Abstract
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 …
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.