Clinical Characterization and Ancillary Tests in Susac Syndrome: A Systematic Review.

IF 7.8 1区 医学 Q1 CLINICAL NEUROLOGY
Adi Wilf-Yarkoni, Ofir Zmira, Assaf Tolkovsky, Barak Pflantzer, Shany G Gofrit, Ilka Kleffner, Friedemann Paul, Jan Dörr
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引用次数: 0

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.

苏萨克综合征的临床特征和辅助检查:系统回顾
苏萨克综合征(Susac Syndrome,SuS)是一种罕见的微血管病变性疾病,以脑病、视网膜分支动脉闭塞导致的视力障碍和感觉神经元性听力损失三联症为特征。我们之前对 2012 年之前报道的所有 SuS 病例进行了系统回顾,从而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,以便及早诊断和治疗 SuS。鉴于过去 10 年中新报告的 SuS 病例不断增加,诊断工具也在不断改进,我们在此旨在更新 SuS 的人口统计学和临床特征,并回顾用于诊断 SuS 的最新辅助检查。因此,根据 2016 年的标准,我们系统地收集并评估了 2013 年 1 月至 2022 年 3 月期间发表的有关 SuS 的数据。
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来源期刊
CiteScore
15.60
自引率
2.30%
发文量
219
审稿时长
8 weeks
期刊介绍: Neurology Neuroimmunology & Neuroinflammation is an official journal of the American Academy of Neurology. Neurology: Neuroimmunology & Neuroinflammation will be the premier peer-reviewed journal in neuroimmunology and neuroinflammation. This journal publishes rigorously peer-reviewed open-access reports of original research and in-depth reviews of topics in neuroimmunology & neuroinflammation, affecting the full range of neurologic diseases including (but not limited to) Alzheimer's disease, Parkinson's disease, ALS, tauopathy, and stroke; multiple sclerosis and NMO; inflammatory peripheral nerve and muscle disease, Guillain-Barré and myasthenia gravis; nervous system infection; paraneoplastic syndromes, noninfectious encephalitides and other antibody-mediated disorders; and psychiatric and neurodevelopmental disorders. Clinical trials, instructive case reports, and small case series will also be featured.
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