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Updated: Aug 3, 2026
14:10
Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
Published on: January 31, 2013
Ventilatory support in facioscapulohumeral muscular dystrophy
M Wohlgemuth1, E L van der Kooi, R G van Kesteren
1Department of Neurology, University Medical Center Nijmegen, Reinier Postlaan 4, 6500 HB Nijmegen, The Netherlands. m.wohlgemuth@neuro.umcn.nl
Neurology
|July 14, 2004
Summary
Respiratory muscle weakness causes respiratory insufficiency in neuromuscular diseases. In facioscapulohumeral muscular dystrophy (FSHD), severe disease, wheelchair use, and kyphoscoliosis are risk factors for respiratory failure.
Area of Science:
- Neurology
- Pulmonology
- Genetics
Background:
- Respiratory muscle weakness is a common complication in neuromuscular diseases.
- The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) remains largely unknown.
- Understanding respiratory complications in FSHD is crucial for patient management.
Purpose of the Study:
- To investigate the prevalence of respiratory failure in the Dutch FSHD population.
- To identify risk factors associated with respiratory failure in FSHD patients.
Main Methods:
- Retrospective analysis of 10 FSHD patients requiring nocturnal ventilatory support.
- Assessment of clinical factors including disease severity, wheelchair dependency, and spinal deformity (kyphoscoliosis).
Main Results:
- Nocturnal ventilatory support was required by approximately 1% of the Dutch FSHD population studied.
- Severe muscle disease, wheelchair dependency, and kyphoscoliosis were identified as significant risk factors for respiratory failure in FSHD.
Conclusions:
- Respiratory failure is a significant concern in FSHD, necessitating ventilatory support in a subset of patients.
- Early identification of risk factors like severe muscle weakness, immobility, and spinal curvature can aid in proactive management of respiratory complications in FSHD.
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