dc.contributor.author | Labayru Isusquiza, Garazi | |
dc.contributor.author | Jiménez Marín, Antonio | |
dc.contributor.author | Fernández, Esther | |
dc.contributor.author | Villanua Bernues, Jorge Alberto | |
dc.contributor.author | Zulaica, Miren | |
dc.contributor.author | Cortés Díaz, Jesús María | |
dc.contributor.author | Díez, Ibai | |
dc.contributor.author | Sepulcre, Jorge | |
dc.contributor.author | López de Munain Arregui, Adolfo José | |
dc.contributor.author | Sistiaga Berrondo, Andone | |
dc.date.accessioned | 2021-03-04T09:26:16Z | |
dc.date.available | 2021-03-04T09:26:16Z | |
dc.date.issued | 2020-10 | |
dc.identifier.citation | Annals Of Clinical And Translational Neurology 7(10) : 1802-1815 (2020) | es_ES |
dc.identifier.issn | 2328-9503 | |
dc.identifier.uri | http://hdl.handle.net/10810/50460 | |
dc.description.abstract | Objective: 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. | es_ES |
dc.description.sponsorship | This study was supported by funding from the Institute of Health Carlos III co‐founded by Fondo Europeo de Desarrollo Regional‐FEDER [grant numbers PI17/01231 and PI17/01841], CIBERNED (grant number: 609) and the Basque Government [SAIO08‐PE08BF01]. G. Labayru was supported by a predoctoral grant from the Basque Government [PRE_2016_1_0187]. A. Jiménez‐Marín was supported by a predoctoral grant from the Basque Government [PRE_2019_1_0070]. | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | Wiley | es_ES |
dc.rights | info:eu-repo/semantics/openAccess | es_ES |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/es/ | * |
dc.subject | neurodegeneration | es_ES |
dc.subject | pediatric | es_ES |
dc.subject | adult | es_ES |
dc.subject | DM1 | es_ES |
dc.subject | MRI | es_ES |
dc.title | Neurodegeneration Trajectory in Pediatric and Adult/Late DM1: a Follow-up MRI Study Across a Decade | es_ES |
dc.type | info:eu-repo/semantics/article | es_ES |
dc.rights.holder | This is an open access article under the terms of the Creative Commons Attribution Non Commercial No Derivs License (CC BY-NC-ND 4.0) | es_ES |
dc.rights.holder | Atribución-NoComercial-SinDerivadas 3.0 España | * |
dc.relation.publisherversion | https://onlinelibrary.wiley.com/doi/10.1002/acn3.51163 | es_ES |
dc.identifier.doi | 10.1002/acn3.51163 | |
dc.departamentoes | Psicología Clínica y de la Salud y Metodología de Investigación | es_ES |
dc.departamentoes | Fisiología | es_ES |
dc.departamentoes | Neurociencias | es_ES |
dc.departamentoeu | Psikologia Klinikoa eta Osasunaren Psikologia eta Ikerketa Metodologia | es_ES |
dc.departamentoeu | Fisiologia | es_ES |
dc.departamentoeu | Neurozientziak | es_ES |