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Expiratory muscle function in amyotrophic lateral sclerosis

M I Polkey1, R A Lyall, M Green

  • 1Respiratory Muscle Laboratory and Department of Clinical Neurosciences, King's College School of Medicine and Dentistry; and Respiratory Muscle Laboratory, Royal Brompton Hospital, London, UK.

American Journal of Respiratory and Critical Care Medicine
|September 10, 1998
PubMed
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Amyotrophic lateral sclerosis (ALS) patients with significant expiratory muscle weakness struggle to cough effectively. However, inspiratory muscle weakness appears to be the main driver of respiratory issues in ALS.

Area of Science:

  • Neurology
  • Pulmonology
  • Muscle Physiology

Background:

  • Limited data exists on expiratory muscle function in amyotrophic lateral sclerosis (ALS).
  • Understanding muscle function is crucial for managing respiratory complications in ALS.

Purpose of the Study:

  • To investigate expiratory muscle function in ALS patients.
  • To correlate expiratory muscle strength with cough effectiveness and respiratory status.

Main Methods:

  • Studied 26 ALS patients, measuring maximal static expiratory mouth pressure (MEP), cough gastric pressure (Cough Pga), and nerve-stimulated gastric pressure (Tw Pga).
  • Assessed cough spikes, blood gases, and inspiratory muscle strength.
  • Performed endoscopic examination of vocal cord motion in symptomatic patients.

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Main Results:

  • Expiratory muscle weakness correlated with an inability to generate cough spikes, with thresholds for Cough Pga (<50 cm H2O) and Tw Pga (<7 cm H2O).
  • Symptomatic ALS patients showed significantly lower inspiratory muscle strength and Tw Pga.
  • Higher PaCO2 was associated with maximal sniff esophageal pressure, not expiratory measures.

Conclusions:

  • Substantial abdominal muscle weakness in ALS impairs the ability to generate a forceful cough.
  • Inspiratory muscle weakness is the primary factor contributing to ventilatory failure and respiratory symptoms in ALS.
  • Abnormal vocal cord motion was noted in a subset of symptomatic patients.