title: Psychomotor development in infants with Prader-Willi syndrome and associations with sleep-related breathing disorders.
abstract: Prader-Willi syndrome (PWS) is a neurogenetic disorder with hypotonia, psychomotor delay, obesity, short stature, and sleep-related breathing disorders. The aim of this study was to evaluate the association between psychomotor development and sleep-related breathing disorders in PWS infants. Bayley Scales of Infant Development were performed in 22 PWS infants, with a median (interquartile range, IQR) age of 1.8 (1.1-3.4) y, and a body mass index SD score (BMISDS) of -0.5 (-1.3 to 1.6). We evaluated psychomotor development in relation to results of polysomnography. Median (IQR) mental and motor development was 73.1% (64.3-79.6%) and 55.2% (46.5-63.1%) of normal children, respectively. All infants had sleep-related breathing disorders, mostly of central origin. The apnea hypopnea index was not associated with psychomotor development. Only four infants had obstructive sleep apnea syndrome (OSAS). They had a significantly delayed mental development of 65.5% (60.0-70.3%) of normal. They had a median BMISDS of 1.4 (0.1-1.6), which tended to be higher than in those without OSAS. Our data indicate that psychomotor development in PWS infants is not related to central sleep-related breathing disorders, but infants with OSAS have more severely delayed mental development, suggesting that PWS infants should be screened for OSAS.
authors.affiliation: Dutch Growth Foundation, 3016AH Rotterdam, The Netherlands. d.festen@erasmusmc.nl
authors.forename: Dederieke A M
authors.initials: DA
authors.lastname: Festen
authors.forename: Maaike
authors.initials: M
authors.lastname: Wevers
authors.forename: Al W
authors.initials: AW
authors.lastname: de Weerd
authors.forename: Renilde A S
authors.initials: RA
authors.lastname: van den Bossche
authors.forename: Hugo J
authors.initials: HJ
authors.lastname: Duivenvoorden
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Jan Maarten
authors.initials: JM
authors.lastname: Wit
authors.forename: Anita C S
authors.initials: AC
authors.lastname: Hokken-Koelega
mesh_terms: D015992:Body Mass Index; D002657:Child Development; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D011597:Psychomotor Performance; D020182:Sleep Apnea, Central / Q000209:etiology* / Q000503:physiopathology / Q000523:psychology; D020181:Sleep Apnea, Obstructive / Q000209:etiology* / Q000503:physiopathology / Q000523:psychology