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一种供非专家临床医生使用的智能工具,用于传播进行性核上性麻痹的MDS标准。

A smart tool for non expert clinicians for the dissemination of the MDS criteria for progressive supranuclear palsy.

作者信息

Picillo Marina, Abate Filomena, Canoro Vincenzo, Tepedino Maria Francesca, Barone Paolo, Erro Roberto

机构信息

Center for Neurodegenerative Diseases (CEMAND), Department of Medicine, Surgery and Dentistry, "Scuola Medica Salernitana", University of Salerno, Salerno, Italy.

Department of Neurology, "Umberto I" Hospital, Nocera Inferiore, Salerno, Italy.

出版信息

Neurol Sci. 2025 May;46(5):1959-1964. doi: 10.1007/s10072-025-07996-0. Epub 2025 Jan 11.

Abstract

Due to the variety of clinical phenotypes and the massive clinical overlap with other neurodegenerative diseases, the diagnosis of Progressive Supranuclear Palsy (PSP) remains a major challenge. Notwithstanding, early and reliable clinical diagnosis of PSP is highly warranted for estimation of prognosis, appropriate allocation to therapeutic trials and development of new diagnostic tools. As reliable biomarkers are lacking, PSP diagnosis relies on the application of the clinical criteria promoted by the International Parkinson and Movement Disorder Society (MDS). Despite providing a framework including all the main PSP cornerstones (ocular dysfunction and postural instability, akinesia and cognitive dysfunction), the application of the MDS PSP criteria is complex and not straightforward to apply in a clinical setting. Herein we propose a practical tool, including a video-guided slide-set and a smartsheet, to disseminate the MDS PSP clinical criteria among healthy practitioners and increase confidence in non expert clinicians towards suspicion and diagnosis of PSP. The video-guided slide set may serve as a teaching resource for both general neurologists and practitioners, while the smartsheet may represent a valid support in attributing the degree of diagnostic certainty and phenotype based on the identified clinical features. Application of our tool may improve early recognition of patients in primary and secondary care and determine a prompt referral to third level movement disorder centers for consideration in clinical trials testing disease-modifying treatments.

摘要

由于临床表型的多样性以及与其他神经退行性疾病存在大量临床重叠,进行性核上性麻痹(PSP)的诊断仍然是一项重大挑战。尽管如此,为了评估预后、合理分配到治疗试验以及开发新的诊断工具,PSP的早期可靠临床诊断非常必要。由于缺乏可靠的生物标志物,PSP的诊断依赖于国际帕金森和运动障碍协会(MDS)推广的临床标准的应用。尽管MDS PSP标准提供了一个包含所有主要PSP基石(眼功能障碍和姿势不稳、运动不能和认知功能障碍)的框架,但其应用复杂,在临床环境中不易应用。在此,我们提出一种实用工具,包括视频引导幻灯片集和智能工作表,以在临床医生中传播MDS PSP临床标准,并增强非专家临床医生对PSP怀疑和诊断的信心。视频引导幻灯片集可作为普通神经科医生和临床医生的教学资源,而智能工作表可在根据已确定的临床特征确定诊断确定性程度和表型方面提供有效支持。应用我们的工具可能会改善初级和二级护理中患者的早期识别,并确定迅速转诊至三级运动障碍中心,以便在测试疾病修饰治疗的临床试验中进行考虑。

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1
Prediagnostic Progressive Supranuclear Palsy - Insights from the UK Biobank.诊断前进行性核上性麻痹——来自英国生物银行的见解
Parkinsonism Relat Disord. 2022 Feb;95:59-64. doi: 10.1016/j.parkreldis.2022.01.004. Epub 2022 Jan 11.
6
The "zig-zag" sign in Progressive Supranuclear Palsy.进行性核上性麻痹中的“锯齿状”征。
Parkinsonism Relat Disord. 2020 Oct;79:86-87. doi: 10.1016/j.parkreldis.2020.08.014. Epub 2020 Aug 23.

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