TY - T1的大脑结构和功能连通性在摩根富林明家族额颞叶痴呆发生前症状-神经学乔-神经病学SP - e19 LP - - 10.1212 / WNL e26做。首页0000000000000583六世- 83 - 2非盟-伊莉斯他夹住AU -哔叽A.R.B. Rombouts AU - Lize c . Jiskoot盟汤姆窝Heijer AU - j·鲁斯a·格拉夫盟英奇德通力AU - Anke r . Hammerschlag盟Harro Seelaar AU -威廉·w·斯利盟Ilya m .转向Patrizia Rizzu AU - Mark a . van Buchem盟盟——约翰·c·范Swieten Y1 - 2014/07/08 UR - //www.ez-admanager.com/content/83/2/e19.abstract N2 -目的:我们的目的是调查是否认知缺陷和结构和功能连通性变化可以在症状出现之前发现一大群运营商MAPT 首页(microtubule-associated tau蛋白质)或入库单(progranulin)突变。方法:病例对照研究中,75名健康个体(20 - 70岁)与50%的风险额颞叶痴呆(FTD)进行DNA检测,神经心理评估,结构磁共振成像和磁共振成像。我们使用分布形态测量学和voxel-wise tract-based空间统计分析的灰质体积和扩散张量成像的措施。利用静息状态功能磁共振成像扫描,我们评估了整个大脑功能连通性frontoinsular, midcingulate前、后扣带回皮层。结果:运营商(39例)和非(n = 36)也有类似的神经心理的表现,除了在运营商字母数字替换测试的得分越低。更糟糕的表现特鲁三世,Rivermead行为记忆测试,和哈佩漫画与更高的年龄相关运营商,而不是控制。降低分数各向异性在正确的钩状的纤维束被发现在运营商与控制。减少功能前midcingulate皮层和frontoinsula之间的连接和其他大脑区域被发现与控制和与更高的年龄相比,航空公司在航空公司,而不是控制。我们没有发现显著差异或年龄相关性后扣带皮层连接。没有发现区域灰质体积的差异,除了小型集群更高的体积在运营商的中央前回。Conclusions: This study demonstrates that alterations in structural and functional connectivity develop before the first symptoms of FTD arise. These findings suggest that diffusion tensor imaging and resting-state fMRI may have the potential to become sensitive biomarkers for early FTD in future clinical trials.aMCC=anterior midcingulate cortex; C9orf72=chromosome 9 open reading frame 72; DA=axial diffusivity; DMN=default mode network; DR=radial diffusivity; DTI=diffusion tensor imaging; FA=fractional anisotropy; FMRIB=Oxford Centre for Functional Magnetic Resonance Imaging of the Brain; FSL=FMRIB's Software Library; FTD=frontotemporal dementia; GRN=progranulin; MAPT=microtubule-associated protein tau; MD=mean diffusivity; MNI=Montreal Neurological Institute; PCC=posterior cingulate cortex; RAVLT=Rey Auditory Verbal Learning Test; SN=salience network; TFCE=threshold-free cluster enhancement; TMT=Trail Making Test; VBM=voxel-based morphometry; WM=white matter ER -