Differences in Symptomatic Presentation and Cognitive Performance Among Participants With LATE-NC Compared to FTLD-TDP.
Differences in Symptomatic Presentation and Cognitive Performance Among Participants With LATE-NC Compared to FTLD-TDP.
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与 FTLD-TDP 相比，LATE-NC 参与者的症状表现和认知表现存在差异。
DOI：
10.1093/jnen/nlab098
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发表时间：
2021
影响因子：
3.2
通讯作者：
Katsumata,Yuriko
中科院分区：
文献类型：
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作者：
Teylan,MerileeA;Mock,Charles;Gauthreaux,Kathryn;Culhane,JessicaE;Jicha,Gregory;Chen,Yen-Chi;Chan,KwunCG;Kukull,WalterA;Nelson,PeterT;Katsumata,Yuriko
交互反应dna结合蛋白43 kDa （TDP-43）在TDP-43型额颞叶变性（FTLD-TDP）和边缘显性年龄相关性TDP-43脑病神经病理改变（LATE-NC）中异常聚集和磷酸化。我们检查了来自国家阿尔茨海默病协调中心的数据，以比较尸检证实的LATE-NC和FTLD-TDP的临床特征。共纳入265名LATE-NC和92名FTLD-TDP参与者。比较认知和行为症状，根据全球临床痴呆评分（CDR）评分按损伤水平分层。晚期nc参与者在死亡时年龄较大，更可能携带apoeε 4，更可能患有阿尔茨海默病神经病理学，并且与FTLD-TDP患者相比，最终CDR总体评分较低（即较轻）。患有FTLD-TDP的参与者更有可能出现原发性进行性失语，或行为问题，如冷漠、去抑制和人格改变。在最终CDR评分为2-3分的参与者中，晚期nc患者更有可能出现视觉空间障碍、妄想和/或视觉幻觉。在排除老年人（死亡年龄≥80岁）、nc晚期3期或严重阿尔茨海默病病例的敏感性分析后，这些差异是稳健的。总体而言，FTLD-TDP在全球范围内更为严重，并且影响年轻参与者，而晚期nc患者更常见精神病。
Transactive response DNA-binding protein 43 kDa (TDP-43) is aberrantly aggregated and phosphorylated in frontotemporal lobar degeneration of the TDP-43 type (FTLD-TDP), and in limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC). We examined data from the National Alzheimer's Coordinating Center to compare clinical features of autopsy-confirmed LATE-NC and FTLD-TDP. A total of 265 LATE-NC and 92 FTLD-TDP participants were included. Cognitive and behavioral symptoms were compared, stratified by level of impairment based on global clinical dementia rating (CDR) score. LATE-NC participants were older at death, more likely to carryAPOEε4, more likely to have Alzheimer disease neuropathology, and had lower (i.e. less severe) final CDR global scores than those with FTLD-TDP. Participants with FTLD-TDP were more likely to present with primary progressive aphasia, or behavior problems such as apathy, disinhibition, and personality changes. Among participants with final CDR score of 2–3, those with LATE-NC were more likely to have visuospatial impairment, delusions, and/or visual hallucinations. These differences were robust after sensitivity analyses excluding older (≥80 years at death), LATE-NC stage 3, or severe Alzheimer cases. Overall, FTLD-TDP was more globally severe, and affected younger participants, whereas psychoses were more common in LATE-NC.