Patterns of White Matter Atrophy in Frontotemporal Lobar Degeneration
Open accessAbstract
BACKGROUND: Structural magnetic resonance imaging (MRI) has been used to investigate the in vivo pathology of frontotemporal lobar degeneration. However, few neuroimaging studies have focused on white matter (WM) alterations in this disease. OBJECTIVES: To use volumetric MRI techniques to identify the patterns of WM atrophy in vivo in 2 clinical variants of frontotemporal lobar degeneration-frontotemporal dementia (FTD) and semantic dementia-and to compare the patterns of WM atrophy with those of gray matter (GM) atrophy in these diseases. DESIGN: Structural MRIs were obtained from patients with FTD (n = 12) and semantic dementia (n = 13) and in cognitively healthy age-matched controls (n = 24). Regional GM and WM were classified automatically from high-resolution T1-, T2-, and proton density-weighted MRIs with Expectation-Maximization Segmentation and compared between the groups using a multivariate analysis of covariance model that included age and WM lesion volumes as covariates. RESULTS: Patients with FTD had frontal WM atrophy and frontal, parietal, and temporal GM atrophy compared with controls, who had none. Patients with semantic dementia had temporal WM and GM atrophy and patients with FTD had frontal GM atrophy. Adding temporal WM volume to temporal GM volume significantly improved the discrimination between semantic dementia and FTD. CONCLUSIONS: These results show that patients with frontotemporal lobar degeneration who are in relatively early stages of the disease (Clinical Dementia Rating score, 1.0-1.2) have WM atrophy that largely parallels the pattern of GM atrophy typically associated with these disorders.
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Genetic variants associated with MOBP may play a role in selectively affecting white matter regions involved with frontotemporal lobar degeneration, most strikingly in CBD.
“In contrast to MAPT and compatible with its known function in normal myelin formation [35], MOBP showed highest expression within white matter indicating that genetic variants associated with MOBP may play a role in selectively affecting white matter regions known to be involved with frontotemporal lobar degeneration [8], most strikingly in CBD.”
From Shared genetic risk between corticobasal degeneration, progressive supranuclear palsy, and frontotemporal dementia (2017) · cites this paper as [8]
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- Authors
- Chao, L. L., Schuff, N., Clevenger, E. M., Mueller, S. G., Rosen, H. J., Gorno-Tempini, M. L., Kramer, J. H., Miller, B. L., & Weiner, M. W.
- Affiliations
- San Francisco VA Medical Center (US)
- ORCID
- Venue
- Archives of Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 2007-11-01
- Volume
- 64
- Issue
- 11
- Pages
- 1619
- ISSN
- 0003-9942 (ISSN-L)
- PMC
- PMC2443735
- OpenAlex
- W2024576158
- Cited by
- 40
- References
- 0
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 1 · 2021: 3 · 2019: 1 · 2018: 3 · 2017: 3 · 2016: 1 · 2014: 4 · 2013: 3 · 2012: 3
- FWCI
- 2.02
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Archives of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health (AG19724)
- National Institute on Aging (P01-AG19724, P01AG019724)
- 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
- frontotemporal lobar degeneration, white matter atrophy, gray matter atrophy, frontotemporal dementia, semantic dementia, volumetric MRI, expectation-maximization segmentation, lesion volume, differential diagnosis, temporal lobe, frontal lobe
- MeSH terms
- Aged, Atrophy, Brain Mapping, Cerebral Cortex, Dementia, Female, Humans, Image Processing, Computer-Assisted, Magnetic Resonance Imaging, Male, Middle Aged, Neuroglia, Species Specificity
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