Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia
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 off...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2021-09-01
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| Series: | Frontiers in Aging Neuroscience |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fnagi.2021.714220/full |
| _version_ | 1819133745109663744 |
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| author | Christian Sandøe Musaeus Jette Stokholm Pedersen Troels Wesenberg Kjær Troels Wesenberg Kjær Peter Johannsen Gunhild Waldemar Gunhild Waldemar Maria Joy Normann Haverberg Theis Bacher Jørgen Erik Nielsen Jørgen Erik Nielsen Peter Roos The FReJA Consortium S Gydesen J Brown AM Isaacs J Collinge A Gade E Englund E Fisher TT Nielsen T Thusgaard I Holm |
| author_facet | Christian Sandøe Musaeus Jette Stokholm Pedersen Troels Wesenberg Kjær Troels Wesenberg Kjær Peter Johannsen Gunhild Waldemar Gunhild Waldemar Maria Joy Normann Haverberg Theis Bacher Jørgen Erik Nielsen Jørgen Erik Nielsen Peter Roos The FReJA Consortium S Gydesen J Brown AM Isaacs J Collinge A Gade E Englund E Fisher TT Nielsen T Thusgaard I Holm |
| author_sort | Christian Sandøe Musaeus |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T09:52:10Z |
| format | Article |
| id | doaj.art-cc78cd044125440b9c28ce0618e30014 |
| institution | Directory Open Access Journal |
| issn | 1663-4365 |
| language | English |
| last_indexed | 2024-12-22T09:52:10Z |
| publishDate | 2021-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Aging Neuroscience |
| spelling | doaj.art-cc78cd044125440b9c28ce0618e300142022-12-21T18:30:22ZengFrontiers Media S.A.Frontiers in Aging Neuroscience1663-43652021-09-011310.3389/fnagi.2021.714220714220Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal DementiaChristian Sandøe Musaeus0Jette Stokholm Pedersen1Troels Wesenberg Kjær2Troels Wesenberg Kjær3Peter Johannsen4Gunhild Waldemar5Gunhild Waldemar6Maria Joy Normann Haverberg7Theis Bacher8Jørgen Erik Nielsen9Jørgen Erik Nielsen10Peter Roos11The FReJA ConsortiumS GydesenJ BrownAM IsaacsJ CollingeA Gade E EnglundE FisherTT NielsenT ThusgaardI HolmDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDepartment of Neurology, Zealand University Hospital, Roskilde, DenmarkDepartment of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDepartment of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDepartment of Neurology, Zealand University Hospital, Roskilde, DenmarkDepartment of Neurology, Zealand University Hospital, Roskilde, DenmarkDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDepartment of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkDanish Dementia Research Centre (DDRC), Rigshospitalet and Department of Clinical Medicine, University of Copenhagen, Copenhagen, DenmarkA 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.https://www.frontiersin.org/articles/10.3389/fnagi.2021.714220/fullEEGmicrostatesspectral powerFTDFrontotemporal dementiaCHMP2B |
| spellingShingle | Christian Sandøe Musaeus Jette Stokholm Pedersen Troels Wesenberg Kjær Troels Wesenberg Kjær Peter Johannsen Gunhild Waldemar Gunhild Waldemar Maria Joy Normann Haverberg Theis Bacher Jørgen Erik Nielsen Jørgen Erik Nielsen Peter Roos The FReJA Consortium S Gydesen J Brown AM Isaacs J Collinge A Gade E Englund E Fisher TT Nielsen T Thusgaard I Holm Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia Frontiers in Aging Neuroscience EEG microstates spectral power FTD Frontotemporal dementia CHMP2B |
| title | Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia |
| title_full | Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia |
| title_fullStr | Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia |
| title_full_unstemmed | Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia |
| title_short | Cortical Frontoparietal Network Dysfunction in CHMP2B-Frontotemporal Dementia |
| title_sort | cortical frontoparietal network dysfunction in chmp2b frontotemporal dementia |
| topic | EEG microstates spectral power FTD Frontotemporal dementia CHMP2B |
| url | https://www.frontiersin.org/articles/10.3389/fnagi.2021.714220/full |
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