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Additionally, patients underwent neuropsychological, genetic, and muscular impairment assessment. Inter\u2010group comparisons of total and voxel\u2010level regional brain volume were conducted through Voxel Based Morphometry (VBM); cross\u2010sectionally and longitudinally, analyzing the associations between brain changes and demographic, clinical, and cognitive outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The percentage of GM loss did not significantly differ in any of the groups compared with HC and when assessed independently, adult\/late DM1 patients and their HC group suffered a significant loss in WM volume. Regional VBM analyses revealed subcortical GM damage in both DM1 groups, evolving to frontal regions in the pediatric onset patients. Muscular impairment and the outcomes of certain neuropsychological tests were significantly associated with follow\u2010up GM damage, while visuoconstruction, attention, and executive function tests showed sensitivity to WM degeneration over time.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Distinct patterns of brain atrophy and its progression over time in pediatric and adult\/late onset DM1 patients are suggested. Results indicate a possible neurodevelopmental origin of the brain abnormalities in DM1, along with the possible existence of an additional neurodegenerative process. Fronto\u2010subcortical networks appear to be involved in the disease progression at young adulthood in pediatric onset DM1 patients. The involvement of a multimodal integration network in DM1 is discussed.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51163","type":"journal-article","created":{"date-parts":[[2020,9,2]],"date-time":"2020-09-02T20:04:54Z","timestamp":1599077094000},"page":"1802-1815","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Neurodegeneration trajectory in pediatric and adult\/late DM1: A follow\u2010up MRI study across a decade"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7304-320X","authenticated-orcid":false,"given":"Garazi","family":"Labayru","sequence":"first","affiliation":[{"name":"Personality, Assessment and psychological treatment department; Psychology Faculty University of the Basque Country (UPV\/EHU) San Sebasti\u00e1n Gipuzkoa Spain"},{"name":"Neuroscience Area Biodonostia Research Institute, Osakidetza Donostia\u2010San Sebasti\u00e1n Gipuzkoa Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas (CIBERNED) Institute Carlos III Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Jimenez\u2010Marin","sequence":"additional","affiliation":[{"name":"Biocruces\u2010Bizkaia Health Research Institute Barakaldo Bizkaia Spain"},{"name":"Biomedical Research Doctorate Program University of the Basque Country (UPV\/EHU) Leioa Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Osatek Donostia University Hospital Donostia\u2010 San Sebasti\u00e1n Gipuzkoa Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Villanua","sequence":"additional","affiliation":[{"name":"Osatek Donostia University Hospital Donostia\u2010 San Sebasti\u00e1n Gipuzkoa Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miren","family":"Zulaica","sequence":"additional","affiliation":[{"name":"Neuroscience Area Biodonostia Research Institute, Osakidetza Donostia\u2010San Sebasti\u00e1n Gipuzkoa Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas (CIBERNED) Institute Carlos III Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesus M.","family":"Cortes","sequence":"additional","affiliation":[{"name":"Biocruces\u2010Bizkaia Health Research Institute Barakaldo Bizkaia Spain"},{"name":"Cell Biology and Histology Department University of the Basque Country (UPV\/EHU) Leioa Spain"},{"name":"IKERBASQUE The Basque Foundation for Science Bilbao Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5769-0178","authenticated-orcid":false,"given":"Ibai","family":"D\u00edez","sequence":"additional","affiliation":[{"name":"Gordon Center for Medical Imaging Department of Radiology Massachusetts General Hospital and Harvard Medical School Boston Massachusetts USA"},{"name":"Athinoula A. 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