title: 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 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.
authors: Radboud University Nijmegen Medical Centre, Nijmegen Centre for Evidence Based Practice, Department of Rehabilitation, Nijmegen, The Netherlands. M.Jansen@reval.umcn.nl
authors: Merel
authors: M
authors: Jansen
authors: Nens
authors: N
authors: van Alfen
authors: Maria W G
authors: MW
authors: Nijhuis van der Sanden
authors: Johannes P
authors: JP
authors: van Dijk
authors: Sigrid
authors: S
authors: Pillen
authors: Imelda J M
authors: IJ
authors: de Groot
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014463:Ultrasonography