Motor speech disorders in primary progressive aphasia: Clinical presentation and neuroanatomical correlates

原发性进行性失语症的运动性言语障碍：临床表现和神经解剖学相关性

• 批准号: 384746448
• 负责人: Professorin Dr. Janine Diehl-Schmid
• 金额: --
• 依托单位: Tagesklinik für kognitive Neurologie
• 依托单位国家: 德国
• 项目类别: Research Grants
• 财政年份: 2018
• 资助国家: 德国
• 起止时间: 2017-12-31 至 2023-12-31
• 项目状态: 已结题
• 来源: https://gepris.dfg.de/gepris/projekt/384746448?language=en
• 关键词: Motor; speech; disorders; primary; progressive

Motor speech disorders (dysarthria, apraxia of speech) are ascribed a high predictive value for the underlying pathology and the course of the disease in primary progressive aphasia (PPA). The overarching goal of the proposed project is to extend knowledge of motor speech disorders in PPA, contributing to a better understanding of this neurodegenerative disease and to further advances in clinical assessment and treatment.The study comprises two major parts. First, we will investigate data from an existing sample of more than 100 patients with PPA of all subtypes from the Research Consortium of Frontotemporal Lobar Degeneration (retrospective study). The database comprises speech recordings as well as study MRIs from each participant. Neurophonetic analyses will be performed with expert raters blinded for PPA subtype (nonfluent, semantic, logopenic variant). The primary objectives of this subproject are the unbiased assessment of the prevalence of motor speech disorders in PPA (dysarthria syndromes, apraxia of speech, mesiofrontal speech motor syndromes) according to standardized criteria, and the identification of their neuroanatomical substrates.Second, we will collect speech data from 40 further patients with different variants of PPA (prospective study). Our aim is to study these patients speech profiles more comprehensively by using a number of well-established instruments specifically developed for the assessment of neurogenic speech disorders, and thereby to implement new diagnostic standards in the clinical assessment of motor speech disorders in PPA. Moreover, the speech data obtained in this way provides the basis for answering further important research questions: For the first time it will be possible to compare the speech profiles of PPA patients with those of patients with dysarthria or apraxia of speech following other degenerative or non-degenerative diseases. For this purpose, we can access an existing database of more than 300 patients with dysarthria and more than 150 patients with apraxia of speech from the Clinical Neuropsychology Research Group (EKN database). Using classificatory approaches, we will also be able to investigate phonetic variables that can contribute to the intricate differential diagnosis of nonfluent and logopenic variant PPA.This study aims at providing, for the first time, an in-depth knowledge of motor speech disorders in German-speaking individuals with PPA.

运动言语障碍（构音障碍、言语失用）被认为对原发性进行性失语症（PPA）的潜在病理和病程具有很高的预测价值。拟议项目的总体目标是扩展PPA中运动语言障碍的知识，有助于更好地理解这种神经退行性疾病，并进一步推进临床评估和治疗。本研究包括两个主要部分。首先，我们将调查来自额颞叶变性研究联盟的100多名PPA所有亚型患者的现有样本数据（回顾性研究）。该数据库包括每个参与者的语音记录以及研究核磁共振成像。神经语音分析将由PPA亚型（不流利，语义，词性变异）的盲法专家评分者进行。本子项目的主要目标是根据标准化标准对PPA（构音障碍综合征、言语失用症、中额叶言语运动综合征）中运动言语障碍的患病率进行公正的评估，并确定其神经解剖学基础。其次，我们将进一步收集40名不同变体PPA患者的语言数据（前瞻性研究）。我们的目的是通过使用一些专门用于评估神经源性语言障碍的成熟仪器，更全面地研究这些患者的语言特征，从而在PPA运动语言障碍的临床评估中实施新的诊断标准。此外，通过这种方式获得的言语数据为回答进一步重要的研究问题提供了基础：首次有可能将PPA患者的言语特征与其他退行性或非退行性疾病后的构音障碍或言语失用患者的言语特征进行比较。为此，我们可以从临床神经心理学研究小组（EKN数据库）中获取300多名构音障碍患者和150多名言语失用患者的现有数据库。使用分类方法，我们也将能够研究语音变量，可以有助于复杂的鉴别诊断不流利和词性变异PPA。这项研究的目的是提供，第一次，深入了解运动语言障碍的个体讲德语的PPA。