title: Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome).
abstract: BACKGROUND
  Sleep disturbance is part of the behavioural phenotype of the rare genetic condition mucopolysaccharidosis (MPS) type III. A growing body of evidence suggests that underlying disturbance in circadian rhythm functioning may explain sleep problems within the MPS III population.
  
  
  METHODS
  Actigraphic data were recorded in eight children with MPS III over 7-10 days and compared to age-matched typically developing controls. Parameters of circadian rhythmicity and activity levels across a 24-h period were analysed.
  
  
  RESULTS
  Statistically and clinically significant differences between the two groups were noted. Analysis indicated that children with MPS III showed significantly increased fragmentation of circadian rhythm and reduced stability with external cues (zeitgebers), compared to controls. Average times of activity onset and offset were indicative of a phase delayed sleep-wake cycle for some children in the MPS III group. Children with MPS III had significantly higher activity levels during the early morning hours (midnight-6 am) compared to controls.
  
  
  CONCLUSIONS
  Results are consistent with previous research into MPS III and suggest that there is an impairment in circadian rhythm functioning in children with this condition. Implications for clinical practice and the management of sleep difficulties are discussed.
authors.affiliation: Psychological Services, Alder Hey Children's Hospital, Liverpool, UK.
authors.forename: Rachel A
authors.initials: RA
authors.lastname: Mumford
authors.affiliation: Manchester Mental Health & Social Care NHS Trust, Manchester, UK.
authors.forename: Louise V
authors.initials: LV
authors.lastname: Mahon
authors.affiliation: Genetic Medicine Unit, St Mary's Hospital, Manchester, UK.
authors.forename: Simon
authors.initials: S
authors.lastname: Jones
authors.affiliation: Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
authors.forename: Brian
authors.initials: B
authors.lastname: Bigger
authors.affiliation: Faculty of Life Sciences, University of Manchester, Manchester, UK.
authors.forename: Maria
authors.initials: M
authors.lastname: Canal
authors.affiliation: School of Psychology, Cardiff University, Cardiff, UK.
authors.forename: Dougal Julian
authors.initials: DJ
authors.lastname: Hare
keywords: Actigraphy; Circadian rhythms; Mucopolysaccharidosis type III; Sanfilippo; Sleep