Clinical Neuropsychology Research Group (EKN), Institute of Phonetics and Speech Processing, Ludwig-Maximilians-Universität (LMU) München, München, Germany.
Max Planck Institute for Human Cognitive and Brain Sciences Leipzig & Clinic for Cognitive Neurology, University Hospital Leipzig, Germany.
Cortex. 2021 Jul;140:66-79. doi: 10.1016/j.cortex.2021.03.017. Epub 2021 Apr 2.
Motor speech disorders (MSDs) are characteristic for nonfluent primary progressive aphasia (nfvPPA). In primary progressive aphasia (PPA) of the semantic (svPPA) and of the logopenic type (lvPPA), speech motor function is considered typically intact. However, knowledge on the prevalence of MSDs in svPPA and lvPPA is mainly based on studies with a priori knowledge of PPA syndrome diagnosis. This fully blinded retrospective study aims to provide data on the prevalence of all types of MSDs in a large sample of German-speaking patients with different subtypes of PPA.
Two raters, blinded for PPA subtype, independently evaluated connected speech samples for MSD syndrome and severity from 161 patients diagnosed with nfvPPA, svPPA or lvPPA in the database of the German Consortium of Frontotemporal Lobar Degeneration (FTLDc). In case of disagreement, a third experienced rater re-evaluated the speech samples, followed by a consensus procedure. Consensus was reached for 160 patients (74 nfvPPA, 49 svPPA, 37 lvPPA).
Across all PPA syndromes, 43.8% of the patients showed MSDs. Patients with nfvPPA demonstrated the highest proportion of MSDs (62.2%), but MSDs were also identified in svPPA (26.5%) and lvPPA (29.7%), respectively. Overall, dysarthria was the most common class of MSDs, followed by apraxia of speech. In addition, we identified speech abnormalities presenting as "syllabic speech", "dysfluent speech", and "adynamic speech".
Our study confirmed MSDs as frequently occurring in PPA. The study also confirmed MSDs to be most common in patients with nfvPPA. However, MSDs were also found in substantial proportions of patients with svPPA and lvPPA. Furthermore, our study identified speech motor deficits that have not received attention in previous studies on PPA. The results are discussed against the background of the existing literature on MSDs in PPA, including theoretical considerations of the neuroanatomical conditions described for each of the different subtypes of PPA.
运动性言语障碍（MSD）是非流利性原发性进行性失语症（nfvPPA）的特征。在语义性原发性进行性失语症（svPPA）和外侧裂失语症（lvPPA）中，言语运动功能被认为是典型完整的。然而，关于 svPPA 和 lvPPA 中 MSD 患病率的知识主要基于对 PPA 综合征诊断有先验知识的研究。本项完全盲法回顾性研究旨在为德国大型 PPA 患者不同亚型的 MSD 各种类型的患病率提供数据。
两名盲法评估者对数据库中 161 名诊断为 nfvPPA、svPPA 或 lvPPA 的患者的连续言语样本进行 MSD 综合征和严重程度的独立评估。在意见不一致的情况下，第三名经验丰富的评估者重新评估言语样本，然后进行共识程序。对 160 名患者（74 名 nfvPPA、49 名 svPPA、37 名 lvPPA）达成了共识。
在所有 PPA 综合征中，有 43.8%的患者存在 MSD。nfvPPA 患者的 MSD 比例最高（62.2%），但在 svPPA（26.5%）和 lvPPA（29.7%）中也存在 MSD。总体而言，构音障碍是最常见的 MSD 类型，其次是言语失用。此外，我们还发现了以“音节性言语”、“不流畅性言语”和“无力性言语”为特征的言语异常。
本研究证实 MSD 在 PPA 中频繁发生。研究还证实 MSD 在 nfvPPA 患者中最常见。然而，svPPA 和 lvPPA 患者中也存在相当比例的 MSD。此外，我们的研究还确定了一些在之前关于 PPA 的研究中未受到关注的言语运动缺陷。研究结果是在 PPA 中 MSD 的现有文献的基础上讨论的，包括对每种不同亚型 PPA 描述的神经解剖条件的理论考虑。
