Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease

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“How much survival and quality-of-life benefit NIV brings depends heavily on how affected the bulbar (speech and swallowing) muscles are.”

In the trial, people whose bulbar muscles were normal or only moderately affected lived a median of about 205 days longer and kept better quality of life. People with severe bulbar weakness gained neither.

Survival and quality of life were significantly improved in the subgroup of people with better bulbar function, but not in those with severe bulbar impairment.

Authors' conclusions

!Qualifies this

“NIV is the ALS/MND breathing treatment with the strongest trial evidence behind it, though that evidence rests on one small randomised trial.”

The systematic review of randomised trials of ventilation in ALS/MND. It found only one trial with usable data, of 41 people. That trial showed NIV prolongs survival, but the evidence base is a single small study rather than a large one, and further trials are unlikely because withholding NIV is no longer considered ethical.

Moderate-quality evidence from a single RCT of NIV in 41 participants suggests that it significantly prolongs survival, and low-quality evidence indicates that it improves or maintains quality of life in people with ALS.

Authors' conclusions

iAdds context

“Some people get symptom and sleep benefit from NIV even where it does not extend survival.”

In the trial group with severe bulbar weakness, NIV did not lengthen life or hold up overall quality of life, but a sleep-related symptom score still improved. The review measures scores, so it shows a sleep benefit persisting without a survival gain rather than what people themselves say they value.

In the 21 participants with severe bulbar weakness, NIV did not prolong survival or maintain quality of life scores, although a sleep-related symptoms score improved.

Key results and quality of the evidence

!Qualifies this

“Invasive ventilation can lengthen survival for some people with ALS/MND.”

This Cochrane review looked for randomised trials of tracheostomy ventilation as well as mask ventilation. Every trial it found tested mask ventilation, so the survival benefit of invasive ventilation has not been measured in a randomised trial and rests on observational studies.

Randomised controlled trials (RCTs) and quasi-RCTs involving non-invasive or tracheostomy-assisted ventilation in participants with a clinical diagnosis of ALS, independent of the reported outcomes.

Selection criteria

!Qualifies this

“Invasive ventilation can add time for some people, but it does not slow or stop ALS/MND itself.”

This Cochrane review set out to assess tracheostomy ventilation as well as mask ventilation, and every randomised trial it found tested mask ventilation. So the survival claim for invasive ventilation rests on observation rather than on randomised evidence.

Randomised controlled trials (RCTs) and quasi-RCTs involving non-invasive or tracheostomy-assisted ventilation in participants with a clinical diagnosis of ALS, independent of the reported outcomes.

Selection criteria