Quantitative muscle ultrasound is a promising longitudinal follow-up tool in Duchenne muscular dystrophy

Merel Jansen, Nens van Alfen, Maria W G Nijhuis van der Sanden, Johannes P van Dijk, Sigrid Pillen, Imelda J M de Groot

Research output: Contribution to journalArticleAcademicpeer-review

96 Citations (Scopus)

Abstract

Responsive outcome measures are needed to follow the disease status of Duchenne muscular dystrophy (DMD) patients, as new therapeutic approaches become available for affected boys. Quantitative muscle ultrasound (QMUS) is potentially an attractive follow up tool for DMD because it reflects the severity of the dystrophic process without the need for invasive procedures, by quantifying echo intensity (i.e., mean grey level of muscle images) and muscle thickness. We performed a longitudinal follow-up of lower and upper extremity QMUS in 18 DMD patients and compared this with physical functioning in 11 of these patients. QMUS could be performed in every patient, and no patient was subjected to more than a total of 20min of ultrasound scanning time for this study. As expected we found a significant increase of echo intensity with age, reflecting increasing dystrophic muscle changes. This increase was related to ambulatory status, functional grading, muscle strength and motor ability. Our study establishes QMUS as a practical and child-friendly tool for the longitudinal follow up of DMD patients.

Original languageEnglish
Pages (from-to)306-317
Number of pages12
JournalNeuromuscular Disorders
Volume22
Issue number4
DOIs
Publication statusPublished - Apr 2012
Externally publishedYes

Keywords

  • Adolescent
  • Age Factors
  • Child
  • Child, Preschool
  • Follow-Up Studies
  • Humans
  • Longitudinal Studies
  • Muscle Strength
  • Muscle, Skeletal/diagnostic imaging
  • Muscular Dystrophy, Duchenne/diagnostic imaging
  • Ultrasonography

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