TY - T1的免疫学特征区分FTLD-TDP FTLD-tau疾病在临床上定义,基因和病理证实军团。乔(S48.006) JF -首页神经-神经学六世- 90 - 15补充SP - S48.006 AU -扎卡里·米勒AU -温迪瑞盟-大卫·佩里盟-维吉尼亚Sturm盟苏珊娜郭盟Shoshannah鲁宾AU -罗宾Ketelle盟赖利义务非盟-希拉里·豪雅盟尼古拉斯奥尔尼非盟-安娜Karydas盟詹妮弗Yokoyama盟-拉莫斯玛丽莎盟艾丽亚娜一直乔凡尼·科波拉AU -丹尼尔Geschwind AU -罗莎说Rademakers AU -丹尼斯·迪克森盟-尼尔Graff-Radford AU -萨尔瓦多脊柱非盟-埃里克·黄盟- Lea格林贝格盟——凯瑟琳·兰金盟——霍华德·罗森AU -布拉德利Boeve盟-亚当拳击手AU -玛丽亚Gorno Tempini AU -威廉·斯利盟布鲁斯·米勒Y1 - 2018/04/10 UR - //www.ez-admanager.com/content/90/15_Supplement/S48.006.abstract N2 -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) cohort, p<0.001 as well as within all subgroups of clinically, genetic, and pathology proven defined cohorts. We also observed a statistical increased prevalence of neoplastic diseases (24%; 67/279) in the total FTLD-tau cohort compared to the total FTLD-TDP cohort (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. ER -