Progression in Behavioral Variant Frontotemporal Dementia: A Longitudinal Study
Open accessAbstract
IMPORTANCE: A gap in the literature exists regarding progression in behavioral variant frontotemporal dementia (BVFTD). Guidance is needed concerning markers that will enable clinicians to discriminate FTD more effectively from phenocopies and to identify factors that determine progression and thereby prognosis. OBJECTIVES: To observe longitudinal outcomes and progression in probable and possible BVFTD in accordance with international diagnostic criteria and to identify features that may aid clinicians to prognosticate better in cases of possible BVFTD. DESIGN, SETTING, AND PARTICIPANTS: Longitudinal cohort study performed in a specialist tertiary FTD research clinic. Fifty-eight consecutive patients were followed up longitudinally from January 1, 2008, through December 31, 2013, and classified as having possible, probable, or definite BVFTD at presentation and latest review. Final follow-up was completed on December 31, 2013, and data were analyzed from January 1 to August 1, 2014. MAIN OUTCOMES AND MEASURES: Clinical, pathological, genetic, neuropsychological, and neuroimaging data were analyzed to categorize patients, to compare differences between groups with changed and unchanged diagnoses, to determine rates of progression in BVFTD, and to identify prognostic features in possible BVFTD. RESULTS: At presentation, 38 of the 58 patients fulfilled criteria for probable BVFTD; of these, 36 continued to satisfy probable criteria or underwent conversion to definite criteria over time. The remaining 20 patients satisfied possible criteria only, and 11 of these patients changed categories over time to probable or definite BVFTD and showed progression on cognitive and functional measures (termed changed status). Of these 11 patients, 8 (73%) carried the C9orf72 expansion. A positive family history, memory impairment, and clinical abnormalities at presentation were key features of progression (P < .05). A continuum of neuropsychological scores, progression rates, and atrophy severity emerged across patients in probable, possible, changed status, and nonchanged status groups; patients with probable BVFTD exhibited the most severe abnormalities. CONCLUSIONS AND RELEVANCE: Behavioral variant FTD shows variable progression over time. Clinicians can use a detailed neurologic and cognitive assessment to identify key predictive features of progression when faced with possible BVFTD, whereas a diagnosis of probable BVFTD is accurate in a clinical setting.
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bvFTD shows highly variable disease progression.
“BvFTD shows highly variable disease progression (Devenney et al., 2015).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Devenney et al., 2015
Longitudinal studies on FTD-FRS captured a multi-domain deterioration over time in sporadic and genetic bvFTD (1-5 years).
“Longitudinal studies on FTD-FRS captured a multi-domain deterioration over time in sporadic and genetic bvFTD (1–5 years) (Devenney et al., 2015; Schubert et al., 2016; Lima-Silva et al., 2021).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Devenney et al., 2015
A minority of bvFTD (20%) may present with intact cognition at first visit, and thereafter cognitive decline is variable.
“A minority of bvFTD (20%) may present with intact cognition at first visit, and thereafter, cognitive decline is variable (Hornberger et al., 2008; Diehl-Schmid et al., 2011; Devenney et al., 2015).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Devenney et al., 2015
Due to its comprehensive, yet feasible design, the ACE-R is a more valid cognitive progression screener for bvFTD, with marked rates of decline over follow-up (1-5 years).
“Due to its comprehensive, yet feasible design, the ACE-R is a more valid cognitive progression screener for bvFTD, with marked rates of decline over follow-up (1–5 years) (Mioshi and Hodges, 2009; Devenney et al., 2015; Schubert et al., 2016).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Devenney et al., 2015
Literature demonstrated declines of MMSE, FAB and ACE-R total scores in bvFTD.
“Literature demonstrated declines of MMSE, FAB and ACE-R total scores in bvFTD (Mioshi and Hodges, 2009; Devenney et al., 2015; Schubert et al., 2016; Reus et al., 2018), but a principal component analysis of these measures (reflecting global cognitive status) showed no association with mortality (Lansdall et al., 2019).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Devenney et al., 2015
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- Authors
- Devenney, E., Bartley, L., Hoon, C., O’Callaghan, C., Kumfor, F., Hornberger, M., Kwok, J. B., Halliday, G. M., Kiernan, M. C., Piguet, O., & Hodges, J. R.
- Affiliations
- UNSW Sydney (AU)
- Neuroscience Research Australia (AU)
- University of Cambridge (GB)
- ARC Centre of Excellence in Cognition and its Disorders (AU)
- Corresponding author
- Devenney, Emma
- ORCID
- Venue
- JAMA Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 2015-12-01
- Volume
- 72
- Issue
- 12
- Pages
- 1501
- ISSN
- 2168-6149 (ISSN-L)
- OpenAlex
- W1882871811
- Cited by
- 61
- References
- 41
- Citations by year
- 2026: 4 · 2025: 3 · 2024: 8 · 2023: 5 · 2022: 3 · 2021: 9 · 2020: 8 · 2019: 4 · 2018: 8 · 2017: 5 · 2016: 4
- FWCI
- 4.60
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- JAMA Neurology (publishedVersion)
- PubMed (publishedVersion)
- UEA Digital Repository (University of East Anglia) (acceptedVersion)
- UNSWorks (UNSW Sydney) (submittedVersion, cc-by-nc-nd)
- UNSWorks (University of New South Wales, Sydney, Australia) (submittedVersion, cc-by-nc-nd)
- Funders
- Neuroscience Research Australia
- Motor Neurone Disease Association
- University of New South Wales
- Centre of Excellence in Cognition and its Disorders, Australian Research Council (CE110001021, 1037746)
- Isaac Newton Trust
- Medical Research Council
- National Health and Medical Research Council (1022684, 1037746)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- behavioral variant frontotemporal dementia, disease progression, prognostic factors, C9orf72 expansion, family history, memory impairment, brain atrophy, neuropsychological assessment, frontotemporal lobar degeneration
- MeSH terms
- C9orf72 Protein, Adult, Aged, Mental Disorders, Brain, Female, Humans, Image Processing, Computer-Assisted, Magnetic Resonance Imaging, Male, Middle Aged, Neuropsychological Tests, Proteins, Severity of Illness Index, Cohort Studies, Disease Progression, Statistics, Nonparametric, Frontotemporal Dementia
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