Cited by 238Dementia and Cognitive Impairment ResearchAlzheimer's disease research and treatmentsAmyotrophic Lateral Sclerosis Research

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Estimating the Number of Persons with Frontotemporal Lobar Degeneration in the US Population

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Knopman, David S., Roberts, Rosebud O.·2011·Journal of Molecular Neuroscience

DOI 10.1007/s12031-011-9538-yPMC PMC3208074

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Abstract

There are many challenges for determining the prevalence and incidence of frontotemporal lobar degenerations (FTLD). Consequently, the number of cases of behavioral variant frontotemporal dementia (bvFTD) or primary progressive aphasia (PPA) in the USA is unknown. Our objective was to derive a consensus estimate of bvFTD and PPA prevalence and thereby to estimate the total number of these syndromes in the USA. We identified five prevalence and three incidence studies of FTLD based on passive surveillance and seven studies of survival in FTLD. Data from these studies were used to estimate the number of cases of PPA or bvFTD in the USA. Because prevalence and incidence estimates outside of the 45-64-year age range were either not available or widely divergent, we used data from clinical and pathological series to estimate the proportion of FTLD cases aged <45 or >64 years. The prevalence estimates in the age categories of 45-64 years old have ranged from 15 to 22 per 100,000 person-years in studies where both bvFTD and PPA were identified. The incidence estimates for the same age group ranged from 2.7 to 4.1 per 100,000 person-years. Using a survival rate of 6 to 9 years from onset and rates from the incidence studies, a calculated prevalence estimate (prevalence = incidence × duration) was similar to the previously reported prevalence rates. We estimated that 10% of cases were less than age 45 years and 30% were 65 years and older. We estimate that there are approximately 20,000 to 30,000 cases of the cognitive syndromes of FTLD in the USA. The main threat to the accuracy of the estimates is the difficulty in diagnosing the clinical syndromes that comprise the FTLD group of disorders.

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Rascovsky K, Hodges JR, Knopman D, Mendez M, Kramer JH, Van Swieten JC et al (2011) Sensitivity of diagnostic criteria for autopsy-confirmed patients with behavioral variant frontotemporal dementia (bvFTD): first report of the international bvFTD criteria consortium (FTDC). Neurology 76(Suppl 4):A262–A263

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Authors: Knopman, D. S., & Roberts, R. O.
Affiliations: Mayo Clinic (US)
Corresponding author: Knopman, David S.
ORCID: Knopman, David S.
Venue: Journal of Molecular Neuroscience
Publisher: Springer Science and Business Media LLC
Published: 2011-05-17
Volume: 45
Issue: 3
Pages: 330-335
ISSN: 0895-8696 (ISSN-L)
1559-1166
DOI: 10.1007/s12031-011-9538-y
PMC: PMC3208074
OpenAlex: W2074608237
Cited by: 236
References: 32
Citations by year: 2026: 8 · 2025: 14 · 2024: 17 · 2023: 19 · 2022: 22 · 2021: 24 · 2020: 20 · 2019: 19 · 2018: 8 · 2017: 24 · 2016: 14 · 2015: 14 · 2014: 14 · 2013: 14 · 2012: 4
FWCI: 7.22
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
Locations: Journal of Molecular Neuroscience (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: Abbott Laboratories
Eli Lilly and Company
National Institutes of Health
National Institute on Aging (P50‐AG16574, U01AG06786, U01AG006786, R01AG023195, P50AG016574)
Topics: Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords: frontotemporal lobar degeneration, behavioral variant frontotemporal dementia, primary progressive aphasia, prevalence estimation, incidence estimation, age distribution of cases, United States
MeSH terms: Adult, Aged, Humans, Middle Aged, United States, Cohort Studies, Survival Rate, Aphasia, Primary Progressive, Frontotemporal Lobar Degeneration

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Estimating the Number of Persons with Frontotemporal Lobar Degeneration in the US Population

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