Susac syndrome: microangiopathy of the retina, cochlea and brain.
VP Saw, PA Canty, CM Green, RJ Briggs, PD Cremer, B Harrisberg, P McCluskey, J O'Day, M Paine, D Wakefield, JD Watson
Clin Exp Ophthalmol | Published : 2000
BACKGROUND: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. METHODS: Analysis of two male and two female cases of Susac syndrome recognized in Australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus ..View full abstract