TY -的T1 -扣带低灌注是一种常见的特征家族额颞痴呆(P05.064) JF -神经学乔-神经病学SP - P05.064 LP - P05.064六世- 78 - 1补充AU -大卫·琼斯AU -布拉德利Boeve盟David首页 Knopman AU -罗纳德·彼得森AU -克利福德杰克盟罗莎说Rademakers AU - Val劳Y1 - 2012/04/25 UR - //www.ez-admanager.com/content/78/1_Supplement/P05.064.abstract N2 -目的:描述SPECT灌注家族额颞叶痴呆的遗传变异。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)。The spatially normalized and smoothed images were entered into a two-sample t-test(p<0.05) comparing all cases of familial FTD to the control group with age and gender as nuisance covariates. The region of greatest hypoperfusion was then used to extract the SPECT intensity for each subject.Results: Individual SPECT scans revealed varying degree of regional hypoperfusion within the inferior lateral and medial frontal, temporal, lateral parietal, and cingulate regions. Group analysis of familial FTD versus controls revealed the greatest reduction in cingulate regions. Cingulate region of interest analysis revealed that cingulate hypometabolism was commonly distributed across the three genetic mutations.Conclusions: Given the small sample size, we were unable to perform an adequately powered investigation of the difference between familial FTD groups. 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 ER -