Incidence and Prevalence of Frontotemporal DementiaA Systematic Review and Meta-Analysis

Daniele Urso, MD, MPH1,2; Stefano Giannoni-Luza, MD, MPH1; Carol Brayne, MD, MSc3; et al

2025년09월08일

JAMA Neurol. Published online: September 8, 2025.

DOI:10.1001/jamaneurol.2025.3307

Abstract

Importance  Comprehensive incidence and prevalence rates of frontotemporal dementia are currently not available.

Objective  To estimate the incidence and prevalence of frontotemporal dementia and its clinical variants in the overall population and age subgroups.

Data Sources and Study Selection  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.

Data Extraction and Synthesis  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.

Main Outcomes and Measures  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.

Results  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).

Conclusions and Relevance  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.

Key Points

Question  What is the incidence and prevalence of frontotemporal dementia (FTD)?

Findings  In this systematic review and meta-analysis of population-based studies with an analyzed population of approximately 31 million person-years for incidence and 6 million individuals for prevalence spread in more than 12 regions around the world, FTD pooled incidence was 2.28 (95% CI, 1.55-3.36) per 100 000 person-years, and pooled prevalence was 9.17 (95% CI, 3.59-23.42) per 100 000 people.

Meaning  With these results providing a robust pooled estimate of FTD incidence and prevalence, future research should include underrepresented populations and other world regions to better comprehend the global burden of FTD.

Figure 1.  Preferred Reporting Items for Systematic Reviews and Meta-Analyses Guideline Flow Diagram of the Identification, Screening, Eligibility Assessment, and Inclusion of Studies

요약

·         중요성 : 현재까지 전두측두치매(Frontotemporal Dementia, FTD) 및 그 임상 아형(clinical variants)에 대한 전 세계적 발생률(incidence) 및 유병률(prevalence)의 포괄적인 추정치는 부족한 상황이다.

·         목적 : 전체 인구 및 연령별 하위 집단을 대상으로 FTD 및 임상 아형의 발생률과 유병률을 추정하는 것

·         자료원 및 연구 선정 :

-데이터베이스: PubMed, EMBASE, Scopus

-검색 기간: 1990년 1월 1일 ~ 2024년 10월 22일

-연구 대상: FTD의 발생률 및/또는 유병률을 추정한 인구 기반(population-based) 연구

-선정 논문 수: 스크리닝한 1,854편 중 32편 포함

·         자료 추출 및 합성 :

-연구 선정 및 데이터 추출은 2명의 연구자가 PRISMA 가이드라인에 따라 독립적으로 수행

-무작위 효과(random-effects) 메타분석을 통해 발생률 및 유병률 통합 추정치(pooled estimates) 산출

-FTD 아형별 및 위험 인구집단별 이질성(heterogeneity) 차이를 분석

·         주요 평가지표 : 

-분자(Numerator): 임상적으로 잘 정의된 기준에 따라 확정된 FTD 사례 수

-분모(Denominator): 공인된 센서스(census) 인구 자료 또는 연구자가 정의한 위험 인구(population at risk) 자료

·          결과 : 총 32개 인구 기반 연구분석

(16개: 유병률, 22개: 발생률, 일부 연구는 중복 보고)

-전체 FTD

▪ 발생률: 2.28명 / 10만 인년 (95% CI, 1.55–3.36)

▪ 유병률: 9.17명 / 10만명 (95% CI, 3.59–23.42)

-행동 변이형 FTD(Behavioral-variant FTD, bvFTD)

▪ 발생률: 1.20명 / 10만 인년 (95% CI, 0.67–2.16)

▪ 유병률: 9.74명 / 10만명 (95% CI, 2.90–32.73)

-원발성 진행성 실어증(Primary Progressive Aphasia, PPA)

▪ 발생률: 0.52명 / 10만 인년 (95% CI, 0.35–0.79)

▪ 유병률: 3.67명 / 10만명 (95% CI, 3.05–4.43)

-65세 미만(Sub-65) FTD

▪ 발생률: 1.84명 / 10만 인년 (95% CI, 0.79–4.30)

▪ 유병률: 7.47명 / 10만명 (95% CI, 4.13–13.49)

-인구 기준 설정에 따른 이질성 차이(I²)

▪ 센서스 기반 분모 사용 시:

발생률 I²: 91.6%, 유병률 I²: 98.8%

▪ 저자 정의 분모 사용 시:

발생률 I²: 97.6%, 유병률 I²: 99.2%

→ 공식 센서스 데이터를 사용할수록 연구 간 변동성이 낮음

·         결론 및 의의 : 본 체계적 문헌고찰 및 메타분석에 따르면

-FTD의 발생률 및 유병률은 루이소체 치매(Dementia with Lewy Bodies, DLB)와 비슷하며, 진행성 핵상마비(PSP), 피질기저증후군(CBS), 근위축성 측삭경화증(ALS) 보다 더 높은 빈도를 보임.

-이번 연구 결과는 FTD 및 하위 아형의 발생률·유병률에 대한 가장 견고한 통합 추정치를 제공하며, 향후 공중보건 전략 및 연구 설계의 기반으로 활용될 수 있음.

-특히 저대표성 집단(underrepresented populations)에서의 FTD 질병부담(global burden) 이해를 개선할 수 있는 자료를 제공함.