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

运动性言语障碍（构音障碍、言语失用症）被认为对原发性进行性失语症（PPA）的潜在病理和病程具有较高的预测价值。该项目的总体目标是扩展PPA运动性言语障碍的知识，有助于更好地了解这种神经退行性疾病，并进一步推进临床评估和治疗。首先，我们将调查来自额颞叶变性研究联盟（回顾性研究）的100多名所有亚型PPA患者的现有样本数据。该数据库包括每个参与者的语音记录以及研究MRI。神经语音学分析将由对PPA亚型（非流利、语义、逻辑变体）设盲的专家评估者进行。这个子项目的主要目标是根据标准化标准对PPA中运动性言语障碍（构音障碍综合征、言语失用症、近中额言语运动综合征）的患病率进行无偏评估，并确定其神经解剖学基础。我们的目的是研究这些患者的语音配置文件更全面地通过使用一些成熟的仪器专门开发的神经源性言语障碍的评估，从而实施新的诊断标准，在PPA运动性言语障碍的临床评估。此外，以这种方式获得的语音数据为回答进一步的重要研究问题提供了基础：第一次将有可能比较PPA患者的语音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症患者的语音特征。为此，我们可以访问现有的数据库，其中包括300多名构音障碍患者和150多名来自临床神经心理学研究组（EKN数据库）的言语失用症患者。使用分类的方法，我们也将能够调查语音变量，可以有助于复杂的鉴别诊断nonfluent和logopenic变量PPA.本研究的目的是提供，第一次，在深入了解运动言语障碍在德语个人PPA。

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.