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不同临床进行性核上性麻痹(PSP)表型的言语特征:一项听觉感知研究。

Speech profile in different clinical PSP phenotypes: an acoustic-perceptual study.

作者信息

Di Rauso Giulia, Cavallieri Francesco, Gessani Annalisa, Fontanesi Davide, Coniglio Serena, Fioravanti Valentina, Contardi Sara, Menozzi Elisa, Antonelli Francesca, Rispoli Vittorio, Valzania Franco, Budriesi Carla

机构信息

Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.

Neurology, Neuroscience Head Neck Department, Azienda Ospedaliero-Universitaria di Modena, Modena, Italy.

出版信息

Neurol Sci. 2025 Feb;46(2):769-774. doi: 10.1007/s10072-024-07833-w. Epub 2024 Oct 25.

Abstract

Progressive supranuclear palsy (PSP) is a neurodegenerative disease with pathological hallmarks and different clinical presentations. Recently, the Movement Disorder Society (MDS) promoted a new classification; specific combinations of the core clinical features identify different phenotypes, including PSP with Richardson's syndrome (PSP-RS) and PSP with predominant parkinsonism (PSP-P). Since speech disorders are very common in PSP, they were included in the MDS-PSP criteria as a supportive clinical feature in the form of hypokinetic, spastic dysarthria. However, little is known about how dysarthria presents across the different PSP variants. The aim of the present study is to evaluate the presence of differences in speech profile in a cohort of PSP-RS and PSP-P patients diagnosed according to the MDS-PSP criteria. Each patient underwent a neurological evaluation and perceptual and acoustic analysis of speech. Disease severity was rated using the Natural History and Neuroprotection in Parkinson plus syndromes-Parkinson plus scale (NNIPPS-PPS), including global score and sub-scores. Twenty-five patients (mean disease duration [standard deviation] = 3.32 [1.79]) were classified as PSP-RS, while sixteen as PSP-P (mean disease duration [standard deviation] = 3.47 [2.00]). These subgroups had homogeneous demographical and clinical characteristics, including disease severity quantified by the NNIPPS-PPS total score. Only the NNIPPS-PPS oculomotor function sub-score significantly differed, being more impaired in PSP-RS patients. No significant differences were found in all speech variables between the two groups. Speech evaluation is not a distinguishing feature of PSP subtypes in mid-stage disease.

摘要

进行性核上性麻痹(PSP)是一种具有病理特征和不同临床表现的神经退行性疾病。最近,运动障碍协会(MDS)推出了一种新的分类方法;核心临床特征的特定组合可识别不同的表型,包括伴有理查森综合征的PSP(PSP-RS)和以帕金森症状为主的PSP(PSP-P)。由于言语障碍在PSP中非常常见,它们以运动减少型、痉挛性构音障碍的形式作为支持性临床特征被纳入MDS-PSP标准。然而,对于构音障碍在不同PSP变体中的表现知之甚少。本研究的目的是评估一组根据MDS-PSP标准诊断的PSP-RS和PSP-P患者在言语特征方面是否存在差异。每位患者都接受了神经学评估以及言语的感知和声学分析。使用帕金森叠加综合征的自然史和神经保护-帕金森叠加量表(NNIPPS-PPS)对疾病严重程度进行评分,包括总体评分和分项评分。25名患者(平均病程[标准差]=3.32[1.79])被归类为PSP-RS,16名患者被归类为PSP-P(平均病程[标准差]=3.47[2.00])。这些亚组具有相似的人口统计学和临床特征,包括由NNIPPS-PPS总分量化的疾病严重程度。只有NNIPPS-PPS动眼功能分项评分存在显著差异,PSP-RS患者的受损程度更严重。两组在所有言语变量上均未发现显著差异。言语评估并非中期疾病中PSP亚型的鉴别特征。

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