Orphanet Journal of Rare Diseases (Mar 2009)

Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency

  • Scoditti Cristina,
  • Lacedonia Donato,
  • Gadaleta Felice,
  • Maniscalco Mauro,
  • Cassano Anna,
  • Spicuzza Lucia,
  • Carratù Pierluigi,
  • Boniello Ester,
  • Di Maria Giuseppe,
  • Resta Onofrio

DOI
https://doi.org/10.1186/1750-1172-4-10
Journal volume & issue
Vol. 4, no. 1
p. 10

Abstract

Read online

Abstract Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, which rapidly leads to chronic respiratory failure requiring mechanical ventilation. Currently, forced vital capacity (FVC) 75%, independently by any treatment. Aim To assess the role of NPPV in improving outcome of ALS, a retrospective analysis was performed to investigate 1 year survival of ALS patients with FVC Methods We investigated seventy-two consecutive ALS patients who underwent pulmonary function test. Forty-four presented a FVC > 75% and served as control group. Twenty-eight patients presented a FVC Results Increased survival rate at 1 year in patients with FVC Conclusion This report demonstrates that early treatment with NPPV prolongs survival and reduces decline of FVC% in ALS.