文摘
摘要目的:执行我们的研究提出一个框架,用于检测早期临床变化额颞叶的变性(FTLD)谱的疾病。
背景:广泛使用的标准6-domain临床痴呆评定(CDRstd)主要集中在广告,从而让更多的重量对记忆和定向障碍,和减少对行为和语言问题。扩大的效用CDRstd捕捉FTLD患者的早期症状,CDRstd扩大添加和行为/态度/个性(BEHAV)和语言(LANG)域创建8-domain FTLD-modified CDR (FTLD-CDR)。一些研究已经证明了FTLD-CDR FTLD参与者的效用。
设计/方法:我们分析了CDRstd FTLD-CDR 861零星和家族参与者的基线访问ARTFL / LEFFTDS财团。
结果:CDRstd-SB < 4轻度受损的参与者,BEHAV评级的频率≥0.5和≥1,分别是:行为变异额颞叶痴呆(bvFTD) 100.0% / 79.6%,轻微的行为变化(MCI-beh) 100.0% / 61.5%,迟滞型/ agrammatic变体原发性进行性失语(nfvPPA) 43.9% / 7.3%,和语义变体PPA (svPPA) 80.6% / 32.3%。朗的评级(评分≥0.5和≥1)是:bvFTD 53.7% / 13.0%, MCI-beh 23.1% / 0%, nfvPPA 100.0% / 92.7%, svPPA 100.0% / 90.3%。没有明显差异的家族和零星bvFTD参与者之间FTLD-CDR概要文件。所有11个MCI-beh bvFTD / PPA参与者被评为正常(CDRstd 0)异常FTLD-CDR评级。
结论:添加BEHAV和朗域FTLD-CDR能够捕捉FTLD的早期症状。
披露:Miyagawa博士没有披露。Brushaber博士没有披露。德弗博士没有披露。博士字段没有披露。Forsberg博士没有披露。Gavrilova博士没有披露。博士豪雅没有披露。Knopman博士已经收到个人赔偿咨询、担任科学顾问委员会说,或其他活动与华盛顿大学殿的研究。Knopman博士接到生原体制药和礼来制药研究支持。Kornak博士没有披露。 Dr. Kramer has nothing to disclose. Dr. Kremers has received research support from Astra Zeneca, Biogen and Roche. Dr. Rankin has nothing to disclose. Dr. Rascovsky has nothing to disclose. Dr. Syrjanen has nothing to disclose. Dr. Boeve has received research support from Biogen. Dr. Boxer has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with AbbVie, Asceneuron, Cellgene, Ipierian, Ionis, Janssen, Merck, Novartis, Toyama and UCB. Dr. Boxer holds stock and/or stock options in Alector and Aeton Therapeutics. Dr. Boxer has received research support from NIH, the Tau Consortium, the University of California Cures Alzheimer’s Disease program, the Bluefield Project to Cure FTD, Corticobasal Degeneration Solutions, and the following companies: Avid, Biogen, Bristol Myers Squibb, C2N Diagnostics, Cortice Biosciences, Eli Lilly, Forum Pharmaceuticals, Genentech, Roche and TauRx. Dr. Rosen has nothing to disclose.
