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An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.

Cross EM, Grant S, Jones S, Bigger BW, Wraith JE, Mahon LV, Lomax M, Hare DJ - J Neurodev Disord (2014)

Bottom Line: 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.The frequency of challenging behaviours displayed by children with MPS III and their adaptive skills was found to decrease with age.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.

View Article: PubMed Central - PubMed

Affiliation: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.

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.

No MeSH data available.


Related in: MedlinePlus

Graph showing the relationship between age anddisability score.
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Fig1: Graph showing the relationship between age anddisability score.

Mentions: As seen in Figure 1, there was anoutlier in the MPS III group with a high level of skills aged 11 years. Subsequentanalyses were conducted both with and without this outlier, but the latter are onlyreported if these differed from those conducted with the whole dataset. Skillsincreased with age for the ID group (green line) but decreased with age for the MPSIII group (blue line), with LDCS A score being significantly correlated with age inthe MPS III group (r = 0.728, p = 0.01).Figure 1


An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.

Cross EM, Grant S, Jones S, Bigger BW, Wraith JE, Mahon LV, Lomax M, Hare DJ - J Neurodev Disord (2014)

Graph showing the relationship between age anddisability score.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4318134&req=5

Fig1: Graph showing the relationship between age anddisability score.
Mentions: As seen in Figure 1, there was anoutlier in the MPS III group with a high level of skills aged 11 years. Subsequentanalyses were conducted both with and without this outlier, but the latter are onlyreported if these differed from those conducted with the whole dataset. Skillsincreased with age for the ID group (green line) but decreased with age for the MPSIII group (blue line), with LDCS A score being significantly correlated with age inthe MPS III group (r = 0.728, p = 0.01).Figure 1

Bottom Line: 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.The frequency of challenging behaviours displayed by children with MPS III and their adaptive skills was found to decrease with age.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.

View Article: PubMed Central - PubMed

Affiliation: Section for Clinical and Health Psychology, School of Psychological Sciences, University of Manchester, Zochonis Building, Brunswick Street, Manchester, M13 9PL UK.

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.

No MeSH data available.


Related in: MedlinePlus