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Effectiveness of diffusion tensor imaging in assessing disease severity in Duchenne muscular dystrophy: preliminary study

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Abstract

Background

There is currently a lack of suitable objective endpoints to measure disease progression in Duchenne muscular dystrophy (DMD). Emerging research suggests that diffusion tensor imaging (DTI) has potential as an outcome measure for the evaluation of skeletal muscle injury.

Objective

The objective of this study was to evaluate the potential of DTI as quantitative magnetic resonance imaging (MRI) markers of disease severity in DMD.

Materials and methods

Thirteen consecutive boys (8.9 years ± 3.0 years) with DMD were evaluated using DTI. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were compared with clinical outcome measures of manual muscle testing and MRI determinations of muscle fat fraction (MFF) in the right lower extremity.

Results

Both MRI measures of FA and ADC strongly correlated with age and muscle strength. Values for FA positively correlated with age and negatively correlated with muscle strength (r = 0.78 and −0.96; both P ≤ 0.002) while measures of ADC negatively correlated age, but positively correlated with muscle strength (r = −0.87 and 0.83; both P ≤ 0.0004). Additionally, ADC and FA strongly correlated with MFF (r = −0.891 and 0.894, respectively; both P ≤ 0.0001). Mean MMF was negatively correlated with muscle strength (r = −0.89, P = 0.0001).

Conclusion

DTI measures of muscle structure strongly correlated with muscle strength and adiposity in boys with DMD in this pilot study, although these markers may be more reflective of fat replacement rather than muscle damage in later stages of the disease. Further studies in presymptomatic younger children are needed to assess the ability of DTI to detect early changes in DMD.

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Conflicts of interest

Dr. T. G. Perkins and Mr. J. M. Chia are employees of Philips Healthcare.

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Authors and Affiliations

  1. Department of Radiology, Children’s Hospital Los Angeles, MS #81, 4650 Sunset Blvd., Los Angeles, CA, 90027, USA

    Skorn Ponrartana, Tishya Anne Leong Wren, Houchun Harry Hu & Vicente Gilsanz

  2. Department of Neurology, Children’s Hospital Los Angeles, Los Angeles, CA, 90027, USA

    Leigh Ramos-Platt

  3. Department of Orthopaedic Surgery, Children’s Hospital Los Angeles, Los Angeles, CA, 90027, USA

    Tishya Anne Leong Wren & Vicente Gilsanz

  4. Philips Healthcare North America, Cleveland, OH, USA

    Thomas Gardner Perkins & Jonathan Mawlin Chia

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  1. Skorn Ponrartana
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  2. Leigh Ramos-Platt
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  4. Houchun Harry Hu
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  5. Thomas Gardner Perkins
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Correspondence to Skorn Ponrartana.

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Ponrartana, S., Ramos-Platt, L., Wren, T.A.L. et al. Effectiveness of diffusion tensor imaging in assessing disease severity in Duchenne muscular dystrophy: preliminary study. Pediatr Radiol 45, 582–589 (2015). https://doi.org/10.1007/s00247-014-3187-6

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  • DOI: https://doi.org/10.1007/s00247-014-3187-6

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