We describe a 25-year-old woman that presented with frequent rotational dizziness, visual loss of the right eye one month later, and unilateral deafness one year after. After 2 years, she presented with a right hemiparesis and deafness greater for low frequency tones. Magnetic resonance imaging with angiography of the brain showed constriction in the first segment of the cerebral anterior artery diagnosed as Susac's syndrome.
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Susac's Syndrome: A Tale of Disability Due to Late Recognition.
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Susac's syndrome is a rare inflammatory microangiopathy characterized by the triad of retinopathy, encephalopathy, and hearing loss. The syndrome causes recurrent microinfarcts in these organs, which in turn manifests with repeated attacks of visual field loss, hearing loss and tinnitus, and various brain syndromes. These often lead to the significant accumulation of disability over time, particularly if there is a delay or failure in diagnosis.
View Article and Find Full Text PDFClinical Characterization and Prognostic Risk Factors of Susac Syndrome: A Retrospective Multicenter Study.
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March 2025
The Faculty of Medical & Health Sciences, Tel Aviv University.
Background And Objectives: Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. Recent updates to diagnostic criteria and treatment guidelines have been made. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to work in patients with SuS.
View Article and Find Full Text PDFBrain microstructural damage through serial diffusion tensor imaging and outcomes in Susac syndrome: A prospective cohort study.
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Department of Internal Medicine, Hospital Bichat-Claude Bernard, Assistance Publique Hôpitaux de Paris, Université Paris Cité, Paris, France.
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