TY - JOUR

T1 - Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia

AU - Musaeus, Christian Sandøe

AU - Pedersen, Jette Stokholm

AU - Kjær, Troels Wesenberg

AU - Johannsen, Peter

AU - Waldemar, Gunhild

AU - Haverberg, Maria Joy Normann

AU - Bacher, Theis

AU - Nielsen, Jørgen Erik

AU - Roos, Peter

AU - The FReJA Consortium

N1 - Publisher Copyright: © Copyright © 2021 Musaeus, Pedersen, Kjær, Johannsen, Waldemar, Haverberg, Bacher, Nielsen, Roos and The FReJA Consortium.

PY - 2021

Y1 - 2021

N2 - 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.

AB - 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.

KW - CHMP2B

KW - EEG

KW - Frontotemporal dementia

KW - FTD

KW - microstates

KW - microstates analysis

KW - spectral power

U2 - 10.3389/fnagi.2021.714220

DO - 10.3389/fnagi.2021.714220

M3 - Journal article

C2 - 34588974

AN - SCOPUS:85115868053

VL - 13

JO - Frontiers in Aging Neuroscience

JF - Frontiers in Aging Neuroscience

SN - 1663-4365

M1 - 714220

ER -