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dc.contributor.authorLabayru, Garazi
dc.contributor.authorJimenez‐Marin, Antonio
dc.contributor.authorFernández, Esther
dc.contributor.authorVillanua, Jorge
dc.contributor.authorZulaica, Miren
dc.contributor.authorCortes, Jesus M.
dc.contributor.authorDíez, Ibai
dc.contributor.authorSepulcre, Jorge
dc.contributor.authorLópez de Munain, Adolfo
dc.contributor.authorSistiaga, Andone
dc.date.accessioned2020-09-11T10:44:30Z
dc.date.available2020-09-11T10:44:30Z
dc.date.issued2020-10
dc.identifier.citationLabayru, Garazi, Antonio Jimenez‐Marin, Esther Fernández, Jorge Villanua, Miren Zulaica, Jesus M. Cortes, Ibai Díez, Jorge Sepulcre, Adolfo López de Munain, and Andone Sistiaga. “Neurodegeneration Trajectory in Pediatric and Adult/late DM1: A Follow‐up MRI Study Across a Decade.” Annals of Clinical and Translational Neurology 7, no. 10 (September 2, 2020): 1802–1815. doi:10.1002/acn3.51163. Feedback: support@crossref.org
dc.identifier.urihttp://hdl.handle.net/11556/977
dc.description.abstractObjective: To characterize the progression of brain structural abnormalities in adults with pediatric and adult/late onset DM1, as well as to examine the potential predictive markers of such progression. Methods: 21 DM1 patients (pediatric onset: N = 9; adult/late onset: N = 12) and 18 healthy controls (HC) were assessed longitudinally over 9.17 years through brain MRI. Additionally, patients underwent neuropsychological, genetic, and muscular impairment assessment. Inter-group comparisons of total and voxel-level regional brain volume were conducted through Voxel Based Morphometry (VBM); cross-sectionally and longitudinally, analyzing the associations between brain changes and demographic, clinical, and cognitive outcomes. Results: 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-up GM damage, while visuoconstruction, attention, and executive function tests showed sensitivity to WM degeneration over time. Interpretation: 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-subcortical 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.en
dc.description.sponsorshipCIBERNED609 Eusko JaurlaritzaPRE_ 2016_1_0187PRE_2019_1_0070SAIO08- PE08BF01 Institute of Health Carlos III cofounded by Fondo Europeo de Desarrollo Regional-FEDERPI17/01231 PI17/01841en
dc.language.isoengen
dc.publisherWiley-Blackwellen
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.titleNeurodegeneration trajectory in pediatric and adult/late DM1: A follow‐up MRI study across a decadeen
dc.typearticleen
dc.identifier.doi10.1002/acn3.51163en
dc.rights.accessRightsopenAccessen
dc.subject.keywordsHealthen
dc.subject.keywordsNeurodegenerationen
dc.identifier.essn2328-9503en
dc.issue.number10
dc.journal.titleAnnals of Clinical and Translational Neurologyen
dc.page.final1815
dc.page.initial1802
dc.volume.number7


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