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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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Symptoms


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What are the signs and symptoms of Susac syndrome?

The specific symptoms and severity of Susac syndrome can vary from one person to another. Headache is often one of the earliest symptoms of Susac syndrome.  Recurrent headaches are almost always associated with encephalopathy.[1][2] Other neurological symptoms may develop including walking difficulties, slurred speech (dysarthria), and cognitive changes including memory loss, confusion and personality or behavioral changes. 

Patients may also have branch retinal artery occlusions (BRAO) usually in both eyes. Impairment of vision can occur in some cases; in other cases, people may have no symptoms with the presence of BRAO.

Low-frequency hearing loss can develop due to damage to the cochlea because of poor circulation; ringing in the ears (tinnitus) may also be present. [3]

The three main symptoms (encephalopathy, branched retinal arterial occlusions, and hearing loss) are not always present at the onset of symptoms and all three do not necessarily develop in all cases.

Susac syndrome can go away on its own, even without treatment.  It usually runs its course in two to three years in which individuals experience recurrent episodes of symptoms.  Although Susac syndrome may resolve on its own, some people can develop persistent neurological damage, hearing or vision loss.[4]
Last updated: 4/25/2011

References
  1. 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.
  2. Susac JO. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/351. Accessed 12/18/2008.
  3. Eggers SDZ, Zee DS. Central Vestibular Disorders. In: Cummings et al. eds. Otolaryngology: Head & Neck Surgery, 4th ed. Philadelphia, Pennsylvania: Mosby, Inc; 2005;
  4. Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441. Accessed 1/1/1900.