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Clinical characterization and ancillary tests in Susac syndrome: a systematic review

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Item Type:Review
Title:Clinical characterization and ancillary tests in Susac syndrome: a systematic review
Creators Name:Wilf-Yarkoni, A. and Zmira, O. and Tolkovsky, A. and Pflantzer, B. and Gofrit, S.G. and Kleffner, I. and Paul, F. and Dörr, J.
Abstract:Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of encephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Our previous systematic review on all cases of SuS reported until 2012 allowed for a better understanding of clinical presentation and diagnostic findings. Based on these data, we suggested diagnostic criteria in 2016 to allow early diagnosis and treatment of SuS. In view of the accumulation of new SuS cases reported in the last 10 years and improved diagnostic tools, we here aimed at updating the demographic and clinical features of SuS and to review the updated ancillary tests being used for SuS diagnosis. Therefore, based on the 2016 criteria, we systematically collected and evaluated data on SuS published from January 2013 to March 2022.
Keywords:Brain Diseases, Differential Diagnosis, Magnetic Resonance Imaging, Susac Syndrome, Vision Disorders
Source:Neurology Neuroimmunology & Neuroinflammation
Publisher:American Academy of Neurology
Page Range:e200209
Date:May 2024
Official Publication:https://doi.org/10.1212/NXI.0000000000200209
PubMed:View item in PubMed

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