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Psychomotor development in infants with Prader-Willi syndrome and associations with sleep-related breathing disorders

Dederieke A M Festen1, Maaike Wevers, Al W de Weerd

  • 1Dutch Growth Foundation, 3016AH Rotterdam, The Netherlands. d.festen@erasmusmc.nl

Pediatric Research
|June 29, 2007
PubMed

Insights

Prader-Willi syndrome (PWS) infants

Area of Science:

  • Neurogenetics
  • Pediatrics
  • Sleep Medicine

Background:

  • Prader-Willi syndrome (PWS) is a complex neurogenetic disorder.
  • PWS is characterized by hypotonia, psychomotor delay, obesity, short stature, and sleep disorders.
  • Sleep-related breathing disorders are common in PWS infants.

Purpose of the Study:

  • To investigate the association between psychomotor development and sleep-related breathing disorders in infants with PWS.
  • To determine if central or obstructive sleep apnea impacts neurodevelopment in PWS.
  • To provide recommendations for screening and management of PWS infants.

Main Methods:

  • 22 infants with PWS underwent psychometric evaluation using Bayley Scales of Infant Development.
  • Polysomnography was used to assess sleep-related breathing disorders.
  • Psychomotor development was correlated with sleep study results and body mass index.

Main Results:

  • All PWS infants exhibited sleep-related breathing disorders, predominantly central.
  • No association was found between the apnea-hypopnea index and psychomotor development.
  • Infants with obstructive sleep apnea syndrome (OSAS) showed significantly delayed mental development (65.5% of normal) and higher BMI SDS.

Conclusions:

  • Psychomotor development in PWS infants is not linked to central sleep disorders.
  • Obstructive sleep apnea syndrome (OSAS) is associated with more severe mental developmental delay in PWS.
  • Routine screening for OSAS in PWS infants is recommended.

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