RT期刊文章SR电子T1免疫学特征区分FTLD-TDP FTLD-tau疾病在临床上定义,基因和病理证实军团。乔(S48.006)摩根富首页林明神经学神经病学FD Lippincott Williams &威尔金斯SP S48.006 VO 90 15补充A1扎卡里·米勒A1温迪丹瑞A1大卫·佩里A1弗吉尼亚Sturm A1苏珊娜郭A1 Shoshannah鲁宾A1罗宾Ketelle A1赖利义务A1希拉里·豪雅A1尼古拉斯奥尔尼A1安娜Karydas A1詹妮弗Yokoyama A1艾丽亚娜一直玛丽莎拉莫斯A1乔凡尼·科波拉A1丹尼尔Geschwind A1罗莎说Rademakers A1丹尼斯·迪克森A1尼尔Graff-Radford A1萨尔瓦多脊柱A1埃里克·黄A1 Lea格林贝格A1凯瑟琳·兰金A1霍华德·罗森A1布拉德利Boeve A1亚当拳击手A1玛丽亚Gorno Tempini A1威廉·斯利A1布鲁斯·米勒年2018 UL //www.ez-admanager.com/content/90/15_Supplement/S48.006.abstract AB目的:比较流行的免疫障碍的临床和基因预测以及病理证实额颞叶大叶性退行性(FTLD)军团。背景:以前,我们从根本上提出免疫失调与FTLD-TDP病理生理学。在这里,我们提供最全面的测试这个假说。设计/方法:我们回顾了在有症状的个体中所扮演的临床免疫紊乱的历史诊断为条件,具有较高的临床病理的相关性FTLD-TDP或FTLD-tau病理学(svPPA FTD / MND, PSP)突变携带者FTLD-TDP和FTLD-tau障碍(C9orf72,入库单,TARDBP MAPT)和解剖情况下拥有FTLD-TDP和/或FTLD-tau病态。我们补充收集个人进入ARTFL回答问卷调查和LEFFTDS财团计划我们专门设计用于捕捉自身免疫性疾病的历史,导致436 FTLD-TDP和316 FTLD-tau例比较。作为探索性措施,限制图审查情况下,我们筛选肿瘤疾病,癌症和自身免疫存在于一系列的免疫监视。结果:在总FTLD-TDP Non-thyroid自身免疫性疾病(15%;57/382)在统计学上高架相比总FTLD-tau (4%;13/306)组,p < 0.001,以及在临床的所有子组,遗传、群体定义和病理证实。我们还观察到一个统计增加肿瘤疾病的患病率(24%;67/279)相比,总FTLD-tau队列总FTLD-TDP队列(18%; 68/386), p=0.04, but not in any of the subgroup comparisons.Conclusions: The association between autoimmunity and FTLD pathology appears to be specific for conditions with underlying TDP-43-positive inclusions across clinical, genetic, and pathologically proven cases. Here we provide the greatest amount of evidence thus far in support of an FTLD-TDP immune hypothesis and the larger concept that specific underlying neurodegenerative pathologies may associate with distinct systemic immunological profiles, with resultant consequences for disease prediction, prevention, monitoring, and therapeutic intervention.Study Supported by: This work was supported by National Institutes of Health grants (K23 AG048291, P01 AG019724, P50 AG023501, and P50AG16574; R01 NS050915-05A1) and its contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Institute on Aging or NIH. Additional funds include the Hellman Research Scientist Award, the Arking Foundation for Frontotemporal Dementia, the Consortium for Frontotemporal Dementia Research, and the Tau Research Consortium.Disclosure: Dr. Miller has nothing to disclose. Dr. Shwe has nothing to disclose. Dr. Perry has nothing to disclose. Dr. Sturm has nothing to disclose. Dr. Kwok has nothing to disclose. Dr. Rubin has nothing to disclose. Dr. Ketelle has nothing to disclose. Dr. Dever has nothing to disclose. Dr. Heuer has nothing to disclose. Dr. Olney has nothing to disclose. Dr. Karydas has nothing to disclose. Dr. Yokoyama has nothing to disclose. Dr. Ramos has nothing to disclose. Dr. Coppola has nothing to disclose. Dr. Geschwind has nothing to disclose. Dr. Rademakers has nothing to disclose. Dr. Dickson has nothing to disclose. Dr. Graff-Radford has nothing to disclose. Dr. Spina has nothing to disclose. Dr. Huang has nothing to disclose. Dr. Grinberg has nothing to disclose. Dr. Rankin has nothing to disclose. Dr. Rosen has nothing to disclose. Dr. Boeve has received research support from GE Heathcare and Axovant. Dr. Boxer has nothing to disclose. Dr. Gorno Tempini has nothing to disclose. Dr. Seeley has nothing to disclose. Dr. Miller has nothing to disclose.