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Brief Report: Contrasting Profiles of Everyday Executive Functioning in Smith–Magenis Syndrome and Down Syndrome

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Abstract

Everyday executive function (EF) was examined in Smith–Magenis syndrome (SMS), associated with high risk of behaviour disorder, and Down syndrome (DS), associated with relatively low risk of behaviour disorder. Caregivers of 13 children with SMS and 17 with DS rated everyday EF using the Behavioral Rating Inventory of Executive Functioning-Preschool. Greater everyday EF deficits relative to adaptive ability were evident in SMS than in DS. The SMS profile of everyday EF abilities was relatively uniform; in DS emotional control strengths and working memory weaknesses were evident. Findings implicate broad everyday EF difficulties in SMS compared to DS, corresponding with increased rates of behaviour disorder in SMS. Findings further suggest that everyday EF profiles may, in part, be syndrome related.

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Fig. 1

Notes

  1. This difference was also significant in Mann–Whitney analysis, U = 17.5, p < .001.

  2. This difference was also significant in a Wilcoxon analysis Z = −2.54, p = .011.

  3. These differences were also significant in a Wilcoxon analysis; Inhibit Z = −3.43, p = .001, Plan/Organize Z = −3.32, p = .001 and Working Memory Z = −3.62, p < .001 but not Shift Z = −2.38, p = .017.

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Acknowledgments

The research reported here was supported by funding from Cerebra. The authors would like to thank all participants and their families. The authors are indebted to the Smith-Magenis Foundation UK and the Down’s Syndrome Association for their assistance with recruitment of children with Smith–Magenis syndrome and Down syndrome, respectively.

Author Contributions

LW conceived of the study, participated in its design and coordination, performed the statistical analyses, participated in the interpretation of the data, and drafted the manuscript. CO conceived of the study, participated in its design and interpretation of the data, and helped to draft the manuscript. Both authors read and approved the final manuscript.

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  1. Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, B15 2TT, UK

    Lucy Wilde & Chris Oliver

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  1. Lucy Wilde
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Correspondence to Lucy Wilde.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Wilde, L., Oliver, C. Brief Report: Contrasting Profiles of Everyday Executive Functioning in Smith–Magenis Syndrome and Down Syndrome. J Autism Dev Disord 47, 2602–2609 (2017). https://doi.org/10.1007/s10803-017-3140-2

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