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

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...

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Bibliographic Details
Published in:Neuromuscular disorders : NMD 2012-04, Vol.22 (4), p.306-317
Main Authors: Jansen, Merel, van Alfen, Nens, Nijhuis van der Sanden, Maria W.G, van Dijk, Johannes P, Pillen, Sigrid, de Groot, Imelda J.M
Format: Article
Language:English
Subjects:
Adolescent
Age
Age Factors
Child
Child, Preschool
Duchenne muscular dystrophy
Duchenne's muscular dystrophy
Follow-Up Studies
Follow-up study
Humans
Longitudinal Studies
Muscle Strength
Muscle, Skeletal - diagnostic imaging
Muscle, Skeletal - physiopathology
Muscular dystrophy
Muscular Dystrophy, Duchenne - diagnostic imaging
Muscular Dystrophy, Duchenne - physiopathology
Muscular strength
Neurology
neuromuscular system
Quantitative muscle ultrasound
Scanning
Study outcome
Ultrasonography
Ultrasound
Validation
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Summary: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 20 min 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.
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2011.10.020