The online version of this article (doi:10.1186/1866-1955-6-46) contains supplementary material, which is available to authorized users.
The authors declare they have no competing interests.
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
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.
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.
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.
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.
Héron B, Mikaeloff Y, Froissart R, Caridade G, Maire I, Caillaud C, Levade T, Chabrol B, Feillet F, Ogier H, Valayannopoulos V, Michelakakis H, Zafeiriou D, Lavery L, Wraith E, Danos O, Heard J-M, Tardieu M: Incidence and natural history of mucopolysaccharidosis type III in France and comparison with United Kingdom and Greece. Am J Med Genet. 2010, 155A (1): 58-68.
Valstar MJ, Neijs S, Bruggenwirth HT, Olmer R, Ruijter GJG, Wevers RA, van Diggelen OP, Poorthuis BJ, Halley DJ, Wijburg FA: Mucopolysaccharidosis type IIIA: clinical spectrum and genotype-phenotype correlations. Am Neurol Assoc. 2010, 68: 876-887. 10.1002/ana.22092. CrossRef
Nidiffer F, Kelly T: Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study. J Ment Defic Res. 1983, 27 (3): 185-203. PubMed
Mahon L, Lomax M, Grant S, Cross E, Jones S, Wraith E, Bigger B, Canal M, Hare DJ: Actigraphic assessment of sleep in children with mucopolysaccharidosis Type III. PLoS One. 2014, 9 (2): 1-7. CrossRef
Grant S, Cross E, Jones S, Wraith E, Bigger B, Hare DJ: Parental stress, coping, resilience, anxiety, depression, and social support in parents with a child with Mucopolysaccharidosis Type III (MPS III, Sanfilippo syndrome) and parents of children with intellectual disabilities (I.D.). J Inherit Metab Dis. 2013, 36: 281-291. 10.1007/s10545-012-9558-y. PubMedCrossRef
Pendaries C: Pilot study on the development of the learning disability Healthcare Resource Groups. Br J Learn Disabil. 1997, 25 (3): 122-126. 10.1111/j.1468-3156.1997.tb00024.x. CrossRef
Kushlick A, Blunden R, Cox GR: A method of rating behaviour characteristics for use in large scale surveys of mental handicap. Psychol Med. 1973, 3 (4): 446-478. CrossRef
Sparrow SS, Cicchetti VD, Balla AD: Vineland Adaptive Behavior Scales. 2005, Circle Pines, MN: American Guidance Service, 2
Aman MG, Singh NN, Stewart AW, Field CJ: The Aberrant Behavior Checklist: a behavior rating scale for the assessment of treatment effects. Am J Ment Defic. 1985, 89 (5): 485-491. PubMed
Aman MG, Burrow W, Wolford PL: The Aberrant Behavior Checklist-Community: factor validity and effect of subject variables for adults in group homes. Am J Ment Retard. 1995, 100 (3): 283-292. PubMed
Newton J, Sturmey P: The Aberrant Behaviour Checklist: a British replication and extension of its psychometric properties. J Intellect Disabil Res. 1988, 32 (2): 87-92. CrossRef
Eyberg S, Pincus D: Eyberg Child Behavior Inventory (ECBI). 1999, Odessa FL: Psychological Assessment Resources
Eyberg SM, Ross AW: Assessment of child behavior problems: the validation of a new inventory. J Clin Child Adolesc Psychol. 1978, 7 (2): 113-116. 10.1080/15374417809532835. CrossRef
Burns GL, Patterson DR: Conduct problem behaviors in a stratified random sample of children and adolescents: new standardization data on the Eyberg Child Behavior Inventory. Psychol Assess: J Consult Clin Psychol. 1990, 2 (4): 391- CrossRef
Shapiro E: Personal Correspondence. 2010, University of Minnesota
Nakagawa S: A farewell to Bonferroni: the problems of low statistical power and publication bias. Behav Ecol. 2004, 15: 1044-1045. 10.1093/beheco/arh107. CrossRef
Rosenthal R: Meta-analytic procedures for social research. 1991, Newbury Park, CA: Sage, 2 CrossRef
Rumsey RK, Rudser K, Delaney K, Potegal M, Whitley CB, Shapiro EG: Acquired autistic behaviors in children with mucopolysaccharidosis type IIIA. J Pediatrics. 2014, 164 (5): 1147-1151. e1. 10.1016/j.jpeds.2014.01.007. CrossRef
National Institute for Health and Clinical Excellence: Attention Deficit Hyperactivity Disorder (ADHD). CG72. 2008, London: NICE
- An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III
Elaine M Cross
Brian W Bigger
James E Wraith
Louise V Mahon
Dougal J Hare
- BioMed Central
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