Eskildsen Simon F, Østergaard Lasse R, Rodell Anders B, Østergaard Leif, Nielsen Jørgen E, Isaacs Adrian M, Johannsen Peter
Department of Health Science and Technology, Aalborg University, Aalborg East, Denmark.
Neuroimage. 2009 Apr 15;45(3):713-21. doi: 10.1016/j.neuroimage.2008.12.024. Epub 2008 Dec 25.
Frontotemporal dementia constitutes the third most prevalent neurodegenerative disease with dementia. We compared cortical structural changes in nine presymptomatic CHMP2B frontotemporal dementia mutation positive individuals with seven mutation negative family members. Using serial MRI scans with a mean interval of 16 months and surface based cortical segmentation we measured cortical thickness and volume, and quantified atrophy rates. Cortical thickness and atrophy rates were averaged within major lobes and focal effects were determined by parametric statistical maps. The volumetric atrophy rates in the presymptomatic CHMP2B mutation carriers were statistically significant, though of a lower magnitude than those previously reported in patients of other types of frontotemporal dementia. Cortical thickness measurements revealed cortical thinning in mutation carriers bilaterally in the frontal and occipital lobes, and in the left temporal lobe. Results indicated that cortical thickness has a higher sensitivity for detecting small changes than whole-brain volumetric measures. Comparing mutation carriers with non-carriers revealed increased atrophy rates in mutation carriers bilaterally in the inferio-temporal cortex, the superior frontal cortex, and the insular cortex. These findings indicated impairment of regions involved in both behaviour and language. The symptoms previously reported in clinical CHMP2B frontotemporal dementia patients are associated with the anatomically affected regions here found in the presymptomatic mutation carriers.
额颞叶痴呆是第三常见的伴有痴呆的神经退行性疾病。我们比较了9名CHMP2B型额颞叶痴呆突变阳性的症状前个体与7名突变阴性家庭成员的皮质结构变化。使用平均间隔为16个月的系列MRI扫描和基于表面的皮质分割，我们测量了皮质厚度和体积，并量化了萎缩率。在主要脑叶内对皮质厚度和萎缩率进行平均，并通过参数统计图谱确定局灶性效应。症状前CHMP2B突变携带者的体积萎缩率具有统计学意义，尽管其程度低于先前报道的其他类型额颞叶痴呆患者。皮质厚度测量显示，突变携带者双侧额叶、枕叶以及左侧颞叶的皮质变薄。结果表明，与全脑体积测量相比，皮质厚度在检测微小变化方面具有更高的敏感性。将突变携带者与非携带者进行比较发现，突变携带者双侧颞下回皮质、额上回皮质和岛叶皮质的萎缩率增加。这些发现表明涉及行为和语言的区域存在损害。先前在临床CHMP2B型额颞叶痴呆患者中报道的症状与在此症状前突变携带者中发现的解剖学上受影响的区域相关。
