PT -期刊文章盟-米歇尔Offit盟天下吴盟-玫琳凯Floeter盟Tanya Lehky TI -电阻抗Myography (EIM)研究对象C9ORF72基因突变(P5.104) DP - 2017年4月18日TA -神经病学PG - P5.104 VI - 88 IP - 16补充4099 - //www.ez-admanager.com/content/88/16_Supplement/P5.104.short 410首页0 - //www.ez-admanager.com/content/88/16_Supplement/P5.104.full所以Neurology2017 4月18日;88 AB -目的:比较8肌肉平均EIM发现4亚种群C9ORF72主题——无症状(ASYMP)、肌萎缩的lateralsclerosis(肌萎缩性侧索硬化症),额颞叶痴呆(FTD)和ALS-FTD correlateEIM发现和医学研究委员会(MRC)肌肉力量测试(MRC0-5)。背景:EIM是一种非侵入性的电诊法的技术使用高频率,低强度电流获得电阻、电抗,肌肉和相位值。已经研究了很多神经肌肉疾病,包括肌肉萎缩症,作为一个可能的生物标志物。C9ORF72基因突变是最常见的ALS的家庭原因。设计/方法:32,肌萎缩性侧索硬化症(n = 14), ALS-FTD (n = 7) FTD (n = 3),和ASYMP (n = 8)进行评估(0)6,18个月。EIM是由测试4手臂和腿部肌肉。EIM和MRC,绝对改变计算6/18个月- 0个月之间的区别。斯皮尔曼相关系数是用来评估EIM变化和MRC变化之间的关系。ALS和ALS-FTD组池作为比较的一个族群。两个示例学习任务是用来比较的均值从ASYMP EIM变化从分组人口。Results: For all subjects, Spearman correlation between EIM change and MRC change did not show significance at 6 months (r=0.449, p=0.0706, n=17), but showed significance for 18 months (r=0.861, p=0.0129, n=7). For the subpopulation, there was a trend downward at both 6 months and 18 months in EIM (n=9 and n=5, respectively). The mean of the EIM change at 6 months of the ALS and ALS-FTD subpopulation (n=9)versus ASYMP (n=5) was significant (p=0.0339).Conclusions: Our exploratory assessment suggests that EIM maybe a useful technique to follow the progression of C9ORF72 subjects with ALS or ALS-FTD over time. Decrease in EIM over time could be used as a biomarker in ALS/ALS-FTD with the C9ORF72 genetic mutation.Study Supported by:This research was supported [in part] by the Intramural Research Program of the NIH, NINDSDisclosure: Dr. Offit, M.S. has nothing to disclose. Dr. Wu, PhD. has nothing to disclose. Dr. Floeter, M.D., PhD. has nothing to disclose. Dr. Lehky, M.D. has nothing to disclose.