title: Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  Noninvasive positive-pressure ventilation may benefit patients with amyotrophic lateral sclerosis and respiratory insufficiency.
  
  
  OBJECTIVE
  To determine 1) whether patients tolerant of noninvasive positive-pressure ventilation have better survival than intolerant patients and 2) whether bulbar symptoms account for intolerance of noninvasive positive-pressure ventilation.
  
  
  METHODS
  Observational cohort study.
  
  
  METHODS
  Tertiary care referral center.
  
  
  METHODS
  39 patients with amyotrophic lateral sclerosis who were treated with noninvasive positive-pressure ventilation.
  
  
  METHODS
  Noninvasive positive-pressure ventilation was started for patients with new orthopnea, new hypercapnia, or both. Patients were divided into two groups: those tolerant of and those intolerant of noninvasive positive-pressure ventilation.
  
  
  RESULTS
  The risk for death from onset of respiratory insufficiency was higher for intolerant patients than for tolerant patients (relative risk, 3.1 [95% CI, 1.8 to 9.6]). Moderate or severe bulbar symptoms were more prevalent among intolerant patients than among tolerant patients (67% compared with 33%; P = 0.04).
  
  
  CONCLUSIONS
  Among patients with amyotrophic lateral sclerosis, those who are tolerant of noninvasive positive-pressure ventilation have better survival than do those who are intolerant. Bulbar symptoms partially account for intolerance of noninvasive positive-pressure ventilation.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, Detroit, MI 48201, USA.
authors.forename: L S
authors.initials: LS
authors.lastname: Aboussouan
authors.forename: S U
authors.initials: SU
authors.lastname: Khan
authors.forename: D P
authors.initials: DP
authors.lastname: Meeker
authors.forename: K
authors.initials: K
authors.lastname: Stelmach
authors.forename: H
authors.initials: H
authors.lastname: Mitsumoto
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D015993:Life Tables; D008297:Male; D008875:Middle Aged; D009830:Olfactory Bulb / Q000503:physiopathology; D011175:Positive-Pressure Respiration; D012131:Respiratory Insufficiency / Q000628:therapy*; D016019:Survival Analysis