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Multicenter Study
. 2004 Jul 13;63(1):176-8.
doi: 10.1212/01.wnl.0000133126.86377.e8.

Ventilatory support in facioscapulohumeral muscular dystrophy

M Wohlgemuth  1 , E L van der Kooi, R G van Kesteren, S M van der Maarel, G W Padberg
Affiliations
Multicenter Study

Ventilatory support in facioscapulohumeral muscular dystrophy

M Wohlgemuth et al. Neurology. .

Abstract

Respiratory insufficiency due to respiratory muscle weakness is a common complication of many neuromuscular diseases. The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) is unknown. The authors identified 10 FSHD patients on nocturnal ventilatory support at home, representing approximately 1% of the Dutch FSHD population. Severe muscle disease, wheelchair dependency, and kyphoscoliosis appeared to be risk factors for respiratory failure.

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