Respiratory muscle weakness in facioscapulohumeral muscular dystrophy.
Muscle Nerve
; 60(6): 679-686, 2019 12.
Article
in En
| MEDLINE
| ID: mdl-31566774
ABSTRACT
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 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 = .02).DISCUSSION:
In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.Key words
Full text:
1
Database:
MEDLINE
Main subject:
Respiratory Muscles
/
Diaphragm
/
Muscle Weakness
/
Muscular Dystrophy, Facioscapulohumeral
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
Limits:
Adult
/
Aged
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
Muscle Nerve
Year:
2019
Type:
Article
Affiliation country:
Germany