Longitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1

dc.contributor.authorKoscik, Timothy R.
dc.contributor.authorvan der Plas, Ellen
dc.contributor.authorLong, Jeffrey D.
dc.contributor.authorCross, Stephen
dc.contributor.authorGutmann, Laurie
dc.contributor.authorCumming, Sarah A.
dc.contributor.authorMonckton, Darren G.
dc.contributor.authorShields, Richard K.
dc.contributor.authorMagnotta, Vincent
dc.contributor.authorNopoulos, Peggy C.
dc.contributor.departmentNeurology, School of Medicine
dc.date.accessioned2024-10-11T10:44:25Z
dc.date.available2024-10-11T10:44:25Z
dc.date.issued2023
dc.description.abstractMyotonic 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.versionAuthor's manuscript
dc.identifier.citationKoscik 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.urihttps://hdl.handle.net/1805/43897
dc.language.isoen_US
dc.publisherElsevier
dc.relation.isversionof10.1016/j.nmd.2023.05.010
dc.relation.journalNeuromuscular Disorders
dc.rightsPublisher Policy
dc.sourcePMC
dc.subjectCognitive function
dc.subjectDM1
dc.subjectDTI
dc.subjectDiffusion imaging
dc.subjectDisease progression
dc.subjectWhite matter
dc.titleLongitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1
dc.typeArticle
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