Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA.
Neurobiol Aging. 2013 Feb;34(2):636-9. doi: 10.1016/j.neurobiolaging.2012.03.009. Epub 2012 Apr 11.
We aimed to assess associations between clinical, imaging, pathologic, and genetic features and frontal lobe asymmetry in behavioral variant frontotemporal dementia (bvFTD). Volumes of the left and right dorsolateral, medial, and orbital frontal lobes were measured in 80 bvFTD subjects and subjects were classified into 3 groups according to the degree of asymmetry (asymmetric left, asymmetric right, symmetric) using cluster analysis. The majority of subjects were symmetric (65%), with 20% asymmetric left and 15% asymmetric right. There were no clinical differences across groups, although there was a trend for greater behavioral dyscontrol in right asymmetric compared with left asymmetric subjects. More widespread atrophy involving the parietal lobe was observed in the symmetric group. Genetic features differed across groups with symmetric frontal lobes associated with C9ORF72 and tau mutations, while asymmetric frontal lobes were associated with progranulin mutations. These findings therefore suggest that neuroanatomical patterns of frontal lobe atrophy in bvFTD are influenced by specific gene mutations.
我们旨在评估行为变异型额颞叶痴呆(bvFTD)中临床、影像、病理和遗传特征与额叶不对称之间的关联。对 80 名 bvFTD 患者的左侧和右侧背外侧、内侧和眶额叶进行体积测量,并使用聚类分析根据不对称程度(左侧不对称、右侧不对称、对称)将患者分为 3 组。大多数患者为对称型(65%),20%为左侧不对称,15%为右侧不对称。各组之间没有临床差异,尽管右侧不对称患者的行为失控程度较左侧不对称患者有增加的趋势。在对称组中观察到更广泛的涉及顶叶的萎缩。不同组之间的遗传特征也存在差异,对称额叶与 C9ORF72 和 tau 突变相关,而不对称额叶与颗粒蛋白基因突变相关。因此,这些发现表明 bvFTD 中额叶萎缩的神经解剖模式受特定基因突变的影响。