- 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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Affected people 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 the condition, 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 people experience recurrent episodes of symptoms. Although it may resolve on its own, some people can develop long-term neurological damage, hearing loss, or vision loss.
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.
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.
The disease is usually self-limiting, and it often stabilizes in 2-4 years. However, it often results in various cognitive, visual and/or hearing problems.
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.
- Eggers SDZ, Zee DS. Central Vestibular Disorders. In: Cummings et al. eds. Otolaryngology: Head & Neck Surgery, 4th ed. Philadelphia, Pennsylvania: Mosby, Inc; 2005;
- Rennebohm, Robert; Susac, John. Journal of Neurological Sciences. February 26,2007; http://www.ncbi.nlm.nih.gov/pubmed/17324441.
- 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.
- Sandhya V, Anand N. Eye. 2002 Nov; http://www.nature.com/eye/journal/v16/n6/pdf/6700170a.pdf. Accessed 12/18/2008.
- Susac JO, Egan RA, Rennebohm RN, Lubow M. J Neurol Sci. June 15, 2007; http://tinyurl.com/yph8kg. Accessed 12/18/2008.