Key result
22q11.2 deletion syndrome is linked to ~32-point lower IQ compared to sibling controls.
Case-Control (n=119)
Absolute Event Rate: 76.8% vs 108.6%
p-value: p=<0.001
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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Niarchou et al. (2013) conducted a case-control in 22q11.2 deletion syndrome (n=119). 22q11.2 deletion syndrome vs. Sibling controls was evaluated on IQ (p=<0.001). 22q11.2 deletion syndrome was associated with a 54% prevalence of psychiatric disorders and significantly lower IQ (mean 76.8 vs 108.6, P<0.001) compared to sibling controls.
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