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
Abstract
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.