Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years

Susac syndrome (retinocochleocerebral vasculopathy) is a syndrome of unknow n pathogenesis. The triad of multifocal encephalopathy, visual loss, and he aring loss is caused by microangiopathy of the brain, retina, and cochlea. The illness tends to be monophasic, and to our knowledge, recurrence after years of remission has not been reported. We describe a 51-year-old woman w ith symptoms, signs, and brain magnetic resonance imaging findings consiste nt with recurrence of Susac syndrome 18 years after remission. Clinicians s hould be aware of the possibility of late recurrence of Susac syndrome when evaluating patients with a distant history of the syndrome who present wit h complaints referable to the brain, retina, and cochlea.