How is respiratory failure treated in limb-girdle muscular dystrophy (LGMD)?

Updated: Aug 15, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Many LGMDs may have early respiratory involvement (LGMD1A, LGMD1B, LGMD1D, LGMD1E, LGMD1F, LGMD2B, LGMD2C-F, LGMD2G, LGMD2I, LGMD2J, LGMD2K, LGMD2M, LGMD2N, LGMD2O, LGMD2R, LGMD2T, LGMD2U, LGMD2V, LGMD2W).

Pulmonary function testing should be done in the neurology clinic or through referral to a pulmonologist in most LGMD patients at time of presentation or when symptomatic.

Patients with excessive daytime sleepiness, frequent arousals, morning headache, or with shortness of breath or abnormal pulmonary function tests should be referred to a pulmonary or sleep medicine clinic for consideration of non-invasive ventilation.

Early intervention to treat respiratory insufficiency with non-invasive ventilation can help improve function and prolong the patient's life expectancy.


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