ABSTRACT
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neuromuscular disease, with a median survival from diagnosis of about two years (1). Death is usually secondary to respiratory failure or respiratory complications (2). Riluzole, the only specific treatment for ALS, prolongs survival by about three months (3) but recent advances in the management of respiratory complications can have a major impact both on survival and quality of life underscoring the need to recognize respiratory muscle weakness and the signs of respiratory failure so that treatment can be initiated in a timely fashion. This chapter reviews normal respiratory function and the resultant changes with weakness of the respiratory muscles as ALS progresses. This is followed by a review of the general strategy for the respiratory management of patients with ALS including non-invasive management and tracheostomy with invasive ventilation.
