Susac Syndrome: Report of the first case in Honduras

Abstract

Background: Susac syndrome is a rare entity, described as a clinical triad of encephalopathy, sensorineural deafness and visual disorder due to occlusion of retinal artery branches, although it is not always presented as a triad, it is characterized by prolonged evolution and not necessarily sequential or correlated with the clinical presentation, which is variable, so the diagnosis can be late and influence the prognosis. It’s more common in women. The pathogenesis is considered as an microangiopathic autoimmune disease of cerebral, retinal and cochlear vessels. Clinical case description: A case of a 36-yearold male patient is presented, with headache, behavioral changes, motor and sensory deficit of the right body, followed by visual disturbances and decreased auditory perception. Diagnosis was made with cerebral Magnetic resonance imaging with rounded hyperintense lesions in the corpus callosum and cortico-subcortical regions; audiometry with sensorineural deafness; and retinal fluorescein angiography with retinal artery obstruction, confirming this last resource the diagnosis of Susac syndrome. Conclusions: This is probably an underdiagnosed disease, where we should be alert to consider within the differential diagnosis of cerebrovascular diseases and demyelinating disease, the possibility of Susac syndrome as etiology, and establish the treatment, that should be aggressive and early to minimize lesions.