title: Psychopathology and cognition in children with 22q11.2 deletion syndrome.
abstract: BACKGROUND
  Children with 22q11.2 deletion syndrome (22q11.2DS) have been reported to have high rates of cognitive and psychiatric problems.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: Maria Niarchou, MSc, Stanley Zammit, MRCPsych, PhD, Institute of Psychological Medicine and Clinical Neurosciences, MRC Centre for Neuropsychiatric Genetics and Genomics, Cardiff University; Stephanie H. M. van Goozen, PhD, School of Psychology, Cardiff University; Anita Thapar, FRCPsych, PhD, Hayley M. Tierling, BSc, Michael J. Owen, FRCPsych, PhD, Marianne B. M. van den Bree, PhD, Institute of Psychological Medicine and Clinical Neurosciences, MRC Centre for Neuropsychiatric Genetics and Genomics, Cardiff University, Cardiff, UK.
authors.forename: Maria
authors.initials: M
authors.lastname: Niarchou
authors.forename: Stanley
authors.initials: S
authors.lastname: Zammit
authors.forename: Stephanie H M
authors.initials: SH
authors.lastname: van Goozen
authors.forename: Anita
authors.initials: A
authors.lastname: Thapar
authors.forename: Hayley M
authors.initials: HM
authors.lastname: Tierling
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Owen
authors.forename: Marianne B M
authors.initials: MB
authors.lastname: van den Bree
mesh_terms: D058165:22q11 Deletion Syndrome / Q000453:epidemiology* / Q000235:genetics / Q000523:psychology; D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D015897:Comorbidity; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000453:epidemiology* / Q000235:genetics; D007361:Intelligence Tests / Q000706:statistics & numerical data; D007406:Interview, Psychological; D008297:Male; D001523:Mental Disorders / Q000453:epidemiology* / Q000235:genetics; D015233:Models, Statistical; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D015995:Prevalence; D011599:Psychopathology; D035781:Siblings