Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia

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  • Christian Sandøe Musaeus
  • Jette Stokholm Pedersen
  • Troels Wesenberg Kjær
  • Peter Johannsen
  • Waldemar, Gunhild
  • Maria Joy Normann Haverberg
  • Theis Bacher
  • Nielsen, Jørgen Erik
  • Peter Roos
  • The FReJA Consortium

A rare cause of inherited frontotemporal dementia (FTD) is a mutation in the CHMP2B gene on chromosome 3 leading to the autosomal dominantly inherited FTD (CHMP2B-FTD). Since CHMP2B-FTD is clinically well-characterized, and patients show a distinct pattern of executive dysfunction, the condition offers possible insight in the early electroencephalographic (EEG) changes in the cortical networks. Specifically, EEG microstate analysis parses the EEG signals into topographies believed to represent discrete network activations. We investigated the EEG dynamics in patients with symptomatic CHMP2B-FTD (n = 5) as well as pre-symptomatic mutation carriers (n = 5) compared to non-carrier family members (n = 6). The data was parsed into four archetypal microstates and global power was calculated. A trend was found for lower occurrence in microstate D in CHMP2B-FTD (p-value = 0.177, F-value = 2.036). Patients with recent symptom onset (<1 year) showed an increased duration of microstate D, whereas patients who had been symptomatic for longer periods (>2 years) showed decreased duration. Patients with CHMP2B-FTD present with executive dysfunction, and microstate D has previously been shown to be associated with the fronto-parietal network. The biphasic pattern may represent the pathophysiological changes in brain dynamics during neurodegeneration, which may apply to other neurodegenerative diseases.

Original languageEnglish
Article number714220
JournalFrontiers in Aging Neuroscience
Volume13
ISSN1663-4365
DOIs
Publication statusPublished - 2021

Bibliographical note

Publisher Copyright:
© Copyright © 2021 Musaeus, Pedersen, Kjær, Johannsen, Waldemar, Haverberg, Bacher, Nielsen, Roos and The FReJA Consortium.

    Research areas

  • CHMP2B, EEG, Frontotemporal dementia, FTD, microstates, microstates analysis, spectral power

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