How is respiratory weakness treated in congenital myopathy?

Updated: Mar 11, 2019
  • Author: Matthew Harmelink, MD; Chief Editor: Amy Kao, MD  more...
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Each congenital myopathy can have a varied degree of respiratory involvement secondary to the pulmonary components of the disease; being aware of signs is relevant. Neuromuscular weakness is often more prevelant during sleep. As such, evaluation of signs of hypoventilation at night, including morning headaches, snoring, or daytime sleepiness, should be undertaken. In infants, respiratory support for frequent or prolonged respiratory illnesses may be needed.

Based upon the subtype, the degree of limb weakness does not always correlate to the severity of respiratory weakness. As such, when respiratory involvement is suspected, it may be helpful to consult a pulmonologist and perform pulmonary function tests (if appropriate for the patient's age). Commonly, patients will have a restrictive pattern of lung disease.

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