Noninvasive positive pressure ventilation prolongs survival in ALS but its effect on quality of life is unknown. The authors prospectively studied quality of life using the SF-36 questionnaire in a cohort of 16 ventilated patients with ALS. Noninvasive positive pressure ventilation improved scores in the "Vitality" domain by as much as 25%, for periods of up to 15 months, despite disease progression. Noninvasive positive pressure ventilation did not cause reduced quality of life, as any fall in scores in the ventilated group were comparable to those seen in a control group. In conclusion, noninvasive positive pressure ventilation enhances quality of life when used to treat sleep-disordered breathing in patients with ALS.

Inspiratory pressure support (IPS) decreases the work of breathing in patients with hyperinflation secondary to airways obstruction when weaning from mechanical ventilation.'0 Non-invasive assisted ventilation has been shown to reduce breathlessness during acute ventilatory failure resulting from COPD. " These patients have increased loads on the respiratory muscles, particularly during exercise, inducing extreme breathlessness. We hypothesised that the application of non-invasive IPS during exercise may reduce breathlessness and improve exercise tolerance by reducing the load on the respiratory muscles. This hypothesis was tested by measuring the effects of non-invasive IPS by face mask on the tolerance to treadmill exercise in eight patients with stable severe COPD. Walks with IPS were compared with those with oxygen and CPAP. MethodsEight men with stable COPD who complained of disabling breathlessness on exertion were recruited from the chest clinic (age range 60-74 years, mean (SD) FEV, 0 73 (02) 1).All patients underwent a period of familiarisation before data collection. This involved several visits to the laboratory where treadmill walks, with and without breathing equipment, were performed so that the patients became completely familiar with the use of the face masks, CPAP, IPS, the Borg scale, and the treadmill. During this training period a comfortable walking speed was found for each patient matching as closely as possible their usual walking speed. Once this had been established, all subsequent walks were performed at this speed. Patients were asked to walk on the treadmill until their perceived rating of

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