The British Journal of Psychiatry (2008) 193: 466-470. doi: 10.1192/bjp.bp.107.044370
© 2008 The Royal College of Psychiatrists
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Behavioural phenotype of Cornelia de Lange syndrome: case–control study

Chris Oliver, BSc, MPhil, PhD, Kate Arron, BSc, MPhil, ClinPsyD and Jenny Sloneem, BSc, PhD, DClinPsy

School of Psychology, University of Birmingham, UK

Scott Hall, PhD

Department of Psychiatry and Behavioral Science, Stanford University, USA

Correspondence: Chris Oliver, Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK. Email: c.oliver{at}bham.ac.uk

Declaration of interest

None. Funding detailed in Acknowledgements.

Background

Cornelia de Lange syndrome is associated with abnormalities on chromosomes 5, 10 and X.

Aims

To delineate the behavioural phenotype of Cornelia de Lange syndrome with specific reference to autistic-spectrum disorder.

Method

A total of 54 individuals with Cornelia de Lange syndrome (mean age 13.88 years; s.d.=8.58) and 46 comparable individuals with intellectual disability (mean age 13.74 years; s.d.=7.99) were assessed on measures of autistic-spectrum disorder, and adaptive, compulsive and disordered behaviour.

Results

There was no difference between the groups in global behaviour disorder. Severe autism was significantly more prevalent in the syndrome group (32.1%) than the comparison group (7.1%). In addition, the syndrome group also evidenced significantly higher levels of compulsive behaviour.

Conclusions

These data suggest that autistic-spectrum disorder is part of the behavioural phenotype of Cornelia de Lange syndrome and that compulsive behaviours are evident. Future research should investigate this behavioural phenotype using contemporary diagnostic algorithms for autism with detailed examination of the phenomenology of compulsive behaviours.


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