Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum.

Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum.
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DOI:
10.1371/journal.pone.0043993
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发表时间:
2012
期刊:
影响因子:
3.7
通讯作者:
Hornberger M
Hornberger M
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Lillo P;Mioshi E;Burrell JR;Kiernan MC;Hodges JR;Hornberger M

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越来越多的证据表明,肌萎缩性侧索硬化症(ALS)和额颞叶痴呆(FTD)存在临床、病理和遗传连续体,其中一种疾病的患者表现出另一种疾病的特征。然而,迄今为止,ALS-FTD疾病连续体中潜在的灰质和白质变化尚未被探索。在这项研究中,53名ALS (n = 10)、ALS-FTD (n = 10)和行为变异性FTD (bvFTD; n = 15)以及对照组(n = 18)的参与者进行了详细的临床评估,并使用基于体素的形态测量(VBM)和扩散张量成像(DTI)分析脑磁共振成像来检查灰质和白质在连续体中的差异和共性。重要的是,患者组的年龄、教育程度、性别和病程相匹配。VBM和DTI结果显示,ALS组的变化主要局限于运动皮质和前扣带及其下的白质束。ALS-FTD和bvFTD表现为额叶和颞叶广泛的灰质和白质改变。与其他亚型相比,广泛的前额皮质变化是bvFTD的标志,而ALS- ftd可以通过额外的颞叶灰质和白质变化与ALS区分。最后,ALS与其他两组的主要区别是皮质脊髓束变性。本研究首次表明,FTD和ALS在整个连续体的前扣带、运动皮层和相关白质束的变化中重叠。然而,额叶和颞叶萎缩以及皮质脊髓束变性成为亚型分类的标志,这将为未来的诊断和目标疾病管理提供信息。
There is increasing evidence that amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) lie on a clinical, pathological and genetic continuum with patients of one disease exhibiting features of the other. Nevertheless, to date, the underlying grey matter and white matter changes across the ALS-FTD disease continuum have not been explored. In this study fifty-three participants with ALS (n = 10), ALS-FTD (n = 10) and behavioural variant FTD (bvFTD; n = 15) as well as controls (n = 18), underwent detailed clinical assessment plus structural imaging using voxel-based morphometry (VBM) and diffusion tensor imaging (DTI) analysis of magnetic resonance brain imaging to examine grey and white matter differences and commonalities across the continuum. Importantly, patient groups were matched for age, education, gender and disease duration. VBM and DTI results showed that changes in the ALS group were confined mainly to the motor cortex and anterior cingulate as well as their underlying white matter tracts. ALS-FTD and bvFTD showed widespread grey matter and white matter changes involving frontal and temporal lobes. Extensive prefrontal cortex changes emerged as a marker for bvFTD compared to other subtypes, while ALS-FTD could be distinguished from ALS by additional temporal lobe grey and white matter changes. Finally, ALS could be mainly distinguished from the other two groups by corticospinal tract degeneration. The present study shows for the first time that FTD and ALS overlap in anterior cingulate, motor cortex and related white matter tract changes across the whole continuum. Nevertheless, frontal and temporal atrophy as well as corticospinal tract degeneration emerged as marker for subtype classification, which will inform future diagnosis and target disease management across the continuum.
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发表时间: 2010-04
期刊: Neuropathology : official journal of the Japanese Society of Neuropathology
影响因子: --
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