Susac syndrome: microangiopathy of the retina, cochlea and brain

Background: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The under lying process is believed to be a small vessel vasculitis causing microinfa rcts in the retina, brain and cochlea. Methods: Analysis of two male and two female cases of Susac syndrome recogn ized in Australia. Results: in this series the epidemiology, mode of presentation, ophthalmolo gic features, neurologic and cochleovestibular features, radiologic charact eristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic diffe rential diagnoses include systemic lupus erythematosis (SLE), Behcet's synd rome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as mult iple sclerosis. Conclusion: Susac syndrome, first described in 1979, is becoming an increas ingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent co gnitive, audiologic and visual sequelae.