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Psychopathology and cognition in children with 22q11.2 deletion syndrome

  • Maria Niarchou (a1), Stanley Zammit (a1), Stephanie H. M. van Goozen (a2), Anita Thapar (a3), Hayley M. Tierling (a3), Michael J. Owen (a3) and Marianne B. M. Van Den Bree (a3)...
Abstract
Background

Children with 22q11.2 deletion syndrome (22q11.2DS) have been reported to have high rates of cognitive and psychiatric problems.

Aims

To establish the nature and prevalence of psychiatric disorder and neurocognitive impairment in children with 22q11.2DS and test whether risk of psychopathology is mediated by the children's intellectual impairment.

Method

Neurocognition and psychopathology were assessed in 80 children with 22q11.2DS (mean age 10.2 years, s.d. = 2.1) and 39 sibling controls (mean age 10.9 years, s.d. = 2.0).

Results

More than half (54%) of children with 22q11.2DS met diagnostic criteria for one or more DSM-IV-TR psychiatric disorder. These children had lower IQ (mean 76.8, s.d. = 13.0) than controls (mean 108.6, s.d. = 15.2) (P<0.001) and showed a range of neurocognitive impairments. Increased risk of psychopathology was not mediated by intellectual impairment.

Conclusions

22q11.2DS is not related to a specific psychiatric phenotype in children. Moreover, the deletion has largely independent effects on IQ and risk of psychopathology, indicating that psychopathology in 22q11.2DS is not a non-specific consequence of generalised cognitive impairment.

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Copyright
This is an Open Access article, distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives licence (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits noncommercial re-use, distribution, and reproduction in any medium, provided the original work is unaltered and is properly cited. The written permission of Cambridge University Press must be obtained for commercial re-use or in order to create a derivative work.
Corresponding author
Dr Marianne van den Bree, Institute of Psychological Medicine and Clinical Neurosciences, MRC Centre for Neuropsychiatric Genetics and Genomics, Cardiff University, Cathays, Maindy Road, Hadyn Ellis Building, Cardiff CF24 4HQ, UK. Email: vandenbreemb@cf.ac.uk
Footnotes
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This study was funded by the Baily Thomas Charitable Trust, the Waterloo Foundation and the Wellcome Trust.

Declaration of interest

None.

Footnotes
References
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Psychopathology and cognition in children with 22q11.2 deletion syndrome

  • Maria Niarchou (a1), Stanley Zammit (a1), Stephanie H. M. van Goozen (a2), Anita Thapar (a3), Hayley M. Tierling (a3), Michael J. Owen (a3) and Marianne B. M. Van Den Bree (a3)...
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