title: Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency.
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) < 50% is considered as physiologic marker for admitting patients to Noninvasive Positive Pressure Ventilation (NPPV) intervention, although it has been recently shown the median survival of patients with baseline FVC < 75% much shorter than median survival of patients with baseline FVC > 75%, independently by any treatment.
  
  
  OBJECTIVE
  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 < 75% and nocturnal respiratory insufficiency, treated with NPPV, compared to a well-matched population of ALS patients, who refused or was intolerant to NPPV.
  
  
  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 < 75% and showed, at polysomnography analysis, nocturnal respiratory insufficiency, requiring NPPV; sixteen were treated with NPPV, while twelve refused or were intolerant.
  
  
  RESULTS
  Increased survival rate at 1 year in patients with FVC < 75% treated with NPPV, as compared to those who refused or could not tolerate NPPV (p = 0.02), was observed. The median rate of decline in FVC% was slower in NPPV patients than in patients who did not use NPPV (95% CI: 0.72 to 1.85; p < 0.0001).
  
  
  CONCLUSIONS
  This report demonstrates that early treatment with NPPV prolongs survival and reduces decline of FVC% in ALS.
authors.affiliation: Institute of Pulmonary Disease, University of Bari, Bari, Italy. pierluigicarratu@yahoo.com
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Carratù
authors.forename: Lucia
authors.initials: L
authors.lastname: Spicuzza
authors.forename: Anna
authors.initials: A
authors.lastname: Cassano
authors.forename: Mauro
authors.initials: M
authors.lastname: Maniscalco
authors.forename: Felice
authors.initials: F
authors.lastname: Gadaleta
authors.forename: Donato
authors.initials: D
authors.lastname: Lacedonia
authors.forename: Cristina
authors.initials: C
authors.lastname: Scoditti
authors.forename: Ester
authors.initials: E
authors.lastname: Boniello
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Di Maria
authors.forename: Onofrio
authors.initials: O
authors.lastname: Resta
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000401:mortality* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011175:Positive-Pressure Respiration; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy*; D013534:Survival; D016019:Survival Analysis; D016896:Treatment Outcome; D014797:Vital Capacity