[0]期刊文章%A Rachel M Butler Pagnotti %A Shehroo B Pudumjee %A Chad L Cross PStat(R) C-MDI %A Justin B Miller ABPP-CN %T边缘显性年龄相关性TDP-43脑病患者的认知和临床特征%D 2023 %R 10.1212/WNL。0000000000207159 %J神首页经病学%P 10.1212/WNL。背景和目的:边缘优势年龄相关的TDP-43 (LATE)影响与阿尔茨海默病(AD)相似的神经解剖网络，并且经常与AD共病，尽管在临床诊断中经常被遗漏。本研究的主要目的是阐明尸检证实的LATE患者与AD合并LATE+AD患者在基线时的临床和认知差异。方法:从国家阿尔茨海默病协调中心(NACC)索取临床和神经病理学数据集。死亡时75岁以上无额颞叶退行性变神经病理指征的个体的基线数据纳入分析。病理定义组反映LATE, AD和合并症LATE+AD被确定。通过使用统一数据集测量的方差分析和卡方分析，探讨临床特征和认知的组间差异。结果:病理组包括31例LATE (Mage: 80.6±5.4)，393例AD (Mage: 77.8±6.4)和262例LATE+AD (Mage: 77.8±6.6)，在性别、教育程度和种族方面无显著差异。与患有AD和LATE+AD病理的参与者相比，LATE病理的参与者寿命明显更长(就诊次数:LATE=7.3±3.7;广告= 5.8±3.0;晚+广告= 5.8±3.0; F(2,683)=3.7, p<.05), reported later onset of cognitive decline (Monset: LATE=78.8±5.7; AD=72.5±7.0; LATE+AD=72.9±7.0; F(2,516)=6.2, p<.01), and were more likely to be diagnosed as cognitively normal at baseline (LATE=41.9%; AD=25.4%; LATE+AD=12%; χ2=38.7, p<.001). Individuals with LATE (45.2%) also reported fewer memory complaints than those with AD (74.4%) or LATE+AD (66.4%; χ2=13.3, p=.001) and were less likely to be classified as Impaired on the Mini Mental State Exam (LATE=6.5%; AD=24.2%; LATE+AD=40.1%; χ2=29.20, p<.001). Across all neuropsychological measures, participants with LATE+AD pathology performed significantly worse than the AD and LATE groups.Conclusions: Those with LATE pathology were older when cognitive symptoms began and lived longer than participants with AD or LATE+AD pathology. Participants with LATE pathology were also more likely to be classified as “cognitively normal” based on objective screening and self-report measures, and they had higher scores on neuropsychological testing. Consistent with prior literature, comorbid pathologies led to more significant cognitive and functional impairment. Early disease characteristics based on clinical presentation alone were insufficient for differentiating LATE from AD, reiterating the need for a validated biomarker. %U //www.ez-admanager.com/content/neurology/early/2023/03/20/WNL.0000000000207159.full.pdf