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Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes
Coyle-Gilchrist IT, Dick KM, Patterson K, Vázquez Rodríquez P, Wehmann E, Wilcox A, Lansdall CJ, Dawson KE et al.. Neurology, 2016-04-01
Topic: Risk, prevention and care in population studies
Objectives To estimate the lifetime risk, prevalence, incidence, and mortality of the principal clinical syndromes associated with frontotemporal lobar degeneration (FTLD) using revised diagnostic criteria and including intermediate clinical phenotypes. Methods Multisource referral over 2 years to identify all diagnosed or suspected cases of frontotemporal dementia (FTD), progressive supranuclear palsy (PSP), or corticobasal syndrome (CBS) in 2 UK counties (population 1.69 million). Diagnostic confirmation used current consensus diagnostic criteria after interview and reexamination. Results were adjusted to the 2013 European standard population. Results The prevalence of FTD, PSP, and CBS was 10.8/100,000. The incidence and mortality were very similar, at 1.61/100,000 and 1.56/100,000 person-years, respectively. The estimated lifetime risk is 1 in 742. Survival following diagnosis varied widely: from PSP 2.9 years to semantic variant FTD 9.1 years. Age-adjusted prevalence peaked between 65 and 69 years at 42.6/100,000: the age-adjusted prevalence for persons older than 65 years is double the prevalence for those between 40 and 64 years. Fifteen percent of those screened had a relevant genetic mutation. Conclusions Key features of this study include the revised diagnostic criteria with improved specificity and sensitivity, an unrestricted age range, and simultaneous assessment of multiple FTLD syndromes. The prevalence of FTD, PSP, and CBS increases beyond 65 years, with frequent genetic causes. The time from onset to diagnosis and from diagnosis to death varies widely among syndromes, emphasizing the challenge and importance of accurate and timely diagnosis. A high index of suspicion for FTLD syndromes is required by clinicians, even for older patients.
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APA 7th
Coyle-Gilchrist, I. T., Dick, K. M., Patterson, K., Vázquez Rodríquez, P., Wehmann, E., Wilcox, A., et al. (2016). Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. https://doi.org/10.1212/wnl.0000000000002638
Vancouver
Coyle-Gilchrist IT, Dick KM, Patterson K, Vázquez Rodríquez P, Wehmann E, Wilcox A, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. 2016. doi:10.1212/wnl.0000000000002638. PMID: 27037234.
BibTeX
@article{coylegilchrist2016-27037234,
author = {Coyle-Gilchrist, I. T. and Dick, K. M. and Patterson, K. and Vázquez Rodríquez, P. and Wehmann, E. and Wilcox, A. and Lansdall, C. J. and Dawson, K. E. and others},
title = {{Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes}},
journal = {Neurology},
year = {2016},
doi = {10.1212/wnl.0000000000002638},
pmid = {27037234},
url = {https://doi.org/10.1212/wnl.0000000000002638}
}
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Key findings
- Objectives To estimate the lifetime risk, prevalence, incidence, and mortality of the principal clinical syndromes associated with frontotemporal lobar degeneration (FTLD) using revised diagnostic criteria and including intermediate clinical phenotypes.
- Results were adjusted to the 2013 European standard population.
- Results The prevalence of FTD, PSP, and CBS was 10.8/100,000.
Why it matters
This study estimated the prevalence, incidence, mortality and survival of frontotemporal lobar degeneration syndromes. It provides epidemiological information about these conditions, not about treating, curing or preventing Alzheimer's disease.
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Study details
- Evidence strengthLow
How this study scored 58/100
Published in a leading specialty journal. Scored zero for study design, sample size, effect reported, studied in people and randomized / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (Neurology) |
| Journal's citation record | 4 / 4 | Neurology: journal h-index 525 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2016 — within 20 year(s) of the newest study here |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomized / blinded | 0 / 2 | No randomization or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 546 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 45.5 citations a year over 12 year(s) since 2016 |
| Impact for its field and year | 5 / 6 | Cited 31.4× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 3 / 6 | Cited by 26 other studies in this library |
| Still being cited | 3 / 4 | Cited 142 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author James Benedict Rowe: h-index 101 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University College London: institution h-index 2,029 (OpenAlex) |
| Multicenter collaboration | 2 / 3 | OpenAlex lists 5 institution(s) in 2 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 27037234) |
| Free to read | 2 / 2 | Free to read |
| Total | 58 / 100 | — |
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