RT Journal Article
T1 Neurodegeneration trajectory in pediatric and adult/late DM1: A follow‐up MRI study across a decade
A1 Labayru, Garazi
A1 Jimenez‐Marin, Antonio
A1 Fernández, Esther
A1 Villanua, Jorge
A1 Zulaica, Miren
A1 Cortes, Jesus M.
A1 Díez, Ibai
A1 Sepulcre, Jorge
A1 López de Munain, Adolfo
A1 Sistiaga, Andone
AB 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.
PB Wiley-Blackwell
YR 2020
FD 2020-10
LK http://hdl.handle.net/11556/977
UL http://hdl.handle.net/11556/977
LA eng
NO Labayru, 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
NO CIBERNED609 Eusko JaurlaritzaPRE_ 2016_1_0187PRE_2019_1_0070SAIO08- PE08BF01 Institute of Health Carlos III cofounded by Fondo Europeo de Desarrollo Regional-FEDERPI17/01231 PI17/01841
DS TECNALIA Publications
RD 23 jul 2024