Susac's syndrome or retinocochleocerebral vasculopathy: a misdiagnosed and overlooked disorder.

Abstract

We report a case of Susac's syndrome (retinocochleocerebral vasculopathy) that was initially successfully treated with steroids. The patient had a recurrence of the disorder 2 years after the onset of the first clinical manifestations; this was treated with steroids and both calcium channel blockers and aspirin. Our findings underline the importance of recognizing patients with retinocochleocerebral vasculopathy at an early stage so that it may be accurately managed and, therefore, result in both decreased morbidity and disability related to severe sequelae. We also stress the need for patients with Susac's syndrome to have both close and long-term follow-up in order to disclose both symptomatic and asymptomatic recurrences of the disorder.