900.
  2. Do TH, Fisch C, Evoy F. AJNR Am J Neuroradiol. 2004 Mar; Accessed 12/18/2008.
  3. Susac JO. AJNR Am J Neuroradiol. 2004 Mar; Accessed 12/18/2008.
  4. Sandhya V, Anand N. Eye. 2002 Nov; Accessed 12/18/2008.