Ash Sharon, Nevler Naomi, Irwin David J, Shellikeri Sanjana, Rascovsky Katya, Shaw Leslie, Lee Edward B, Trojanowski John Q, Grossman Murray
Penn Frontotemporal Degeneration Center and Department of Neurology, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA, USA.
Department of Pathology and Laboratory Medicine, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA, USA.
J Alzheimers Dis Rep. 2023 Jun 8;7(1):589-604. doi: 10.3233/ADR-220089. eCollection 2023.
Apraxia of speech (AOS) is a core feature of nonfluent/agrammatic primary progressive aphasia (naPPA), but its precise characteristics and the prevalence of AOS features in spontaneous speech are debated.
To assess the frequency of features of AOS in the spontaneous, connected speech of individuals with naPPA and to evaluate whether these features are associated with an underlying motor disorder such as corticobasal syndrome or progressive supranuclear palsy.
We examined features of AOS in 30 patients with naPPA using a picture description task. We compared these patients to 22 individuals with behavioral variant frontotemporal dementia and 30 healthy controls. Each speech sample was evaluated perceptually for lengthened speech segments and quantitatively for speech sound distortions, pauses between and within words, and articulatory groping. We compared subgroups of naPPA with and without at least two features of AOS to assess the possible contribution of a motor impairment to speech production deficits.
naPPA patients produced both speech sound distortions and other speech sound errors. Speech segmentation was found in 27/30 (90%) of individuals. Distortions were identified in 8/30 (27%) of individuals, and other speech sound errors occurred in 18/30 (60%) of individuals. Frequent articulatory groping was observed in 6/30 (20%) of individuals. Lengthened segments were observed rarely. There were no differences in the frequencies of AOS features among naPPA subgroups as a function of extrapyramidal disease.
Features of AOS occur with varying frequency in the spontaneous speech of individuals with naPPA, independently of an underlying motor disorder.
言语失用(AOS)是非流畅性/语法缺失型原发性进行性失语(naPPA)的核心特征,但其确切特征以及自发言语中AOS特征的发生率仍存在争议。
评估naPPA患者自发连贯言语中AOS特征的频率,并评估这些特征是否与潜在的运动障碍如皮质基底节综合征或进行性核上性麻痹相关。
我们使用图片描述任务检查了30例naPPA患者的AOS特征。我们将这些患者与22例行为变异型额颞叶痴呆患者和30名健康对照者进行了比较。对每个言语样本进行感知评估,以确定延长的言语片段,并进行定量评估,以确定语音失真、单词之间和单词内部的停顿以及发音摸索情况。我们比较了有和没有至少两种AOS特征的naPPA亚组,以评估运动障碍对言语产生缺陷的可能影响。
naPPA患者出现了语音失真和其他语音错误。在30名个体中的27名(90%)发现了言语分割。在30名个体中的8名(27%)发现了失真,在30名个体中的18名(60%)出现了其他语音错误。在30名个体中的6名(20%)观察到频繁的发音摸索。很少观察到延长的片段。作为锥体外系疾病的一个函数,naPPA亚组中AOS特征的频率没有差异。
AOS特征在naPPA患者的自发言语中以不同频率出现,与潜在的运动障碍无关。