Respiratory management of amyotrophic lateral sclerosis : Approaches to respiratory dysfunction based on current knowledge

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of upper and lower motor neurons that also affects the diaphragm and other respiratory muscles. Respiratory dysfunction, including hypoventilation and respiratory failure and respiratory infection are common causes of death and respiratory function significantly affects both survival and quality of life in patients with ALS. Progress in devices such as ventilators and mechanical insufflation–exsufflation and respiratory physiotherapy are recognized to prolong survival without tracheostomy and to maintain or improve quality of life and maintaining or improving quality of life. Due to ethical concerns, intervention studies for non–invasive ventilation cannot be conducted, making observational studies the only viable option. Additionally, the wide variety of ventilator models and settings makes it challenging to evaluate appropriate settings, though several settings have been suggested in observational studies. Respiratory management of ALS is now expected to be a disease–modifying therapy.