Quantitative muscle MRI as a sensitive marker of early muscle pathology in myotonic dystrophy type 1

dc.contributor.authorvan der Plas, Ellen
dc.contributor.authorGutmann, Laurie
dc.contributor.authorThedens, Dan
dc.contributor.authorShields, Richard K.
dc.contributor.authorLangbehn, Kathleen
dc.contributor.authorGuo, Zhihui
dc.contributor.authorSonka, Milan
dc.contributor.authorNopoulos, Peggy
dc.contributor.departmentNeurology, School of Medicineen_US
dc.date.accessioned2023-06-14T12:14:15Z
dc.date.available2023-06-14T12:14:15Z
dc.date.issued2021
dc.description.abstractBackground: Quantitative muscle MRI as a sensitive marker of early muscle pathology and disease progression in adult-onset myotonic dystrophy type 1. The utility of muscle MRI as a marker of muscle pathology and disease progression in adult-onset myotonic dystrophy type 1 (DM1) was evaluated. Methods: This prospective, longitudinal study included 67 observations from 36 DM1 patients (50% female), and 92 observations from 49 healthy adults (49% female). Lower-leg 3T magnetic resonance imaging (MRI) scans were acquired. Volume and fat fraction (FF) were estimated using a three-point Dixon method, and T2-relaxometry was determined using a multi-echo spin-echo sequence. Muscles were segmented automatically. Mixed linear models were conducted to determine group differences across muscles and image modality, accounting for age, sex, and repeated observations. Differences in rate of change in volume, T2-relaxometry, and FF were also determined with mixed linear regression that included a group by elapsed time interaction. Results: Compared with healthy adults, DM1 patients exhibited reduced volume of the tibialis anterior, soleus, and gastrocnemius (GAS) (all, P < .05). T2-relaxometry and FF were increased across all calf muscles in DM1 compared to controls. (all, P < .01). Signs of muscle pathology, including reduced volume, and increased T2-relaxometry and FF were already noted in DM1 patients who did not exhibit clinical motor symptoms of DM1. As a group, DM1 patients exhibited a more rapid change than did controls in tibialis posterior volume (P = .05) and GAS T2-relaxometry (P = .03) and FF (P = .06). Conclusions: Muscle MRI renders sensitive, early markers of muscle pathology and disease progression in DM1. T2 relaxometry may be particularly sensitive to early muscle changes related to DM1.en_US
dc.eprint.versionAuthor's manuscripten_US
dc.identifier.citationvan der Plas E, Gutmann L, Thedens D, et al. Quantitative muscle MRI as a sensitive marker of early muscle pathology in myotonic dystrophy type 1. Muscle Nerve. 2021;63(4):553-562. doi:10.1002/mus.27174en_US
dc.identifier.urihttps://hdl.handle.net/1805/33747
dc.language.isoen_USen_US
dc.publisherWileyen_US
dc.relation.isversionof10.1002/mus.27174en_US
dc.relation.journalMuscle & Nerveen_US
dc.rightsPublisher Policyen_US
dc.sourcePMCen_US
dc.subjectMagnetic Resonance Imaging (MRI)en_US
dc.subjectMyotonic Dystrophy (DM1)en_US
dc.subjectMuscle segmentationen_US
dc.subjectT2-relaxometry Fat fraction (FF)en_US
dc.titleQuantitative muscle MRI as a sensitive marker of early muscle pathology in myotonic dystrophy type 1en_US
dc.typeArticleen_US
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