Epidemiology of Frontotemporal Lobar Degeneration in Northern Finland

Luukkainen, Laura; Bloigu, Risto; Moilanen, Virpi; Remes, Anne M.

doi:10.1159/000440858

Cited by 6 publications

(7 citation statements)

References 23 publications

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“…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 studies 44 - 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.…”

Section: Discussionmentioning

confidence: 99%

The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

Hogan

Jetté

Fiest

et al. 2016

Can. J. Neurol. Sci.

188

124

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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...

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Section: Discussionmentioning

confidence: 99%

The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

Hogan

Jetté

Fiest

et al. 2016

Can. J. Neurol. Sci.

188

124

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“…ALS is the most frequently occurring form of motor neuron disease, affecting approximately 2–4/100,000 individuals (Johnston et al , 2006), and is characterized by progressive paralysis typically leading to death within two to three years after onset. FTD is the second most common form of presenile dementia and affects approximately 20/100,000 individuals between the ages of 45 and 65 (Onyike and Diehl-Schmid, 2013; Luukkainen et al , 2015). FTD presents heterogeneously and is divided into three clinical syndromes; behavioral variant, semantic dementia, and progressive nonfluent aphasia (Neary et al , 1998).…”

Section: Ran Translation At Ggggcc and Ggcccc Repeats In C9 Als/ftdmentioning

confidence: 99%

RAN translation—What makes it run?

2016

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Nucleotide-repeat expansions underlie a heterogeneous group of neurodegenerative and neuromuscular disorders for which there are currently no effective therapies. Recently, it was discovered that such repetitive RNA motifs can support translation initiation in the absence of an AUG start codon across a wide variety of sequence contexts, and that the products of these atypical translation initiation events contribute to neuronal toxicity. This review examines what we currently know and don’t know about repeat associated non-AUG (RAN) translation in the context of established canonical and non-canonical mechanisms of translation initiation. We highlight recent findings related to RAN translation in three repeat expansion disorders: CGG repeats in fragile X-associated tremor ataxia syndrome (FXTAS), GGGGCC repeats in C9orf72 associated amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) and CAG repeats in Huntington disease. These studies suggest that mechanistic differences may exist for RAN translation dependent on repeat type, repeat reading frame, and the surrounding sequence context, but that for at least some repeats, RAN translation retains a dependence on some of the canonical translational initiation machinery.

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“…1 Frontotemporal dementia is the second cause of presenile dementia, and bvFTD represents the most frequent subtype. 2,3 This form is clinically characterized by prominent behavioral disturbances, including disinhibition, impulsivity, perseverations, and loss of empathy, that are associated with deterioration in social conduct and impairment of cognition. 1,4 Recently, a complex and intriguing relationship between bvFTD and several psychiatric disorders has been described.…”

Section: Introductionmentioning

confidence: 99%

High Risk of Suicide in Behavioral Variant Frontotemporal Dementia

Zucca

Rubino

Vacca

et al. 2018

Am J Alzheimers Dis Other Demen

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Background: Recent studies reported an increased risk of suicidality in behavioral variant Frontotemporal Dementia (bvFTD). Aim of the study was to determine the prevalence of suicidal ideation and attempts in bvFTD patients, evaluating possible risk factors for suicidality. Methods: Risk of suicide was assessed using the Scale for Suicide Ideation (SSI) in 35 bvFTD patients and 25 controls. Results: According to SSI, 40% of bvFTD patients had suicidal ideation in comparison to 8% of controls (p=.009). Four bvFTD patients have attempted suicide versus none control (p=.006). BvFTD patients with suicide risk showed higher levels of anxiety, depression, stress and hopelessness than patients without suicide risk (p<.001). Patients who attempted suicide were younger, and had a longer disease duration than those with only suicide ideation. Intriguingly, 40% of patients with parkinsonism presented high level of suicide ideation. Conclusions: Our findings show that bvFTD patients have a high risk of suicide. Additional studies in larger population are needed to confirm our results.

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Epidemiology of Frontotemporal Lobar Degeneration in Northern Finland

Cited by 6 publications

References 23 publications

The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

The Prevalence and Incidence of Frontotemporal Dementia: a Systematic Review

RAN translation—What makes it run?

High Risk of Suicide in Behavioral Variant Frontotemporal Dementia

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