% 0期刊文章%弗朗西斯卡卡索%玛雅亨利%一个斯蒂芬·威尔逊Benno Gesierich % %一个布鲁斯·米勒尼娜Dronkers % %一个玛利亚路易莎Gorno-Tempini威廉·斯利% % T临床神经影像,在一群迟滞型和病理特性变异原发性进行性失语(nfvPPA)前瞻性研究(P07.172) % D J神经病学2012% % P P07.172-P07.172 % V 78% N 1补充% X目的:检查临床之间的关系,神经影像学和病理特性在迟滞型变体原发性进行性失语(nfvPPA)。首页背景病理诊断与FTLD-tau病理学nfvPPA主要有关联但TDP-43病理学也被报道。有人提议nfvPPA例τ和TDP-43显示不同的可能协助预测病理的临床特征。具体来说,语法缺失已经与TDP-43而参与与τ有关电动机演讲。设计/方法:在这里,我们目前的详细,nfvPPA 9例前瞻性纵向数据。病人临床随访时间平均为4.5年,直到尸检。分布形态测量学(VBM)被用来评估区域灰质和白质萎缩与一组140名健康对照组。结果:患者尸检显示两个TDP-43病理学(22%)和剩余情况下与τ病理学(78%),(4 cortico-basal退化,2进行性核上的麻痹,1选择病)。首先评估、TDP-43例功能静音,但写作样本并没有透露弗兰克语法缺失。τ病人所有显示失用症的演讲。有轻微的语法缺失六τ病例和构音障碍四。TDP-43和四个τ病人开发了一个温和的锥体外系综合征而戏剧性的运动参与观察三τ1例(2 PSP和CBD。VBM nfvPPA病人的整个组的结果显示左半球的普遍参与,包括前运动皮层、中央盖,脑岛。τ患者表现出更大的偏侧性相对于TDP43情况下,显示额外的参与中央盖。 In patients with tau pathology, grey matter loss was also observed in subcortical structures, including the left thalamus and caudate.Finally, tau patients showed extended cortico-subcortical white matter damage compared to TDP-43 patients.Conclusions: NfvPPA with TDP-43 or Tau pathology have clinical and neuroimaging commonalities.However,they also show distinctive features that,if confirmed in a larger cohort,may be helpful in-vivo prediction of pathology.Supported by: R01 NS050915,NIA P50 AG03006,NIA P01 AG019724;DHS04-35516;03-75271 DHS/AD/ARCC;Larry L. Hillblom Foundation;John Douglas French Alzheimer's Foundation; Koret Family Foundation;McBean Family Foundation.Disclosure: Dr. Caso has nothing to disclose. Dr. Henry has nothing to disclose. Dr. Gesierich has nothing to disclose. Dr. Wilson has nothing to disclose. Dr. Dronkers has nothing to disclose. Dr. Miller has received personal compensation for activities with Allon Therapeutics, Inc. and TauRx Therapeutics, Ltd. Dr. Miller has received research support from Novartis. Dr. Seeley has received personal compensation for activities with Korea Novartis. Dr. Gorno Tempini has nothing to disclose.Thursday, April 26 2012, 14:00 pm-18:30 pm %U