The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

Abstract

Background: Population-based prevalence and incidence studies are essential for understanding the burden of frontotemporal dementia (FTD). Methods: The MEDLINE and EMBASE databases were searched to identify population-based publications from 1985 to 2012, addressing the incidence and/or prevalence of FTD. References of included articles and prior systematic reviews were searched for additional studies. Two reviewers screened all abstracts and full-text reviews, abstracted data and performed quality assessments. Results: Twenty-six studies were included. Methodological limitations led to wide ranges in the estimates for prevalence (point prevalence 0.01-4.6 per 1000 persons; period prevalence 0.16-31.04 per 1000 persons) and incidence (0.0-0.3 per 1000 person-years). FTD accounted for an average of 2.7% (range 0-9.1%) of all dementia cases among prevalence studies that included subjects 65 and older compared to 10.2% (range 2.8-15.7%) in studies restricted to those aged less than 65. The cumulative numbers of male (373 [52.5%]) and female (338 [47.5%]) cases from studies reporting this information were nearly equal (p = 0.18). The behavioural variant FTD (bvFTD) was almost four times as common as the primary progressive aphasias. Conclusions: Population-based estimates for the epidemiology of FTD varied widely in the included studies. Refinements in the diagnostic process, possibly by the use of validated biomarkers or limiting case ascertainment to specialty services, are needed to obtain more precise estimates of the prevalence and incidence of FTD.RÉSUMÉ: Prévalence et incidence de la démence fronto-temporale : une revue systématique du sujet. Contexte : Les études de population sur la prévalence et l'incidence sont essentielles à la compréhension du fardeau associé à la démence fronto-temporale (DFT). Méthodologie : Nous avons cherché dans les bases de données MEDLINE et EMBASE les articles publiés entre 2000 et 2012 portant sur l'incidence et/ou la prévalence de la DFT dans la population. Nous avons également examiné les références des articles inclus dans notre étude ainsi que celles des revues systématiques antérieures. Deux évaluateurs ont examiné tous les résumés et le texte intégral des publications et l'extraction des données, et ils en ont évalué la qualité. Résultats : Vingt-six études ont été retenues. Des limites méthodologiques expliquent les écarts dans les estimations de prévalence (prévalence ponctuelle de 0,01 à 4,6 par 1 000 ; prévalence d'une période donnée de 0,16 à 31,04 par 1 000) et incidence (0,0 à 0,3 par 1 000 personnes-années). La DFT constituait en moyenne 2,7% (écart de 0 à 9,1%) de tous les cas de démence dans les études de prévalence qui incluaient des sujets de 65 ans et plus par rapport à 10,2% (2,8 à 15,7%) dans les études portant sur des sujets âgés de moins de 65 ans. Les nombres cumulatifs d'hommes (373 [52,5%]) et de femmes (338 [47,5%]) tirés des études dans lesquelles cette information était mentionnée étaient pratiquement égaux (p = 0,18). La variante comporte...

Dignity in people with frontotemporal dementia and similar disorders — a qualitative study of the perspective of family caregivers

“…These statements indicate a disapproval of the subtle categorization and the associated restrictions that seemed to unite the patient group. Uneasiness related to the frames of care may be of particular relevance with regard to patients with FTD because these patients are often quite young [1]. In a study on how relatives of people with frontotemporal dementia experience the illness, all of the participants found that they needed to be assertive to gain access to what they considered appropriate services.…”

“…Frontotemporal dementia (FTD) is a pathologically and clinically heterogeneous group of non-Alzheimer neurodegenerative dementias [1]. People with neuronal degeneration in frontal and temporal lobes demonstrate a decline in social conduct, and/or language aphasias, apathy, and loss of insight that is gradual and progressive [1, 2].…”

“…People with neuronal degeneration in frontal and temporal lobes demonstrate a decline in social conduct, and/or language aphasias, apathy, and loss of insight that is gradual and progressive [1, 2]. These symptoms can overlap with a range of other conditions, such as a frontal variant of Alzheimer’s disease or atypical parkinsonian disorders, and the diagnostic process is considered complex [1]. Earlier studies show that FTD constitutes 10–20% of dementia in younger persons (≤65 years old) [3, 4], but it can also affect older people.…”

“…According to this review, FTD accounted for an average of 2.7% (range 0–9.1) of all dementia cases in prevalence studies that included persons aged 65 and older, compared to an average of 10. 2% (range 2.8–15.7) in studies including those aged less than 65 years [1]. …”

Validação da escala de estadiamento e progressão da demência frontotemporal (FTD-FRS)

“…No estudo de Takada et al (2003), a DFT foi diagnosticada em 5,1% dos pacientes atendidos no São descritas na literatura três condições principais na DFT: DFTvc (Rascovsky et al, 2011;Hornberger et al, 2012), APPvs, APPvnf, APPvl (Hodges et al, 2001;Boxer et al, 2005;Gorno-Tempini et al, 2011).Em estudo de revisão, Hogan et al (2016) documentaram que há uma média de 2,7% de pacientes com DFTvc em estudos de prevalência de demência no mundo. Os dados indicaram que houve predominância do sexo masculino (52,5%).…”