Respiratory involvement in inherited primary muscle conditions

J Neurol Neurosurg Psychiatry. 2006 Oct;77(10):1108-15. doi: 10.1136/jnnp.2005.078881.


Patients with inherited muscle disorders can develop respiratory muscle weakness leading to ventilatory failure. Predicting the extent of respiratory involvement in the different types of inherited muscle disorders is important, as it allows clinicians to impart prognostic information and offers an opportunity for early interventional management strategies. The approach to respiratory assessment in patients with muscle disorders, the current knowledge of respiratory impairment in different muscle disorders and advice on the management of respiratory complications are summarised.

Publication types

  • Review

MeSH terms

  • Humans
  • Muscle Weakness / etiology
  • Muscle Weakness / physiopathology*
  • Muscular Diseases / complications*
  • Muscular Diseases / genetics*
  • Respiratory Function Tests
  • Respiratory Insufficiency / etiology*
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy