Disrupted functional connectivity in primary progressive apraxia of speech
Disrupted functional connectivity in primary progressive apraxia of speech
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DOI：
10.1016/j.nicl.2018.02.036
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发表时间：
2018-01-01
影响因子：
4.2
通讯作者：
Jones, David T.
中科院分区：
文献类型：
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作者：
Botha, Hugo;Utianski, Rene L.;Jones, David T.
语言失用症是一种运动语言障碍，被认为是由发音运动的计划或规划受损造成的。它可以是一种退行性疾病的初始或唯一表现，称为原发性进行性语言失用症（PPAOS）。本研究的目的是利用无任务功能磁共振成像（fMRI）评估PPAOS的大尺度脑网络病理生理。从退行性言语和语言障碍患者的前瞻性队列中确定了22名PPAOS参与者。所有参与者都进行了全面、标准化的评估，包括语言病理学家的评估、行为神经学家的检查和多模态成像协议，其中包括无任务功能磁共振成像序列。PPAOS参与者的年龄和性别与淀粉样蛋白阴性、认知正常的参与者匹配，比例为1:2。我们从大量样本外独立成分分析中选择了一组假设驱动的、预定义的内在连通性网络（ICNs），然后使用它们初始化时空对偶回归来估计这些ICNs内参与者水平的连通性。具体而言，我们以多元方式评估了语音和语言、面部和手部感觉运动、左工作记忆、显著性、顶叶上、边缘上、岛状和深灰色ICNs之间的连通性。然后在PPAOS和对照参与者之间比较每个ICN的空间图。我们使用言语失用严重程度的临床测量来评估在PPAOS和对照参与者之间发现的不同区域的临床连接相关性。与对照组相比，PPAOS参与者的右侧辅助运动区和左侧颞后回与其他语音和语言ICN的连通性降低。右侧辅助运动区的连通性与发音错误评分呈负相关。PPAOS参与者的左侧辅助运动区与面部感觉运动ICN、左侧外侧前额皮质与突出ICN、左侧颞枕交界处与左侧工作记忆ICN之间的连通性也降低。后一种连通性与言语失用严重程度评定量表相关，尽管这一发现没有经过多次比较的修正。在PPAOS参与者中，后扣带背侧和左工作记忆ICN之间的连通性增加。我们的研究结果支持辅助运动区在PPAOS病理生理学中的重要性，该区域似乎与言语和语言区域断开。辅助运动区连通性可能作为退行性言语失用严重程度的生物标志物。
Apraxia of speech is a motor speech disorder thought to result from impaired planning or programming of articulatory movements. It can be the initial or only manifestation of a degenerative disease, termed primary progressive apraxia of speech (PPAOS). The aim of this study was to use task-free functional magnetic resonance imaging (fMRI) to assess large-scale brain network pathophysiology in PPAOS. Twenty-two PPAOS participants were identified from a prospective cohort of degenerative speech and language disorders patients. All participants had a comprehensive, standardized evaluation including an evaluation by a speech-language pathologist, examination by a behavioral neurologist and a multimodal imaging protocol which included a task-free fMRI sequence. PPAOS participants were age and sex matched to amyloid-negative, cognitively normal participants with a 1:2 ratio. We chose a set of hypothesis driven, predefined intrinsic connectivity networks (ICNs) from a large, out of sample independent component analysis and then used them to initialize a spatiotemporal dual regression to estimate participant level connectivity within these ICNs. Specifically, we evaluated connectivity within the speech and language, face and hand sensorimotor, left working memory, salience, superior parietal, supramarginal, insular and deep gray ICNs in a multivariate manner. The spatial maps for each ICN were then compared between PPAOS and control participants. We used clinical measures of apraxia of speech severity to assess for clinical-connectivity correlations for regions found to differ between PPAOS and control participants. Compared to controls, PPAOS participants had reduced connectivity of the right supplementary motor area and left posterior temporal gyrus to the rest of the speech and language ICN. The connectivity of the right supplementary motor area correlated negatively with an articulatory error score. PPAOS participants also had reduced connectivity of the left supplementary motor area to the face sensorimotor ICN, between the left lateral prefrontal cortex and the salience ICN and between the left temporal-occipital junction and the left working memory ICN. The latter connectivity correlated with the apraxia of speech severity rating scale, although the finding did not survive correction for multiple comparisons. Increased connectivity was noted in PPAOS participants between the dorsal posterior cingulate and the left working memory ICN. Our results support the importance of the supplementary motor area in the pathophysiology of PPAOS, which appears to be disconnected from speech and language regions. Supplementary motor area connectivity may serve as a biomarker of degenerative apraxia of speech severity.