Abstract

The clinical triad of branch retinal artery occlusions, hearing loss and encephalopathy constitutes Susac's Syndrome. This rare, immune mediated, microangiopathic disorder shows a variable course with a preponderance of 3 to 1 for women, mainly in their 2nd to 4th decade. The condition relies on diagnosis and early aggressive immunosuppressive therapy to avoid severe sequale. Unfortunately, due to the paucity of experience, and a variable disease course, often lacking all elements of the triad concurrently, diagnosis continues to be delayed.

We present five cases (Age 34–45, M:F–1:4) seen at our regional neurosciences centre, which highlight the diagnostic differences and challenges and the variety of management techniques adopted. Three patients had a monophasic illness and two had a relapsing remitting course. In addition, we provide an up-to-date literature review on the pathophysiology, diagnosis and management of this rare and fascinating condition.

Susac's disease, otherwise described as retinocochleocerebral vasculopathy, RED-M (retinopathy, encephalopathy, deafness associated microangiopathy) and SICRET (small infarcts of cochlear, retinal and encephalic tissues), is a syndromic pathology requiring the recognition of a triad of deficits to make a diagnosis. The relevance of this and the difficulty in diagnosis is exemplified in the cases presented here.