% 0期刊文章%一个大卫琼斯%布拉德利Boeve %罗纳德·彼得森David Knopman % % Clifford杰克%罗莎说Rademakers % Val劳% T扣带低灌注是一种常见的家庭额颞痴呆(P05.064) % D J神经病学2012% % P P05.064-P05.064 % V 78% N 1补充% X目的:描述SPECT灌注家族额颞叶痴呆的遗传变异。首页后台功能神经影像学研究SPECT和PET等敏感的诊断额颞叶(FTD)。额颞叶低灌注SPECT模式特征在家族和FTD的散在病例。最近鉴定的基因异常与家庭相关的额颞叶痴呆和/或肌萎缩性脊髓侧索硬化症,我们进行了描述性分析家族FTD的SPECT灌注与此相关hexanucleotide重复扩张C9ORF72, progranulin (PGRN)和微管相关蛋白(MAPT)情况下,SPECT扫描可用。设计/方法:我们确定了四个主题相关家族FTD C9ORF72 (3 f, 1米),三个与PGRN (3 f)和两个与MAPT (1 f, 1米)。SPECT图像空间归一化MNI空间,强度归一化相对于脑桥,空间平滑之前被转换成z分数地图使用5对照组的平均值和标准偏差(3 f; 2米)。空间归一化、平滑图像进入了两个示例t检验比较(p < 0.05)的所有情况下家族FTD对照组与年龄和性别作为协变量妨害。最大的灌注不足的地区被用来提取每个主题的SPECT强度。结果:个人SPECT扫描显示不同程度的区域内低灌注下外侧和内侧额叶,颞侧顶叶和扣带区域。组分析家族FTD显示扣带地区最大的减少和控制。扣带的分析发现,扣带代谢减退常分布在三个基因突变。结论:鉴于小样本大小,我们无法进行充分的调查家族FTD组之间的差异。 However, it appears as if cingulate hypoperfusion is a common feature of familial FTD associated with MAPT, PGRN, and C9ORF72. This would indicate the cingulate hypoperfusion is a stable biomarker of FTD and not of genetic variants of familial FTD.Disclosure: Dr. Jones has nothing to disclose. Dr. Boeve has nothing to disclose. Dr. Knopman has received personal compensation for activities with Lilly Pharmaceuticals as a data safety monitoring board member. Dr. Knopman has received personal compensation in an editorial capacity for Neurology. Dr. Knopman has received research support from Elan, Forest, and Baxter. Dr. Petersen has received personal compensation for activities with Pfizer, Inc., Janssen Alzheimer's Immunotherapy, Elan Pharmaceuticals, and GE Healthcare. Dr. Jack has received personal compensation for activities with Janssen Pharmaceutica, Eisai Inc, General Electric, Johnson and Johnson, and Eli Lilly & Company as a consultant.Dr. Jack has received research support from Pfizer Inc, Allon, and Baxter, Inc. Dr. Rademakers has nothing to disclose. Dr. Lowe has nothing to disclose.Wednesday, April 25 2012, 14:00 pm-19:00 pm %U