Susac syndrome (SuS) is a rare poorly characterised disorder that affects the brain, retina, and cochlea. Here, we present a case of a 31-year-old pregnant female with a new diagnosis of SuS that was successfully managed to 36 weeks of gestation with minimal disease burden to both the mother and newborn. She was treated initially using intravenous methylprednisolone followed by oral prednisone, and intravenous immunoglobulin (IVIg). We stress the importance of a multidisciplinary approach, involving both neurology and maternal-fetal medicine, and provide guidance in navigating the various options for immunosuppressive therapy during pregnancy.
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http://dx.doi.org/10.1155/2020/6049126 | DOI Listing |
Cureus
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.
Can J Neurol Sci
December 2024
Department of Clinical Neurological Sciences, London Health Sciences Centre, Western University, London, ON, Canada.
Eye (Lond)
November 2024
Ophthalmology Department, Ulucanlar Eye Training and Research Hospital, Ankara, Turkey.
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