Locus Coeruleus Degeneration Differs Between Frontotemporal Lobar Degeneration Subtypes

Nathalie Matti, Keivan Javanshiri, Mattias Haglund, Xavier Saenz-Sardá, Elisabet Englund

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)463-471
Number of pages9
JournalJournal of Alzheimer's Disease
Issue number2
Publication statusPublished - 2022 Jul 20

Subject classification (UKÄ)

  • Neurology


  • Frontotemporal dementia
  • locus coeruleus
  • neuronal counts
  • tau
  • tauopathy
  • TDP-43


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