Quantitative assessment of skeletal muscle degeneration in patients with myotonic dystrophy type 1 using MRI.

Abstract

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.

• Authors: Bassem Hiba, Nathalie Richard, Luc J Hébert, Chantal Coté, Mimoun Nejjari, Christoph Vial, Françoise Bouhour, Jack Puymirat, Marc Janier
• Journal: Journal of magnetic resonance imaging : JMRI (J Magn Reson Imaging) Vol. 35 Issue 3 Pg. 678-85 (Mar 2012) ISSN: 1522-2586 [Electronic] United States
• PMID: 22069222 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2011 Wiley Periodicals, Inc.

Chemical References

• Biomarkers

Topics

• Adult
• Ankle Joint (pathology)
• Biomarkers (analysis)
• Female
• Humans
• Image Processing, Computer-Assisted
• Magnetic Resonance Imaging (methods)
• Male
• Middle Aged
• Muscle Strength (physiology)
• Muscle, Skeletal (pathology)
• Muscular Atrophy (pathology)
• Myotonic Dystrophy (pathology)
• Reproducibility of Results
• Statistics, Nonparametric