期刊
MUSCLE & NERVE
卷 60, 期 6, 页码 679-686出版社
WILEY
DOI: 10.1002/mus.26717
关键词
diaphragm ultrasound; facioscapulohumeral muscular dystrophy; phrenic nerve conduction studies; respiratory muscle strength; transdiaphragmatic pressure
资金
- Sanofi-Genzyme, Neu-Isenburg, Germany
Introduction The purpose of this study was to comprehensively evaluate respiratory muscle function in adults with facioscapulohumeral muscular dystrophy (FSHD). Methods Fourteen patients with FSHD (9 men, 53 +/- 16 years of age) and 14 matched controls underwent spirometry, diaphragm ultrasound, and measurement of twitch gastric and transdiaphragmatic pressures (twPgas and twPdi; n = 10) after magnetic stimulation of the lower thoracic nerve roots and the phrenic nerves. The latter was combined with recording of diaphragm compound muscle action potentials (CMAPs; n = 14). 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 cmH(2)O) and twPgas (7 +/- 3 vs 25 +/- 20 cmH(2)O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (r = -0.63, P = .02). Discussion In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.
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