Abstract
AbstractSusac's syndrome is a primary vasculitis of the central nervous system. It is a microangiopathy affecting the brain, the inner ear and the retina. Its clinical presentation usually consists in behavior and memory disturbances, hearing loss and visual loss due to multiple branch arteries occlusions. It affects mostly women between 18 and 40 years of age. There are no specific radiological or laboratory abnormalities. Lumbar puncture reveals non specific minimal pleocytosis and elevation of proteins. The differential diagnosis includes granulomatous angiitis of the central nervous system and Cogan's syndrome. The pathogenesis is unknown but immune complex and antibody‐mediated endothelial damage are possible mechanisms. There is no universally recognized therapy, but immunosuppressants are frequently used.
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