Quantitative Muscle MRI Depicts Increased Muscle Mass after a Behavioral Change in Myotonic Dystrophy Type 1.

Abstract

Background Patients with myotonic dystrophy type 1 (DM1) increased their physical activity and exercise capacity following a behavioral intervention. However, it is unknown what is altered in muscles of patients with DM1 as a result of this intervention. The increased exercise capacity suggests that decelerated fat infiltration or increased muscle cross-sectional area (CSA) could be involved. Purpose To assess the effect of this activity-stimulating behavioral intervention on the lower extremity muscles of patients with DM1 with longitudinal quantitative muscle MRI. Materials and Methods In this prospective trial, participants with DM1 were randomized to a behavioral intervention (<i>n</i> = 14) or continued regular care (standard care; <i>n</i> = 13); no age-matched pairing was performed. Participants underwent MRI of the lower extremities at baseline and 10-month follow-up (January 2015 to March 2016). Fat fraction (FF), muscle CSA, and muscle water T2 (T2_water) as markers for fat infiltration, muscle mass, and alteration in tissue water distribution (edema), respectively, were assessed with a chemical shift-encoded Dixon sequence and multiecho spin-echo sequence. Longitudinal within-group and between-group changes were assessed with paired-samples <i>t</i> tests and multivariable regression models. Results A total of 27 patients with DM1 (15 men) were evaluated. Patient age was comparable between groups (intervention, 45 years ± 13 [standard deviation]; standard care, 5 years ± 12; <i>P</i> = .96). Muscle CSA increased 5.9 cm² ± 7.8 in the intervention group during the 10-month follow-up (<i>P</i> = .03) and decreased 3.6 cm² ± 7.2 in the standard care group (<i>P</i> = .13). After 10 months, the mean difference between the groups was 9.5 cm² (<i>P</i> = .01). This effect was stronger in muscles with baseline FF below the mean ± standard deviation of unaffected volunteers (-0.4 cm² ± 0.15; <i>P</i> < .001). FF increased 0.9% ± 1.0 in the intervention group (<i>P</i> = .02) and 1.2% ± 1.2 for standard care (<i>P</i> = .02), with no between-group difference (<i>P</i> = .56). T2_water did not change significantly in either group (intervention, <i>P</i> = .08; standard care, <i>P</i> = .88). Conclusion A behavioral intervention targeting physical activity increased lower extremity muscle cross-sectional area in patients with myotonic dystrophy, preferentially in healthy-appearing muscle. © RSNA, 2020 <i>Online supplemental material is available for this article.</i>