Respiratory failure is the leading cause of death in patients with amy
otrophic lateral sclerosis (ALS). We review the physiology of respirat
ory compromise in ALS and techniques of monitoring respiratory functio
n. Treatment options, including pharmacologic interventions, aspiratio
n precautions, and invasive and noninvasive modes of mechanical ventil
ation are reviewed. Our clinical experience with respiratory failure i
n ALS demonstrates significantly prolonged survival in subjects who el
ect to receive noninvasive mechanical ventilation (19.25 vs. 80.4 days
, p < 0.01). Four of 18 patients who elected to receive noninvasive ve
ntilation decided to discontinue treatment. Four of 13 patients who we
re receiving mechanical ventilation elected to discontinue life suppor
t. The decision to utilize these modalities must be made with realisti
c considerations of the patient's quality of life.