title: Sleep architecture in infants with spinal muscular atrophy type 1.
abstract: OBJECTIVE
  Few reports on sleep patterns of patients with spinal muscular atrophy type 1 (SMA1) have been published and none on sleep microstructure. The aim of this study was to analyze sleep architecture and microstructure in a group of infants with SMA1, compared with age- and sex-matched controls.
  
  
  METHODS
  Twelve SMA1 patients (six males, mean age 5.9 months) and 10 controls (five males, mean age 4.8 months) underwent full polysomnography to evaluate their sleep architecture and microstructure by means of the cyclic alternating pattern (CAP).
  
  
  RESULTS
  Compared with control children, SMA1 patients showed increased sleep latency and apnea/hypopnea index. CAP analysis revealed a significant increase in the percentage of A1 CAP subtypes, a reduction of that of A3 subtypes and of A2 and A3 indexes (number/h), indicating a dysfunction of the arousal system in these patients.
  
  
  CONCLUSIONS
  The results indicate the presence of an abnormality of sleep microstructure in SMA1 patients, characterized by a reduction of A2 and A3 CAP subtypes. We hypothesize that SMA1 patients have reduced arousability during non-rapid eye movement sleep, which could be interpreted as additional evidence of central nervous system involvement in this disease.
authors.affiliation: Respiratory Unit, Pediatric Department, Bambino Gesù Children's Research Hospital, Rome, Italy. Electronic address: elisabettaverrillo@hotmail.com.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Verrillo
authors.affiliation: Department of Developmental and Social Psychology, Sapienza University, Rome, Italy.
authors.forename: Oliviero
authors.initials: O
authors.lastname: Bruni
authors.affiliation: Respiratory Unit, Pediatric Department, Bambino Gesù Children's Research Hospital, Rome, Italy.
authors.forename: Martino
authors.initials: M
authors.lastname: Pavone
authors.affiliation: Department of Neurology, Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina, Italy.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Ferri
authors.affiliation: Respiratory Unit, Pediatric Department, Bambino Gesù Children's Research Hospital, Rome, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Caldarelli
authors.affiliation: Department of Developmental and Social Psychology, Sapienza University, Rome, Italy.
authors.forename: Luana
authors.initials: L
authors.lastname: Novelli
authors.affiliation: Respiratory Unit, Pediatric Department, Bambino Gesù Children's Research Hospital, Rome, Italy.
authors.forename: Maria Beatrice Chiarini
authors.initials: MB
authors.lastname: Testa
authors.affiliation: Respiratory Unit, Pediatric Department, Bambino Gesù Children's Research Hospital, Rome, Italy.
authors.forename: Renato
authors.initials: R
authors.lastname: Cutrera
mesh_terms: D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017286:Polysomnography; D012890:Sleep / Q000502:physiology; D020921:Sleep Arousal Disorders / Q000209:etiology / Q000503:physiopathology; D012892:Sleep Deprivation / Q000209:etiology / Q000503:physiopathology; D012893:Sleep Wake Disorders / Q000209:etiology* / Q000503:physiopathology; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications* / Q000503:physiopathology
keywords: Arousability; Central nervous system; Cyclic alternating pattern; Sleep; Sleep breathing disorder; Spinal muscular atrophy type 1