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

2011; Elsevier BV; Volume: 22; Issue: 4 Linguagem: Inglês

10.1016/j.nmd.2011.10.020

1873-2364

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

Neurogenetic and Muscular Disorders Research

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.