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A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes

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Abstract

Unique socio-behavioural phenotypes are reported for individuals with different neurodevelopmental disorders. Here, the effects of adult familiarity and nature of interaction on social anxiety and social motivation were investigated in individuals with fragile X (FXS; n = 20), Cornelia de Lange (CdLS; n = 20) and Rubinstein-Taybi (RTS; n = 20) syndromes, compared to individuals with Down syndrome (DS; n = 20). The Social Anxiety and Motivation Rating Scale was employed whilst participants completed four social tasks, each administered separately by a familiar adult, and also by an unfamiliar adult. Compared to participants with DS, those with FXS and RTS exhibited high levels of social anxiety but similar levels of social motivation. Participants with CdLS showed heightened social anxiety and reduced social motivation only during interactions with an unfamiliar adult when active participation was voluntary.

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Acknowledgments

The authors would like to thank all participants and their families. The authors are indebted to the Fragile X Society, the Cornelia de Lange Foundation UK & Ireland, and the Rubinstein-Taybi Syndrome Support Group for their assistance with recruitment of participants.

Funding

This study was funded by a grant from the Economic and Social Research council (Grant Number: ES/I901825/1) and by Cerebra.

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Author notes

  1. Hayley Crawford

    Present address: Faculty of Health and Life Sciences, Coventry University, Richard Crossman Fourth Floor, Priory Street, Coventry, CV1 5FB, UK

  2. Robyn Dowlen

    Present address: School of Nursing, Midwifery and Social Work, The University of Manchester, Manchester, UK

  3. Lisa Nelson

    Present address: Derby Royal Hospital, Derby, UK

Authors and Affiliations

  1. Cerebra Centre for Neurodevelopmental Disorders, University of Birmingham, Edgbaston, UK

    Hayley Crawford

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

    Joanna Moss, Laura Groves, Robyn Dowlen, Lisa Nelson, Donna Reid & Chris Oliver

  3. Institute of Cognitive Neuroscience, University College London, 17 Queen Square, London, WC1N 3AR, UK

    Joanna Moss

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Contributions

HC was involved in study design, data collection, data analysis, data interpretation and drafting of the manuscript. JM was involved in study design and editing the manuscript. LG was involved in participant recruitment, data collection and data coding. RD was involved in participant recruitment and data collection. LN was involved in task development, and recruitment and data collection of the CdLS and DS participant groups. DR was involved in recruitment and data collection of the CdLS and DS participant groups. CO was involved in study design, data analysis, data interpretation and editing of the manuscript.

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Correspondence to Hayley Crawford.

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

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Informed consent was obtained from all individual participants included in the study. Participants aged 16 and above provided written consent to participate. Parents of children aged below 16 years provided written consent on behalf of their children.

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Crawford, H., Moss, J., Groves, L. et al. A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes. J Autism Dev Disord 50, 127–144 (2020). https://doi.org/10.1007/s10803-019-04232-5

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