Susac syndrome--clinical insight and strategies of therapy.

Susac syndrome is an uncommon autoimmune microangiopathy characterized mainly by neurological disorders and, to date, 304 clinical cases have been described. The background of this syndrome is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear resulting in encephalopathy, hearing loss, and branch retinal artery occlusions. However, the cause and the pathogenesis of this microangiopathy remain unclear. Magnetic resonance imaging, retinal fluorescein angiography, and audiography findings enable the diagnosis of this syndrome. In this review, we have demonstrated the epidemiology and pathology of Susac syndrome with detailed description of clinical signs, diagnostic procedures and therapeutic possibilities.