FRONTotemporal dementia Incidence European Research Study—FRONTIERS: Rationale and design

Borroni, Barbara; Graff, Caroline; Hardiman, Orla; Ludolph, Albert C.; Moreno, Fermín; Otto, Markus; Piccininni, Marco; Remes, Anne M.; Rowe, James B.; Seelaar, Harro; Stefanova, Elka; Traykov, Latchezar; Logroscino, Giancarlo; Frontiers,

doi:10.1002/alz.12414

Cited by 14 publications

(13 citation statements)

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“…Accuracy of data about FTD is hampered by underdiagnosis and different criteria adopted. For better estimation, a multicenter prospective observational study designed in Europe is underway [21].…”

Section: Epidemiology Of Tauopathiesmentioning

confidence: 99%

“…Only approximately 31% have been reported with family history [102], which is even lower in PSP and GGT [69]. Familial inheritance is usually in an autosomal dominant pattern in FTD and is associated with three different genes (C9orf72, GRN, or MAPT), of which MAPT occupies 5-10% [21,102]. While in secondary tauopathies, including AD, myotonic dystrophy, Down syndrome, etc., no pathogenic MAPT mutation has been found.…”

Section: Mapt Mutationsmentioning

confidence: 99%

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Tauopathies: new perspectives and challenges

Zhang

Liu

et al. 2022

Mol Neurodegeneration

132

114

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Background Tauopathies are a class of neurodegenerative disorders characterized by neuronal and/or glial tau-positive inclusions. Main body Clinically, tauopathies can present with a range of phenotypes that include cognitive/behavioral-disorders, movement disorders, language disorders and non-specific amnestic symptoms in advanced age. Pathologically, tauopathies can be classified based on the predominant tau isoforms that are present in the inclusion bodies (i.e., 3R, 4R or equal 3R:4R ratio). Imaging, cerebrospinal fluid (CSF) and blood-based tau biomarkers have the potential to be used as a routine diagnostic strategy and in the evaluation of patients with tauopathies. As tauopathies are strongly linked neuropathologically and genetically to tau protein abnormalities, there is a growing interest in pursuing of tau-directed therapeutics for the disorders. Here we synthesize emerging lessons on tauopathies from clinical, pathological, genetic, and experimental studies toward a unified concept of these disorders that may accelerate the therapeutics. Conclusions Since tauopathies are still untreatable diseases, efforts have been made to depict clinical and pathological characteristics, identify biomarkers, elucidate underlying pathogenesis to achieve early diagnosis and develop disease-modifying therapies.

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Section: Epidemiology Of Tauopathiesmentioning

confidence: 99%

Section: Mapt Mutationsmentioning

confidence: 99%

Tauopathies: new perspectives and challenges

Zhang

Liu

et al. 2022

Mol Neurodegeneration

132

114

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“…This observation prompted the formation of the Frontotemporal Dementia Incidence European Research Study (FRONTIERS), a collaborative European research initiative aimed at assessing FTLD incidence across Europe, aligning multinational population-based disease registries. 20 In the present retrospective analysis, FRONTIERS aimed to address 6 critical issues in the field: (1) to assess the incidence of FTLD-related disorders across Europe; (2) to assess the geographic heterogeneity of the disease presentations; (3) to define age-and sex-specific incidence rates of FTLD; (4) to describe the distribution of different FTLD phenotypes, related clinical features, and phenotype heterogeneity; (5) to assess the frequency of a positive family history across phenotypes; and (6) to estimate the numbers of new cases in Europe on the basis of the data obtained.…”

mentioning

confidence: 99%

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries

et al. 2023

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ImportanceDiagnostic incidence data for syndromes associated with frontotemporal lobar degeneration (FTLD) in multinational studies are urgent in light of upcoming therapeutic approaches.ObjectiveTo assess the incidence of FTLD across Europe.Design, Setting, and ParticipantsThe 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.Main Outcomes and MeasuresRandom-intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.ResultsBased 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.Conclusions and RelevanceThe 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.

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“…Both DLB and FTD are rare neurodegenerative diseases. The epidemiological collection of data should be based on the reconstructed cohort design, an approach that takes advantage of clinical collection of data within a large population of residents in a well-defined geographic area [ 24 , 25 ]. This approach could also be useful to identify proper biomarkers for the clinical diagnosis of these conditions [ 25 ].…”

Section: What Disease Definition Should We Use For Projections?mentioning

confidence: 99%

Descriptive Epidemiology of Neurodegenerative Diseases: What Are the Critical Questions?

2022

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The field of neurodegenerative diseases is a major challenge faced by Public Health and is still in need of robust preventive measures and disease-modifying treatments. Population-based studies can offer the framework in the context primary and secondary prevention of neurodegenerative diseases. The epidemiology of neurodegenerative disorders in the last decades has focused on descriptive studies mainly based on the use of clinical criteria. However, clinical definition is basically insufficient both to well characterize different phenotypes and to make an early diagnosis. Descriptive epidemiology needs a new framework to update the area of neurodegenerative research, based on advancement of both clinical and biological diagnostic criteria and the urgency for an early diagnosis of the disease. In here, we address the present and future of population-based studies in neurodegenerative disorders and discuss the shift of paradigms in the diagnosis of disease and disease definition. We further debate the changes in biomarker implementation models and type of biomarkers used in population-based studies. Descriptive epidemiology of neurogenerative disorders is rapidly evolving. These implementations will improve the future design and outcome of population-based studies and policy-making in Public Health intervention.

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FRONTotemporal dementia Incidence European Research Study—FRONTIERS: Rationale and design

Cited by 14 publications

References 44 publications

Tauopathies: new perspectives and challenges

Tauopathies: new perspectives and challenges

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries

Descriptive Epidemiology of Neurodegenerative Diseases: What Are the Critical Questions?

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