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Cambridge University Press, British Journal of Psychiatry, 1(204), p. 46-54, 2014

DOI: 10.1192/bjp.bp.113.132324

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

Journal article published in 2013 by Maria Niarchou, Marianne B. M. van den Bree, Stanley Zammit ORCID, Stephanie H. M. van Goozen, Stephanie Helena Maria van Goozen, Anita Thapar ORCID, Hayley M. Tierling, Michael John Owen, Marianne Bernadette van den Bree
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This paper is available in a repository.

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Abstract

BackgroundChildren with 22q11.2 deletion syndrome (22q11.2DS) have been reported to have high rates of cognitive and psychiatric problems.AimsTo 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.MethodNeurocognition 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).ResultsMore 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.Conclusions22q11.2DS 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.