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Related Experiment Videos

Respiratory muscle function in myasthenia gravis.

A K Mier-Jedrzejowicz1, C Brophy, M Green

  • 1Respiratory Muscle Laboratory, Brompton Hospital, London, United Kingdom.

The American Review of Respiratory Disease
|October 1, 1988
PubMed
Summary

Patients with myasthenia gravis often experience respiratory muscle weakness. Edrophonium hydrochloride (Tensilon) improved respiratory muscle strength, but not vital capacity, in these individuals.

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Area of Science:

  • Neurology
  • Pulmonology
  • Clinical Physiology

Background:

  • Generalized myasthenia gravis (MG) can cause respiratory muscle weakness and breathlessness.
  • Assessing respiratory muscle function is crucial for managing MG patients.

Purpose of the Study:

  • To evaluate global respiratory muscle function and diaphragmatic strength in MG patients with breathlessness.
  • To compare respiratory muscle strength with quadriceps femoris muscle strength.
  • To assess the response of respiratory muscles to edrophonium hydrochloride (Tensilon).

Main Methods:

  • 17 patients with mild-to-moderate generalized MG and breathlessness were studied.
  • Maximal static mouth pressures, quadriceps femoris strength, vital capacity (VC), sniff transdiaphragmatic pressure (sniff Pdi), and twitch transdiaphragmatic pressure (twitch Pdi) were measured.
  • Measurements were repeated after Tensilon administration.

Main Results:

  • Reduced maximal static expiratory and inspiratory mouth pressures and quadriceps strength were common.
  • VC was abnormal in most patients, but sniff Pdi was reduced in only half.
  • Tensilon significantly improved mouth pressures, quadriceps strength, and sniff Pdi, but not VC or twitch Pdi.
  • Twitch Pdi only detected diaphragmatic weakness in severe cases.

Conclusions:

  • Expiratory and inspiratory muscle weakness is frequent in MG.
  • Respiratory muscle strength improves with Tensilon, indicating cholinergic responsiveness.
  • Mouth pressures and sniff Pdi are more sensitive indicators of respiratory muscle strength than VC in MG.
  • Twitch Pdi is less sensitive for detecting diaphragmatic involvement in MG unless weakness is severe.

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