Display options
Cite
Mumford RA, Mahon LV, Jones S, et al. Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome). J Neurodev Disord. 2015;7(1):31doi: 10.1186/s11689-015-9126-5.
Mumford, R. A., Mahon, L. V., Jones, S., Bigger, B., Canal, M., & Hare, D. J. (2015). Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome). Journal of neurodevelopmental disorders, 7(1), 31. https://doi.org/10.1186/s11689-015-9126-5
Mumford, Rachel A, et al. "Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome)." Journal of neurodevelopmental disorders vol. 7,1 (2015): 31. doi: https://doi.org/10.1186/s11689-015-9126-5
Mumford RA, Mahon LV, Jones S, Bigger B, Canal M, Hare DJ. Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome). J Neurodev Disord. 2015;7(1):31. doi: 10.1186/s11689-015-9126-5. Epub 2015 Sep 01. PMID: 26388955; PMCID: PMC4574189.
Copy Download .nbib Format: NLM
Share it on

J Neurodev Disord. 2015;7(1):31. doi: 10.1186/s11689-015-9126-5. Epub 2015 Sep 01.

Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome).

Journal of neurodevelopmental disorders

Rachel A Mumford, Louise V Mahon, Simon Jones, Brian Bigger, Maria Canal, Dougal Julian Hare

Affiliations

  1. Psychological Services, Alder Hey Children's Hospital, Liverpool, UK.
  2. Manchester Mental Health & Social Care NHS Trust, Manchester, UK.
  3. Genetic Medicine Unit, St Mary's Hospital, Manchester, UK.
  4. Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
  5. Faculty of Life Sciences, University of Manchester, Manchester, UK.
  6. School of Psychology, Cardiff University, Cardiff, UK.

PMID: 26388955 PMCID: PMC4574189 DOI: 10.1186/s11689-015-9126-5

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.

Keywords: Actigraphy; Circadian rhythms; Mucopolysaccharidosis type III; Sanfilippo; Sleep

References

  1. Arch Dis Child. 2005 Dec;90(12):1239-42 - PubMed
  2. J Neurosci Methods. 2001 Feb 15;105(2):185-91 - PubMed
  3. Dev Med Child Neurol. 2006 Aug;48(8):650-5 - PubMed
  4. Arch Dis Child. 1995 Jul;73(1):77-81 - PubMed
  5. Mol Genet Metab. 2009 Jan;96(1):20-6 - PubMed
  6. J Inherit Metab Dis. 2013 Mar;36(2):281-91 - PubMed
  7. Sleep. 1999 Feb 1;22(1):95-103 - PubMed
  8. Dev Med Child Neurol. 2004 Jun;46(6):372-80 - PubMed
  9. Sleep Med. 2012 Oct;13(9):1164-70 - PubMed
  10. Eur J Pediatr. 1993 Sep;152(9):754-9 - PubMed
  11. J Intellect Disabil Res. 2007 Jan;51(Pt 1):2-13 - PubMed
  12. Mol Genet Metab. 2008 Feb;93(2):104-11 - PubMed
  13. Brain Dev. 2005 Nov;27 Suppl 1:S35-S42 - PubMed
  14. Sleep Med. 2004 Nov;5(6):541-50 - PubMed
  15. Pediatrics. 2008 Dec;122(6):1343-50 - PubMed
  16. J Med Genet. 2003 Jan;40(1):74-8 - PubMed
  17. Arch Dis Child. 1993 Sep;69(3):403-6 - PubMed
  18. Mol Psychiatry. 2008 Jan;13(1):90-8 - PubMed
  19. Sleep Med. 2007 Sep;8(6):681-7 - PubMed
  20. Sleep. 2003 May 1;26(3):342-92 - PubMed
  21. J Inherit Metab Dis. 2008 Apr;31(2):240-52 - PubMed
  22. Am J Med Genet A. 2011 Jan;155A(1):58-68 - PubMed
  23. PLoS One. 2014 Feb 04;9(2):e84128 - PubMed
  24. J Intellect Disabil Res. 2002 Oct;46(Pt 7):537-47 - PubMed
  25. Chronobiol Int. 1999 Jul;16(4):505-18 - PubMed
  26. J Pineal Res. 2006 Mar;40(2):192-3 - PubMed
  27. Child Care Health Dev. 2001 May;27(3):201-21 - PubMed
  28. BMJ. 2012 Nov 05;345:e6664 - PubMed
  29. Arch Dis Child. 2004 Feb;89(2):125-30 - PubMed
  30. J Intellect Disabil Res. 1996 Dec;40 ( Pt 6):518-28 - PubMed
  31. Neurosci Biobehav Rev. 2010 Apr;34(5):755-68 - PubMed
  32. Dev Med Child Neurol. 1996 Jun;38(6):538-44 - PubMed
  33. Brain Dev. 2010 Feb;32(2):156-61 - PubMed
  34. Pediatr Pulmonol. 2010 Dec;45(12):1205-12 - PubMed
  35. Child Care Health Dev. 2004 Mar;30(2):139-50 - PubMed
  36. Dev Med Child Neurol. 2004 Nov;46(11):776-82 - PubMed
  37. Sleep Med Rev. 2009 Feb;13(1):47-60 - PubMed
  38. J Intellect Disabil Res. 2006 Oct;50(Pt 10):701-10 - PubMed
  39. J Neurodev Disord. 2014;6(1):46 - PubMed
  40. J Pediatr. 2004 May;144(5 Suppl):S27-34 - PubMed
  41. J Biol Rhythms. 1991 Fall;6(3):235-48 - PubMed
  42. Mol Cell Endocrinol. 2006 Jun 27;252(1-2):88-91 - PubMed
  43. Clin Genet. 2002 Nov;62(5):418-21 - PubMed
  44. Behav Brain Res. 2010 Jun 19;209(2):212-20 - PubMed
  45. Epilepsia. 1999 Nov;40(11):1557-65 - PubMed
  46. PLoS One. 2007 Aug 22;2(8):e772 - PubMed
  47. J Sleep Res. 2008 Jun;17(2):197-206 - PubMed
  48. Autism. 2006 Nov;10(6):565-75 - PubMed
  49. Clin Electroencephalogr. 2003 Jan;34(1):18-22 - PubMed
  50. J Pediatr. 2001 Jul;139(1):111-6 - PubMed
  51. Res Dev Disabil. 2009 Nov-Dec;30(6):1512-22 - PubMed
  52. Sleep Med Rev. 2012 Apr;16(2):117-27 - PubMed
  53. Curr Pharm Biotechnol. 2011 Jun;12(6):923-30 - PubMed

Publication Types