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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 of the relationship between ECBI Intensity scoreand age.
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Fig2: Graph of the relationship between ECBI Intensity scoreand age.

Mentions: Figure 2 shows the relationship betweenthe ECBI behaviour intensity score and age. Forthe MPS III group (blue line), the frequency of behavioural problems reduced withage, while for the ID group (green line) the frequency increases. Age and intensity score were significantly negatively correlatedfor children with MPS III (r = -0.639, p = 0.008), but this was non-significant when the outlierwas removed.Figure 2


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 of the relationship between ECBI Intensity scoreand age.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig2: Graph of the relationship between ECBI Intensity scoreand age.
Mentions: Figure 2 shows the relationship betweenthe ECBI behaviour intensity score and age. Forthe MPS III group (blue line), the frequency of behavioural problems reduced withage, while for the ID group (green line) the frequency increases. Age and intensity score were significantly negatively correlatedfor children with MPS III (r = -0.639, p = 0.008), but this was non-significant when the outlierwas removed.Figure 2

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