title: [Nocturnal noninvasive ventilation of children and adolescents with neuromuscular diseases: effect on sleep and symptoms].
abstract: OBJECTIVE
  Sleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disorders (NMD). Aim of the study was to prospectively investigate the impact of three months of non-invasive ventilation (NIV) on sleep and SDB-associated symptoms in children and adolescents with advanced NMD.
  
  
  METHODS
  Twenty-two patients (11.1 +/- 4.3 years) with symptomatic SDB were enrolled in the study and evaluated with polysomnography and a symptom questionnaire. Twenty patients were treated with pressure assisted ventilation during sleep. The impact of NIV on SDB, sleep and symptoms was re-evaluated after 3 months of NIV.
  
  
  RESULTS
  In 2/22 patients, after adenotomy, SDB was no longer demonstrable. In the remaining 20 patients NIV improved SDB (Respiratory Disturbance Index 1.8 +/- 1.6 vs. 9.2 +/- 4.5/h; minimal SpO2 91 +/- 3 vs. 79 +/- 12%). Three months of treatment resulted in shortened sleep latency (17 +/- 13 vs. 42 +/- 38 minutes), improved sleep efficiency (95 +/- 3 vs. 89 +/- 9%) and less EEG-arousals (11 +/- 5 vs. 22 +/- 9/h, p < 0.001 for all). Sleep-stage proportion did not change. Patients reported significant improvements of sleep quality, morning headaches, mood, concentration and daytime sleepiness (p < 0.001 for all).
  
  
  CONCLUSIONS
  In children with NMD intermittent nocturnal NIV results in objective and subjective improvements of sleep quality and symptoms associated with SDB.
authors.affiliation: Abteilung allgemeine Kinderheilkunde/Schwerpunkt Neuropädiatrie, Zentrum für Kinderheilkunde, Universtitätsklinikum Essen, Deutschland. uwe.mellies@uni-essen.de
authors.forename: Uwe
authors.initials: U
authors.lastname: Mellies
authors.forename: Christian
authors.initials: C
authors.lastname: Dohna-Schwake
authors.forename: Regine
authors.initials: R
authors.lastname: Ragette
authors.forename: Helmut
authors.initials: H
authors.lastname: Teschler
authors.forename: Thomas
authors.initials: T
authors.lastname: Voit
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D045422:Continuous Positive Airway Pressure; D005260:Female; D006801:Humans; D007320:Inspiratory Capacity; D008297:Male; D008365:Manometry; D009468:Neuromuscular Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D017286:Polysomnography; D011446:Prospective Studies; D011788:Quality of Life; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D013147:Spirometry; D016896:Treatment Outcome; D014797:Vital Capacity