Urso Daniele, Giannoni-Luza Stefano, Brayne Carol, Ray Nicolas, Logroscino Giancarlo
Center for Neurodegenerative Diseases and the Aging Brain, University of Bari 'Aldo Moro,' "Pia Fondazione Cardinale G. Panico," Tricase, Lecce, Italy.
GeoHealth Group, Institute of Global Health, Faculty of Medicine, University of Geneva, Geneva, Switzerland.
JAMA Neurol. 2025 Sep 8. doi: 10.1001/jamaneurol.2025.3307.
Comprehensive incidence and prevalence rates of frontotemporal dementia are currently not available.
To estimate the incidence and prevalence of frontotemporal dementia and its clinical variants in the overall population and age subgroups.
We systematically searched PubMed, EMBASE, and Scopus between January 1, 1990, and October 22, 2024, for population-based studies estimating the incidence and/or prevalence of FTD.
Studies and data were screened and extracted independently by 2 investigators in accordance with PRISMA guidelines. Incident and prevalent cases together with the population at risk were pooled using random-effects meta-analysis. Differences in heterogeneity by FTD variants and populations at risk were estimated.
Prevalent and incident cases as numerator were based on well-defined clinical criteria. Denominators were derived either from census population data or from author-defined populations at risk.
From 1854 screened articles, 32 eligible population-based studies were identified. Sixteen were on prevalence and 22 on incidence reporting FTD measures, including those with estimates for the whole population and for specific age subgroups. Pooled crude incidence for FTD was 2.28 (95% CI, 1.55-3.36) per 100 000 person-years and prevalence, 9.17 (95% CI, 3.59-23.42) per 100 000 people. The behavioral-variant FTD pooled crude incidence was 1.20 (95% CI, 0.67-2.16) per 100 000 person-years and prevalence, 9.74 (95% CI, 2.90-32.73) per 100 000 people. The primary progressive aphasia variant pooled crude incidence was 0.52 (95% CI, 0.35-0.79) per 100 000 person-years and prevalence, 3.67 (95% CI, 3.05-4.43). FTD incidence among individuals younger than 65 years was 1.84 (95% CI, 0.79-4.30) per 100 000 person-years and prevalence, 7.47 (95% CI, 4.13-13.49) per 100 000 people. The denominator based on census data showed less heterogeneity than the population at risk defined by the authors (I2: for incidence, 91.6% vs 97.6%, respectively, and for prevalence, 98.8% vs 99.2%, respectively).
In this systematic review and meta-analysis, estimates indicate that FTD is comparable in frequency to dementia with Lewy bodies and occurs at higher rates than progressive supranuclear palsy, corticobasal syndrome, and amyotrophic lateral sclerosis. These results provide a foundation for future research and public health strategy, especially for underrepresented populations, to better comprehend the global burden of FTD. Our findings provide robust pooled estimates of the incidence and prevalence of FTD and its subtypes, offering a foundation for future research and public health planning.
目前尚无额颞叶痴呆的综合发病率和患病率数据。
估计总体人群及年龄亚组中额颞叶痴呆及其临床变异型的发病率和患病率。
我们系统检索了1990年1月1日至2024年10月22日期间的PubMed、EMBASE和Scopus数据库，以查找基于人群的研究，这些研究估计了额颞叶痴呆的发病率和/或患病率。
两名研究人员根据PRISMA指南独立筛选和提取研究及数据。采用随机效应荟萃分析汇总发病病例、患病病例以及处于风险中的人群。估计了额颞叶痴呆变异型和处于风险中的人群之间的异质性差异。
作为分子的患病和发病病例基于明确的临床标准。分母来自人口普查数据或作者定义的处于风险中的人群。
从1854篇筛选的文章中，确定了32项符合条件的基于人群的研究。16项关于患病率，22项关于发病率，报告了额颞叶痴呆的测量指标，包括对总体人群和特定年龄亚组的估计。额颞叶痴呆的合并粗发病率为每10万人年2.28（95%CI，1.55 - 3.36），患病率为每10万人9.17（95%CI，3.59 - 23.42）。行为变异型额颞叶痴呆的合并粗发病率为每10万人年1.20（95%CI，0.67 - 2.16），患病率为每10万人9.74（95%CI，2.90 - 32.73）。原发性进行性失语变异型的合并粗发病率为每10万人年0.52（95%CI，0.35 - 0.79），患病率为3.67（95%CI，3.05 - 4.43）。65岁以下个体中额颞叶痴呆的发病率为每10万人年1.84（95%CI, 0.79 - 4.30），患病率为每10万人7.47（95%CI，4.13 - 13.49）。基于人口普查数据的分母显示出的异质性低于作者定义的处于风险中的人群（I²：发病率分别为91.6%对97.6%，患病率分别为98.8%对99.2%）。
在这项系统评价和荟萃分析中，估计表明额颞叶痴呆的发病频率与路易体痴呆相当，且高于进行性核上性麻痹、皮质基底节综合征和肌萎缩侧索硬化症。这些结果为未来研究和公共卫生策略提供了基础，特别是对于代表性不足的人群，以更好地理解额颞叶痴呆的全球负担。我们的研究结果提供了额颞叶痴呆及其亚型发病率和患病率的可靠汇总估计，为未来研究和公共卫生规划奠定了基础。
