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The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

Published online by Cambridge University Press:  16 June 2016

David B. Hogan ,
Nathalie Jetté ,
Kirsten M. Fiest ,
Jodie I. Roberts ,
Dawn Pearson ,
Eric E. Smith ,
Pamela Roach ,
Andrew Kirk ,
Tamara Pringsheim  and
Colleen J. Maxwell
David B. Hogan
Affiliation:
Brenda Strafford Chair in Geriatric Medicine, University of Calgary, Calgary, Alberta, Canada Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
Nathalie Jetté*
Affiliation:
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada O’Brien Institute for Public Health, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Kirsten M. Fiest
Affiliation:
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada O’Brien Institute for Public Health, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Jodie I. Roberts
Affiliation:
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada O’Brien Institute for Public Health, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Dawn Pearson
Affiliation:
Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Eric E. Smith
Affiliation:
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Pamela Roach
Affiliation:
Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Andrew Kirk
Affiliation:
Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
Tamara Pringsheim
Affiliation:
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Department of Clinical Neurosciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada
Colleen J. Maxwell
Affiliation:
Department of Community Health Sciences, University of Calgary, University of Calgary, Calgary, Alberta, Canada Schools of Pharmacy and Public Health and Health Systems, University of Waterloo, Waterloo, Ontario, Canada Institute for Clinical Evaluative Sciences, Toronto, Ontario, Canada
*
Correspondence to: Nathalie Jetté, Foothills Medical Center, Department of Clinical Neurosciences, 1403-29th Street NW, Calgary, Alberta T2N 4N1, Canada. Email: [email protected].
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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 comportementale DFT était presque quatre fois plus fréquente que les aphasies progressives primaires. Conclusions: Les estimations basées sur la population en ce qui concerne l’épidémiologie de la DFT étaient très variables dans les études que nous avons retenues. Il faudra raffiner le processus diagnostique, possiblement par l’utilisation de biomarqueurs validés ou limitant la constatation des cas à ceux confirmés par des services spécialisés, pour obtenir des estimations plus précises de la prévalence et de l’incidence de la DFT.

Keywords

DementiaAlzheimer’s DiseaseLewy Body DementiaFrontotemporal Dementiasystematic reviewmeta-analysis
Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 43 , Supplement S1: The Epidemiology of Dementia and its Commoner Neurodegenerative Causes Systematic Reviews and Meta-Analyses of the Incidence and Prevalence of Dementia, Alzheimer’s Disease, Dementia with Lewy Bodies and Frontotemporal Dementia , April 2016 , pp. S96 - S109
Creative Commons
Creative Common License - CCCreative Common License - BY
This is an open access article, distributed under the terms of the creative commons attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited.
Copyright
Copyright © The Canadian Journal of Neurological Sciences Inc. 2016

Introduction

Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous group of non-Alzheimer neurodegenerative dementias characterized by progressive decline in behaviour (behavioural variant FTD [bvFTD]) and/or language (primary progressive aphasias, such as semantic dementia and progressive non-fluent aphasia [PNFA]) associated with degeneration of the frontal and anterior temporal lobes.Reference Rabinovici and Miller 1 , Reference Warren, Rohrer and Rossor 2 These presentations can overlap with atypical parkinsonian disorders (i.e., corticobasal syndrome [CBS], progressive supranuclear palsy [PSP]) and amyotrophic lateral sclerosis [ALS]). Though a variety of terms have been employed to describe these conditions (e.g., frontotemporal degeneration, frontotemporal lobar degeneration [which some restrict to pathologically confirmed cases], dementia of the frontal type, Pick complex disorder), in this paper we will use FTD. First described in the late 19th century,Reference Warren, Rohrer and Rossor 2 FTD is generally held to be a relatively common cause of early-onset (<65 years) dementia.Reference Rossor, Fox, Mummery, Schott and Warren 3 Dementia clinic-based studies suggest that FTD may account for approximately 5% of all dementia cases,Reference Feldman, Levy, Hsiung, Peters, Donald and Black 4 but this may represent an inaccurate estimate of the proportion of dementia cases arising from this condition because of selection or referral bias.Reference Chow, Hodges, Dawson, Miller, Smith and Mendez 5

In the absence of autopsy confirmation, the diagnosis of FTD is dependent on searching for clinical diagnostic features. In research and practice, the most commonly used criteria for detection of FTD over the last 20 years were those developed by the Lund and Manchester Groups, 6 Gregory and Hodges,Reference Gregory and Hodges 7 Neary et al.Reference Neary, Snowden, Gustafson, Passant, Stuss and Black 8 and the Work Group of Frontotemporal Dementia and Pick’s Disease (also referred to as the McKhann criteria).Reference McKhann, Albert, Grossman, Miller, Dickson and Trojanowski 9 Revised diagnostic criteria have been recently developed.Reference Gorno-Tempini, Hillis, Weintraub, Kertesz, Mendez and Cappa 10 , Reference Rascovsky, Hodges, Knopman, Mendez, Kramer and Neuhaus 11

Population-based prevalence and incidence studies are essential for understanding the societal burden of FTD and planning for the range of healthcare services needed for those with this condition. In this paper, we report on a systematic review of population-based prevalence and incidence studies of FTD.

Methods

This is one in a series of systematic reviews on the prevalence and incidence of priority neurological conditions identified by the Public Health Agency of Canada and the Neurological Health Charities Canada as part of the National Population Health Study of Neurological Conditions.Reference Caesar-Chavannes and MacDonald 12

Search Strategy

The systematic review was conducted according to a predetermined protocol based on the PRISMA Statement for systematic reviews and meta-analyses.Reference Moher, Liberati, Tetzlaff and Altman 13 The search strategy (see Appendix A) was developed by the study authors, who possess extensive expertise in dementia and/or epidemiology, in consultation with a research librarian experienced in the performance of systematic reviews. The initial MEDLINE and EMBASE search was conducted in February of 2011 and then updated in May of 2012. The review was restricted to studies written in English or French and published from the year 2000 or later for international studies and 1985 or later for Canadian studies. References were exported and managed using EndNote X5.

Study Selection

Two reviewers independently screened all abstracts in order to identify original research that appeared to be reporting on the prevalence or incidence of dementia. These papers were selected for full-text review. Studies were excluded at this stage if the abstract clearly indicated that the study was not population-based.

Two reviewers independently performed the full-text reviews. Articles were included in this systematic review if they met the following criteria: (1) represented original research; (2) were population-based (i.e., involved a defined “general population” as opposed to a specific hospital- or clinic-based population); and (3) reported an incidence and/or prevalence estimate of FTD. English and French articles were screened and reviewed in a similar fashion by reviewers fluent in English and French, respectively. The references of included articles were hand searched for additional articles. All additional articles were evaluated in a manner identical to what has been previously outlined. The references of systematic reviews and literature reviews on the epidemiology of dementia were also hand searched. Disagreements were resolved by consensus, with involvement of a third party if necessary (this step was never required).

Data Extraction and Study Quality

Two reviewers extracted data from included articles using a standard data collection form. Agreement was reached on all items. If multiple articles reported data on the same study population, the most comprehensive data were utilized. In cases where the studies reported on different time frames or subgroups (e.g., by sex and/or age), all data were included. Demographic data retrieved included age, sex and study location. Source/type of clinical data and the definition/diagnostic criteria used for the diagnosis of FTD were noted. Incidence and prevalence estimates of dementia from each study were recorded, along with any stratification by age, sex or year of data collection. The quality of the included studies was evaluated using an assessment instrumentReference Boyle 14 , Reference Loney, Chambers, Bennett, Roberts and Stratford 15 (see Appendix B). This instrument included an evaluation of sample representativeness, condition assessment and statistical methods. Each study was given a quality score that ranged from 0 to 8 (higher being better).

Data Analysis

Pooled meta-analyses were not done due to significant between-study heterogeneity and small sample size. Forest plots presenting the distribution of study estimates were produced. As it is held that FTD disproportionately affects middle-aged individuals,Reference Warren, Rohrer and Rossor 2 we compared studies that included older participants (65+) with those that restricted themselves to younger (<65) individuals. All statistical analyses were carried out in R version 2.14. The meta package was employed to produce the forest plots. Depending on the methodology of the study, incidence proportion, incidence rate, period prevalence and point prevalence are provided.

Results

Identification and Description of Studies

The search strategy yielded 16,066 citations (8743 from MEDLINE, 7323 from EMBASE, with 7923 remaining after the removal of duplicates) (Figure 1). A total of 707 articles were selected for full-text review. Of the 176 studies (total of the original and updated searches plus the hand search of included articles) that presented data on the prevalence and/or incidence of dementia and its subtypes, 26 reported on FTD and were included in our systematic review.

Figure 1 Study flow diagram.

The characteristics of the 26 included studies are summarized in Tables 1-3. NineteenReference Banerjee, Mukherjee, Dutt, Shekhar and Hazra 16 - Reference Yamada, Hattori, Miura, Tanabe and Yamori 34 reported on prevalence, sixReference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 - Reference Ravaglia, Forti, Maioli, Martelli, Servadei and Brunetti 40 on incidence and one study provided data on both the incidence and prevalence of FTD.Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 Sixteen studies presented data from Europe, seven from Asia, two from South America, and one from North America. While a variety of approaches was employed, most studies were either surveys of residents of a specific location (n=12) or based on cases identified by specialty services serving a defined catchment area (n=8). The NearyReference Neary, Snowden, Gustafson, Passant, Stuss and Black 8 (n=16) and/or Lund and Manchester 6 (n=9) criteria were the diagnostic criteria most commonly used, with diagnosis typically based on an assessment by a healthcare professional (n=22), often coupled with imaging studies (n=18) and/or laboratory investigations (n=15), and/or health record review (n=10).

Table 1 Studies Reporting on the Prevalence of Frontotemporal Dementia

AD=Alzheimer’s disease; bvFTD=behavioural variant FTD; CBD=corticobasal degeneration; CI95%=95% confidence interval; CT=computed tomography; DSM–III–R=Diagnostic and Statistical Manual of Mental Disorders, 3rd ed., revised; EEG=electroencephalography; FTD=frontotemporal dementia; LP=lumbar puncture; SD=standard deviation.

Prevalence of Frontotemporal Dementia

Fourteen articles reported on point prevalenceReference Borroni, Alberici, Grassi, Rozzini, Turla and Zanetti 17 , Reference Borroni, Alberici, Grassi, Turla, Zanetti and Bianchetti 18 , Reference Gilberti, Turla, Alberici, Bertasi, Civelli and Archetti 20 , Reference Gurvit, Emre, Tinaz, Bilgic, Hanagasi, Sahin and Gurol 22 - Reference Herrera, Caramelli, Silveira and Nitrini 24 , Reference Ikeda, Hokoishi, Maki, Nebu, Tachibana and Komori 26 - Reference Kivipelto, Helkala, Laakso, Hänninen, Hallikainen and Alhainen 28 , Reference Ratnavalli, Brayne, Dawson and Hodges 30 - Reference Yamada, Hattori, Miura, Tanabe and Yamori 34 (Figure 2). Estimates ranged widely from 0.01 to 4.61 per 1000. Among studies restricted to individuals less than 65 years of age,Reference Borroni, Alberici, Grassi, Rozzini, Turla and Zanetti 17 , Reference Harvey, Skelton-Robinson and Rossor 23 , Reference Ikejima, Yasuno, Mizukami, Sasaki, Tanimukai and Asada 27 , Reference Ratnavalli, Brayne, Dawson and Hodges 30 point prevalence was in a narrower range (0.07-0.30 per 1000). Studies that surveyed a defined populationReference Gurvit, Emre, Tinaz, Bilgic, Hanagasi, Sahin and Gurol 22 , Reference Herrera, Caramelli, Silveira and Nitrini 24 , Reference Ikeda, Hokoishi, Maki, Nebu, Tachibana and Komori 26 , Reference Stevens, Livingston, Kitchen, Manela, Walker and Katona 32 - Reference Yamada, Hattori, Miura, Tanabe and Yamori 34 generally reported higher estimates than those based on enumerating cases identified by specialty servicesReference Borroni, Alberici, Grassi, Rozzini, Turla and Zanetti 17 , Reference Borroni, Alberici, Grassi, Turla, Zanetti and Bianchetti 18 , Reference Gilberti, Turla, Alberici, Bertasi, Civelli and Archetti 20 , Reference Ratnavalli, Brayne, Dawson and Hodges 30 (range of estimates in surveys 0.13-4.61 per 1000 compared to 0.15-0.40 per 1000 for specialty services), but the latter were in a narrower range.

Figure 2 Point prevalence of frontotemporal dementia.

Six studiesReference Banerjee, Mukherjee, Dutt, Shekhar and Hazra 16 , Reference Gascon-Bayarri, Reñé, Del Barrio, De Pedro-Cuesta, Ramón and Manubens 19 , Reference Gislason, Sjögren, Larsson and Skoog 21 , Reference Ibach, Koch, Koller and Wolfersdorf 25 , Reference Lee, Lee, Ju, Lee, Kim and Jhoo 29 , Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 reported on period prevalence (Figure 3). Period prevalence estimates ranged from 0.16 to 31.04 per 1000. The Gislason studyReference Gislason, Sjögren, Larsson and Skoog 21 reported a strikingly high prevalence. A number of features of this study are unique: a single-phase survey approach was used; only a relatively small number of participants were examined (n=494); the age range studied (85-86) was both narrow and quite advanced; and the focus of the study was detecting what was termed a frontal lobe syndrome, with these individuals diagnosed as having bvFTD if they did not meet exclusionary criteria, irrespective of whether they met DSM–III–R criteria for a dementia (9/14 cases did not). Estimates derived from surveysReference Banerjee, Mukherjee, Dutt, Shekhar and Hazra 16 , Reference Gascon-Bayarri, Reñé, Del Barrio, De Pedro-Cuesta, Ramón and Manubens 19 , Reference Gislason, Sjögren, Larsson and Skoog 21 , Reference Lee, Lee, Ju, Lee, Kim and Jhoo 29 tended to be higher than those based on cases identified by specialty servicesReference Ibach, Koch, Koller and Wolfersdorf 25 , Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 (range 0.15-31.04 per 1000 vs. 0.27-0.48 per 1000), but the latter were in a narrower range.

Figure 3 Period prevalence of frontotemporal dementia.

FTD accounted for 2.7% (range 0-9.1%) of all dementia cases in the prevalence studies that included individuals older than 65 years of ageReference Banerjee, Mukherjee, Dutt, Shekhar and Hazra 16 , Reference Gascon-Bayarri, Reñé, Del Barrio, De Pedro-Cuesta, Ramón and Manubens 19 , Reference Gislason, Sjögren, Larsson and Skoog 21 , Reference Gurvit, Emre, Tinaz, Bilgic, Hanagasi, Sahin and Gurol 22 , Reference Herrera, Caramelli, Silveira and Nitrini 24 - Reference Ikeda, Hokoishi, Maki, Nebu, Tachibana and Komori 26 , Reference Kivipelto, Helkala, Laakso, Hänninen, Hallikainen and Alhainen 28 , Reference Lee, Lee, Ju, Lee, Kim and Jhoo 29 , Reference Stevens, Livingston, Kitchen, Manela, Walker and Katona 32 - Reference Yamada, Hattori, Miura, Tanabe and Yamori 34 , Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 compared to 10.2% (2.8-15.7%) among studies restricted to those younger than 65.Reference Harvey, Skelton-Robinson and Rossor 23 , Reference Ikejima, Yasuno, Mizukami, Sasaki, Tanimukai and Asada 27 , Reference Ratnavalli, Brayne, Dawson and Hodges 30

Incidence of Frontotemporal Dementia

Only one study reported on incidence proportion.Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 This community-based study of subjects between 42 to 92 years of age consisted of 29,357 persons followed for 6 years. Incidence proportion was estimated to be 0.11 per 1000.

Six studiesReference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 - Reference Ravaglia, Forti, Maioli, Martelli, Servadei and Brunetti 40 reported on incidence rate (Figure 4). Participants were followed for between 3 and 25 years. Incidence rate estimates ranged from 0.00 to 0.33 per 1000 person-years. Among studies restricted to individuals less than 65Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 or 70Reference Knopman, Petersen, Edland, Cha and Rocca 36 years of age, incidence rate was in a narrower range (0.00-0.06 per 1000 person-years) compared to studies that included older subjects (0.17-0.33 per 1000 person-years). Estimates derived from surveysReference Nitrini, Caramelli, Herrera, Bahia, Caixeta and Radanovic 38 , Reference Ravaglia, Forti, Maioli, Martelli, Servadei and Brunetti 40 were higher than specialty service-based onesReference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 (range 0.28-0.55 per 1000 person-years vs. 0.03-0.05 per 1000 person-years), but the latter were in a narrower range.

Figure 4 Incidence rate of frontotemporal dementia.

FTD accounted for 2.0% (range 0.2-3.9%) of all dementia cases in incidence studies including older subjectsReference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Nitrini, Caramelli, Herrera, Bahia, Caixeta and Radanovic 38 - Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 and 15.3% (range 6.7-29.6%) in studies restricted to those younger than 65Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 or 70Reference Knopman, Petersen, Edland, Cha and Rocca 36 years of age.

Type of Frontotemporal Dementia

Among studies that supplied information on the frequency of the type of FTD detected,Reference Borroni, Alberici, Grassi, Turla, Zanetti and Bianchetti 18 , Reference Gilberti, Turla, Alberici, Bertasi, Civelli and Archetti 20 , Reference Ratnavalli, Brayne, Dawson and Hodges 30 , Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 - Reference Mercy, Hodges, Dawson, Barker and Brayne 37 bvFTD (n=299, 79.7% of the total of 375 cases, which included one classified as FTD with ALS) was approximately four times as common as the primary progressive aphasias (n=75, 20%; 32 semantic dementia, 43 PNFA). A study that provided population estimates based on FTD cases admitted to state psychiatric hospitals serving defined catchment areasReference Ibach, Koch, Koller and Wolfersdorf 25 not surprisingly reported that all had behavioural symptoms (61% had behavioural only and 39% both behavioural and language symptoms). Two studiesReference Rosso, Donker Kaat, Baks, Joosse, de Koning, Pijnenburg and de Jong 31 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 reporting on a total of 356 individuals with FTD noted that 12 (4.6% of the total number of FTD cases) also had a diagnosis of ALS (note that individuals with ALS were included in the FTD total). Five studiesReference Harvey, Skelton-Robinson and Rossor 23 , Reference Wada-Isoe, Uemura, Suto, Doi, Imamura and Hayashi 33 - Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 reporting on a total of 95 individuals with FTD found an additional 5, and 17 study participants diagnosed with CBS and PSP, respectively (note that individuals with these conditions were not included in the FTD total).

Study Quality

The median study quality score was 6 (range 4-8) (Table 4).

Table 2 Studies Reporting on the Incidence Rate of Frontotemporal Dementia

bvFTD=behavioural variant FTD; CBD=corticobasal degeneration; EOD=early-onset dementia; FTD=frontotemporal dementia; ICD=International Classification of Diseases; LOD=late-onset dementia; PSP=progressive supranuclear palsy.

Table 3 Studies Reporting on the Incidence Proportion of Frontotemporal Dementia

EEG=electroencephalography; FTD=frontotemporal dementia.

Table 4 Quality Assessment Scores of Frontotemporal Dementia Incidence and Prevalence Studies

NR=not reported; NC=not clear; NA=not applicable.

Discussion

We found that population-based prevalence and incidence estimates for FTD were generally low and varied widely, especially among older individuals. FTD accounted for a higher proportion of dementia cases among younger (<65) individuals. In the studies reviewed, there was no apparent predisposition based on sex, and bvFTD was the most common form encountered, and relatively few individuals were felt to have CBS, PSP or ALS.

Two previous systematic reviewsReference Knopman and Roberts 42 , Reference Onyike and Diehl-Schmid 43 based on a smaller number of studies reported similar incidence and prevalence estimates. Knopman and RobertsReference Knopman and Roberts 42 reviewed five prevalence and three incidence studies. For those 45-64 years of age, point prevalence estimates varied tenfold, from 0.02 to 0.22 per 1000, while incidence rates were between 0.027 and 0.041 per 1000. Onyike and Diehl-SchmidReference Onyike and Diehl-Schmid 43 identified seven prevalence and three incidence studies. Their reported prevalence (0.02-0.31 per 1000) and incidence (0.013 and 0.167 per 1000) rates also ranged widely. After completion of our systematic review, three otherwise eligible studiesReference Wada-Isoe, Ito, Adachi, Yamawaki, Nakashita and Kusumi 44 - Reference Luukkainen, Bloigu, Moilanen and Remes 46 that reported on the incidence and/or prevalence of FTD were published. Their estimates fall within the range we report and do not change our main findings.

We suspect that the limitations of the clinical diagnostic criteria used in included studies 6 - Reference McKhann, Albert, Grossman, Miller, Dickson and Trojanowski 9 and concerns about how the criteria were operationalized partially explain the wide ranges in the estimates of prevalence and incidence. These criteria have been criticized for (among other things): (1) their large number of features (some of which were very rare or of debatable validity); (2) lack of guidance as to the number of features required for diagnosis and the relative importance of symptoms; (3) placing greater emphasis on behavioural manifestations compared to language (one of the studies reviewedReference Gascon-Bayarri, Reñé, Del Barrio, De Pedro-Cuesta, Ramón and Manubens 19 only considered behavioural symptoms, while the five studies that solely utilized the Lund and Manchester criteriaReference Harvey, Skelton-Robinson and Rossor 23 , Reference Herrera, Caramelli, Silveira and Nitrini 24 , Reference Ikejima, Yasuno, Mizukami, Sasaki, Tanimukai and Asada 27 , Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 , Reference Andreasen, Blennow, Sjödin, Winblad and Svärdsudd 41 would be expected to preferentially detect bvFTD); (4) ambiguity in the description and time frame of behavioural manifestations; (5) need to infer some aspects of the person’s state (e.g., assessment of the lack of insight); (6) uncertainty on how best to assess cognitive (traditional executive measures vs. social cognition and decision-making tasks) and behavioural (e.g., objective vs. subjective) characteristics; (7) rigidity in how criteria were applied; (8) limited role for supportive features; (9) no estimate for level of diagnostic certainty (e.g., probable or possible); (10) impact of the exclusionary criteria; (11) relative neglect of imaging and genetic characteristics; and (12) their insensitivity for early disease.Reference Rascovsky, Hodges, Knopman, Mendez, Kramer and Neuhaus 11 , Reference Neary, Snowden and Mann 47 - Reference Rascovsky, Hodges, Kipps, Johnson, Seeley and Mendez 49 Patients with bvFTD are frequently misdiagnosed as suffering from a psychiatric illness early in the course of their illnessReference Woolley, Khan, Murthy, Miller and Rankin 50 , Reference Landqvist Waldö, Gustafson, Passant and Englund 51 and referred to mental health services.Reference Johnson, Diehl, Mendez, Neuhaus, Shapira and Forman 52 As an accurate clinical diagnosis of FTD can be very difficult to make upon presentation, long-term follow-up may be needed to establish its presence.Reference Landqvist Waldö, Gustafson, Passant and Englund 51 , Reference Mendez 53 , Reference Silva 54

Autopsy studies indicate that the criteria used in our included studies lack sensitivity. For example, Neary criteriaReference Neary, Snowden, Gustafson, Passant, Stuss and Black 8 were positive in only 79 of 152 (52%) autopsy-confirmed cases of bvFTD,Reference Rascovsky, Hodges, Knopman, Mendez, Kramer and Neuhaus 11 with particular issues among those over the age of 65. The exclusionary features (e.g., early severe amnesia, spatial disorientation) eliminated 26 confirmed cases. Another studyReference Mendez, Shapira, McMurtray, Licht and Miller 55 of these criteria found that they had a low sensitivity (36.5%) at the time of the person’s initial presentation compared to their final clinical diagnosis. The revised diagnostic criteriaReference Gorno-Tempini, Hillis, Weintraub, Kertesz, Mendez and Cappa 10 , Reference Rascovsky, Hodges, Knopman, Mendez, Kramer and Neuhaus 11 will likely have improved sensitivity,Reference Rascovsky, Hodges, Knopman, Mendez, Kramer and Neuhaus 11 , Reference Costa, Suárez-Calvet, Antón, Dols-Icardo, Clarimón and Alcolea 56 but possibly at the cost of worse specificity and a heightened risk of misclassifying individuals as suffering from FTD when they in fact have a frontal variant of Alzheimer’s disease,Reference Woodward, Jacova, Black, Kertesz, Mackenzie and Feldman 57 other neurological causes or a psychiatric diagnosis.Reference Krudop, Kerssens, Dols, Prins, Möller and Schouws 58 Further validation of these revised criteria is qrequired. The newer criteria were not utilized in the studies we reviewed.

Experienced clinicians working in specialty clinics are able to accurately diagnose FTD.Reference Knopman, Boeve, Parisi, Dickson, Smith and Ivnik 59 , Reference Snowden, Thompson, Stopford, Richardson, Gerhard and Neary 60 Clinical acumen can be supplemented by appropriate use of investigations. For example, finding frontotemporal abnormalities without corresponding ones in more posterior brain areas on functional (e.g., single-photon emission computed tomography, positron emission tomography) or structural (e.g., magnetic resonance imaging) neuroimaging studies can improve on the sensitivity of clinical criteria in detecting FTD.Reference Mendez, Shapira, McMurtray, Licht and Miller 61 Basing estimates on patients seen by specialty services who are comprehensively investigated would partially address concerns about the validity of the diagnosis, but at the potential cost of missing cases. A proportion of affected individuals in the catchment area of the study may not be seen by the services utilized in identifying cases, as they may be referred elsewhere (though some authors minimize this possibility because of their conviction that the high regard local practitioners have for their service means that suspected cases will be referred to themReference Borroni, Alberici, Grassi, Rozzini, Turla and Zanetti 17 , Reference Borroni, Alberici, Grassi, Turla, Zanetti and Bianchetti 18 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 ), are not experiencing the types of symptoms that would lead to a referral, or are unwilling to be seen.

We did not find a lower FTD prevalence or incidence among older (65+) compared to younger (<65) individuals. Five studiesReference Borroni, Alberici, Grassi, Turla, Zanetti and Bianchetti 18 , Reference Gilberti, Turla, Alberici, Bertasi, Civelli and Archetti 20 , Reference Ratnavalli, Brayne, Dawson and Hodges 30 , Reference Rosso, Donker Kaat, Baks, Joosse, de Koning, Pijnenburg and de Jong 31 , Reference Knopman, Petersen, Edland, Cha and Rocca 36 reporting estimates by age subgroups found the highest prevalence and incidence rates between 60 and the mid-70s. The studyReference Gislason, Sjögren, Larsson and Skoog 21 with the oldest subjects included in our systematic review reported the highest prevalence of FTD, but this investigation had other unique features that may have contributed to the high estimate. Studies published subsequent to our review have reported high prevalenceReference Wada-Isoe, Ito, Adachi, Yamawaki, Nakashita and Kusumi 44 and incidenceReference Nilsson, Landqvist Waldö, Nilsson, Santillo and Vestberg 45 rates in older populations. While we found that FTD made up a larger proportion of dementia cases among those less than 65 compared to older patients, this appeared to be driven more by the exponential increase in prevalence and incidence with advancing age of other neurodegenerative causes of dementia, in particular Alzheimer’s disease (AD), than a higher incidence of FTD among those less than 65 compared to older individuals. Differentiating FTD from AD can be particularly challenging at more advanced ages. Compared to younger individuals with FTD, older (65+) persons with this condition tend to have more memory and visuospatial deficits suggestive of AD while showing less pronounced frontal and temporal lobar atrophy on imaging studies.Reference Baborie, Griffiths, Jaros, Momeni, McKeith and Burn 62 , Reference Shinagawa, Toyota, Ishikawa, Fukuhara, Hokoishi and Komori 63 The perception that FTD becomes less common as we age may be due to the increasing difficulty in differentiating it from other forms of dementia.

Our systematic review was not restricted to population-based studies with autopsy confirmation of the clinical diagnosis. There are but few of these studies—a systematic review published in 2006Reference Zaccai, Ince and Brayne 64 could only identify six. Their restriction to high-income countries, questions about the generalizability of their results and the relative rarity of FTD coupled with the limited number of brains being collected mean that these extremely valuable studies cannot fully address questions about the population-based prevalence and incidence of FTD. As well, in an era without specific disease-modifying therapies, the patient’s clinical profile rather than their underlying pathology will be driving service provision.

Notwithstanding the limitations noted above, our systematic review of the incidence and prevalence of FTD updates and expands on prior work. Because of the nature of their symptoms, it has been argued that individuals with FTD, especially early-onset cases,Reference Borroni, Alberici, Grassi, Rozzini, Turla and Zanetti 17 , Reference Garre-Olmo, Genís Batlle, del Mar Fernández, Marquez Daniel, de Eugenio Huélamo and Casadevall 35 will be referred to assessment and management services. While relying on figures from these sources will underestimate the overall prevalence and incidence of this condition, collating data from specialty services might still be an efficient way of capturing data on patients requiring assistance from the healthcare system. The Cambridgeshire studies of early-onset dementia found that all those with FTD in contact with the healthcare system were known to local specialist services,Reference Ratnavalli, Brayne, Dawson and Hodges 30 , Reference Mercy, Hodges, Dawson, Barker and Brayne 37 while in SwedenReference Nilsson, Landqvist Waldö, Nilsson, Santillo and Vestberg 45 diagnoses of FTD were almost exclusively made by specialist clinics. Standardization of methods and refinements in the diagnostic process, possibly by the use of validated biomarkers, will hopefully improve the precision of prevalence and incidence estimates of this challenging condition.

Acknowledgments

We thank Ms. Diane Lorenzetti, librarian at the University of Calgary, who guided the development of the search strategy for this systematic review. Our study is part of the National Population Health Study of Neurological Conditions. We acknowledge the membership of Neurological Health Charities Canada and the Public Health Agency of Canada for their contribution to the success of this initiative. Funding for the study was provided by the Public Health Agency of Canada. The opinions expressed in this publication are those of the authors/researchers and do not necessarily reflect the official views of the Public Health Agency of Canada.

Disclosures

Kirsten Fiest, Jodie Roberts, Dawn Pearson, Pamela Roach, Andrew Kirk, Colleen Maxwell, Eric Smith and Tamara Pringsheim do not have anything to disclose.

David Hogan has the following disclosures: He holds the Brenda Strafford Foundation Chair in Geriatric Medicine, though receives no salary support from this.

Nathalie Jetté has the following disclosures: Public Health Agency of Canada, Principal Investigator, research support; Canada Research Chair, Researcher, research support; Alberta Innovates Health Solutions, Researcher, research support.

Statement of Authorship

DBH, NJ, KMF, JIR, TP and CJM contributed to study conception and design. DBH, NJ, KMF, JIR, DP, EES, PR, AK and CJM contributed to the acquisition of data. KMF and DBH conducted the data analysis. DBH, NJ, KMF, JIR, EES and CJM participated in the interpretation of study data. All authors participated in critically revising the manuscript for important intellectual content and gave final approval for the submission of this manuscript and any further submissions of this work.

Supplementary Material

To view the supplementary material that exist for this study (Appendix e-1 and e-2), please visit http://dx.doi.org/10.1017/cjn.2016.25.

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Figure 0

Figure 1 Study flow diagram.

Figure 1

Table 1 Studies Reporting on the Prevalence of Frontotemporal Dementia

Figure 2

Figure 2 Point prevalence of frontotemporal dementia.

Figure 3

Figure 3 Period prevalence of frontotemporal dementia.

Figure 4

Figure 4 Incidence rate of frontotemporal dementia.

Figure 5

Table 2 Studies Reporting on the Incidence Rate of Frontotemporal Dementia

Figure 6

Table 3 Studies Reporting on the Incidence Proportion of Frontotemporal Dementia

Figure 7

Table 4 Quality Assessment Scores of Frontotemporal Dementia Incidence and Prevalence Studies

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