Department of Psychology, Sapienza University of Rome, Via dei Marsi 78, 00185, Rome, Italy.
Ataxia Laboratory, IRCCS Fondazione Santa Lucia, Via Ardeatina 306-354, 00179, Rome, Italy.
J Neurol. 2023 Dec;270(12):5756-5772. doi: 10.1007/s00415-023-11993-8. Epub 2023 Sep 26.
Autonomic dysfunctions are prevalent in several cerebellar disorders, but they have not been systematically investigated in spinocerebellar ataxias (SCAs). Studies investigating autonomic deficits in SCAs are fragmented, with each one focusing on different autonomic dysfunctions and different SCA subtypes.
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, we conducted a systematic review of the literature to assess the presence of autonomic dysfunctions in various SCAs. PubMed served as the primary database, and the Rayyan web application was employed for study screening.
We identified 46 articles investigating at least one autonomic function in patients with SCA. The results were analyzed and categorized based on the genetic subtype of SCA, thereby characterizing the specific autonomic deficits associated with each subtype.
This review confirms the presence of autonomic dysfunctions in various genetic subtypes of SCA, underscoring the cerebellum's role in the autonomic nervous system (ANS). It also emphasizes the importance of investigating these functions in clinical practice.
自主功能障碍在几种小脑疾病中很常见,但在脊髓小脑共济失调(SCA)中尚未进行系统研究。研究 SCA 自主功能障碍的研究分散,每个研究都集中在不同的自主功能障碍和不同的 SCA 亚型上。
根据系统评价和荟萃分析的首选报告项目(PRISMA)声明,我们对文献进行了系统综述,以评估各种 SCA 中自主功能障碍的存在。PubMed 作为主要数据库,使用 Rayyan 网络应用程序进行研究筛选。
我们确定了 46 篇研究 SCA 患者至少一种自主功能的文章。根据 SCA 的遗传亚型对结果进行了分析和分类,从而描述了与每个亚型相关的特定自主功能障碍。
本综述证实了各种遗传亚型的 SCA 中存在自主功能障碍,强调了小脑在自主神经系统(ANS)中的作用。它还强调了在临床实践中研究这些功能的重要性。