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Muscle ultrasound quantifies disease progression over time in infants and young boys with duchenne muscular dystrophy
Authors:Craig M Zaidman MD  Elizabeth C Malkus PT  MHS  Anne M Connolly MD
Institution:1. Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA;2. Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA
Abstract:Introduction: Quantitative muscle ultrasound (QUS) in boys with Duchenne muscular dystrophy (DMD) shows increased echointensity as muscle is replaced with fat and fibrosis. Studies of quantitative ultrasound in infants/young boys with DMD over time have not been reported. Methods: We used calibrated muscle backscatter (cMB), a reproducible measure of ultrasound echointensity, to quantify muscle pathology in 5 young boys with DMD (ages 0.5–2.8 years) over 17–29 months. We compared the results with repeated assessments of function (n = 4) and with muscle ultrasound images from a cross‐section of 6 male controls (0.6–3.1 years). Results: cMB in boys with DMD increased (worsened) over time (P < 0.001), whereas function improved. After age 2 years, cMB in most (4 of 5) boys with DMD was higher than in any control. Conclusions: QUS measures disease progression in young boys with DMD despite functional improvements. QUS could be employed as an outcome measure for serial assessment of young boys with DMD. Muscle Nerve 52 : 334–338, 2015
Keywords:backscatter  children  Duchenne muscular dystrophy  muscle  myopathy  ultrasound
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