Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy

R. J. Willcocks, W. T. Triplett, S. C. Forbes, H. Arora, C. R. Senesac, D. J. Lott, T. R. Nicholson, William Rooney, G. A. Walter, K. Vandenborne

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

There is a pressing need for biomarkers and outcomes that can be used across disease stages in Duchenne muscular dystrophy (DMD), to facilitate the inclusion of a wider range of participants in clinical trials and to improve our understanding of the natural history of DMD. Quantitative magnetic resonance imaging (qMRI) and spectroscopy (MRS) biomarkers show considerable promise in both the legs and forearms of individuals with DMD, but have not yet been examined in functionally important proximal upper extremity muscles such as the biceps brachii and deltoid. The primary objective of this study was to examine the feasibility of implementing qMRI and MRS biomarkers in the proximal upper extremity musculature, and the secondary objective was to examine the relationship between MR measures of arm muscle pathology and upper extremity functional endpoints. Biomarkers included MRS and MRI measures of fat fraction and transverse relaxation time (T2). The MR exam was well tolerated in both ambulatory and non-ambulatory boys. qMR biomarkers differentiated affected and unaffected participants and correlated strongly with upper extremity function (r = 0.91 for biceps brachii T2 versus performance of upper limb score). These qMR outcome measures could be highly beneficial to the neuromuscular disease community, allowing measurement of the quality of functionally important muscles across disease stages to understand the natural history of DMD and particularly to broaden the opportunity for clinical trial participation.

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalJournal of Neurology
DOIs
StateAccepted/In press - Oct 24 2016

Fingerprint

Duchenne Muscular Dystrophy
Upper Extremity
Biomarkers
Magnetic Resonance Imaging
Muscles
Magnetic Resonance Spectroscopy
Clinical Trials
Neuromuscular Diseases
Forearm
Spectrum Analysis
Leg
Arm
Fats
Outcome Assessment (Health Care)
Pathology

Keywords

  • Biomarker
  • Magnetic resonance spectroscopy
  • Non-ambulatory
  • Transverse relaxation time

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Willcocks, R. J., Triplett, W. T., Forbes, S. C., Arora, H., Senesac, C. R., Lott, D. J., ... Vandenborne, K. (Accepted/In press). Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy. Journal of Neurology, 1-8. https://doi.org/10.1007/s00415-016-8311-0

Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy. / Willcocks, R. J.; Triplett, W. T.; Forbes, S. C.; Arora, H.; Senesac, C. R.; Lott, D. J.; Nicholson, T. R.; Rooney, William; Walter, G. A.; Vandenborne, K.

In: Journal of Neurology, 24.10.2016, p. 1-8.

Research output: Contribution to journalArticle

Willcocks, RJ, Triplett, WT, Forbes, SC, Arora, H, Senesac, CR, Lott, DJ, Nicholson, TR, Rooney, W, Walter, GA & Vandenborne, K 2016, 'Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy', Journal of Neurology, pp. 1-8. https://doi.org/10.1007/s00415-016-8311-0
Willcocks, R. J. ; Triplett, W. T. ; Forbes, S. C. ; Arora, H. ; Senesac, C. R. ; Lott, D. J. ; Nicholson, T. R. ; Rooney, William ; Walter, G. A. ; Vandenborne, K. / Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy. In: Journal of Neurology. 2016 ; pp. 1-8.
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