title: Assessment of sleep in children with mucopolysaccharidosis type III.
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.
authors: Division of Clinical Psychology, University of Manchester, Manchester, United Kingdom.
authors: Louise Victoria
authors: LV
authors: Mahon
authors: Division of Clinical Psychology, University of Manchester, Manchester, United Kingdom.
authors: Michelle
authors: M
authors: Lomax
authors: Division of Clinical Psychology, University of Manchester, Manchester, United Kingdom.
authors: Sheena
authors: S
authors: Grant
authors: Division of Clinical Psychology, University of Manchester, Manchester, United Kingdom.
authors: Elaine
authors: E
authors: Cross
authors: Division of Clinical Psychology, University of Manchester, Manchester, United Kingdom.
authors: Dougal Julian
authors: DJ
authors: Hare
authors: Genetic Medicine, St. Mary's Hospital, Manchester, United Kingdom.
authors: James Ed
authors: JE
authors: Wraith
authors: Genetic Medicine, St. Mary's Hospital, Manchester, United Kingdom.
authors: Simon
authors: S
authors: Jones
authors: Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom.
authors: Brian
authors: B
authors: Bigger
authors: Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom.
authors: Kia
authors: K
authors: Langford-Smith
authors: Faculty of Life Sciences, University of Manchester, Manchester, United Kingdom.
authors: Maria
authors: M
authors: Canal
mesh_terms: D056044:Actigraphy; D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008550:Melatonin / Q000378:metabolism; D009084:Mucopolysaccharidosis III / Q000503:physiopathology*; D012890:Sleep / Q000502:physiology*; D013997:Time Factors
chemical_list: D008550:Melatonin