Susac syndrome: The effectiveness of Rituximab monotherapy

Susac syndrome (SS) or retino-cochleo-cerebral vasculopathy is an extremely rare, severe, and potentially disabling condition. Underlying occlusive microangiopathy in SS clinically characterized by the triad of encephalopathy, sensorineural hearing loss branch retinal arterial occlusion. therapy envisages simultaneous use high doses glucocorticoids, intravenous immunoglobulins, cyclophosphamide rituximab (RTХ). This article presents a case remitting-relapsing slow-progressive with typical clinical manifestations demonstrating successful treatment RTХ monotherapy; it also discuss focus monotherapy should be targeted at cases contraindications for glucocorticoids cytostatics use, early stages.