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ALS is a progressive and uniformly fatal neuromuscular disease, with a median survival from diagnosis of about 3 years. Death is usually secondary to respiratory failure or respiratory complications. Riluzole, the only specific treatment for ALS, prolongs survival by only 3 months, but recent advances in the management of respiratory complications have shown a much greater effect of noninvasive positive pressure ventilation (NIPPV), on both survival and quality of life. In this issue of Neurology, Lo Coco et al.1 examined 71 patients who were offered NIPPV according to standard guidelines. They demonstrated that there was improved survival in patients with ALS who were compliant (61.9%) with NIPPV (≥4 h/day) and that there was a slowing of the rate of decline in forced vital capacity (FVC) after treatment initiation in patients who were tolerant but not in those patients unable to tolerate NIPPV. They found that tolerance of NIPPV and survival in the ALS population could be predicted by the severity of bulbar impairment and the nutritional status of the patients at the start of NIPPV. Patients with severe bulbar involvement were less likely to tolerate NIPPV. Finally, a body …
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