Neurológia pre prax 6/2020

Susac’s syndrome

Susac’s syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, arterial retinal occlusion, and neurosensory hearing loss based on microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by a typical radiological finding on MRI, which, together with clinical symptoms, may allow diagnosis. Retinal artery occlusion is best evaluated by fluorescein angiography, which may exhibit typical multifocal fluorescence. SS is an autoimmune endothelopathy that requires longterm treatment with corticosteroids, immunosuppressants, or even monoclonal antibodies, in acute cases intravenous immunoglobulin or plasmapheresis. Therapy leads to significant clinical and radiological improvement. Early diagnosis and treatment is important for delaying disease progression and preventing permanent disability. The article is illustrated by a case report of a woman with typical SS manifestations.

Keywords: Susac´s syndrome, encephalopathy, branch retinal artery occlusion, sensorineural hearing loss