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Attention challenges in Kabuki syndrome.
A J Kalinousky1, T Rapp2, J R Harris1,3
1McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Journal of Intellectual Disability Research : JIDR
|November 3, 2023
Summary
Children with Kabuki syndrome (KS) show significantly elevated attention problems, impacting adaptive function. Clinicians should screen for attention deficit hyperactivity disorder (ADHD) in these children.
Area of Science:
- Neurodevelopmental disorders
- Rare genetic diseases
- Behavioral genetics
Background:
- Kabuki syndrome (KS) is a rare genetic disorder associated with intellectual disability and neurodevelopmental issues.
- Previous research indicated attentional deficits in some individuals with KS, but studies were limited by small sample sizes and retrospective data.
- Standardized assessments are crucial for accurately characterizing neurobehavioral phenotypes in rare genetic conditions.
Purpose of the Study:
- To investigate the neurobehavioral profile and adaptive functioning in individuals with molecularly confirmed Kabuki syndrome.
- To compare behavioral assessments of children with KS to their unaffected biological siblings.
- To identify specific behavioral challenges, such as attention problems, and their correlation with adaptive function in KS.
Main Methods:
- Fifty-five individuals with molecularly confirmed KS and 23 unaffected siblings underwent standardized behavioral and adaptive function assessments.
- Caregiver reports were utilized for individuals with KS.
- Data analysis focused on comparing behavioral categories between the KS group and the control group, and examining correlations between attention and adaptive function.
Main Results:
- Attention Problems emerged as the only clinically significant behavioral category when averaged across all children with KS.
- Nearly 50% of children with KS exhibited scores in the problematic range for attention.
- Children with KS scored significantly higher on nearly all behavioral categories compared to their siblings, with a notable negative correlation between attention problems and adaptive function.
Conclusions:
- Attentional deficits are significantly more prevalent in children with Kabuki syndrome than in the general population.
- Attention challenges in KS are negatively correlated with adaptive function, independent of general cognitive ability.
- Routine screening for attention deficit hyperactivity disorder (ADHD) is recommended for children with Kabuki syndrome to improve care and support.
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