PURPOSE: To identify MRI biomarkers that could be used to follow disease progression and therapeutic efficacy in one individual muscle in patients with myotonic dystrophy type 1 (DM1). MATERIALS AND METHODS: Lower limb MRI and maximal ankle dorsiflexor strength assessment, using a hand-held dynamometer, were performed in 19 DM1 patients and 6 control subjects. The volume of residual muscle tissue of Tibialis Anterior (TA) muscle was chosen as an index for muscle atrophy, and the T2-relaxation-time of the residual muscle tissue was measured to evaluate edema-like lesions. The fat-to-water ratio was assessed using three-point Dixon images to quantify fat infiltration in the entire muscle. RESULTS: The intra-observer variability of MRI indices (∼5.2% for the residual muscle tissue volume and 2.5% for the fat-to-water ratio) was lower than that of the dorsiflexor torque measurement (∼11.5%). A high correlation (r = 0.91) was found between maximal ankle dorsiflexor strength and residual TA muscle tissue volume in DM1 patients. Increases in the fat-to-water ratio and T2-relaxation-time were associated with a decrease in maximal ankle dorsiflexor strength. CONCLUSION: MRI appears as a noninvasive method which can be used to follow disease progression and therapeutic efficacy.
PURPOSE: To identify MRI biomarkers that could be used to follow disease progression and therapeutic efficacy in one individual muscle in patients with myotonic dystrophy type 1 (DM1). MATERIALS AND METHODS: Lower limb MRI and maximal ankle dorsiflexor strength assessment, using a hand-held dynamometer, were performed in 19 DM1patients and 6 control subjects. The volume of residual muscle tissue of Tibialis Anterior (TA) muscle was chosen as an index for muscle atrophy, and the T2-relaxation-time of the residual muscle tissue was measured to evaluate edema-like lesions. The fat-to-water ratio was assessed using three-point Dixon images to quantify fat infiltration in the entire muscle. RESULTS: The intra-observer variability of MRI indices (∼5.2% for the residual muscle tissue volume and 2.5% for the fat-to-water ratio) was lower than that of the dorsiflexor torque measurement (∼11.5%). A high correlation (r = 0.91) was found between maximal ankle dorsiflexor strength and residual TA muscle tissue volume in DM1patients. Increases in the fat-to-water ratio and T2-relaxation-time were associated with a decrease in maximal ankle dorsiflexor strength. CONCLUSION: MRI appears as a noninvasive method which can be used to follow disease progression and therapeutic efficacy.
Authors: Marianna S Thomas; David Newman; Olof Dahlqvist Leinhard; Bahman Kasmai; Richard Greenwood; Paul N Malcolm; Anette Karlsson; Johannes Rosander; Magnus Borga; Andoni P Toms Journal: Eur Radiol Date: 2014-05-29 Impact factor: 5.315
Authors: Pierre G Carlier; Benjamin Marty; Olivier Scheidegger; Paulo Loureiro de Sousa; Pierre-Yves Baudin; Eduard Snezhko; Dmitry Vlodavets Journal: J Neuromuscul Dis Date: 2016-03-03
Authors: William T Triplett; Celine Baligand; Sean C Forbes; Rebecca J Willcocks; Donovan J Lott; Soren DeVos; Jim Pollaro; William D Rooney; H Lee Sweeney; Carsten G Bönnemann; Dah-Jyuu Wang; Krista Vandenborne; Glenn A Walter Journal: Magn Reson Med Date: 2013-09-04 Impact factor: 4.668