Department of Neurology, University of California, Irvine, CA, USA.
Institute for Memory Impairments and Neurological Disorders, University of California, Irvine, CA, USA.
J Alzheimers Dis. 2023;96(1):113-124. doi: 10.3233/JAD-230238.
Limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) is a clinicopathological construct proposed to facilitate studying TDP-43 pathology in older individuals.
Our aim was to describe clinical and cognitive characteristics of LATE-NC without Alzheimer's disease neuropathologic change (ADNC) and Lewy body (LB) and to compare this with ADNC and primary age related tauopathy (PART).
In 364 autopsies of the oldest old of The 90+ Study, we identified those with LATE-NC without ADNC and LB. Control groups were participants with ADNC and PART.
Of 31% of participants who had LATE-NC, only 5 (1.4%) had LATE-NC without ADNC and LB, all of whom had tau. These participants had a gradual and progressive cognitive decline. Four (80%) had dementia at death, a rate that was higher than ADNC (50%) and PART (21.7%). Mean duration of cognitive impairment was twice as long in LATE-NC without ADNC and LB (6.2 years) compared to ADNC (2.9 years) and PART (3 years). LATE-NC without ADNC and LB group had a higher prevalence of syncope, depression, and extrapyramidal signs than the ADNC and PART groups.
Despite the high prevalence of LATE-NC, LATE-NC without ADNC and LB was rare in this large oldest-old cohort, highlighting the very high prevalence of multiple pathologic changes in the oldest old. Slowly progressive cognitive decline, ubiquitous memory impairment, history of syncope and depression, and extrapyramidal signs were prominent features among our LATE-NC without ADNC and LB group.
边缘为主型年龄相关性 TDP-43 脑蛋白病（LATE-NC）是一种临床病理构建体，旨在促进研究老年个体中的 TDP-43 病理学。
我们的目的是描述无阿尔茨海默病病理改变（ADNC）和路易体（LB）的 LATE-NC 的临床和认知特征，并将其与 ADNC 和原发性年龄相关性 tau 病（PART）进行比较。
在 90+ 研究中对最年长个体的 364 例尸检中，我们确定了无 ADNC 和 LB 的 LATE-NC。对照组为 ADNC 和 PART 的参与者。
在 31%的 LATE-NC 参与者中，仅有 5 例（1.4%）为无 ADNC 和 LB 的 LATE-NC，所有这些参与者均存在 tau。这些参与者认知能力逐渐且逐渐下降。4 例（80%）在死亡时患有痴呆症，这一比例高于 ADNC（50%）和 PART（21.7%）。与 ADNC（2.9 年）和 PART（3 年）相比，无 ADNC 和 LB 的 LATE-NC 认知障碍的平均持续时间长一倍。无 ADNC 和 LB 的 LATE-NC 组比 ADNC 和 PART 组更常见晕厥、抑郁和锥体外系体征。
尽管 LATE-NC 的患病率很高，但在这个最大的最年长队列中，无 ADNC 和 LB 的 LATE-NC 却很少见，这突显了最年长个体中多种病理改变的极高患病率。缓慢进行性认知下降、普遍存在的记忆障碍、晕厥和抑郁病史以及锥体外系体征是我们无 ADNC 和 LB 的 LATE-NC 组的突出特征。
