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Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients

Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder caused by absence of dystrophin protein. Dystrophin is expressed in muscle, but also in the brain. Difficulties with attention/inhibition, working memory and information processing are well described in DMD patients bu...

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Autores principales: Doorenweerd, Nathalie, de Rover, Mischa, Marini-Bettolo, Chiara, Hollingsworth, Kieren G., Niks, Erik H., Hendriksen, Jos G. M., Kan, Hermien E., Straub, Volker
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8500880/
https://www.ncbi.nlm.nih.gov/pubmed/33389442
http://dx.doi.org/10.1007/s11682-020-00422-3
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author Doorenweerd, Nathalie
de Rover, Mischa
Marini-Bettolo, Chiara
Hollingsworth, Kieren G.
Niks, Erik H.
Hendriksen, Jos G. M.
Kan, Hermien E.
Straub, Volker
author_facet Doorenweerd, Nathalie
de Rover, Mischa
Marini-Bettolo, Chiara
Hollingsworth, Kieren G.
Niks, Erik H.
Hendriksen, Jos G. M.
Kan, Hermien E.
Straub, Volker
author_sort Doorenweerd, Nathalie
collection PubMed
description Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder caused by absence of dystrophin protein. Dystrophin is expressed in muscle, but also in the brain. Difficulties with attention/inhibition, working memory and information processing are well described in DMD patients but their origin is poorly understood. The default mode network (DMN) is one of the networks involved in these processes. Therefore we aimed to assess DMN connectivity in DMD patients compared to matched controls, to better understand the cognitive profile in DMD. T1-weighted and resting state functional MRI scans were acquired from 33 DMD and 24 male age-matched controls at two clinical sites. Scans were analysed using FMRIB Software Library (FSL). Differences in the DMN were assessed using FSL RANDOMISE, with age as covariate and threshold-free cluster enhancement including multiple comparison correction. Post-hoc analyses were performed on the visual network, executive control network and fronto-parietal network with the same methods. In DMD patients, the level of connectivity was higher in areas within the control DMN (hyperconnectivity) and significant connectivity was found in areas outside the control DMN. No hypoconnectivity was found and no differences in the visual network, executive control network and fronto-parietal network. We showed differences both within and in areas outside the DMN in DMD. The specificity of our findings to the DMN can help provide a better understanding of the attention/inhibition, working memory and information processing difficulties in DMD. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s11682-020-00422-3.
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spelling pubmed-85008802021-10-19 Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients Doorenweerd, Nathalie de Rover, Mischa Marini-Bettolo, Chiara Hollingsworth, Kieren G. Niks, Erik H. Hendriksen, Jos G. M. Kan, Hermien E. Straub, Volker Brain Imaging Behav Original Research Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder caused by absence of dystrophin protein. Dystrophin is expressed in muscle, but also in the brain. Difficulties with attention/inhibition, working memory and information processing are well described in DMD patients but their origin is poorly understood. The default mode network (DMN) is one of the networks involved in these processes. Therefore we aimed to assess DMN connectivity in DMD patients compared to matched controls, to better understand the cognitive profile in DMD. T1-weighted and resting state functional MRI scans were acquired from 33 DMD and 24 male age-matched controls at two clinical sites. Scans were analysed using FMRIB Software Library (FSL). Differences in the DMN were assessed using FSL RANDOMISE, with age as covariate and threshold-free cluster enhancement including multiple comparison correction. Post-hoc analyses were performed on the visual network, executive control network and fronto-parietal network with the same methods. In DMD patients, the level of connectivity was higher in areas within the control DMN (hyperconnectivity) and significant connectivity was found in areas outside the control DMN. No hypoconnectivity was found and no differences in the visual network, executive control network and fronto-parietal network. We showed differences both within and in areas outside the DMN in DMD. The specificity of our findings to the DMN can help provide a better understanding of the attention/inhibition, working memory and information processing difficulties in DMD. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s11682-020-00422-3. Springer US 2021-01-03 2021 /pmc/articles/PMC8500880/ /pubmed/33389442 http://dx.doi.org/10.1007/s11682-020-00422-3 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Research
Doorenweerd, Nathalie
de Rover, Mischa
Marini-Bettolo, Chiara
Hollingsworth, Kieren G.
Niks, Erik H.
Hendriksen, Jos G. M.
Kan, Hermien E.
Straub, Volker
Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title_full Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title_fullStr Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title_full_unstemmed Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title_short Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients
title_sort resting-state functional mri shows altered default-mode network functional connectivity in duchenne muscular dystrophy patients
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8500880/
https://www.ncbi.nlm.nih.gov/pubmed/33389442
http://dx.doi.org/10.1007/s11682-020-00422-3
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