Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa.
Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy.
Acta Biomed. 2022 Mar 21;92(S4):e2021414. doi: 10.23750/abm.v92iS4.12663.
Sydenham's Chorea (SC) is a hyperkinetic movement disorder associated with neuropsychiatric manifestations. It is believed to be caused by the autoimmune response following a group A beta-hemolytic streptococcal (GABHS) pharyngitis, and it is one of the major diagnostic criteria for Acute Rheumatic Fever (ARF) diagnosis. Despite having been known and studied for centuries, there are still no standardized therapies or official guidelines for SC treatment, so that it is necessarily left to physicians' clinical experience. Antibiotic treatment, symptomatic therapies, and immunomodulatory treatment are the three pillars upon which SC patients' management is currently based, but they still lack a solid scientific basis. The aim of this writing is precisely to review the state of the art of SC's treatment, with an overview of the advances made in the last 5 years. However, since the therapeutic uncertainties are a mere reflection of the severe gap of knowledge that concerns SC's pathogenesis and manifestations, the importance of high-quality research studies based on homogenized methodologies, instruments, and measured outcomes will also be stressed.
舞蹈症（Sydenham's Chorea，SC）是一种与神经精神表现相关的运动障碍。据信，它是由 A 组β溶血性链球菌（Group A beta-hemolytic streptococcal，GABHS）咽炎后的自身免疫反应引起的，是急性风湿热（Acute Rheumatic Fever，ARF）诊断的主要标准之一。尽管 SC 已经被认识和研究了几个世纪，但目前仍然没有针对 SC 治疗的标准化疗法或官方指南，因此，它必然依赖于医生的临床经验。抗生素治疗、对症治疗和免疫调节治疗是目前 SC 患者管理的三大支柱，但它们仍然缺乏坚实的科学基础。本文的目的正是要回顾 SC 治疗的最新进展，概述过去 5 年来取得的进展。然而，由于治疗的不确定性仅仅反映了 SC 的发病机制和临床表现方面的严重知识差距，因此基于同质化方法、工具和测量结果的高质量研究的重要性也将被强调。
