title: An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.
abstract: BACKGROUND
  Mucopolysaccharidosis type-III (MPS III) is an autosomal recessive lysosomal storage disorder. It causes progressive physical and cognitive decline and has been linked to increased incidences of behavioural problems.
  
  
  METHODS
  Data on the behaviour and adaptive skills of 20 children with MPS III and 25 children with intellectual disability (ID) (17 included in analysis) were gathered via parental report questionnaire. The frequencies of different types of behaviour displayed by children with MPS III and children with ID were compared across two age categories.
  
  
  RESULTS
  The total frequency of challenging behaviours displayed by children aged 2-9 years with MPS III and ID was not significantly different. Behaviours associated with hyperactivity, orality, unusual body movements and inattention were seen significantly more frequently in 2-9 year olds with MPS III than in those with ID. Children aged 10-15 years with MPS III showed significantly fewer problem behaviours than a contrasting group with ID. The frequency of challenging behaviours displayed by children with MPS III and their adaptive skills was found to decrease with age.
  
  
  CONCLUSIONS
  Behaviours relating to hyperactivity, orality, unusual body movements and inattention are part of the behavioural phenotype of the middle phase of MPS III. The late phase of MPS III is associated with low rates of problem behaviour and loss of adaptive skills. Therefore, families with a child with MPS III may benefit from a different type of clinical service when the child is aged 2-9 years, than when aged 10-15 years.
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: Elaine M
authors: EM
authors: Cross
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: Sheena
authors: S
authors: Grant
authors: Department of Genetic Medicine, St Mary's Hospital, Manchester, UK.
authors: Simon
authors: S
authors: Jones
authors: Stem Cell & Neurotherapies, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
authors: Brian W
authors: BW
authors: Bigger
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: James E
authors: JE
authors: Wraith
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: Louise V
authors: LV
authors: Mahon
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: Michelle
authors: M
authors: Lomax
authors: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.
authors: Dougal J
authors: DJ
authors: Hare
keywords: Behavioural phenotype; MPS III; Mucopolysaccharidosis; Sanfilippo syndrome
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