Susac syndrome is a rare immune-mediated endotheliopathy affecting the microvasculature. It presents three main symptoms: encephalopathy, branch retinal artery occlusions, and hearing loss . Here we present a new case report focusing on the evolution of the cognitive profile. The patient underwent two neuropsychological examinations. The first, one month after the onset of the symptomatology, highlighted a prevalent involvement of verbal executive functions. The second, conducted six months later, revealed a global improvement in most previously deficient areas, although with the persistence of a difficulty in cognitive estimation. . This case illustrates the importance of a comprehensive analysis of patients with Susac syndrome to appreciate the whole range of cognitive deficits and reliably evaluate symptom evolution.
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http://dx.doi.org/10.1080/13554794.2024.2366457 | DOI Listing |
Rheumatology (Oxford)
January 2025
Department of Pédiatrie Générale, Assistance Publique et Hôpitaux de Marseille (AP-HM), Marseille, France.
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.
View Article and Find Full Text PDFCureus
November 2024
Neurology, King's College Hospital, Dubai, ARE.
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 PDFNeurol Neuroimmunol Neuroinflamm
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 PDFEur J Neurol
January 2025
Department of Internal Medicine, Hospital Bichat-Claude Bernard, Assistance Publique Hôpitaux de Paris, Université Paris Cité, Paris, France.
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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