Center for Neurodegenerative Diseases and the Aging Brain, University of Bari-Aldo Moro, Bari at Pia Fondazione Cardinale Giovanni Panico, Tricase, Lecce, Italy.
Institute of Public Health, Charité-Universitätsmedizin Berlin, Berlin, Germany.
JAMA Neurol. 2023 Mar 1;80(3):279-286. doi: 10.1001/jamaneurol.2022.5128.
Diagnostic incidence data for syndromes associated with frontotemporal lobar degeneration (FTLD) in multinational studies are urgent in light of upcoming therapeutic approaches.
To assess the incidence of FTLD across Europe.
DESIGN, SETTING, AND PARTICIPANTS: The Frontotemporal Dementia Incidence European Research Study (FRONTIERS) was a retrospective cohort study conducted from June 1, 2018, to May 31, 2019, using a population-based registry from 13 tertiary FTLD research clinics from the UK, the Netherlands, Finland, Sweden, Spain, Bulgaria, Serbia, Germany, and Italy and including all new FTLD-associated cases during the study period, with a combined catchment population of 11 023 643 person-years. Included patients fulfilled criteria for the behavioral variant of frontotemporal dementia (BVFTD), the nonfluent variant or semantic variant of primary progressive aphasia (PPA), unspecified PPA, progressive supranuclear palsy, corticobasal syndrome, or frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS). Data were analyzed from July 19 to December 7, 2021.
Random-intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.
Based on 267 identified cases (mean [SD] patient age, 66.70 [9.02] years; 156 males [58.43%]), the estimated annual incidence rate for FTLD in Europe was 2.36 cases per 100 000 person-years (95% CI, 1.59-3.51 cases per 100 000 person-years). There was a progressive increase in FTLD incidence across age, reaching its peak at the age of 71 years, with 13.09 cases per 100 000 person-years (95% CI, 8.46-18.93 cases per 100 000 person-years) among men and 7.88 cases per 100 000 person-years (95% CI, 5.39-11.60 cases per 100 000 person-years) among women. Overall, the incidence was higher among men (2.84 cases per 100 000 person-years; 95% CI, 1.88-4.27 cases per 100 000 person-years) than among women (1.91 cases per 100 000 person-years; 95% CI, 1.26-2.91 cases per 100 000 person-years). BVFTD was the most common phenotype (107 cases [40.07%]), followed by PPA (76 [28.46%]) and extrapyramidal phenotypes (69 [25.84%]). FTD-ALS was the rarest phenotype (15 cases [5.62%]). A total of 95 patients with FTLD (35.58%) had a family history of dementia. The estimated number of new FTLD cases per year in Europe was 12 057.
The findings suggest that FTLD-associated syndromes are more common than previously recognized, and diagnosis should be considered at any age. Improved knowledge of FTLD incidence may contribute to appropriate health and social care planning and in the design of future clinical trials.
鉴于即将出现的治疗方法，在多国研究中，与额颞叶变性（FTLD）相关的综合征的诊断发病率数据非常重要。
评估欧洲的 FTLD 发病率。
设计、地点和参与者：额颞痴呆发病率欧洲研究（FRONTIERS）是一项回顾性队列研究，于 2018 年 6 月 1 日至 2019 年 5 月 31 日进行，使用来自英国、荷兰、芬兰、瑞典、西班牙、保加利亚、塞尔维亚、德国和意大利的 13 个三级 FTLD 研究诊所的基于人群的登记处，包括研究期间所有新的 FTLD 相关病例，合并的集水区人口为 11023643 人年。纳入的患者符合行为变异额颞痴呆（BVFTD）、非流利型或语义性原发性进行性失语（PPA）、未特指的 PPA、进行性核上性麻痹、皮质基底节综合征或额颞痴呆伴肌萎缩侧索硬化症（FTD-ALS）的标准。数据于 2021 年 7 月 19 日至 12 月 7 日进行分析。
使用随机截距泊松模型，对考虑地理异质性的欧洲 FTLD 发病率进行估计。
根据 267 例确诊病例（平均[标准差]患者年龄，66.70[9.02]岁；156 名男性[58.43%]），欧洲 FTLD 的估计年发病率为每 10 万人中有 2.36 例（95%CI，每 10 万人中有 1.59-3.51 例）。FTLD 发病率随年龄呈递增趋势，在 71 岁时达到峰值，男性每 10 万人中有 13.09 例（95%CI，每 10 万人中有 8.46-18.93 例），女性每 10 万人中有 7.88 例（95%CI，每 10 万人中有 5.39-11.60 例）。总体而言，男性的发病率高于女性（男性每 10 万人中有 2.84 例；95%CI，每 10 万人中有 1.88-4.27 例），而女性每 10 万人中有 1.91 例（95%CI，每 10 万人中有 1.26-2.91 例）。BVFTD 是最常见的表型（107 例[40.07%]），其次是 PPA（76 例[28.46%]）和锥体外系表型（69 例[25.84%]）。FTD-ALS 是最罕见的表型（15 例[5.62%]）。共有 95 例 FTLD 患者（35.58%）有痴呆家族史。欧洲每年新发 FTLD 病例估计为 12057 例。
研究结果表明，与额颞叶变性相关的综合征比以前认为的更为常见，应在任何年龄考虑诊断。对 FTLD 发病率的认识提高可能有助于适当的卫生和社会保健规划，并有助于未来临床试验的设计。
