Susac syndrome: remind to see and hear

Case presentation: Man, 23 years old, complaint of dysarthria, paresthesia in the right hand, tinnitus in the right ear, diplopia and sudden scotoma in the left eye, without associated cognitive complaints but with a report of slowing of thought observed by the family. In magnetic resonance imaging of the skull there is evidence of involvement of the corpus callosum by "snow ball", in arteriography there is occlusion of branches of the ophthalmic arteries bilaterally, audiometry showed sensorineural deafness and fluorescent retinography showed occlusion of the arteriolar branch of the right eye and arteriole branches in the lower arch and temporal periphery of the left eye. Clinical presentation and results of complementary exams related with Susac Syndrome. Immunosuppression was performed with corticosteroids, azathioprine and immunoglobulin with partial response. After two months, it evolved with short and long-term memory changes, headache, paresthesias, bilateral tinnitus, and immunosuppression was adjusted for corticosteroids, immunoglobulin and cyclophosphamide with stabilization of the disease. 

Discussion: Susac syndrome is a rare and severe disease, of probable autoimmune etiology, that due to the occlusion of microvessels, causes small infarctions in the central nervous system, inner ear and retina. It is characterized by the clinical triad of encephalopathy, sensorineural hearing loss and visual impairment. The treatment is based on long-term immunosuppression.

Final comments: The diagnosis of Susac Syndrome is challenging given its rarity and the incomplete presentation of the initial phase that expands the possibilities of differential diagnoses which include demyelinating, autoimmune and vascular occlusion diseases. In addition to other neurological pathologies, psychiatric and otorhinolaryngological pathologies are also among the hypotheses to be considered throughout the propaedeutics.