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Abstract
Objective To assess the evidence of a relationship between muscle MRI and disease severity in Duchenne muscular dystrophy (DMD).
Methods We conducted a systematic review of studies that analyzed correlations between MRI measurements and motor function in patients with DMD. PubMed, Cochrane, Scopus, and Web of Science were searched using relevant keywords and inclusion/exclusion criteria (January 1, 1990–January 31, 2019). We evaluated article quality using the Joanna Briggs Institute scale. Information regarding the samples included, muscles evaluated, MRI protocols and motor function tests used was collected from each article. Correlations between MRI measurements and motor function were reported exhaustively.
Results Seventeen of 1,629 studies identified were included. Most patients included were ambulant with a mean age of 8.9 years. Most studies evaluated lower limb muscles. Moderate to excellent correlations were found between MRI measurements and motor function. The strongest correlations were found for quantitative MRI measurements such as fat fraction or mean T2. Correlations were stronger for lower leg muscles such as soleus. One longitudinal study reported that changes in soleus mean T2 were highly correlated with changes in motor function.
Conclusion The findings of this systematic review showed that MRI measurements can be used as biomarkers of disease severity in ambulant patients with DMD. Guidelines are proposed to help clinicians choose the most appropriate MRI measurements and muscles to evaluate. Studies exploring upper limb muscles, other stages of the disease, and sensitivity of measurements to change are needed.
Glossary
- BFLH=
- biceps femoris long head;
- DMD=
- Duchenne muscular dystrophy;
- FF=
- fat fraction;
- HQ=
- high quality;
- MFM=
- Motor Function Measure;
- MQ=
- medium quality;
- MRS=
- magnetic resonance spectroscopy;
- PUL=
- Performance of the Upper Limb;
- TA=
- tibialis anterior;
- TP=
- tibialis posterior;
- VHQ=
- very HQ;
- VL=
- vastus lateralis
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received July 15, 2019.
- Accepted in final form October 29, 2019.
- © 2019 American Academy of Neurology
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