Key PointsQuestionWhat is the difference in clinical outcomes among 3 corticosteroid regimens (0.75 mg/kg of daily prednisone, 0.90 mg/kg of daily deflazacort, or 0.75 mg/kg of intermittent prednisone for 10 days on and then 10 days off) as initial treatment for boys with Duchenne muscular dystrophy?FindingsThis randomized clinical trial included 196 boys with Duchenne muscular dystrophy; the clinical outcome was a global outcome that incorporated a measure of rising from the floor, forced vital capacity, and global satisfaction with treatment assessed over 3 years. Daily prednisone and daily deflazacort resulted in significantly better outcomes compared with intermittent prednisone; there was no significant difference between the 2 daily regimens.MeaningThe findings support the use of a daily corticosteroid regimen over an intermittent prednisone regimen that alternates dosing for 10 days on and 10 days off as initial treatment for boys with Duchenne muscular dystrophy.

DMD pathogenic variants for Duchenne and Becker muscular dystrophy are detectable with high sensitivity by standard clinical exome analyses of genomic DNA. However, up to 7% of DMD mutations are deep intronic and analysis of muscle-derived RNA is an important diagnostic step for patients who have negative genomic testing but abnormal dystrophin expression in muscle. In this study, muscle biopsies were evaluated from 19 patients with clinical features of a dystrophinopathy, but negative clinical DMD

Introduction: Ultrasound can potentially identify nerves and guide recording and stimulating electrode placement for nerve conduction studies (NCS). This prospective study was performed to determine whether ultrasound guidance of sural NCS results in higher action potential amplitude, fewer stimuli required, lower stimulus strength required, and less pain experienced. Methods: Fourteen healthy individuals underwent bilateral sural NCS, both with and without ultrasound guidance. Studies were separated by at least 48 h, and the order of testing was randomly assigned. Results: Ultrasound guidance resulted in significantly fewer stimuli and lower stimuli strength required to obtain supramaximal responses (P < 0.01–0.03). Ultrasound guidance required significantly more time to perform than standard sural NCS (P < 0.01). There was no difference in sural nerve amplitude or pain rating between the 2 groups. Discussion: Neuromuscular ultrasound can be used effectively to guide electrode placement during sural NCS. Muscle Nerve 59:705–707, 2019

Ultrasound is a non-invasive, painless, and radiation-free technology that can be used to evaluate scapular winging. Muscle Nerve 56: 7-14, 2017.

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