Muscle and Nerve 2019-Sep
Respiratory Muscle Weakness in Facioscapulohumeral Muscular Dystrophy.
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RESULTS
The following parameters were significantly lower in patients vs. controls: forced vital capacity (FVC), maximum inspiratory and expiratory pressure, peak cough flow, diaphragm excursion amplitude and thickening ratio on ultrasound, twPdi (11±5 vs. 20±6 cmH2 O), and twPgas (7±3 vs. 25±20 cmH2 O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (r=-0.63, p=0.02).