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Clinical Characterization and Ancillary Tests in Susac Syndrome: A Systematic Review
Adi Wilf-Yarkoni1, Ofir Zmira1, Assaf Tolkovsky1
1From the Departmet of Neurology (A.W.-Y., A.T.), Rabin Medical Center, Petah Tikva; Department of Neurology and Neurosurgery (A.W.-Y., A.T., B.P., S.G.G.), Sackler Faculty of Medicine, Tel Aviv University; Department of Neurology (O.Z.), Hillel Yaffe Medical Center, Hadera; Department of Neurology (O.Z.), Ruth and Bruce Rappaport Faculty of Medicine, Technion Institute of Technology, Haifa; Department of Neurology (B.P., S.G.G.), Sheba Medical Center, Ramat Gan, Israel; Department of Neurology (I.K.), University Hospital Knappschaftskrankenhaus Bochum, Ruhr University Bochum; Experimental and Clinical Research Center (F.P., J.D.), Max Delbrueck Center for Molecular Medicine and Charité Universitaetsmedizin Berlin; and Multiple Sclerosis Center (J.D.), Neurology Department, Oberhavel Kliniken, Hennigsdorf, Germany.
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.
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