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Clinical Manifestations of Early-Onset Dementia With Lewy Bodies Compared With Late-Onset Dementia With Lewy Bodies and Early-Onset Alzheimer Disease.
JAMA neurology Pub Date : 2022-07-01 , DOI: 10.1001/jamaneurol.2022.1133
Jingwei Sim 1 , Huihua Li 2 , Shahul Hameed 1 , Simon Kang Seng Ting 1
Affiliation  

Importance Early-onset dementia, presenting in individuals younger than 65 years, is a diagnosis with significant social and financial implications. The early-onset form of dementia with Lewy bodies (DLB) is poorly understood. Objective To investigate clinical features that distinguish early-onset DLB (onset and diagnosis at age <65 years) from late-onset DLB (onset at age ≥65 years) and from early-onset Alzheimer disease (AD) dementia. Design, Setting, and Participants This is a retrospective case-control study on patients with pathologically confirmed DLB or AD enrolled in the National Alzheimer's Coordinating Center database from January 2005 to July 2017. The National Alzheimer's Coordinating Center Uniform Data Set comprised deidentified data collected by Alzheimer disease centers in the United States. Of patients fulfilling criteria for all-cause dementia at enrollment (n = 1152), those who at post mortem received a pathological diagnosis of either AD (n = 848) or Lewy body disease (n = 218) were selected. Excluding 52 patients owing to missing data and 12 diagnosed with Parkinson disease dementia, remaining patients were classified by age of symptom onset into early-onset AD, early-onset DLB, and late-onset DLB subgroups. Data were analyzed from June to December 2018 and from November to December 2021. Exposures Demographics, cognitive, behavioral, and motor features recorded at first clinic visit and neuropathological characteristics at autopsy were analyzed by disease subgroup. Main Outcomes and Measures Concordance between initial etiologic diagnosis of dementia and final pathological diagnosis was assessed, as was time to death. Results A total of 542 individuals were categorized as having early-onset AD (n = 363; mean [SD] age, 53.0 [5.8] years; 208 [57.3%] male), early-onset DLB (n = 32; mean [SD] age, 57.9 [3.2] years; 23 [71.9%] male), and late-onset DLB (n = 147; mean [SD] age, 73.5 [5.5] years; 103 [70.1%] male). Early-onset DLB was clinically misdiagnosed in 16 individuals (50%). Features that predicted a diagnosis of early-onset DLB over early-onset AD included visual hallucinations (15 [46.9%] vs 42 [11.6%]), slowness (23 [71.9%] vs 95 [26.2%]), apathy (23 [71.9%] vs 189 [52.1%]), and motor deterioration that preceded cognitive and behavioral symptoms (7 [21.9%] vs 6 [1.7%]). Late-onset DLB had more amnestic features, but this was accounted for by a higher proportion of neocortical neuritic plaques and diffuse plaques (frequent in 79 [53.7%] vs 8 [25%]) than seen in early-onset DLB. Conclusions and Relevance This study found that early-onset DLB has clinical features that distinguish it from early-onset AD, whereas features of late-onset DLB are associated with a higher burden of AD copathology.

中文翻译:

早发性路易体痴呆与迟发性路易体痴呆和早发性阿尔茨海默病的临床表现比较。

重要性 早发性痴呆,出现在 65 岁以下的个体中,是一种具有重大社会和经济影响的诊断。对路易体痴呆 (DLB) 的早发形式知之甚少。目的 研究区分早发性 DLB(发病和诊断年龄 <65 岁)与迟发性 DLB(发病年龄≥65 岁)和早发性阿尔茨海默病 (AD) 痴呆的临床特征。设计、设置和参与者 这是一项回顾性病例对照研究,对象是 2005 年 1 月至 2017 年 7 月在国家阿尔茨海默病协调中心数据库中登记的经病理证实的 DLB 或 AD 患者。国家阿尔茨海默病协调中心统一数据集包含由美国的阿尔茨海默病中心。在入组时满足全因痴呆标准的患者 (n = 1152) 中,选择了那些在验尸时接受病理诊断为 AD (n = 848) 或路易体病 (n = 218) 的患者。由于数据缺失,排除了 52 名患者和 12 名被诊断患有帕金森病痴呆的患者,其余患者根据症状发作的年龄分为早发性 AD、早发性 DLB 和晚发性 DLB 亚组。分析了 2018 年 6 月至 2018 年 12 月以及 2021 年 11 月至 12 月的数据。按疾病亚组分析首次就诊时记录的人口统计学、认知、行为和运动特征以及尸检时的神经病理学特征。主要结果和测量 评估了痴呆的初始病因诊断与最终病理诊断之间的一致性,以及死亡时间。结果 共有 542 人被归类为早发性 AD(n = 363;平均 [SD] 年龄,53.0 [5.8] 岁;208 [57.3%] 男性)、早发性 DLB(n = 32;平均 [ SD] 年龄,57.9 [3.2] 岁;23 [71.9%] 男性)和迟发性 DLB(n = 147;平均 [SD] 年龄,73.5 [5.5] 岁;103 [70.1%] 男性)。早发性 DLB 在 16 人 (50%) 中被临床误诊。与早发性 AD 相比,预测早发性 DLB 诊断的特征包括视幻觉(15 [46.9%] 对 42 [11.6%])、行动迟缓(23 [71.9%] 对 95 [26.2%])、冷漠(23 [71.9%] 对 189 [52.1%]),以及先于认知和行为症状的运动能力恶化(7 [21.9%] 对 6 [1.7%])。迟发性 DLB 具有更多的遗忘特征,但这是由于新皮质神经炎斑块和弥漫性斑块的比例较高(常见于 79 [53. 7%] vs 8 [25%]) 比在早发性 DLB 中看到的要多。结论和相关性 本研究发现,早发性 DLB 具有可与早发性 AD 区分开来的临床特征,而晚发性 DLB 的特征与较高的 AD 病理学负担相关。
更新日期:2022-05-23
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