Respiratory training in an individual with amyotrophic lateral sclerosis

Abstract

We examined the impact of expiratory muscle strength training on maximum expiratory pressure, cough spirometry, and disease progression in a 71-year-old male with amyotrophic lateral sclerosis. Maximum expiratory pressure declined 9% over an 8-week sham training period, but subsequently improved by 102% following 8 weeks of expiratory muscle strength training. Improvements in cough spirometry and mitigated disease progression were also observed post expiratory muscle strength training. Improvements in maximum expiratory pressures were maintained 6 months following expiratory muscle strength training and were 79% higher than baseline data obtained 301 days prior. In this spinal-onset amyotrophic lateral sclerosis patient, respiratory training improved subglottic air pressure generation and sequential cough generation.

Figures

Figure 1

Maximum expiratory pressure (MEPs cm H20) in a 71‐year‐old male patient with amyotrophic lateral sclerosis who underwent 8 weeks of sham training followed by 8 weeks of expiratory muscle strength training at 50% of individualized MEP. Three‐ and six‐month follow‐up MEP data are also included. MEPs decreased by 9% between baseline and post‐sham and increased by 102% pre‐ versus post‐EMST. Six‐month follow‐up data indicate that treatment gains were maintained at 100 cm H20, representing a 3% decrease from the immediate post‐EMST assessment and a 79% increase from his initial baseline (pre‐sham) MEPs taken 10 months earlier.

Figure 2

A cough epoch, or sequential cough flow waveform depicting cough total (CrTot), defined as number of coughs within an epoch and cough inspired volume (CIV), defined as the amount of air inspired prior to cough at baseline (A) and post‐EMST (B) evaluations in a 71‐year‐old male with amyotrophic lateral sclerosis. Cr1, Cr2, etc. represent individual coughs within the epoch, which make up the CrTot value.