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额颞叶痴呆综合征行为变异型和皮质基底节变性痴呆综合征中的白质纤维束损伤

White matter tract damage in the behavioral variant of frontotemporal and corticobasal dementia syndromes.

作者信息

Tovar-Moll Fernanda, de Oliveira-Souza Ricardo, Bramati Ivanei Edson, Zahn Roland, Cavanagh Alyson, Tierney Michael, Moll Jorge, Grafman Jordan

机构信息

D'Or Institute for Research and Education (IDOR), Rio de Janeiro, Brazil; Institute of Biomedical Sciences and National Center of Structural Biology and Bioimaging (CENABIO), Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

D'Or Institute for Research and Education (IDOR), Rio de Janeiro, Brazil.

出版信息

PLoS One. 2014 Jul 23;9(7):e102656. doi: 10.1371/journal.pone.0102656. eCollection 2014.

Abstract

The phenotypes of the behavioral variant of frontotemporal dementia and the corticobasal syndrome present considerable clinical and anatomical overlap. The respective patterns of white matter damage in these syndromes have not been directly contrasted. Beyond cortical involvement, damage to white matter pathways may critically contribute to both common and specific symptoms in both conditions. Here we assessed patients with the behavioral variant of frontotemporal dementia and corticobasal syndrome with whole-brain diffusion tensor imaging to identify the white matter networks underlying these pathologies. Twenty patients with the behavioral variant of frontotemporal dementia, 19 with corticobasal syndrome, and 15 healthy controls were enrolled in the study. Differences in tract integrity between (i) patients and controls, and (ii) patients with the corticobasal syndrome and the behavioral variant of frontotemporal dementia were assessed with whole brain tract-based spatial statistics and analyses of regions of interest. Behavioral variant of frontotemporal dementia and the corticobasal syndrome shared a pattern of bilaterally decreased white matter integrity in the anterior commissure, genu and body of the corpus callosum, corona radiata and in the long intrahemispheric association pathways. Patients with the behavioral variant of frontotemporal dementia showed greater damage to the uncinate fasciculus, genu of corpus callosum and forceps minor. In contrast, corticobasal syndrome patients had greater damage to the midbody of the corpus callosum and perirolandic corona radiata. Whereas several compact white matter pathways were damaged in both the behavioral variant of frontotemporal dementia and corticobasal syndrome, the distribution and degree of white matter damage differed between them. These findings concur with the distinctive clinical manifestations of these conditions and may improve the in vivo neuroanatomical and diagnostic characterization of these disorders.

摘要

额颞叶痴呆行为变异型和皮质基底节综合征的表型在临床和解剖学上存在相当大的重叠。这些综合征中白质损伤的各自模式尚未直接对比。除了皮质受累外,白质通路的损伤可能对这两种疾病的共同症状和特定症状都有至关重要的影响。在此,我们对额颞叶痴呆行为变异型和皮质基底节综合征患者进行了全脑弥散张量成像,以确定这些病理状态下的白质网络。本研究纳入了20例额颞叶痴呆行为变异型患者、19例皮质基底节综合征患者和15名健康对照者。通过全脑基于纤维束的空间统计学和感兴趣区域分析,评估了(i)患者与对照之间以及(ii)皮质基底节综合征患者与额颞叶痴呆行为变异型患者之间纤维束完整性的差异。额颞叶痴呆行为变异型和皮质基底节综合征在前连合、胼胝体膝部和体部、放射冠以及长的半球内联合通路中存在双侧白质完整性降低的共同模式。额颞叶痴呆行为变异型患者的钩束、胼胝体膝部和小钳受到的损伤更大。相比之下,皮质基底节综合征患者的胼胝体中部和中央旁放射冠受到的损伤更大。虽然额颞叶痴呆行为变异型和皮质基底节综合征均有几条紧密的白质通路受损,但它们之间白质损伤的分布和程度有所不同。这些发现与这些疾病的独特临床表现一致,可能会改善这些疾病的活体神经解剖学和诊断特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cf2/4108323/9248148eeb76/pone.0102656.g001.jpg

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