title: Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation.
abstract: BACKGROUND
  Non-invasive ventilation (NIV) is part of standard care in amyotrophic lateral sclerosis (ALS). Intolerance or unavailability of NIV, as well as the quality of correction of nocturnal hypoventilation, has a direct impact on prognosis.
  
  
  OBJECTIVE
  We describe the importance of NIV failure due to upper airway obstructive events, the clinical characteristics, as well as their impact on the prognosis of ALS.
  
  
  METHODS
  Retrospective analysis of the data of 190 patients with ALS and NIV in a single centre for the period 2011-2014. 179 patients tolerating NIV for more than 4 h per night without leaks were analysed.
  
  
  RESULTS
  Among the 179 patients, after correction of leaks, 73 remained inadequately ventilated at night (defined as more than 5% of the night spent at <90% of SpO2), as a result of obstructive events in 67% of cases (n=48). Patients who remained inadequately ventilated after optimal adjustment of ventilator settings presented with shorter survival than adequately ventilated patients. Unexpectedly, patients with upper airway obstructive events without nocturnal desaturation and in whom no adjustment of treatment was therefore performed also presented with shorter survival. On initiation of NIV, no difference was demonstrated between patients with and without upper airway obstructive events. In all patients, upper airway obstruction was concomitant with reduction of ventilatory drive.
  
  
  CONCLUSIONS
  This study shows that upper airway obstruction during NIV occurs in patients with ALS and is associated with poorer prognosis. Such events should be identified as they can be corrected by adjusting ventilator settings.
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France.
authors.forename: Marjolaine
authors.initials: M
authors.lastname: Georges
authors.affiliation: Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France Département "R3S", Service des Pathologies du Sommeil AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Attali
authors.affiliation: Département de biostatistiques, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Jean Louis
authors.initials: JL
authors.lastname: Golmard
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France.
authors.forename: Capucine
authors.initials: C
authors.lastname: Morélot-Panzini
authors.affiliation: Unité de la Voix, Service ORL et Chirurgie Cervico-Faciale, AP-HP, Hôpital Européen Georges Pompidou, Paris, France Université Paris Descartes, CNRS UMR 7018, Paris, France.
authors.forename: Lise
authors.initials: L
authors.lastname: Crevier-Buchman
authors.affiliation: Département "R3S", Service des Pathologies du Sommeil AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Collet
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Tintignac
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Elise
authors.initials: E
authors.lastname: Morawiec
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: Valery
authors.initials: V
authors.lastname: Trosini-Desert
authors.affiliation: Département de Neurologie et Centre de Référence pour la SLA, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France.
authors.forename: François
authors.initials: F
authors.lastname: Salachas
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Similowski
authors.affiliation: Département "R3S", Service de Pneumologie et Réanimation Médicale, AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, Paris, France.
authors.forename: Jesus
authors.initials: J
authors.lastname: Gonzalez-Bermejo
mesh_terms: D000368:Aged; D000402:Airway Obstruction / Q000401:mortality* / Q000628:therapy*; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality* / Q000628:therapy*; D003131:Combined Modality Therapy; D015897:Comorbidity; D005260:Female; D006785:Hospitals, University; D006801:Humans; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation; D010100:Oxygen / Q000097:blood; D017286:Polysomnography; D011379:Prognosis; D012131:Respiratory Insufficiency / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D019782:Riluzole / Q000627:therapeutic use; D016019:Survival Analysis
chemical_list: D019782:Riluzole; D010100:Oxygen