文摘
摘要目的:评估纵向模式显示萎缩的磁共振成像(MRI)在大脑皮层和皮层下灰质(GM)的患者不同临床变异的影响额颞叶的变性(FTLD)光谱。
背景:FTLD的发展是伴随着普遍通用的中断,远远超出额叶和颞叶皮层区域。
设计/方法:59例,其中包括26行为变异的额颞叶痴呆(bvFTD), 10 non-fluent / agrammatic变体原发性进行性失语(nfvPPA), 12 PPA的语义变体(svPPA)和11个运动神经元病(MND),在没有已知的致病突变,经历了在3 t MRI扫描器每隔6个月为一年。33健康对照组接受相同的协议。分析了3 d t1加权磁共振成像序列使用分布形态测量学评估通用汽车的纵向演化萎缩的病人,而HC。
结果:在基线,严重的弥漫性萎缩额颞叶皮层区域和基底神经节bvFTD被发现,nfvPPA和svPPA团体,而MND通用不显著萎缩。在6个月随访,bvFTD和PPA组显示萎缩的重要进展在岛(bvFTD nfvPPA和svPPA)和前扣带皮层(bvFTD和nfvPPA),双边以及在左侧尾状核和中颞叶皮层(svPPA)。在12个月的随访,萎缩进展被发现的模式相似,有额外的上额叶脑回参与nfvPPA,双边,svPPA海马。没有明显的萎缩在MND患者被发现。
结论:我们的数据表明,萎缩的岛和前扣带地区密切反映了神经退化的进程在FTD的行为和语言陈述,在与大量保留MND的通用汽车。措施cingulo-insular-striatal网络的退化是有前途的候选人在FTD疾病进展的生物标志物。
披露:博士利沃诺主席没有披露。西尔维亚Basaia没有披露。卡米拉Cividini没有披露。尼洛莉娃没有披露。朱塞佩•马格纳尼没有披露。卡索博士没有披露。Paola Caroppo没有披露。莎拉Prioni没有披露。卢西奥Tremolizzo没有披露。马里兰州Ildebrando h . Appollonio没有披露。 Dr. Silani has nothing to disclose. Paola Carrera has nothing to disclose. Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bayer, Biogen Idec, Merck-Serono, Novartis, Roche, Sanofi Genzyme, Takeda, and Teva Pharmaceutical Industries. Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bayer, Biogen Idec, Merck-Serono, Novartis, Roche, Sanofi Genzyme, Takeda, and Teva Pharmaceutical Industries. Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Filippi has received research support from Biogen Idec, Merck-Serono, Novartis, Roche, Teva Pharmaceutical Industries, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla, and ARiSLA (Fondazione Italiana di Ricerca per la SLA). Federica Agosta has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Philips. Federica Agosta has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier INC.
