Susac syndrome: A rare presentation

Susac syndrome is an autoimmune microangiopathy characterized by a triad of encephalopathy, sensori-neural hearing loss and varying degrees of branch retinal artery occlusion. A young male patient is described with features of focal neurological lesions and small superficial retinal haemorrhages and areas of retinal arteriolar endothelial inflammation appearing as Gass plaques. Susac syndrome presents with varying features involving brain, inner ear and retina. Fundus Fluorescein angiography should be performed in all patients with an unexplained encephalopathy to look for characteristic arteriolar wall changes.
 

Keywords: Susac Syndrome, Branch retinal artery occlusion, Gass plaques, Encephalopathy