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T2 Mapping and Fat Quantification of Thigh Muscles in Children with Duchenne Muscular Dystrophy

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Abstract

Quantitative magnetic resonance image (MRI) in individual muscles may be useful for monitoring disease progression in Duchenne muscular dystrophy (DMD). The purpose of this study was to measure T2 relaxation time of thigh muscles in children with DMD and healthy boys, and to correlate the T2 relaxation time of muscles with the fat fraction (FF) at quantitative magnetic resonance and results of clinical assessment. Thirty-two boys with DMD and 18 healthy boys were evaluated with T2 mapping and three-point Dixon MRI. Age, body mass index (BMI), muscle strength assessment, timed functional tests (time to walk or run 10 metres, rise from the floor and ascend four stairs), and the North Star Ambulatory Assessment (NSAA) were evaluated. Spearman’s correlation was used to assess the relationships between FF and clinical assessments and T2 relaxation time. The mean T2 relaxation time of thigh muscles in DMD was significantly longer than that in the control group (P<0.05), except for the gracilis (P=0.952). The gracilis, sartorius and adductor longus were relatively spared by fatty infiltration in DMD patients. The T2 relaxation time was correlated significantly with the mean FF in all muscles. Age, BMI, total muscle strength score, timed functional tests and NSAA were significantly correlated with the overall mean T2 relaxation time. T2 mapping may prove clinically useful in monitoring muscle changes as a result of the disease process and in predicting the outcome of DMD patients.

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References

  1. Bushby K, Finkel R, Birnkrant DJ, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol, 2010,9(1):77–93

    Article  Google Scholar 

  2. Hoffman EP, Brown RH Jr., Kunkel LM. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell, 1987,51(6):919–928

    Article  CAS  Google Scholar 

  3. McDouall RM, Dunn MJ, Dubowitz V. Nature of the mononuclear infiltrate and the mechanism of muscle damage in juvenile dermatomyositis and Duchenne muscular dystrophy. J Neurol Sci, 1990,99(2–3):199–217

    Article  CAS  Google Scholar 

  4. Moxley RT 3rd, Ashwal S, Pandya S, et al. Practice parameter: corticosteroid treatment of Duchenne dystrophy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology, 2005,64(1):13–20

    Article  CAS  Google Scholar 

  5. Schmidt S, Hafner P, Klein A, et al. Timed function tests, motor function measure, and quantitative thigh muscle MRI in ambulant children with Duchenne muscular dystrophy: A cross–sectional analysis. Neuromuscul Disord, 2018,28(1):16–23

    Article  Google Scholar 

  6. Lerario A, Bonfiglio S, Sormani M, et al. Quantitative muscle strength assessment in duchenne muscular dystrophy: longitudinal study and correlation with functional measures. BMC Neurol, 2012,12:91

    Article  Google Scholar 

  7. Mazzone E, Vasco G, Sormani MP, et al. Functional changes in Duchenne muscular dystrophy: a 12–month longitudinal cohort study. Neurology, 2011,77(3):250–256

    Article  CAS  Google Scholar 

  8. Mazzone E, Martinelli D, Berardinelli A, et al. North Star Ambulatory Assessment, 6–minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy. Neuromuscul Disord, 2010,20(11):712–716

    Article  Google Scholar 

  9. Mayhew AG, Cano SJ, Scott E, et al. Detecting meaningful change using the North Star Ambulatory Assessment in Duchenne muscular dystrophy. Dev Med Child Neurol, 2013,55(11):1046–1052

    Article  Google Scholar 

  10. Kim HK, Laor T, Horn PS, et al. Quantitative assessment of the T2 relaxation time of the gluteus muscles in children with Duchenne muscular dystrophy: a comparative study before and after steroid treatment. Korean J Radiol, 2010,11(3):304–311

    Article  Google Scholar 

  11. Gaeta M, Messina S, Mileto A, et al. Muscle fatfraction and mapping in Duchenne muscular dystrophy: evaluation of disease distribution and correlation with clinical assessments. Preliminary experience. Skeletal Radiol, 2012,41(8):955–961

    Article  Google Scholar 

  12. Johnston JH, Kim HK, Merrow AC, et al. Quantitative Skeletal Muscle MRI: Part 1, Derived T2 Fat Map in Differentiation Between Boys With Duchenne Muscular Dystrophy and Healthy Boys. AJR Am J Roentgenol, 2015,205(2):W207–215

    Article  Google Scholar 

  13. Kim HK, Serai S, Lindquist D, et al. Quantitative Skeletal Muscle MRI: Part 2, MR Spectroscopy and T2 Relaxation Time Mapping–Comparison Between Boys With Duchenne Muscular Dystrophy and Healthy Boys. AJR Am J Roentgenol, 2015,205(2):W216–223

    Article  Google Scholar 

  14. Reeder SB, Wen ZF, Yu HZ, et al. Multicoil Dixon chemical species separation with an iterative leastsquares estimation method. Magn Reson Med, 2004,51(1):35–45

    Article  CAS  Google Scholar 

  15. Hu HH, Kim HW, Nayak KS, et al. Comparison of fatwater MRI and single–voxel MRS in the assessment of hepatic and pancreatic fat fractions in humans. Obesity (Silver Spring), 2010,18(4):841–847

    Article  Google Scholar 

  16. Aoki T, Yamaguchi S, Kinoshita S, et al. Quantification of bone marrow fat content using iterative decomposition of water and fat with echo asymmetry and least–squares estimation (IDEAL): reproducibility, site variation and correlation with age and menopause. Br J Radiol, 2016,89(1065): 20150538

    Article  Google Scholar 

  17. Hu L, Zha YF, Wang L, et al. Quantitative Evaluation of Vertebral Microvascular Permeability and Fat Fraction in Alloxan–induced Diabetic Rabbits. Radiology, 2018,287(1):128–136

    Article  Google Scholar 

  18. Huang Y, Majumdar S, Genant HK, et al. Quantitative MR relaxometry study of muscle composition and function in Duchenne muscular dystrophy. J Magn Reson Imaging, 1994,4(1):59–64

    Article  CAS  Google Scholar 

  19. Florence JM, Pandya S, King WM, et al. Intrarater reliability of manual muscle test (Medical Research Council scale) grades in Duchenne’s muscular dystrophy. Phys Ther, 1992,72(2):115–122

    Article  CAS  Google Scholar 

  20. Brooke MH, Fenichel GM, Griggs RC, et al. Clinical investigation in Duchenne dystrophy: 2. Determination of the "power" of therapeutic trials based on the natural history. Muscle Nerve, 1983,6(2):91–103

    Article  CAS  Google Scholar 

  21. McDonald CM, Abresch RT, Carter GT, et al. Profiles of neuromuscular diseases. Duchenne muscular dystrophy. Am J Phys Med Rehabil, 1995,74(5 Suppl):S70–92

    Article  CAS  Google Scholar 

  22. Bushby K, Connor E. Clinical outcome measures for trials in Duchenne muscular dystrophy: report from International Working Group meetings. Clin Investig (Lond), 2011,1(9):1217–1235

    Article  Google Scholar 

  23. Mazzone ES, Messina S, Vasco G, et al. Reliability of the North Star Ambulatory Assessment in a multicentric setting. Neuromuscul Disord, 2009,19(7):458–461

    Article  CAS  Google Scholar 

  24. Mazzone E, Martinelli D, Berardinelli A, et al. North Star Ambulatory Assessment, 6–minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy. Neuromuscul Disord, 2010,20(11):712–716

    Article  Google Scholar 

  25. Kan HE, Scheenen TW, Wohlgemuth M, et al. Quantitative MR imaging of individual muscle involvement in facioscapulohumeral muscular dystrophy. Neuromuscul Disord, 2009,19(5):357–362

    Article  Google Scholar 

  26. Willis TA, Hollingsworth KG, Coombs A, et al. Quantitative Muscle MRI as an Assessment Tool for Monitoring Disease Progression in LGMD2I: A Multicentre Longitudinal Study. PLos One, 2013,8(8):e70993

    Article  CAS  Google Scholar 

  27. Burakiewicz J, Sinclair CDJ, Fischer D, et al. Quantifying fat replacement of muscle by quantitative MRI in muscular dystrophy. J Neurol, 2017,264(10):2053–2067

    Article  Google Scholar 

  28. Hooijmans MT, Niks EH, Burakiewicz J, et al. Nonuniform muscle fat replacement along the proximodistal axis in Duchenne muscular dystrophy. Neuromuscul Disord, 2017,27(5):458–464

    Article  CAS  Google Scholar 

  29. Hooijmans MT, Damon BM, Froeling M, et al. Evaluation of skeletal muscle DTI in patients with duchenne muscular dystrophy. NMR Biomed, 2015,28(11):1589–1597

    Article  CAS  Google Scholar 

  30. Mankodi A, Bishop CA, Auh S, et al. Quantifying disease activity in fatty–infiltrated skeletal muscle by IDEAL–CPMG in Duchenne muscular dystrophy. Neuromuscul Disord, 2016,26(10):650–658

    Article  Google Scholar 

  31. Wattjes MP, Kley RA, Fischer D. Neuromuscular imaging in inherited muscle diseases. Eur Radiol, 2010,20(10):2447–2460

    Article  Google Scholar 

  32. Forbes SC, Willcocks RJ, Triplett WT, et al. Magnetic resonance imaging and spectroscopy assessment of lower extremity skeletal muscles in boys with Duchenne muscular dystrophy: a multicenter cross sectional study. PLoS One, 2014,9(9):e106435

    Article  Google Scholar 

  33. Garrood P, Hollingsworth KG, Eagle M, et al. MR imaging in Duchenne muscular dystrophy: quantification of T1–weighted signal, contrast uptake, and the effects of exercise. J Magn Reson Imaging, 2009,30(5):1130–1138

    Article  Google Scholar 

  34. Arpan I, Forbes SC, Lott DJ, et al. T(2) mapping provides multiple approaches for the characterization of muscle involvement in neuromuscular diseases: a cross–sectional study of lower leg muscles in 5–15–yearold boys with Duchenne muscular dystrophy. NMR Biomed, 2013,26(3):320–328

    Article  CAS  Google Scholar 

  35. Kim HK, Laor T, Horn PS, et al. T2 mapping in Duchenne muscular dystrophy: distribution of disease activity and correlation with clinical assessments. Radiology, 2010,255(3):899–908

    Article  Google Scholar 

  36. Arpan I, Willcocks RJ, Forbes SC, et al. Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS. Neurology, 2014,83(11):974–980

    Article  CAS  Google Scholar 

  37. Li W, Zheng Y, Zhang W, et al. Progression and variation of fatty infiltration of the thigh muscles in Duchenne muscular dystrophy, a muscle magnetic resonance imaging study. Neuromuscul Disord, 2015,25(5):375–380

    Article  Google Scholar 

  38. Zheng YM, Li W, Du J, et al. The trefoil with single fruit sign in muscle magnetic resonance imaging is highly specific for dystrophinopathies. Eur J Radiol, 2015,84(10):1992–1998

    Article  Google Scholar 

  39. Polavarapu K, Manjunath M, Preethish–Kumar V, et al. Muscle MRI in Duchenne muscular dystrophy: Evidence of a distinctive pattern. Neuromuscul Disord, 2016,26(11):768–774

    Article  Google Scholar 

  40. Wokke BH, Van Den Bergen JC, Hooijmans MT, et al. T2 relaxation times are increased in skeletal muscle of DMD but not BMD patients. Muscle Nerve, 2016,53(1):38–43

    Article  CAS  Google Scholar 

  41. Gaudreault N, Gravel D, Nadeau S, et al. Gait patterns comparison of children with Duchenne muscular dystrophy to those of control subjects considering the effect of gait velocity. Gait Posture, 2010,32(3):342–347

    Article  Google Scholar 

  42. Doglio L, Pavan E, Pernigotti I, et al. Early signs of gait deviation in Duchenne muscular dystrophy. Eur J Phys Rehabil Med, 2011,47(4):587–594

    CAS  PubMed  Google Scholar 

  43. Ganea R, Jeannet PY, Paraschiv–Ionescu A, et al. Gait assessment in children with Duchenne muscular dystrophy during long–distance walking. J Child Neurol, 2012,27(1):30–38

    Article  Google Scholar 

  44. Carlier PG. Global T2 versus water T2 in NMR imaging of fatty infiltrated muscles: different methodology, different information and different implications. Neuromuscul Disord, 2014,24(5):390–392

    Article  Google Scholar 

  45. Bendixen RM, Lott DJ, Senesac C, et al. Participation in daily life activities and its relationship to strength and functional measures in boys with Duchenne muscular dystrophy. Disabil Rehabil, 2014,36(22):1918–1923

    Article  Google Scholar 

  46. Fischer D, Hafner P, Rubino D, et al. The 6–minute walk test, motor function measure and quantitative thigh muscle MRI in Becker muscular dystrophy: A crosssectional study. Neuromuscul Disord, 2016,26(7):414–422

    Article  Google Scholar 

  47. Johnston JH, Kim HK, Merrow AC, et al. Quantitative Skeletal Muscle MRI: Part I, Derived T2 Fat Map in Differentiation Between Boys With Duchenne Muscular Dystrophy and Healthy Boys. Am J Roentgenol, 2015,205(2):W207–W214

    Article  Google Scholar 

  48. Mankodi A, Azzabou N, Bulea T, et al. Skeletal muscle water T–2 as a biomarker of disease status and exercise effects in patients with Duchenne muscular dystrophy. Neuromuscul Disord, 2017,27(8):705–714

    Article  Google Scholar 

  49. Patten C, Meyer RA, Fleckenstein JL. T2 mapping of muscle. Semin Musculoskelet Radiol, 2003,7(4):297–305

    Article  Google Scholar 

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Correspondence to Jiang-xi Xiao or Yun Yuan.

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Yin, L., Xie, Zy., Xu, Hy. et al. T2 Mapping and Fat Quantification of Thigh Muscles in Children with Duchenne Muscular Dystrophy. CURR MED SCI 39, 138–145 (2019). https://doi.org/10.1007/s11596-019-2012-8

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  • DOI: https://doi.org/10.1007/s11596-019-2012-8

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