TY - JOUR T1 -自动检测尸检确认的非流利/语法变体原发性进步性失语症的语音时间改变JF -神经学JO -神经学SP - e500 LP - e511 DO - 10.1212/WNL.0000000000200750半岛投注体育官网六世- 99 - 5 AU -阿道夫•m•加西亚盟阿丽亚娜e·韦尔奇AU -玛丽亚·路易萨盟的铜质把手-玛雅·l·亨利盟Sladjana Lukic AU -玛丽亚·约瑟夫托雷斯先天非盟-杰西卡负责盟Buddhika m . Ratnasiri AU -迭戈l . Lorca-Puls盟布鲁斯·l·米勒AU -威廉·斯利盟-亚当·p·沃格尔AU -玛丽亚·路易萨Gorno-Tempini Y1 - 2022/08/02 UR - //www.ebmtp.com/content/99/5/e500.abstract N2 -背景和目标机动语音功能,包括演讲时间,半岛投注体育官网是诊断非流利/语法变体原发性进行性失语症(nfvPPA)的一个关键领域。然而，标准评估使用主观的、专家依赖的评估，破坏了可靠性和可伸缩性。此外，很少有研究检查了病理确诊患者的相关解剖-临床改变。这项研究在一组独特的经尸检证实的病例中使用自动语音计时分析克服了这些警告。在一项横断面研究中，我们执行了一项公开的阅读任务，并量化了发音率、平均音节和停顿时间，以及音节和停顿时间的可变性。使用皮层厚度和白质(WM)萎缩分析评估神经解剖学破坏。结果我们评估了22例nfvPPA患者(平均年龄:67.3岁;13例女性患者)，并确认潜在的4次重复tau病，15例语义变异性原发性进行性失语症(svPPA;平均年龄:66.5岁; 8 female patients), and 10 healthy controls (HCs; 70 years; 5 female patients). All 5 speech timing measures revealed alterations in persons with nfvPPA relative to both the HC and svPPA groups, controlling for dementia severity. The articulation rate robustly discriminated individuals with nfvPPA from HCs (area under the ROC curve [AUC] = 0.95), outperforming specialist-dependent perceptual measures of dysarthria and apraxia of speech severity. Patients with nfvPPA exhibited structural abnormalities in left precentral and middle frontal as well as bilateral superior frontal regions, including their underlying WM. The articulation rate correlated with atrophy of the left pars opercularis and supplementary/presupplementary motor areas. Secondary analyses showed that, controlling for dementia severity, all measures yielded greater deficits in patients with nfvPPA and corticobasal degeneration (nfvPPA-CBD, n = 12) than in those with progressive supranuclear palsy pathology (nfvPPA-PSP, n = 10). The articulation rate robustly discriminated between individuals in each subgroup (AUC = 0.82). More widespread cortical thinning was observed for the nfvPPA-CBD than the nfvPPA-PSP group across frontal regions.Discussion Automated speech timing analyses can capture specific markers of nfvPPA while potentially discriminating between patients with different tauopathies. Thanks to its objectivity and scalability; this approach could support standard speech assessments.Classification of Evidence This study provides Class III evidence that automated speech analysis can accurately differentiate patients with nonfluent PPA from normal controls and patients with semantic variant PPA.4Rtau=FTLD-4-repeat tauopathy; AUC=area under the ROC curve; BA=Brodmann area; CBD=corticobasal degeneration; CDR=Clinical Dementia Rating; FTLD=frontotemporal lobar degeneration; FWE=family wise error; HCs=healthy controls; MMSE=Mini-Mental State Examination; nfvPPA=nonfluent/agrammatic variant primary progressive aphasia; nfvPPA-CBD=nonfluent/agrammatic primary progressive aphasia with underlying corticobasal degeneration pathology; nfvPPA-PSP=nonfluent/agrammatic parimary progressive aphasia with underlying progressive supranuclear palsy pathology; PMC=primary motor cortex; PPA=primary progressive aphasia; PPAOS=primary progressive apraxia of speech; preSMA=presupplementary motor area; PSP=progressive supranuclear palsy; ROI=region of interest; SMA=supplementary motor area; svPPA=semantic variant primary progressive aphasia; TIV=total intracranial volume; WM=white matter; GM=gray matter; TE=echo time; TR=repetition time ER -