From 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.
Neurol Neuroimmunol Neuroinflamm. 2024 May;11(3):e200209. doi: 10.1212/NXI.0000000000200209. Epub 2024 Feb 16.
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.
苏萨克斯综合征(SuS)是一种罕见的微血管疾病,其特征为脑病、分支视网膜动脉闭塞引起的视力障碍和感觉神经性听力损失三联征。我们之前对截至 2012 年所有报道的 SuS 病例进行的系统综述使我们更好地了解了临床表现和诊断结果。基于这些数据,我们在 2016 年提出了诊断标准,以实现 SuS 的早期诊断和治疗。鉴于过去 10 年中报道的新 SuS 病例不断增加以及诊断工具的改进,我们旨在更新 SuS 的人口统计学和临床特征,并回顾用于 SuS 诊断的更新辅助检查。因此,我们根据 2016 年的标准,系统地收集和评估了 2013 年 1 月至 2022 年 3 月期间发表的关于 SuS 的数据。