Assessment & Research

Exploratory study on cognitive abilities and social responsiveness in children with 22q11.2 deletion syndrome (22q11DS) and children with idiopathic intellectual disability (IID).

Van Den Heuvel et al. (2018) · Research in developmental disabilities 2018
★ The Verdict

Kids with 22q11DS can lose social skills while IQ stays put, so keep testing social responsiveness.

✓ Read this if BCBAs working with 22q11DS or genetic syndromes in clinic or school settings.
✗ Skip if Practitioners focused only on adults or mild learning delays.

01Research in Context

01

What this study did

Van Den Heuvel et al. (2018) tracked the kids with 22q11DS and the kids with idiopathic ID. They gave IQ and social tests twice, 19-30 months apart.

The team wanted to see if social skills slid while thinking scores stayed flat.

02

What they found

Both groups had mixed IQ paths. Only the 22q11DS kids lost social ground. Their social-responsiveness scores dropped even when IQ held steady.

Idiopathic ID kids stayed the same on social measures.

03

How this fits with other research

Iversen et al. (2021) meta-analysis links poor executive function to more repetitive behaviors in autism. Ellen’s 22q11DS kids may share that EF-RRB loop, hinting at shared brain pathways.

Gandhi et al. (2022) found Grade 1-2 ASD students scored far worse on teacher EF ratings than peers. Ellen’s data extend this pattern: EF trouble can ride along with social decline in genetic syndromes too.

Matson et al. (2008) gave us MESSIER cut-offs for adults with severe ID. Ellen adds a child lens and shows scores can worsen over time, so one-time testing isn’t enough.

04

Why it matters

If you serve a child with 22q11DS, schedule quick social check-ups every six months even if IQ looks stable. Slide a social-skills probe into routine visits. Catch dips early and add peer-practice or social stories before gaps widen.

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Add a five-item social-responsiveness probe to your next session and plot the score.

02At a glance

Intervention
not applicable
Design
quasi experimental
Sample size
42
Population
intellectual disability, mixed clinical
Finding
mixed
Magnitude
small

03Original abstract

BACKGROUND: Development of cognitive skills and social responsiveness are areas of concern in children with 22q11.2 deletion syndrome (22q11DS). It remains unclear if the cognitive and social profiles and trajectories are syndrome-specific or similar to those of children with idiopathic intellectual disabilities (IID) with or without comorbid autism spectrum disorder (ASD). AIMS AND METHODS: In this exploratory study, we examined and compared five broad cognitive abilities (BCAs) and the social responsiveness in primary school-aged children with 22q11DS (age 6-13, n = 21) and IQ-matched peers with IID (n = 21). The relative strengths and weaknesses of both groups were re-evaluated after 19 to 30 months. OUTCOMES AND RESULTS: Four different cognitive trajectories (i.e. absolute progress, stability, growing into deficit, and absolute decline) were demonstrated in both groups. Most children showed combined types of trajectories across BCAs resulting in a complex changing cognitive profile. In the 22q11DS group, social responsiveness problems increased, whereas no significant change was observed in the IID group. CONCLUSIONS AND IMPLICATIONS: Results reflect similar cognitive and social responsiveness profiles and trajectories across groups with children with 22q11DS being more at risk for growing into a social deficit. We recommend repeated monitoring of social skills development to adapt the environmental demands to the child's individual social capacities.

Research in developmental disabilities, 2018 · doi:10.1016/j.ridd.2018.04.026