title: Sleep-disordered breathing in children with congenital muscular dystrophies.
abstract: OBJECTIVE
  Most types of neuromuscular diseases are known to be associated with a high risk of sleep-disordered breathing. We performed a prospective study in a well individualized group of muscular disorders, congenital muscular dystrophies (CMD), to characterize the frequency of sleep-disordered breathing and thereby to determine the potential usefulness of sleep studies in such patients.
  
  
  METHODS
  Twenty CMD children (12 F, 8 M, aged 4-17 years) were included. Using overnight polysomnography, we determined the following parameters: sleep stages, sleep latency, sleep efficiency index, wake time duration, total sleep time (TST), apnea/hypopnea index (AHI), arterial blood oxygen saturation, and nocturnal paroxysmal EEG activity.
  
  
  RESULTS
  As compared to healthy controls, we detected in our study group frequent awakenings, a decreased TST (mean 448 ± 44.4 min) and a decreased REM duration (mean 11.5 ± 3.5% of TST). Significant increase in wake time duration (28-90 min) and decrease in REM duration were observed in 12 patients. An apnea/hypopnea syndrome was detected in 13 patients (65%) with central apneas in 8, obstructive apneas in 2 and 3 mixed apneas in 3 patients. AHI was >10 in 3 cases, <10> 5 in 4 cases and were concomitant with blood oxygen de-saturation in four cases. NPA were detected in 10 patients ranging from 10 to 40% of TST.
  
  
  CONCLUSIONS
  Our results confirm the high incidence of sleep disordered breathing in children with CMD, and thereby, the usefulness of overnight polysomnography recordings in such patients.
authors.affiliation: Paediatric Neurology Unit, Paediatric Department, Raymond Poincare Hospital, Assistance Publique - Hôpitaux de Paris, University of Versailles-St-Quentin, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Pinard
authors.forename: Eric
authors.initials: E
authors.lastname: Azabou
authors.forename: Nouha
authors.initials: N
authors.lastname: Essid
authors.forename: Susana
authors.initials: S
authors.lastname: Quijano-Roy
authors.forename: Samir
authors.initials: S
authors.lastname: Haddad
authors.forename: Fawzia
authors.initials: F
authors.lastname: Cheliout-Héraut
mesh_terms: D000293:Adolescent; D001784:Blood Gas Analysis; D002648:Child; D002675:Child, Preschool; D055724:Electrophysiological Phenomena; D005260:Female; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications / Q000151:congenital* / Q000503:physiopathology*; D009460:Neurologic Examination; D017286:Polysomnography; D011446:Prospective Studies; D012129:Respiratory Function Tests; D015656:Respiratory Mechanics / Q000502:physiology; D012890:Sleep / Q000502:physiology; D012891:Sleep Apnea Syndromes / Q000209:etiology / Q000503:physiopathology*; D012895:Sleep, REM / Q000502:physiology; D014851:Wakefulness