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Spinal cord involvement in Susac Syndrome: a unique paediatric case.

Rheumatology (Oxford)

January 2025

Department of Pédiatrie Générale, Assistance Publique et Hôpitaux de Marseille (AP-HM), Marseille, France.

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Susac is a rare systemic disease characterized by ischemic events involving the cochlea, brain, and retina. Delay in the diagnosis leads to sight-threatening complications such as neovascular glaucoma.

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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.

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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.

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Background: Susac syndrome (SuS) is a rare immune-mediated microangiopathy with potential disabling evolution. We aimed to analyze brain microstructural damage through diffusion tensor imaging (DTI) in SuS and determine its association with poor outcomes.

Method: CarESS study is a prospective multicenter national cohort study of patients with SuS.

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