Looking for measures of disease severity in the frontotemporal dementia continuum
Abstract
Frontotemporal dementia (FTD) is characterized by executive dysfunctions, behavioral disturbances, language deficits and extrapyramidal symptoms. Frontotemporal lobar degeneration-modified Clinical Dementia Rating Scale (FTLD modified-CDR) has been proposed to measure disease severity in behavioral variant FTD (bvFTD). No tools of global disease severity are available in the other FTLD phenotypes [primary progressive aphasias (PPAs), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS)]. This would be strategic as outcome measures in clinical trials. To this aim, we evaluated the association between brain volume (voxel based morphometry) and available clinical scales in FTD. In 176 FTD patients (64 bvFTD, 40 PPAs, 32 PSP, 40 CBS), instrumental activities of daily living (ADLs), FTLD-modified CDR, Mini-Mental State Examination (MMSE), Frontal Behavioral Inventory (FBI), and Neuropsychiatry Inventory (NPI) were administered and MRI performed. Whole-brain linear correlation between each clinical rating scale and brain volume was performed. In bvFTD and PPAs, FTLD-modified CDR was associated with regional brain volume, thereby providing evidence for validity of the FTLD-modified CDR. In PSP, none of the clinical indicators were associated with regional brain volume. In CBS, ADLs and MMSE correlated with frontotemporal lower volume. Considering monogenic disease, FTLD-modified CDR was the best measure. In FTD continuum, different measures able to correlate with brain damage should be considered for the different clinical phenotypes or genetic traits.
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The FTLD-CDR score has been associated with bvFTD-specific neuroimaging changes, such as frontotemporal blood flow and atrophy.
“The FTLD-CDR score has been associated with bvFTD specific neuroimaging changes, such as frontotemporal blood flow and atrophy (Borroni et al., 2010; Premi 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 Premi et al., 2016
For MMSE, rates of decline are lacking or modest, and unrelated to frontotemporal changes on multiple neuroimaging modalities.
“For MMSE in specific, rates of decline are known to be lacking or modest, and unrelated to frontotemporal changes on multiple neuroimaging modalities (Borroni et al., 2010; Tan et al., 2013; Premi et al., 2016; Leroy 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 Premi et al., 2016
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- Authors
- Premi, E., Gualeni, V., Costa, P., Cosseddu, M., Gasparotti, R., Padovani, A., & Borroni, B.
- Affiliations
- University of Brescia (IT)
- Corresponding author
- Borroni, Barbara
- ORCID
- Venue
- Journal of Alzheimer’s Disease
- Publisher
- SAGE Publications
- Published
- 2016-04-16
- Volume
- 52
- Issue
- 4
- Pages
- 1227-1235
- ISSN
- 1387-2877 (ISSN-L)
- 1875-8908
- OpenAlex
- W2344927856
- Cited by
- 26
- References
- 33
- Citations by year
- 2026: 2 · 2025: 3 · 2024: 1 · 2023: 2 · 2022: 3 · 2021: 4 · 2020: 2 · 2019: 4 · 2018: 2 · 2017: 3
- FWCI
- 2.66
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Alzheimer s Disease (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
- Keywords
- frontotemporal dementia, disease severity
- MeSH terms
- Activities of Daily Living, Aged, Brain, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neuropsychological Tests, Severity of Illness Index, Frontotemporal Dementia, Neuroimaging
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