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Behavioral problems in relation to intelligence in children with 22q11.2 deletion syndrome: a matched control study
P W Jansen1, S N Duijff, F A Beemer
1Department of Pediatric Psychology, University Medical Centre, Utrecht, The Netherlands.
American Journal of Medical Genetics. Part A
|February 24, 2007
Summary
Children with 22q11.2 deletion syndrome (22q11DS) exhibit more behavioral issues than those with craniofacial anomalies. Notably, intelligence level did not correlate with behavioral problems in 22q11DS, suggesting a distinct behavioral phenotype.
Area of Science:
- Genetics
- Developmental Psychology
- Pediatrics
Background:
- 22q11.2 deletion syndrome (22q11DS) presents with diverse medical and developmental issues.
- Behavioral problems in 22q11DS are debated, with potential links to medical factors or an independent genetic influence.
- Understanding the relationship between intelligence and behavior is crucial for identifying a potential 22q11DS behavioral phenotype.
Purpose of the Study:
- To investigate the association between intelligence level and behavioral problems in children with 22q11DS.
- To compare behavioral problems in children with 22q11DS to those with craniofacial anomalies (CFA) matched for IQ.
- To explore evidence for a distinct behavioral phenotype in 22q11DS independent of intellectual functioning.
Main Methods:
- Compared 69 children with 22q11DS to 69 IQ-matched children with CFA.
- Utilized the Child Behavior Checklist (CBCL) to assess behavioral problems, with norms adjusted for age and sex.
- Analyzed correlations between total IQ scores and CBCL-derived behavioral problem scales within each group.
Main Results:
- Children with 22q11DS displayed significantly more behavioral problems than the CFA group across multiple CBCL subscales.
- No significant correlation was found between IQ score and behavioral problems in the 22q11DS cohort.
- In contrast, intellectual disability predicted behavioral problems in the CFA group.
- Internalizing behaviors were more prevalent in higher-IQ 22q11DS children, while lower-IQ children showed varied problems.
Conclusions:
- The lack of IQ-behavior correlation in 22q11DS tentatively supports a unique behavioral phenotype.
- Behavioral issues in 22q11DS may be intrinsically linked to the genetic deletion, not solely secondary to intellectual deficits.
- Further research is warranted to elucidate the specific genetic and environmental factors contributing to the 22q11DS behavioral phenotype.
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