title: Oral appliance to assist non-invasive ventilation in a patient with amyotrophic lateral sclerosis.
abstract: BACKGROUND
  From the moment the respiratory muscle groups are affected in amyotrophic lateral sclerosis (ALS), respiratory complications will be the major cause of morbidity and mortality. Untreated respiratory muscle impairment leads to respiratory insufficiency and additionally to difficulties in airway secretion clearance. Non-invasive ventilation (NIV) is the first choice in treating respiratory insufficiency in ALS as it improves sleep-related symptoms, quality of life and life expectancy. Nevertheless, NIV is not always effective, probably due to bulbar dysfunction or anatomical abnormalities. As a result, tracheostomy ventilation (TV) may become necessary.
  
  
  METHODS
  In this case report, we present a 60-year-old female with ALS, for whom it was not possible to provide a sufficient tidal volume with NIV. A chin lift was performed while the patient was awake to see if a more anterior jaw position would lead to an increased tidal volume. As this was the case, a mandibular advancement device (MAD) was fabricated.
  
  
  RESULTS
  With a combination of a MAD and NIV, the upper airway obstructions were overcome and a good ventilation and adherence to therapy were seen.
  
  
  CONCLUSIONS
  When there is the presumption of airway obstructions in combination with an ineffective NIV, we advise to perform a chin lift to assess whether the obstructions can be overcome by a more anterior jaw position. If that is the case, NIV may be combined with MAD to establish effective ventilation and avoid the use of TV.
authors.affiliation: Department of Oral and Maxillofacial Surgery, University Medical Center Groningen, University of Groningen, Hanzeplein 1, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands, s.k.b.veldhuis@alumnus.rug.nl.
authors.forename: Steffanie K B
authors.initials: SK
authors.lastname: Veldhuis
authors.forename: Michiel H J
authors.initials: MH
authors.lastname: Doff
authors.forename: Boudewijn
authors.initials: B
authors.lastname: Stegenga
authors.forename: Jellie A
authors.initials: JA
authors.lastname: Nieuwenhuis
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Wijkstra
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000628:therapy*; D045422:Continuous Positive Airway Pressure; D005260:Female; D006801:Humans; D019186:Mandibular Advancement / Q000295:instrumentation*; D008875:Middle Aged; D017090:Occlusal Splints; D016382:Orthodontic Appliance Design; D012131:Respiratory Insufficiency / Q000628:therapy*