Abstract
Susac's syndrome is a rare disease attribuited to a microangiopathy involving the arterioles of the cochlea, retina and brain. Encefalopathy, hearing loss, and visual deficits are the hallmarks of the disease. Visual loss is due to multiple, recurrent branch arterial retinal occlusions. We report a case of a 20-year-old women with Susac syndrome presented with peripheral vestibular syndrome, hearing loss, ataxia, vertigo, and vision loss due occlusion of the retinal branch artery.
Highlights
Susac’s syndrome (SS) is a rare disease characterised by the clinical triad of vision disturbances, encephalopathy, and sensorineural hearing loss
Susac’s Syndrome consists of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss due to a microangiopathy involved the vessel of the brain, cochlea and retina
There is a female predominance of 3 to 1, and the age extending from 16 years to 58 years[7]
Summary
Relatamos o caso de uma paciente de 20 anos com síndrome de Susac apresentando síndrome vestibular periférica, perda auditiva, vertigem, ataxia e baixa da acuidade visual por oclusão de ramo arterial de retina. Susac’s syndrome (SS) is a rare disease characterised by the clinical triad of vision disturbances, encephalopathy, and sensorineural hearing loss. Due recurents brach retinal artery occlusions (BRAO), presenting with scotoma is one of the clinical hallmarks often not predominant.
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