Disease trajectories in behavioural variant frontotemporal dementia, primary psychiatric and other neurodegenerative disorders presenting with behavioural change
Open accessAbstract
Behavioural variant frontotemporal dementia (bvFTD) is characterized by behavioural and social cognitive disturbances, while various psychiatric and neurodegenerative disorders may have similar clinical symptoms. Since neurodegenerative disorders are eventually progressive, whereas primary psychiatric disorders are not, this study aimed to investigate whether the change in clinical symptoms over time differed between groups and which biomarkers predicted rate of decline. Disease trajectories (median follow-up = 3 years) of frontal and stereotyped behaviour, general and frontal cognitive functioning, and social cognition were examined in bvFTD (n = 34), other neurodegenerative (n = 28) and primary psychiatric disorders (n = 43), all presenting with late-onset frontal lobe syndrome (45-75 years), using linear mixed models. To gain more insight in underlying pathological processes driving disease progression, we studied the association of baseline cerebrospinal fluid (CSF) (neurofilament light (NfL) and YKL-40 levels, phosphotau 181 to total tau ratio) and neuroimaging markers with disease trajectories. Frontal behavioural symptoms (e.g., disinhibition, apathy) worsened over time in bvFTD, whereas they improved in psychiatric disorders and remained stable in other neurodegenerative disorders. General and frontal cognitive decline was observed in bvFTD and other neurodegenerative disorders, but not in psychiatric disorders. None of the groups showed change in stereotypy and social cognition. For all diagnostic groups, higher CSF NfL levels were associated with faster frontal cognitive decline. A modest association was observed between caudate volume and stereotyped behaviour. Tracking frontal behavioural symptoms and cognition has potential to distinguish bvFTD from other disorders. CSF NfL levels seem to be associated with decline in frontal cognitive functioning.
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While one study reported no change of stereotypy over time, the SRI predicted progression of frontotemporal atrophy, institutionalization and death.
“While one study reported no change of stereotypy over time, the SRI predicted progression of frontotemporal atrophy, institutionalization and death (Reus et al., 2018; Gossink et al., 2019) (Table 1).”
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 Reus et al., 2018
Other studies did not support decline on the Ekman-60-faces, reporting no change or improvement over 3 years.
“However, other studies did not support this decline, reporting no change or improvement on the Ekman-60-faces over 3 years (Lavenu and Pasquier, 2005; Reus et al., 2018).”
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 Reus et al., 2018
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References 43 · Cited by 26
- Authors
- Reus, L. M., Vijverberg, E. G., Tijms, B. M., Kate, M. T., Gossink, F., Krudop, W. A., Campo, M. D., Teunissen, C. E., Barkhof, F., van der Flier, W. M., Visser, P. J., Dols, A., & Pijnenburg, Y. A.
- Affiliations
- Amsterdam Neuroscience (NL)
- GGZ inGeest (NL)
- University Medical Center Utrecht (NL)
- Amsterdam University Medical Centers (NL)
- Maastricht University (NL)
- Corresponding author
- Reus, Lianne M.
- ORCID
- Venue
- Journal of Psychiatric Research
- Publisher
- Elsevier BV
- Published
- 2018-09
- Volume
- 104
- Pages
- 183-191
- ISSN
- 0022-3956 (ISSN-L)
- OpenAlex
- W2886289674
- Cited by
- 26
- References
- 43
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 3 · 2022: 7 · 2021: 2 · 2020: 6 · 2019: 4 · 2018: 1
- FWCI
- 2.55
- Type
- journal article
- Language
- en
- Open access
- green
- discovery.ucl.ac.uk
- Locations
- Journal of Psychiatric Research (publishedVersion)
- PubMed (publishedVersion)
- Research Publications (Maastricht University) (publishedVersion)
- Pure Amsterdam UMC (publishedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- Research Publications (Maastricht University) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Funders
- Innovative Medicines Initiative 2 (115916)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- behavioral variant frontotemporal dementia, disease trajectories, frontal lobe syndrome, behavior change, neurodegenerative diseases, primary psychiatric disorders, social cognition, stereotypy, disinhibition, apathy, neurofilament light chain, YKL-40, caudate volume, cerebrospinal fluid biomarkers, differential diagnosis
- MeSH terms
- Aged, Mental Disorders, Cognition Disorders, Female, Humans, Image Processing, Computer-Assisted, Longitudinal Studies, Magnetic Resonance Imaging, Male, Middle Aged, Neuropsychological Tests, Psychiatric Status Rating Scales, Stereotyped Behavior, Disease Progression, Neurodegenerative Diseases, Frontotemporal Dementia, Apathy
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