Management of Susac Syndrome

Abstract

Introduction: Susac syndrome is a disease with autoimmune microangiopathic etiology of vascular endothelia that causes occlusion of arterial branches. Due to its high diagnostic complexity, it cannot be detected at the onset of the syndrome because most patients do not present the characteristic triad at a certain time, they start their picture with neurological manifestations which implies several differential diagnoses. Aim: To describe the correct management of Susac Syndrome based on the main diagnostic criteria and the efficacy of pharmacological treatment. Methods: A literature review study of articles from 2017 to 2022 on Susac syndrome was conducted in the following databases: Scopus, SciELO, Pubmed, Cochrane and Lilacs. Results: This disease is characterized by the presence of a pathognomonic triad of encephalopathy, sensorineural hearing loss and retinal arterial occlusion. Consequently, it is vital to use different diagnostic methods: laboratory data, imaging studies and audiometry. Treatment regimens have been proposed, establishing corticosteroids and immunomodulators as the first line of treatment. When considering pharmacological treatments, not only the severity of SS must be contemplate but also the presence of comorbidities and characteristics according to each patient in order to define the appropriate pharmacological scheme for each patient. Conclusions: Audiometric examinations are considered fundamental for the diagnosis since sensorineural hearing loss can be evidenced in low and medium frequencies, it is important to perform surgical interventions with the purpose of avoiding the progression of the syndrome.