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

Cross, Elaine M., Grant, Sheena, Jones, Simon, Bigger, Brian W., Wraith, James E., Mahon, Louise V., Lomax, Michelle and Hare, Dougal Julian 2014. An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III. Journal of Neurodevelopmental Disorders 6 (1) , 46. 10.1186/1866-1955-6-46

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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.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Psychology
Subjects:	R Medicine > RC Internal medicine > RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry
Additional Information:	2014 Cross et al.; licensee BioMed Central. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Publisher:	BioMed Central
ISSN:	1866-1955
Date of First Compliant Deposit:	30 March 2016
Date of Acceptance:	10 December 2014
Last Modified:	07 May 2023 09:05
URI:	https://orca.cardiff.ac.uk/id/eprint/80969

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