Assessment of sleep in children with mucopolysaccharidosis type III

PLoS One. 2014 Feb 4;9(2):e84128. doi: 10.1371/journal.pone.0084128. eCollection 2014.

Abstract

Sleep disturbances are prevalent in mucopolysaccharidosis Type III (MPS III), yet there is a lack of objective, ecologically valid evidence detailing sleep quantity, quality or circadian system. Eight children with MPS III and eight age-matched typically developing children wore an actigraph for 7-10 days/nights. Saliva samples were collected at three time-points on two separate days, to permit analysis of endogenous melatonin levels. Parents completed a sleep questionnaire and a daily sleep diary. Actigraphic data revealed that children with MPS III had significantly longer sleep onset latencies and greater daytime sleep compared to controls, but night-time sleep duration did not differ between groups. In the MPS III group, sleep efficiency declined, and sleep onset latency increased, with age. Questionnaire responses showed that MPS III patients had significantly more sleep difficulties in all domains compared to controls. Melatonin concentrations showed an alteration in the circadian system in MPS III, which suggests that treatment for sleep problems should attempt to synchronise the sleep-wake cycle to a more regular pattern. Actigraphy was tolerated by children and this monitoring device can be recommended as a measure of treatment success in research and clinical practice.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Actigraphy
  • Adolescent
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Melatonin / metabolism
  • Mucopolysaccharidosis III / physiopathology*
  • Sleep / physiology*
  • Time Factors

Substances

  • Melatonin

Grants and funding

The MPS Society UK (http://www.mpssociety.org.uk/) partially funded the study. No additional external funding was received for this study. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.