Susac Syndrome Presenting as Recurrent Strokes

Susac syndrome (SS) is a rare clinical entity that affects primarily young women and might result in significant morbidity. The triad that leads to suspecting the disease has classically been involvement of the brain, retina and inner ear. The likely pathology of the disease is thought to be immune mediated endotheliopathy; given its clinical and, possibly, pathological remission with immunosuppressive therapy. Here we describe an uncommon recurrent stroke in a young female that unfolds to Susac syndrome at the end. We also reviewed the literature behind diagnosis and treatment. Delayed diagnosis is associated with worse morbidity and mortality, and the most important predictor of long-term prognosis in the reported cases is the time to diagnosis. Therapies tried (with variable success) include corticosteroids, IVIG, plasmapheresis, cyclophosphamide, mycophenolate mofetil, and rituximab. The prognosis of SS is difficult to predict given the absence of strong clinical or radiographic features to suggest better/worse prognosis at the time of initial diagnosis. Brain MRIs and hearing/vision impairment have never normalized in previously studied cohort of patients.