Ventilatory support in facioscapulohumeral muscular dystrophy

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

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.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Female
  • Funnel Chest / complications
  • Home Nursing
  • Humans
  • Kyphosis / complications
  • Male
  • Middle Aged
  • Muscular Dystrophy, Facioscapulohumeral / complications
  • Muscular Dystrophy, Facioscapulohumeral / epidemiology
  • Muscular Dystrophy, Facioscapulohumeral / therapy*
  • Netherlands / epidemiology
  • Respiration Disorders / epidemiology
  • Respiration Disorders / etiology
  • Respiration Disorders / therapy*
  • Respiration, Artificial*
  • Respiratory Muscles / physiopathology
  • Risk Factors
  • Scoliosis / complications
  • Wheelchairs