This review highlights recent advances in the critical care management of neuromuscular disease, particularly in the long-term management of chronic respiratory failure occurring as a consequence of neuromuscular disease. Although randomized clinical trial evidence of benefit is sparse, a large volume of nonrandomized clinical trial evidence has accumulated demonstrating that noninvasive positive pressure ventilation prolongs and improves quality of life in conditions such as Duchenne muscular dystrophy and motor neuron disease. Immunomodulatory treatments favorably modify the course of neuromuscular diseases such as Guillain-Barré syndrome, whereas long-term noninvasive positive pressure ventilation has transformed the outlook in previously untreatable conditions such as motor neuron disease and muscular dystrophies. The availability of long-term noninvasive positive pressure ventilation raises major medical, social, economic, and ethical issues that are increasingly being investigated and discussed. Copyright 2003 Lippincott Williams & Wilkins