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Lower leg muscle involvement in Duchenne muscular dystrophy: an MR imaging and spectroscopy study

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Abstract

Objective

To describe the involvement of lower leg muscles in boys with Duchenne muscular dystrophy (DMD) by using MR imaging (MRI) and spectroscopy (MRS) correlated to indices of functional status.

Subjects and methods

Nine boys with DMD (mean age, 11 years) and eight healthy age- and BMI-matched boys (mean age, 13 years) prospectively underwent lower leg MRI, 1H-MRS of tibialis anterior (TA) and soleus (SOL) for lipid fraction measures, and 31P-MRS for pH and high-energy phosphate measures. DMD subjects were evaluated using the Vignos lower extremity functional rating, and tests including 6 min walk test (6MWT) and 10 m walk.

Results

DMD subjects had highest fatty infiltration scores in peroneal muscles, followed by medial gastrocnemius and soleus. Compared to controls, DMD boys showed higher intramuscular fat (P = 0.04), lipid fractions of TA and SOL (P = 0.02 and 0.003, respectively), pH of anterior compartment (P = 0.0003), and lower phosphocreatine/inorganic phosphorus ratio of posterior compartment (P = 0.02). The Vignos rating correlated with TA (r = 0.79, P = 0.01) and SOL (r = 0.71, P = 0.03) lipid fractions. The 6MWT correlated with fatty infiltration scores of SOL (r = −0.76, P = 0.046), medial (r = −0.80, P = 0.03) and lateral (r = −0.84, P = 0.02) gastrocnemius, intramuscular fat (r = −0.80, P = 0.03), and SOL lipid fraction (r = −0.89, P = 0.007). Time to walk 10 m correlated with anterior compartment pH (r = 0.78, P = 0.04).

Conclusion

Lower leg muscles of boys with DMD show a distinct involvement pattern and increased adiposity that correlates with functional status. Lower leg MRI and 1H-MRS studies may help to noninvasively demonstrate the severity of muscle involvement.

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Acknowledgments

We thank the boys and their families for their dedication and cooperation during this study. This work was conducted with support from Harvard Catalyst, The Harvard Clinical and Translational Science Center (NIH Award #UL1 RR 025758 and financial contributions from Harvard University and its affiliated academic health care centers).

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Division of Musculoskeletal Imaging and Intervention, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Martin Torriani, Bijoy J. Thomas, Miriam A. Bredella & Reza H. Ghomi

  2. MGH Institute of Health Professions and Massachusetts General Hospital, Boston, MA, USA

    Elise Townsend

  3. Pediatric Neuromuscular Clinic, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Brian S. Tseng

  4. Novartis Institute of Biomedical Research, Cambridge, MA, USA

    Brian S. Tseng

  5. Musculoskeletal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, 55 Fruit Street, YAW 6048, Boston, MA, 02114, USA

    Martin Torriani

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  1. Martin Torriani
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  2. Elise Townsend
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  3. Bijoy J. Thomas
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  4. Miriam A. Bredella
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  5. Reza H. Ghomi
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Correspondence to Martin Torriani.

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The content is solely the responsibility of the authors and does not necessarily represent the official views of Harvard Catalyst, Harvard University and its affiliated academic health care centers, the National Center for Research Resources, or the National Institutes of Health.

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Torriani, M., Townsend, E., Thomas, B.J. et al. Lower leg muscle involvement in Duchenne muscular dystrophy: an MR imaging and spectroscopy study. Skeletal Radiol 41, 437–445 (2012). https://doi.org/10.1007/s00256-011-1240-1

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  • DOI: https://doi.org/10.1007/s00256-011-1240-1

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