This may account for the low prevalence of the illness. There is also a pathological similarity between the endotheliopathy in Susacs syndrome with that seen in juvenile dermatomyositis. Treatment
Early and aggressive treatment is important to prevent irreversible neurological damage, hearing loss, or vision loss. Medications used include immunosuppressive agents and corticosteroids such a prednisone, or intravenous immunoglobulins (IVIG). Other drugs that have been used are mycophenolate mofetil (Cellcept), azathioprine (Imuran), cyclophosphamide, rituximab, and anti-TNF therapies.Hearing aids or cochlear implants may be necessary in the event of hearing loss. References

Egan RA, Nguyen TH, Gass JDM, Rizzo JF, Tivnan J, Susac JO. Retinal Artery Wall Plaques in Susac Syndrome. American Journal of Ophthalmology  135: 483-6; 2003
Susac JO, Murtagh FR, Egan RA, Berger JR, Fox RJ, Galetta S, Costello F, Lee AG, Bakshi R, Lincoff N, Clark J, Daroff R.  MRI Findings in Susac Syndrome. Neurology  61: 1783-1787; 2003
Egan RA, Hills WL, Susac JO. Gass Plaques and Fluorescein Leakage in Susac Syndrome. Journal of Neurological Sciences  299(1-2): 97-100; 2010
Susac JO, Rennebohm RM, Egan RA, Daroff RB. Susac’s Syndrome – Update. Journal of Neurological Sciences  299(1-2): 86-91; 2010
Susac JO. Susac Syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology  44(4): 591-3; 1994
Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, Aktas O, Ringelstein EB, Paul F, Kleffner I.  Characteristics of Susac Syndrome: a review of all reported cases.
One small, possibly biased study found that the condition was eventually diagnosed in 45% of outpatients with sudden headache, and 46% of outpatients with thunderclap headache.The average age of onset is 42, but RCVS has been observed in patients aged from 19 months to 70 years. Children are rarely affected. It is more common in females, with a female-to-male ratio of 2.4:1. History
Case studies of the condition first appeared in the 1960s, but it was not then recognized as a distinct entity. In 1983, French researchers published a case series of 11 patients, terming the condition acute benign cerebral angiopathy. Gregory Call and Marie Fleming were the first two authors of a report in which doctors from Massachusetts General Hospital, led by C. Miller Fisher, described 4 patients, alongside 12 previous case studies, with the characteristic symptoms and abnormal cerebral angiogram findings. The name Call-Fleming syndrome refers to these researchers.A 2007 review by Leonard Calabrese and colleagues proposed the name reversible cerebral vasoconstriction syndrome, which has since gained widespread acceptance. This name merges various conditions that were previously treated as distinct entities, including Call-Fleming syndrome, postpartum angiopathy, and drug-induced angiopathy. Other names may still be used for particular forms of the condition. References


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