In amyotrophic lateral sclerosis, the onset of respiratory muscle weakness is silent, but survival following symptom recognition may only be a few weeks. Consequently, respiratory function and symptoms should be assessed every 2-3 months. Noninvasive ventilation improves symptoms, quality of life and survival, without increasing carer burden. Lung volume recruitment helps to reverse and prevent atelectasis, improving gas exchange, while techniques to enhance sputum clearance reduce the risk of mucus plugging and lower respiratory tract infections. When noninvasive support fails, often due to severe bulbar impairment, tracheostomy ventilation prolongs life. Most patients receiving tracheostomy ventilation at home report satisfactory quality of life, but at the expense of high carer burden. Diaphragmatic pacing is associated with an increased risk of death.
Keywords: amyotrophic lateral sclerosis; lung function; noninvasive ventilation; respiratory muscle weakness; ventilation.