University of Kentucky, Rm 311 Sanders-Brown Center on Aging, Lexington, KY, USA.
University of Cambridge, Cambridge, UK.
Acta Neuropathol. 2022 Jul;144(1):27-44. doi: 10.1007/s00401-022-02444-1. Epub 2022 Jun 13.
Limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) and Alzheimer's disease neuropathologic change (ADNC) are each associated with substantial cognitive impairment in aging populations. However, the prevalence of LATE-NC across the full range of ADNC remains uncertain. To address this knowledge gap, neuropathologic, genetic, and clinical data were compiled from 13 high-quality community- and population-based longitudinal studies. Participants were recruited from United States (8 cohorts, including one focusing on Japanese-American men), United Kingdom (2 cohorts), Brazil, Austria, and Finland. The total number of participants included was 6196, and the average age of death was 88.1 years. Not all data were available on each individual and there were differences between the cohorts in study designs and the amount of missing data. Among those with known cognitive status before death (n = 5665), 43.0% were cognitively normal, 14.9% had MCI, and 42.4% had dementia-broadly consistent with epidemiologic data in this age group. Approximately 99% of participants (n = 6125) had available CERAD neuritic amyloid plaque score data. In this subsample, 39.4% had autopsy-confirmed LATE-NC of any stage. Among brains with "frequent" neuritic amyloid plaques, 54.9% had comorbid LATE-NC, whereas in brains with no detected neuritic amyloid plaques, 27.0% had LATE-NC. Data on LATE-NC stages were available for 3803 participants, of which 25% had LATE-NC stage > 1 (associated with cognitive impairment). In the subset of individuals with Thal Aβ phase = 0 (lacking detectable Aβ plaques), the brains with LATE-NC had relatively more severe primary age-related tauopathy (PART). A total of 3267 participants had available clinical data relevant to frontotemporal dementia (FTD), and none were given the clinical diagnosis of definite FTD nor the pathological diagnosis of frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP). In the 10 cohorts with detailed neurocognitive assessments proximal to death, cognition tended to be worse with LATE-NC across the full spectrum of ADNC severity. This study provided a credible estimate of the current prevalence of LATE-NC in advanced age. LATE-NC was seen in almost 40% of participants and often, but not always, coexisted with Alzheimer's disease neuropathology.
边缘为主的与年龄相关的 TDP-43 蛋白病脑病理改变(LATE-NC)和阿尔茨海默病脑病理改变(ADNC)在老年人群中均与严重认知障碍相关。然而,ADNC 全范围内 LATE-NC 的患病率尚不确定。为了解决这一知识空白,我们从 13 项高质量的社区和人群纵向研究中收集了神经病理学、遗传学和临床数据。参与者来自美国(8 个队列,包括一个专注于日裔美国人的队列)、英国(2 个队列)、巴西、奥地利和芬兰。总共有 6196 名参与者,死亡时的平均年龄为 88.1 岁。并非每个人都有所有数据,并且队列之间在研究设计和缺失数据量方面存在差异。在已知死亡前认知状态的个体中(n=5665),43.0%认知正常,14.9%有轻度认知障碍,42.4%有痴呆症-与该年龄组的流行病学数据基本一致。大约 99%的参与者(n=6125)有可用的 CERAD 神经原纤维缠结淀粉样斑块评分数据。在这个亚样本中,39.4%有任何阶段的经尸检证实的 LATE-NC。在“频繁”神经原纤维缠结淀粉样斑块的大脑中,54.9%合并有 LATE-NC,而在没有检测到神经原纤维缠结淀粉样斑块的大脑中,27.0%有 LATE-NC。有 3803 名参与者有 LATE-NC 阶段的数据,其中 25%有 LATE-NC 阶段>1(与认知障碍相关)。在 Thal Aβ 阶段=0 的个体亚组(缺乏可检测的 Aβ 斑块)中,有 LATE-NC 的大脑相对有更严重的原发性年龄相关性 tau 病(PART)。共有 3267 名参与者有与额颞叶痴呆(FTD)相关的可用临床数据,没有一个被给予明确的 FTD 临床诊断,也没有被给予额颞叶变性伴 TDP-43 包涵体(FTLD-TDP)的病理诊断。在 10 个有详细神经认知评估的队列中,在接近死亡时,认知功能随着 ADNC 严重程度的全谱 LATE-NC 而恶化。本研究提供了一个可信的当前高龄人群中 LATE-NC 患病率的估计。在近 40%的参与者中观察到 LATE-NC,并且通常(但不总是)与阿尔茨海默病病理学共存。