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Compositional analysis of muscle in boys with Duchenne muscular dystrophy using MR imaging

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Abstract

Objective

Boys with Duchenne muscular dystrophy (DMD) present by age 5 years with weakness and, untreated, stop walking unaided by age 10 or 11 years. We used magnetic resonance (MR) imaging to study age-related changes in the composition and distribution of diseased muscles.

Design and patients

Eleven boys (mean 7.1±1.6 years) with DMD underwent clinical and MR examinations. Quantitative muscle strength and timed functional testing was performed. Thigh muscles were scanned at three levels (hip, mid-thigh, and knee) using T1-weighted spin echo and short-tau inversion recovery (STIR) sequences. Outcome measures included intramuscular fatty infiltration, intermuscle fat deposition, edema, and muscle size.

Results

Ten boys completed the study. Older boys demonstrated more prominent fatty infiltration of muscles. Fatty infiltration occurred in a characteristic pattern with the gluteus and adductor magnus muscles most commonly involved and the gracilis most commonly spared. Similarly, patchy increases in free water content suggested a pattern of intramuscular edema or inflammation. Atrophy occurred in muscles heavily infiltrated with fat, and true hypertrophy selectively occurred in those that were spared.

Conclusions

While fibrofatty changes have been described in DMD, this study further defines differential involvement and additionally suggests widespread edema or inflammation. Improved imaging techniques to quantify the degree and distribution of these changes may provide a basis for exploring mechanisms of action of medications and perhaps another means for selecting treatment regimens and monitoring their effects.

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References

  1. Hoffman EP, Brown RH, Kunkel LM. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell 1987; 51:919–928.

    Article  CAS  PubMed  Google Scholar 

  2. Zuberi SM, Matta N, Nawaz S, Stephenson JB, McWilliam RC, Hollman A. Muscle ultrasound in the assessment of suspected neuromuscular disease in childhood. Neuromuscul Disord 1999; 9:203–207.

    Article  CAS  PubMed  Google Scholar 

  3. Reimers CD, Schlotter B, Eicke BM, Witt TN. Calf enlargement in neuromuscular diseases: a quantitative ultrasound study in 350 patients and review of the literature. J Neurol Sci 1996; 143:46–56.

    Article  CAS  PubMed  Google Scholar 

  4. Manzur AY, Hyde SA, Rodillo E, Heckmatt JZ, Bentley G, Dubowitz V. A randomized controlled trial of early surgery in Duchenne muscular dystrophy. Neuromuscul Disord 1992; 2:379–387.

    CAS  PubMed  Google Scholar 

  5. Heckmatt J, Rodillo E, Doherty M, Willson K, Leeman S. Quantitative sonography of muscle. J Child Neurol 1989; 4 (Suppl):S101–106.

    Google Scholar 

  6. Lamminen A, Jaaskelainen J, Rapola J, Suramo I. High-frequency ultrasonography of skeletal muscle in children with neuromuscular disease. J Ultrasound Med 1988; 7:505–509.

    CAS  PubMed  Google Scholar 

  7. Dock W, Happak W, Grabenwoger F, Toifl K, Bittner R, Gruber H. Neuromuscular diseases: evaluation with high-frequency sonography. Radiology 1990; 177:825–828.

    CAS  PubMed  Google Scholar 

  8. Liu GC, Jong YJ, Chiang CH, Jaw TS. Duchenne muscular dystrophy: MR grading system with functional correlation. Radiology 1993; 186:475–480.

    CAS  PubMed  Google Scholar 

  9. Gong QY, Phoenix J, Kemp GJ, et al. Estimation of body composition in muscular dystrophy by MRI and stereology. J Magn Reson Imaging 2000; 12:467–475.

    Article  CAS  PubMed  Google Scholar 

  10. Matsumura K, Nakano I, Fukuda N, Ikehira H, Tateno Y, Aoki Y. Proton spin-lattice relaxation time of Duchenne dystrophy skeletal muscle by magnetic resonance imaging. Muscle Nerve 1988; 11:97–102.

    CAS  PubMed  Google Scholar 

  11. Murphy WA, Totty WG, Carroll JE. MRI of normal and pathologic skeletal muscle. AJR Am J Roentgenol 1986; 146:565–574.

    CAS  PubMed  Google Scholar 

  12. Scopinaro F, Manni C, Miccheli A, et al. Muscular uptake of Tc-99m MIBI and TI-201 in Duchenne muscular dystrophy. Clin Nucl Med 1996; 21:792–796.

    Article  CAS  PubMed  Google Scholar 

  13. Escolar DM, Henricson EK, Mayhew J, et al. Clinical evaluator reliability for quantitative and manual muscle testing measures of strength in children. Muscle Nerve 2001; 24:787–793.

    Article  CAS  PubMed  Google Scholar 

  14. Connolly AM, Pestronk A, Mehta S, Al-Lozi M. Case of the month: Primary α-sarcoglycan deficiency responsive to immunosuppression over three years. Muscle Nerve 1998; 21:1549–1553.

    Article  CAS  PubMed  Google Scholar 

  15. Connolly AM, Schierbecker J, Renna R, Florence J. High dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy. Neuromuscul Disord 2002; 12:917–925.

    Article  PubMed  Google Scholar 

  16. Stuberg WA, Metcalf WK. Reliability of quantitative muscle testing in healthy children and in children with Duchenne muscular dystrophy using a hand-held dynamometer. Phys Ther 1988; 68:977–982.

    CAS  PubMed  Google Scholar 

  17. Brooke MH, Fenichel GM, Griggs RC, et al. Clinical investigation of Duchenne muscular dystrophy: interesting results in a trial of prednisone. Arch Neurol 1987; 44:812–817.

    CAS  PubMed  Google Scholar 

  18. Fenichel GM, Florence JM, Pestronk A, et al. Long-term benefit from prednisone therapy in Duchenne muscular dystrophy. Neurology 1991; 41:1874–1877.

    CAS  PubMed  Google Scholar 

  19. Brooke MH, Fenichel GM, Griggs RC, et al. Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy. Neurology 1989; 39:475–481.

    CAS  PubMed  Google Scholar 

  20. Arahata K, Engel AG. Monoclonal antibody analysis of mononuclear cells in myopathies. I. Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984; 16:193–208.

    CAS  PubMed  Google Scholar 

  21. Engel AG, Biesecker G. Complement activation in muscle fiber necrosis: demonstration of the membrane attack complex of complement in necrotic fibers. Ann Neurol 1982; 12:289–296.

    CAS  PubMed  Google Scholar 

  22. Gorospe JR, Tharp MD, Hinckley J, Kornegay JN, Hoffman EP. A role for mast cells in the progression of Duchenne muscular dystrophy? Correlations in dystrophin-deficient humans, dogs, and mice. J Neurol Sci 1994; 122:44–56.

    Article  CAS  PubMed  Google Scholar 

  23. Spuler S, Engel AG. Unexpected sarcolemmal complement membrane attack complex deposits on nonnecrotic muscle fibers in muscular dystrophies. Neurology 1998; 50:41–46.

    Article  CAS  PubMed  Google Scholar 

  24. Mercuri E, Pichiecchio A, Counsell S, et al. A short protocol for muscle MRI in children with muscular dystrophies. Eur J Paediatr Neurol 2002; 6:305–307.

    Article  PubMed  Google Scholar 

  25. Pichiecchio A, Uggetti C, Egitto MG, et al. Quantitative MR evaluation of body composition in patients with Duchenne muscular dystrophy. Eur Radiol 2002; 12:2704–2709.

    Google Scholar 

  26. Dubowitz V, Kinali M, Main M, Mercuri E, Mutoni F. Remission of clinical signs in early Duchenne muscular dystrophy on intermittent low-dosage prednisolone therapy. Eur J Paediatr Neurol 2002; 6:153–159.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

The authors would like to thank Jeanine Schierbecker, P.T., and Julaine Florence, P.T., for their assistance in the clinical evaluation of these boys. We would also like to thank Glenn Foster, R.T. (R, MR), and Rich Nagel, R.T. (R, MR), for their assistance performing the MR scans.

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Author notes

  1. Franklin A. Marden

    Present address: Department of Neurosurgery (MC 799), University of Illinois at Chicago, 912 S. Wood Street, Chicago, IL 60612, USA

Authors and Affiliations

  1. Mallinckrodt Institute of Radiology at Washington University Medical Center, 510 South Kingshighway Blvd., St. Louis, MO 63110, USA

    Franklin A. Marden, Marilyn J. Siegel & David A. Rubin

  2. Department of Radiology, Barnes-Jewish Hospital, One Barnes Hospital Plaza, St. Louis, MO 63110, USA

    Franklin A. Marden, Marilyn J. Siegel & David A. Rubin

  3. Department of Pediatrics, St. Louis Children’s Hospital, One Children’s Place, St. Louis, MO 63110, USA

    Anne M. Connolly

  4. Department of Neurology, Barnes-Jewish Hospital, Washington University School of Medicine, 660 S. Euclid, Box 8111, St. Louis, MO 63110, USA

    Anne M. Connolly

Authors
  1. Franklin A. Marden
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  2. Anne M. Connolly
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  4. David A. Rubin
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Corresponding author

Correspondence to Anne M. Connolly.

Additional information

Financial support for imaging: Mallinckrodt Institute of Radiology

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Marden, F.A., Connolly, A.M., Siegel, M.J. et al. Compositional analysis of muscle in boys with Duchenne muscular dystrophy using MR imaging. Skeletal Radiol 34, 140–148 (2005). https://doi.org/10.1007/s00256-004-0825-3

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  • DOI: https://doi.org/10.1007/s00256-004-0825-3

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