[Susac syndrome - an unusual syndrome which can be mistaken for multiple sclerosis]

Abstract

Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most commonly affects young women. Susac syndrome is most likely underdiagnosed, not the least since only 13% have the clinical triad upon presentation. Many are misdiagnosed with multiple sclerosis or another neuroinflammatory entity. Susac syndrome is a microangiopathy affecting the precapillary arterioles causing infarcts of the brain, retina and inner ear. Beside the clinical symptoms, Susac syndrome is diagnosed by typical radiological features on magnetic resonance imaging and branch retinal artery occlusions, which are best evaluated using fluorescein angiography. Early diagnosis and correct immunosuppressive therapy are of utmost importance for clinical improvement and prevention of permanent disability. Diagnosis and treatment of Susac syndrome requires close cooperation between neurologists, radiologists, ophthalmologists and otorhinologists. Here, we present three cases and a review of the literature.