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Locus Coeruleus Degeneration Differs Between Frontotemporal Lobar Degeneration Subtypes

Matti, Nathalie ; Javanshiri, Keivan LU orcid ; Haglund, Mattias LU ; Saenz-Sardá, Xavier and Englund, Elisabet LU orcid (2022) In Journal of Alzheimer's Disease 89(2). p.463-471
Abstract

Background: There are few studies on the locus coeruleus (LC) in frontotemporal lobar degeneration (FTLD) and the potential differences in the LC related to the underlying proteinopathy. Objective: The aim of this study was to investigate the LC in FTLD subgroups. Methods: Neuropathological cases diagnosed with FTLD were included. The subgroups consisted of FTLD with tau, transactive response DNA-binding protein 43 (TDP) and fused in sarcoma (FUS). Micro-and macroscopical degeneration of the LC were assessed with respect to the number of neurons and the degree of depigmentation. A group of cognitively healthy subjects and a group with vascular cognitive impairment (VCI) served as comparison groups. Results: A total of 85 FTLD cases were... (More)

Background: There are few studies on the locus coeruleus (LC) in frontotemporal lobar degeneration (FTLD) and the potential differences in the LC related to the underlying proteinopathy. Objective: The aim of this study was to investigate the LC in FTLD subgroups. Methods: Neuropathological cases diagnosed with FTLD were included. The subgroups consisted of FTLD with tau, transactive response DNA-binding protein 43 (TDP) and fused in sarcoma (FUS). Micro-and macroscopical degeneration of the LC were assessed with respect to the number of neurons and the degree of depigmentation. A group of cognitively healthy subjects and a group with vascular cognitive impairment (VCI) served as comparison groups. Results: A total of 85 FTLD cases were included, of which 44 had FTLD-TDP, 38 had FTLD-tau, and three had FTLD-FUS. The groups were compared with 25 VCI cases and 41 cognitively healthy control cases (N = 151 for the entire study). All FTLD groups had a statistically higher microscopical degeneration of the LC compared to the controls, but the FTLD-tau group had greater micro-and macroscopical degeneration than the FTLD-TDP group. Age correlated positively with the LC score in the FTLD-tau group, but not in the FTLD-TDP group. Conclusion: A greater microscopical degeneration of the LC was observed in all FTLD cases compared to healthy controls and those with VCI. The LC degeneration was more severe in FTLD-tau than in FTLD-TDP. The macroscopically differential degeneration of the LC in FTLD subgroups may facilitate differential diagnostics, potentially with imaging.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Frontotemporal dementia, locus coeruleus, neuronal counts, tau, tauopathy, TDP-43
in
Journal of Alzheimer's Disease
volume
89
issue
2
pages
9 pages
publisher
IOS Press
external identifiers
  • scopus:85138454941
  • pmid:35871340
ISSN
1387-2877
DOI
10.3233/JAD-220276
language
English
LU publication?
yes
id
62ecbdef-36c7-448e-b8ab-809c41705eee
date added to LUP
2022-12-30 14:12:25
date last changed
2024-04-15 00:35:32
@article{62ecbdef-36c7-448e-b8ab-809c41705eee,
  abstract     = {{<p>Background: There are few studies on the locus coeruleus (LC) in frontotemporal lobar degeneration (FTLD) and the potential differences in the LC related to the underlying proteinopathy. Objective: The aim of this study was to investigate the LC in FTLD subgroups. Methods: Neuropathological cases diagnosed with FTLD were included. The subgroups consisted of FTLD with tau, transactive response DNA-binding protein 43 (TDP) and fused in sarcoma (FUS). Micro-and macroscopical degeneration of the LC were assessed with respect to the number of neurons and the degree of depigmentation. A group of cognitively healthy subjects and a group with vascular cognitive impairment (VCI) served as comparison groups. Results: A total of 85 FTLD cases were included, of which 44 had FTLD-TDP, 38 had FTLD-tau, and three had FTLD-FUS. The groups were compared with 25 VCI cases and 41 cognitively healthy control cases (N = 151 for the entire study). All FTLD groups had a statistically higher microscopical degeneration of the LC compared to the controls, but the FTLD-tau group had greater micro-and macroscopical degeneration than the FTLD-TDP group. Age correlated positively with the LC score in the FTLD-tau group, but not in the FTLD-TDP group. Conclusion: A greater microscopical degeneration of the LC was observed in all FTLD cases compared to healthy controls and those with VCI. The LC degeneration was more severe in FTLD-tau than in FTLD-TDP. The macroscopically differential degeneration of the LC in FTLD subgroups may facilitate differential diagnostics, potentially with imaging.</p>}},
  author       = {{Matti, Nathalie and Javanshiri, Keivan and Haglund, Mattias and Saenz-Sardá, Xavier and Englund, Elisabet}},
  issn         = {{1387-2877}},
  keywords     = {{Frontotemporal dementia; locus coeruleus; neuronal counts; tau; tauopathy; TDP-43}},
  language     = {{eng}},
  month        = {{07}},
  number       = {{2}},
  pages        = {{463--471}},
  publisher    = {{IOS Press}},
  series       = {{Journal of Alzheimer's Disease}},
  title        = {{Locus Coeruleus Degeneration Differs Between Frontotemporal Lobar Degeneration Subtypes}},
  url          = {{http://dx.doi.org/10.3233/JAD-220276}},
  doi          = {{10.3233/JAD-220276}},
  volume       = {{89}},
  year         = {{2022}},
}