Longitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1
dc.contributor.author | Koscik, Timothy R. | |
dc.contributor.author | van der Plas, Ellen | |
dc.contributor.author | Long, Jeffrey D. | |
dc.contributor.author | Cross, Stephen | |
dc.contributor.author | Gutmann, Laurie | |
dc.contributor.author | Cumming, Sarah A. | |
dc.contributor.author | Monckton, Darren G. | |
dc.contributor.author | Shields, Richard K. | |
dc.contributor.author | Magnotta, Vincent | |
dc.contributor.author | Nopoulos, Peggy C. | |
dc.contributor.department | Neurology, School of Medicine | |
dc.date.accessioned | 2024-10-11T10:44:25Z | |
dc.date.available | 2024-10-11T10:44:25Z | |
dc.date.issued | 2023 | |
dc.description.abstract | Myotonic dystrophy type 1 is characterized by neuromuscular degeneration. Our objective was to compare change in white matter microstructure (fractional anisotropy, radial and axial diffusivity), and functional/clinical measures. Participants underwent yearly neuroimaging and neurocognitive assessments over three-years. Assessments encompassed full-scale intelligence, memory, language, visuospatial skills, attention, processing speed, and executive function, as well as clinical symptoms of muscle/motor function, apathy, and hypersomnolence. Mixed effects models were used to examine differences. 69 healthy adults (66.2% women) and 41 DM1 patients (70.7% women) provided 156 and 90 observations, respectively. There was a group by elapsed time interaction for cerebral white matter, where DM1 patients exhibited declines in white matter (all p<0.05). Likewise, DM1 patients either declined (motor), improved more slowly (intelligence), or remained stable (executive function) for functional outcomes. White matter was associated with functional performance; intelligence was predicted by axial (r = 0.832; p<0.01) and radial diffusivity (r = 0.291, p<0.05), and executive function was associated with anisotropy (r = 0.416, p<0.001), and diffusivity (axial: r = 0.237, p = 0.05 and radial: r = 0.300, p<0.05). Indices of white matter health are sensitive to progression in DM1. These results are important for clinical trial design, which utilize short intervals to establish treatment efficacy. | |
dc.eprint.version | Author's manuscript | |
dc.identifier.citation | Koscik TR, van der Plas E, Long JD, et al. Longitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1. Neuromuscul Disord. 2023;33(8):660-669. doi:10.1016/j.nmd.2023.05.010 | |
dc.identifier.uri | https://hdl.handle.net/1805/43897 | |
dc.language.iso | en_US | |
dc.publisher | Elsevier | |
dc.relation.isversionof | 10.1016/j.nmd.2023.05.010 | |
dc.relation.journal | Neuromuscular Disorders | |
dc.rights | Publisher Policy | |
dc.source | PMC | |
dc.subject | Cognitive function | |
dc.subject | DM1 | |
dc.subject | DTI | |
dc.subject | Diffusion imaging | |
dc.subject | Disease progression | |
dc.subject | White matter | |
dc.title | Longitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1 | |
dc.type | Article |