Other Names for this Disease
- Retinocochleocerebral vasculopathy
- SICRET (small infarction of cochlear, retinal, and encephalic tissue) syndrome
Your QuestionMy girlfriend's physicians think that she may have Susac syndrome. Can you provide us with some more information about this disease, particularly how it is treated and what we might expect in terms of prognosis?
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Questions on this page
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. 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.
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.
The symptoms of this condition result from damage to these very small blood vessels, which in turn results in decreased or impaired blood flow. Impaired blood flow to the brain, retinas and inner ears (microinfarcts) results in damage to the tissue or organs in these areas.
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.
Susac syndrome can mimic several diseases. These conditions may include 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.
For those with significant hearing loss, a specific type of hearing aid called a cochlear implant may be an option.
A study published in March 2007 entitled, Long Term Outcome in Susac Syndrome by Aubart-Cohen, et.al. followed 9 patients with Susac syndrome for more than 6 years. In this series, patients did not suffer from severe cognitive sequelae. All but 1 patient returned to work. Vision was usually not seriously impaired. Most of the patients had bilateral hearing loss, with limited disability.
- Susac's Syndrome. National Organization for Rare Disorders (NORD). December 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Susac%27s%20Syndrome. Accessed 4/25/2011.
- Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO . Pediatr Rheumatol Online J. January 2008; http://www.ped-rheum.com/content/pdf/1546-0096-6-3.pdf. Accessed 4/25/2011.
- Aubart-Cohen, et.al.. Long-Term Outcome in Susac Syndrome. Medicine. March 2007;
- 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.
- Susac JO. AJNR Am J Neuroradiol. 2004 Mar; http://www.ajnr.org/cgi/content/full/25/3/351. Accessed 12/18/2008.
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- Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441. Accessed 1/1/1900.
- Sandhya V, Anand N. Eye. 2002 Nov; http://www.nature.com/eye/journal/v16/n6/pdf/6700170a.pdf. Accessed 12/18/2008.
- Susac syndrome. Orphanet Database. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=838. Accessed 4/25/2011.
- Susac JO, Egan RA, Rennebohm RN, Lubow M. J Neurol Sci. June 15, 2007; http://tinyurl.com/yph8kg. Accessed 12/18/2008.