患者临床特征可以预测τ病理行为变异额颞叶痴呆(bvFTD) ?(P05.101)

文摘

摘要目的:确定临床特征预测τ病理行为变异的额颞叶痴呆(bvFTD)。

背景:虽然以行为改变和额颞叶萎缩,bvFTD病态异构。随着τ的发展代理治疗FTD,越来越重要的是建立在生活底层τ病理学。因此,我们试图确定早期临床预测τ病理学的多站点示例autopsy-confirmed bvFTD病人。

设计/方法:国际财团bvFTD标准的146例(FTDC)数据库是包括在内。情况下遇到了可能bvFTD标准表示和FTLD病理学尸检。卡方分析相比早期行为和认知功能在τ积极(τ+)和τ- (non-tau)病例。

结果:56例(38%)为τnon-tau +和90 (62%)。更高比例的non-tau情况下面对bvFTD一致(即神经心理学概要文件。,executive/generation deficits with relative sparing of memory and visuospatial functions: tau+=69%, non-tau=84%, X2=4.1, p<.05). This may be driven in part by a high frequency of memory (31%) and visuospatial dysfunction (31%) in patients with tau genetic mutations (n=13). No significant group differences were observed for gender distribution (tau+=62% male, non-tau=56% male), age-at-onset (tau+=56yrs, non-tau=57yrs), or initial MMSE scores (tau+=20.3, non-tau=22.8). Frequency rates of core behavioral symptoms did not differ between groups: early disinhibition (tau+=86%, non-tau=86%), apathy/inertia (tau+=93%, non-tau=89%), loss of empathy (tau+=79%, non-tau=87%), compulsive/perseverative behaviors (tau+=81%, non-tau=76%), or hyperorality (tau+=68%, non-tau=70%). Groups did not differ in the frequency of extrapyramidal symptoms or additional language impairments.

结论:我们的研究结果表明,预测的τ病理学bvFTD很难基于人口、临床或行为特征。准确预测的τ病理学bvFTD可能最终取决于更详细的认知/行为测试加上成像和biofluid生物标记。

支持:AG17586、NS44266 P50 -AG016574P01 -AG019724P50 -AG023501,国土安全部07 - 65807。

披露:Rascovsky博士没有披露。霍奇斯博士没有披露。Knopman博士已经收到个人赔偿与礼来公司和公司活动。Knopman博士已经收到了个人在一篇社论中补偿神经病学的能力。首页Knopman博士接到TauRx研究支持。门德斯博士获得了个人在一篇社论中补偿现时的能力。克莱默博士没有披露。纽豪斯博士没有披露。范博士Swieten没有披露。Seelaar博士没有披露。 Dr. Dopper has nothing to disclose. Dr. Onyike has received research support from Forest Laboratories, Inc. Dr. Hillis has received personal compensation in an editorial capacity for Behavioural Neurology. Dr. Hillis has received research support from Allon Pharmaceutical. Dr. Josephs has nothing to disclose. Dr. Boeve has received research support from Cephalon, Inc.; Allon Therapeutics; and GE Healthcare. Dr. Kertesz has received personal compensation for activities with Pfizer and Janssen as a consultant. Dr. Kertsz has received research support from Sanofi and Janssen. Dr. Seeley has received personal compensation for activities with Korea Novartis, Summer Street Research Partners and Bristol-Myers Squibb for speaker, consulting and scientific advisory boards. Dr. Rankin has nothing to disclose. Dr. Johnson has nothing to disclose. Dr. Gorno Tempini has nothing to disclose. Dr. Rosen has nothing to disclose. Dr. Latham has nothing to disclose. Dr. Lee has nothing to disclose. Dr. Kipps has nothing to disclose. Dr. Lillo has nothing to disclose. Dr. Piguet has nothing to disclose. Dr. Rohrer has nothing to disclose. Dr. Rossor has nothing to disclose. Dr. Warren has nothing to disclose. Dr. Fox has received personal compensation in an editorial capacity for Alxforum. Dr. Fox has received license fee payments from IXICO. Dr. Fox has received research support from Elan/Janssen Alzheimer Immunotherapy, Lundbeck Research USA, Inc., and Pfizer/Wyeth Pharmaceuticals. Dr. Galasko has received personal compensation for activities with United BioSource Corporation, Pfizer, Janssen, Elan Pharmaceuticals, Lundbeck, and Balance Pharma as a consultant. Dr. Galasko has received personal compensation in an editorial capacity for Alzheimer's Disease Research and Treatment. Dr. Galasko has received research support from Pfizer and Eli Lilly, Inc. Dr. Salmon has received personal compensation for activities with Bristol Myer Squibb as consultant. Dr. Black has recieved personal compensation for activities with Novartis Pharmaceuticals, Pfizer, GlaxoSmithKline, Roche Pharmaceuticals, and Bristol-Myers Squibb, and Elan. Dr. Black has received research support from Novartis Pharmaceuticals, Pfizer, Roche, and GlaxoSmithKline. Dr. Mesulam has nothing to disclose. Dr. Weintraub has nothing to disclose. Dr. Dickerson has received personal compensation for activities with Pfizer Inc and En Vivo as a consultant. Dr. Diehl has nothing to disclose. Dr. Pasquier has nothing to disclose. Dr. Deramecourt has nothing to disclose. Dr. Lebert has nothing to disclose. Dr. Pijnenburg has nothing to disclose. Dr. Chow has received personal compensation for activities with Bristol-Myers Squibb Company as a consultant. Dr. Manes has nothing to disclose. Dr. Grafman has nothing to disclose. Dr. Cappa has received personal compensation in an editorial capacity for Behavioral Neurology. Dr. Freedman has been listed on a provisional patent related to methods and kits for differential diagnosis of Alzheimer's disease vs frontotemporal dementia using blood biomarkers. Dr. Freedman has received research support from Lundbeck, Canada. Dr. Miller has received personal compensation for activities with Allon Therapeuctics, Inc. and TauRx Therapeutics, Ltd. Dr. Miller has received research support from Novartis. Dr. Grossman has nothing to disclose.