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多发性硬化症的运动言语表型特征。

Characteristics of motor speech phenotypes in multiple sclerosis.

机构信息

Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University in Prague, Technicka 2, 160 00 Prague 6, Czech Republic; Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Katerinska 30, 120 00 Prague 2, Czech Republic.

Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Katerinska 30, 120 00 Prague 2, Czech Republic.

出版信息

Mult Scler Relat Disord. 2018 Jan;19:62-69. doi: 10.1016/j.msard.2017.11.007. Epub 2017 Nov 8.

Abstract

BACKGROUND

Motor speech disorders in multiple sclerosis (MS) are poorly understood and their quantitative, objective acoustic characterization remains limited. Additionally, little data regarding relationships between the severity of speech disorders and neurological involvement in MS, as well as the contribution of pyramidal and cerebellar functional systems on speech phenotypes, is available.

METHODS

Speech data were acquired from 141 MS patients with Expanded Disability Status Scale (EDSS) ranging from 1 to 6.5 and 70 matched healthy controls. Objective acoustic speech assessment including subtests on phonation, oral diadochokinesis, articulation and prosody was performed.

RESULTS

The prevalence of dysarthria in our MS cohort was 56% while the severity was generally mild and primarily consisted of a combination of spastic and ataxic components. Prosodic-articulatory disorder presenting with monopitch, articulatory decay, excess loudness variations and slow rate was the most salient. Speech disorders reflected subclinical motor impairment with 78% accuracy in discriminating between a subgroup of asymptomatic MS (EDSS < 2.0) and control speakers. Speech disorder severity was related to the severity of neurological involvement. Decreased articulation rate was moderately correlated to EDSS as well as all subtests of the multiple sclerosis functional composite. The strongest correlation was observed between irregular oral diadochokinesis and the 9-Hole Peg Test (r = - 0.65, p < 0.001). Irregular oral diadochokinesis and excess loudness variations significantly separated pure pyramidal and mixed pyramidal-cerebellar MS subgroups.

CONCLUSIONS

Automated speech analyses may provide valuable biomarkers of disease progression in MS as dysarthria represents common and early manifestation that reflects disease disability and underlying pyramidal-cerebellar pathophysiology.

摘要

背景

多发性硬化症(MS)中的运动言语障碍理解不足,其定量、客观的声学特征仍然有限。此外,关于言语障碍的严重程度与 MS 中的神经受累之间的关系,以及关于言语表型的皮质下和小脑功能系统的贡献,数据很少。

方法

从 EDSS 范围为 1 至 6.5 的 141 名 MS 患者和 70 名匹配的健康对照者中获取言语数据。进行客观的言语评估,包括发声、口腔交替运动、发音和韵律等测试。

结果

在我们的 MS 队列中,构音障碍的患病率为 56%,而严重程度通常较轻,主要由痉挛和共济失调成分的组合构成。最突出的是韵律-发音障碍,表现为单音调、发音衰减、过度响度变化和缓慢的语速。言语障碍反映出亚临床运动障碍,在区分无症状 MS 亚组(EDSS < 2.0)和对照组之间具有 78%的准确性。言语障碍严重程度与神经受累的严重程度相关。发音率降低与 EDSS 以及多发性硬化症功能综合测试的所有测试均呈中度相关。不规则的口腔交替运动与 9 孔钉测试之间的相关性最强(r = - 0.65,p < 0.001)。不规则的口腔交替运动和过度响度变化可明显区分单纯皮质下和混合皮质下-小脑 MS 亚组。

结论

自动言语分析可能为 MS 的疾病进展提供有价值的生物标志物,因为构音障碍是常见且早期的表现,反映了疾病残疾和潜在的皮质下-小脑病理生理学。

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