Respiratory muscle training in children and adults with neuromuscular disease

Review question

Does respiratory muscle training have beneficial effects for children and adults with neuromuscular disease?


Neuromuscular disease is a very broad term that covers many diseases that either directly or indirectly affect muscles or nerves. Children and adults with neuromuscular diseases can present with muscle weakness, loss of movement control, and muscle wasting. Some neuromuscular diseases cause weakness of respiratory muscles (diaphragm and accessory muscles of respiration). The decline of respiratory muscle function in these diseases affects activities of daily living and quality of life. Respiratory muscle training could potentially be considered as an extra therapy for people with suspected or confirmed respiratory muscle weakness.

Study characteristics

This review included 11 studies with a total of 250 randomized participants with neuromuscular disease. Six studies included 112 young males (including children) with Duchenne muscular dystrophy, which is an inherited muscle disease. One trial involved 23 adults with other muscle diseases (Becker muscular dystrophy and limb-girdle muscular dystrophy). Three trials involved 88 people with amyotrophic lateral sclerosis, a progressive condition that affects the nerves controlling movement. One trial involved 27 people with myasthenia gravis, a condition that affects the signals between nerves and muscles.

Key results

The studies showed that respiratory muscle training may result in some improvements in lung function for people with amyotrophic lateral sclerosis and Duchenne muscular dystrophy. However, this finding was not consistent between studies. Physical function and quality of life were only assessed in one amyotrophic lateral sclerosis trial, which indicated that RMT may have no clear effect. One trial reported on adverse events, but the certainty of evidence was too low for conclusions to be drawn. The studies did not report the number of unscheduled hospitalisations for sudden infection or worsening of chronic respiratory failure.

Certainty of the evidence

The certainty of the evidence examined as part of this review was low or very low. Low-certainty evidence means that our confidence in the effect of respiratory muscle training is limited, and the true effect may be substantially different. When the evidence is of very low-certainty, the true effect is likely to be substantially different. Given the low or very low-certainty of the evidence presented in the studies, we believe that there is a need for more well-conducted studies in order to assess the efficacy of respiratory muscle training in people with NMD.

The evidence is current to November 2018.

Authors' conclusions: 

RMT may improve lung capacity and respiratory muscle strength in some NMDs. In ALS there may not be any clinically meaningful effect of RMT on physical functioning or quality of life and it is uncertain whether it causes adverse effects. Due to clinical heterogeneity between the trials and the small number of participants included in the analysis, together with the risk of bias, these results must be interpreted very cautiously.

Read the full abstract...

Neuromuscular diseases (NMDs) are a heterogeneous group of diseases affecting the anterior horn cell of spinal cord, neuromuscular junction, peripheral nerves and muscles. NMDs cause physical disability usually due to progressive loss of strength in limb muscles, and some NMDs also cause respiratory muscle weakness. Respiratory muscle training (RMT) might be expected to improve respiratory muscle weakness; however, the effects of RMT are still uncertain. This systematic review will synthesize the available trial evidence on the effectiveness and safety of RMT in people with NMD, to inform clinical practice.


To assess the effects of respiratory muscle training (RMT) for neuromuscular disease (NMD) in adults and children, in comparison to sham training, no training, standard treatment, breathing exercises, or other intensities or types of RMT.

Search strategy: 

On 19 November 2018, we searched the Cochrane Neuromuscular Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase. On 23 December 2018, we searched the US National Institutes for Health Clinical Trials Registry (, the World Health Organization International Clinical Trials Registry Platform, and reference lists of the included studies.

Selection criteria: 

We included randomized controlled trials (RCTs) and quasi-RCTs, including cross-over trials, of RMT in adults and children with a diagnosis of NMD of any degree of severity, who were living in the community, and who did not need mechanical ventilation. We compared trials of RMT (inspiratory muscle training (IMT) or expiratory muscle training (EMT), or both), with sham training, no training, standard treatment, different intensities of RMT, different types of RMT, or breathing exercises.

Data collection and analysis: 

We followed standard Cochrane methodological procedures.

Main results: 

We included 11 studies involving 250 randomized participants with NMDs: three trials (N = 88) in people with amyotrophic lateral sclerosis (ALS; motor neuron disease), six trials (N = 112) in Duchenne muscular dystrophy (DMD), one trial (N = 23) in people with Becker muscular dystrophy (BMD) or limb-girdle muscular dystrophy, and one trial (N = 27) in people with myasthenia gravis.

Nine of the trials were at high risk of bias in at least one domain and many reported insufficient information for accurate assessment of the risk of bias. Populations, interventions, control interventions, and outcome measures were often different, which largely ruled out meta-analysis. All included studies assessed lung capacity, our primary outcome, but four did not provide data for analysis (1 in people with ALS and three cross-over studies in DMD). None provided long-term data (over a year) and only one trial, in ALS, provided information on adverse events. Unscheduled hospitalisations for chest infection or acute exacerbation of chronic respiratory failure were not reported and physical function and quality of life were reported in one (ALS) trial.

Amyotrophic lateral sclerosis (ALS)

Three trials compared RMT versus sham training in ALS. Short-term (8 weeks) effects of RMT on lung capacity in ALS showed no clear difference in the change of the per cent predicted forced vital capacity (FVC%) between EMT and sham EMT groups (mean difference (MD) 0.70, 95% confidence interval (CI) -8.48 to 9.88; N = 46; low-certainty evidence). The mean difference (MD) in FVC% after four months' treatment was 10.86% in favour of IMT (95% CI -4.25 to 25.97; 1 trial, N = 24; low-certainty evidence), which is larger than the minimal clinically important difference (MCID, as estimated in people with idiopathic pulmonary fibrosis). There was no clear difference between IMT and sham IMT groups, measured on the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALFRS; range of possible scores 0 = best to 40 = worst) (MD 0.85, 95% CI -2.16 to 3.85; 1 trial, N = 24; low-certainty evidence) or quality of life, measured on the EuroQol-5D (0 = worst to 100 = best) (MD 0.77, 95% CI -17.09 to 18.62; 1 trial, N = 24; low-certainty evidence) over the medium term (4 months). One trial report stated that the IMT protocol had no adverse effect (very low-certainty evidence).

Duchenne muscular dystrophy (DMD)

Two DMD trials compared RMT versus sham training in young males with DMD. In one study, the mean post-intervention (6-week) total lung capacity (TLC) favoured RMT (MD 0.45 L, 95% CI -0.24 to 1.14; 1 trial, N = 16; low-certainty evidence). In the other trial there was no clear difference in post-intervention (18 days) FVC between RMT and sham RMT (MD 0.16 L, 95% CI -0.31 to 0.63; 1 trial, N = 20; low-certainty evidence). One RCT and three cross-over trials compared a form of RMT with no training in males with DMD; the cross-over trials did not provide suitable data. Post-intervention (6-month) values showed no clear difference between the RMT and no training groups in per cent predicted vital capacity (VC%) (MD 3.50, 95% CI -14.35 to 21.35; 1 trial, N = 30; low-certainty evidence).

Becker or limb-girdle muscular dystrophy

One RCT (N = 21) compared 12 weeks of IMT with breathing exercises in people with Becker or limb-girdle muscular dystrophy. The evidence was of very low certainty and conclusions could not be drawn.

Myasthenia gravis

In myasthenia gravis, there may be no clear difference between RMT and breathing exercises on measures of lung capacity, in the short term (TLC MD -0.20 L, 95% CI -1.07 to 0.67; 1 trial, N = 27; low-certainty evidence). Effects of RMT on quality of life are uncertain (1 trial; N = 27).

Some trials reported effects of RMT on inspiratory and/or expiratory muscle strength; this evidence was also of low or very low certainty.