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01.12.2014 | Research | Ausgabe 1/2014 Open Access

Journal of Neurodevelopmental Disorders 1/2014

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

Zeitschrift:
Journal of Neurodevelopmental Disorders > Ausgabe 1/2014
Autoren:
Elaine M Cross, Sheena Grant, Simon Jones, Brian W Bigger, James E Wraith, Louise V Mahon, Michelle Lomax, Dougal J Hare
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1866-1955-6-46) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare they have no competing interests.

Authors’ contributions

Conceived and designed the experiments: EMC, LVM, ML, SG, DJH, JEW and SJ. Performed the experiments: EMC and SG. Analysed the data: EMC. Wrote the paper: EMC and DJH. Recruited participants: EMC, SG, LVM, ML, JEW and SJ. Data entry: EMC. Edited the manuscript: DJH, BWB and EMC. All authors read and approved the final manuscript

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.
Zusatzmaterial
Authors’ original file for figure 1
11689_2013_94_MOESM1_ESM.pdf
Authors’ original file for figure 2
11689_2013_94_MOESM2_ESM.pdf
Literatur
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