Psychomotor development in infants with Prader-Willi syndrome and associations with sleep-related breathing disorders.
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.
Festen
Dederieke A M
DA
Dutch Growth Foundation, 3016AH Rotterdam, The Netherlands. d.festen@erasmusmc.nl
Wevers
Maaike
M
de Weerd
Al W
AW
van den Bossche
Renilde A S
RA
Duivenvoorden
Hugo J
HJ
Otten
Barto J
BJ
Wit
Jan Maarten
JM
Hokken-Koelega
Anita C S
AC
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