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行为变异型额颞叶痴呆的运动言语特征:对表型的精细化。

Motor speech signature of behavioral variant frontotemporal dementia: Refining the phenotype.

机构信息

From the Centre for Neuroscience of Speech (A.P.V., M.L.P., M.W.J.C.) and Florey Institute of Neuroscience and Mental Health (H.P., F.M.C.B., D.D.), University of Melbourne, Australia; Department of Neurodegeneration (A.P.V.), Hertie Institute for Clinical Brain Research, University of Tübingen, Germany; Eastern Cognitive Disorders Clinic (A.P.V., M.L.P., D.D., A.B.), Box Hill Hospital, Monash University; Redenlab Pty. Ltd. (A.P.V., E.L.); and Neuroscience Research Unit (E.L.), Department of Neurology, Sunshine Hospital, Western Health, Melbourne, Australia.

出版信息

Neurology. 2017 Aug 22;89(8):837-844. doi: 10.1212/WNL.0000000000004248. Epub 2017 Jul 21.

Abstract

OBJECTIVE

To provide a comprehensive description of motor speech function in behavioral variant frontotemporal dementia (bvFTD).

METHODS

Forty-eight individuals (24 bvFTD and 24 age- and sex-matched healthy controls) provided speech samples. These varied in complexity and thus cognitive demand. Their language was assessed using the Progressive Aphasia Language Scale and verbal fluency tasks. Speech was analyzed perceptually to describe the nature of deficits and acoustically to quantify differences between patients with bvFTD and healthy controls. Cortical thickness and subcortical volume derived from MRI scans were correlated with speech outcomes in patients with bvFTD.

RESULTS

Speech of affected individuals was significantly different from that of healthy controls. The speech signature of patients with bvFTD is characterized by a reduced rate (75%) and accuracy (65%) on alternating syllable production tasks, and prosodic deficits including reduced speech rate (45%), prolonged intervals (54%), and use of short phrases (41%). Groups differed on acoustic measures derived from the reading, unprepared monologue, and diadochokinetic tasks but not the days of the week or sustained vowel tasks. Variability of silence length was associated with cortical thickness of the inferior frontal gyrus and insula and speech rate with the precentral gyrus.

CONCLUSIONS

One in 8 patients presented with moderate speech timing deficits with a further two-thirds rated as mild or subclinical. Subtle but measurable deficits in prosody are common in bvFTD and should be considered during disease management. Language function correlated with speech timing measures derived from the unprepared monologue only.

摘要

目的

全面描述行为变异型额颞叶痴呆(bvFTD)患者的言语运动功能。

方法

48 名个体(24 名 bvFTD 患者和 24 名年龄和性别匹配的健康对照者)提供了言语样本。这些样本的复杂性不同,因此认知需求也不同。他们的语言使用渐进性失语症语言量表和言语流畅性任务进行评估。通过感知分析来描述缺陷的性质,并通过声学分析来量化 bvFTD 患者和健康对照组之间的差异。从 MRI 扫描中得出的皮质厚度和皮质下体积与 bvFTD 患者的言语结果相关。

结果

受影响个体的言语与健康对照组明显不同。bvFTD 患者的言语特征是交替音节产生任务的语速(75%)和准确性(65%)降低,以及韵律缺陷,包括语速降低(45%)、间隔延长(54%)和使用短短语(41%)。组间在朗读、无准备独白和双音节词任务中得出的声学测量值存在差异,但在星期天数和持续元音任务中无差异。静默长度的可变性与额下回和脑岛的皮质厚度相关,而语速与中央前回相关。

结论

每 8 名患者中就有 1 名患者存在中度言语时程缺陷,另有 2/3 名患者的言语时程缺陷为轻度或亚临床。在 bvFTD 中,韵律方面存在微妙但可测量的缺陷,在疾病管理过程中应予以考虑。语言功能仅与无准备独白中得出的言语时程测量值相关。

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