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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.<\/jats:p>","DOI":"10.1007\/s11682-020-00422-3","type":"journal-article","created":{"date-parts":[[2021,1,3]],"date-time":"2021-01-03T12:02:46Z","timestamp":1609675366000},"page":"2297-2307","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Resting-state functional MRI shows altered default-mode network functional connectivity in Duchenne muscular dystrophy patients"],"prefix":"10.1007","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2932-4327","authenticated-orcid":false,"given":"Nathalie","family":"Doorenweerd","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5703-666X","authenticated-orcid":false,"given":"Mischa","family":"de Rover","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Marini-Bettolo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3135-8131","authenticated-orcid":false,"given":"Kieren G.","family":"Hollingsworth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5892-5143","authenticated-orcid":false,"given":"Erik H.","family":"Niks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos G. 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RA3\/3079\/1); and Gratama Foundation Netherlands (Leiden University, grant no. 10.13). The sponsors had no role in study design, data collection, data analyses, data interpretation, or writing of the report. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit for publication. This study took place in two sites, at LUMC, Leiden, The Netherlands and Newcastle University, Newcastle upon Tyne, in the United Kingdom, and was approved by the local medical ethic committees in both sites. Participants were recruited from patient registries, local clinics and flyers. Written informed consent was received from all participants and\/or their legal guardians. ND reports grants from Duchenne Parent Project and grants from Gratama Stichting during the conduct of the study; EN report grants from Duchenne Parent Project, ZonMW and AFM, consultancies for BioMarin and Summit, and worked as local investigator of clinical trials of BioMarin, GSK, Lilly, Santhera, Givinostat, and Rocheoutside the submitted work. KGH reports grants from the United Kingdom Medical Research Council, Diabetes UK, the European Union (H2020, 667,078) and the Newcastle Healthcare Charity, consultancy for Summit pharmaceuticals and trial support from ImagingDMD-UF outside the submitted work. HK reports grants from ZonMW, AFM, Duchenne Parent Project, Gratama Stichting, NWO and the EU and research support from Philips Healthcare during the conduct of the study, consultancy for BioMarin and aTyr Pharma, and trial support from ImagingDMD-UF outside the submitted work. All reimbursements were received by the LUMC or Newcastle University. No personal financial benefits were received. VS has participated in advisory boards for Audentes Therapeutics, Biogen, Exonics Therapeutics, Italfarmaco S.p.A., Roche, Sanofi Genzyme, Sarepta Therapeutics, Summit Therapeutics, UCB, and Wave Therapeutics. He has research collaborations with Ultragenyx and Sanofi Genzyme. Several of the authors are members of the European Reference Network for Rare Neuromuscular Diseases [ERN EURO-NMD]. All other authors have nothing to declare.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical statement"}}]}}