Susac syndrome: remember to see and hear

Address for correspondence: Elisangela Ferraz Pazinato (email: [e-mail]).

Abstract

Case Presentation: We herein report the case of a 23-year-old male patient, with complaints 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. On a magnetic resonance imaging scan of the skull, there was evidence of involvement of the corpus callosum by “snow ball”; on arteriography, there was 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. The clinical presentation and results of the complementary exams were related with Susac syndrome. Immunosuppression was performed with corticosteroids, azathioprine, and immunoglobulin, with partial response. After two months, the patient evolved with short- and long-term memory changes, headache, paresthesias, and 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, which, 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, which 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 propedeutics.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
02 October 2024