Susac's syndrome: An immune mediated endotheliopathy laden with challenges and controversies
Malhotra, A., Reyneke, E. and Needham, M. (2013) Susac's syndrome: An immune mediated endotheliopathy laden with challenges and controversies. BMJ Case Reports, 2013 .
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Susac's syndrome is a microangiopathy of the retina, inner ear and brain manifesting as a triad of encephalopathy, hearing loss and branch retinal artery occlusion. The pathological mechanism is thought to be an immune-mediated small vessel vasculitis with some pathophysiological similarity to dermatomyositis. Awareness and early recognition of this syndrome is important as early treatment with immunosuppression can minimise cognitive, audiological and visual sequelae. We report a case of a 33-year-old woman who presented with the characteristic syndrome. She was treated with immunomodulatory therapy and remains well 2 years postpresentation with no new events.
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