Memory and Aging Center, Department of Neurology, University of California, San Francisco, 350 Parnassus Avenue, Suite 905, San Francisco, CA 94143, USA.
Brain. 2010 Jul;133(Pt 7):2069-88. doi: 10.1093/brain/awq129. Epub 2010 Jun 11.
Primary progressive aphasia is a clinical syndrome defined by progressive deficits isolated to speech and/or language, and can be classified into non-fluent, semantic and logopenic variants based on motor speech, linguistic and cognitive features. The connected speech of patients with primary progressive aphasia has often been dichotomized simply as 'fluent' or 'non-fluent', however fluency is a multidimensional construct that encompasses features such as speech rate, phrase length, articulatory agility and syntactic structure, which are not always impacted in parallel. In this study, our first objective was to improve the characterization of connected speech production in each variant of primary progressive aphasia, by quantifying speech output along a number of motor speech and linguistic dimensions simultaneously. Secondly, we aimed to determine the neuroanatomical correlates of changes along these different dimensions. We recorded, transcribed and analysed speech samples for 50 patients with primary progressive aphasia, along with neurodegenerative and normal control groups. Patients were scanned with magnetic resonance imaging, and voxel-based morphometry was used to identify regions where atrophy correlated significantly with motor speech and linguistic features. Speech samples in patients with the non-fluent variant were characterized by slow rate, distortions, syntactic errors and reduced complexity. In contrast, patients with the semantic variant exhibited normal rate and very few speech or syntactic errors, but showed increased proportions of closed class words, pronouns and verbs, and higher frequency nouns, reflecting lexical retrieval deficits. In patients with the logopenic variant, speech rate (a common proxy for fluency) was intermediate between the other two variants, but distortions and syntactic errors were less common than in the non-fluent variant, while lexical access was less impaired than in the semantic variant. Reduced speech rate was linked with atrophy to a wide range of both anterior and posterior language regions, but specific deficits had more circumscribed anatomical correlates. Frontal regions were associated with motor speech and syntactic processes, anterior and inferior temporal regions with lexical retrieval, and posterior temporal regions with phonological errors and several other types of disruptions to fluency. These findings demonstrate that a multidimensional quantification of connected speech production is necessary to characterize the differences between the speech patterns of each primary progressive aphasic variant adequately, and to reveal associations between particular aspects of connected speech and specific components of the neural network for speech production.
原发性进行性失语是一种以言语和/或语言进行性缺陷为特征的临床综合征,根据运动言语、语言和认知特征可分为非流利型、语义型和流利型。原发性进行性失语患者的连贯言语通常简单地分为“流利”或“非流利”,然而流利是一个多维结构,包括语速、短语长度、发音敏捷度和句法结构等特征,这些特征并不总是平行受到影响。在这项研究中,我们的第一个目标是通过同时量化言语输出的多个运动言语和语言维度来改善每个原发性进行性失语变体的连贯言语产生的特征描述。其次,我们旨在确定这些不同维度变化的神经解剖学相关性。我们记录、转录和分析了 50 名原发性进行性失语患者的言语样本,以及神经退行性疾病和正常对照组的言语样本。患者接受了磁共振成像扫描,体素形态计量学用于识别与运动言语和语言特征显著相关的萎缩区域。非流利型患者的言语样本特征为语速慢、发音扭曲、句法错误和复杂度降低。相比之下,语义型患者的语速正常且言语或句法错误很少,但封闭类词汇、代词和动词的比例增加,名词出现频率增加,反映出词汇检索缺陷。在失语法型患者中,语速(流利的常见指标)介于其他两种变体之间,但发音扭曲和句法错误比非流利型患者少见,而词汇获取障碍比语义型患者轻。语速降低与广泛的前后语言区域的萎缩相关,但特定的缺陷与更具局限性的解剖学相关。额区与运动言语和句法过程相关,额和下颞区与词汇检索相关,颞后区与语音错误和其他几种流利障碍相关。这些发现表明,多维的连贯言语产生量化对于充分描述每个原发性进行性失语变体的言语模式差异以及揭示连贯言语的特定方面与言语产生神经网络的特定成分之间的关联是必要的。