Assessment & Research

Cognitive and behavioral trajectories in 22q11DS from childhood into adolescence: a prospective 6-year follow-up study.

Duijff et al. (2013) · Research in developmental disabilities 2013
★ The Verdict

In 22q11DS, cognitive decline and behavioral problems emerge independently—track both, not just IQ.

✓ Read this if BCBAs working with 22q11DS in clinic or school settings
✗ Skip if Practitioners who only serve clients with ASD or no genetic diagnoses

01Research in Context

01

What this study did

The team tracked kids with 22q11.2 deletion syndrome for six years. They started in childhood and checked again in adolescence.

Each visit gave IQ tests and behavior checklists. No treatment was tested; they just watched change over time.

02

What they found

About one-third lost a lot of IQ points. At the same time, worry and sadness went up, but rule-breaking and aggression went down.

Surprise: the kids who lost IQ did not automatically show more behavior problems. The two paths moved on their own.

03

How this fits with other research

Marchese et al. (2012) saw the same kids at one time point and found poor social skills linked to anxiety, not IQ. The new study agrees: behavior and cognition split apart.

Raspail et al. (2025) show that in mild ID, bad inhibition predicts social cue errors. N et al. find no link at all in 22q11DS, hinting the syndrome plays by different rules.

Whitehouse et al. (2014) warn that even autistic adults with higher IQ can struggle with daily life. The IQ drop seen here gives the same warning for 22q11DS: plan for life skills, not just test scores.

04

Why it matters

If you serve a child with 22q11DS, watch both lines on the graph. A stable IQ does not promise stable mood, and falling scores do not guarantee worse behavior. Run separate probes: yearly IQ plus frequent anxiety and social checks. Build goals for daily living now, before the teen years hit.

Free CEUs

Want CEUs on This Topic?

The ABA Clubhouse has 60+ free CEUs — live every Wednesday. Ethics, supervision & clinical topics.

Join Free →
→ Action — try this Monday

Add a quick anxiety rating to your next session even if the child's IQ scores look fine.

02At a glance

Intervention
not applicable
Design
pre post no control
Sample size
53
Population
other
Finding
mixed
Magnitude
medium

03Original abstract

Patients with 22q11DS are at risk of behavioral problems and cognitive impairment. Recent studies suggest a possible intellectual decline in 22q11DS children. To date it is unknown if cognitive development is related to the behavioral problems in 22q11DS. We studied 53 children with 22q11DS who underwent cognitive and behavioral assessments at 9.5 years (T1) and 15.3 years (T2). In about one third, IQ data obtained at 7.5 years (T0) were also available. Results showed that internalizing behaviors intensified while externalizing behaviors decreased. Simultaneously, in about a third a significant decline in IQ was found, which, surprisingly, was unrelated to the behavioral changes. It can be concluded that children with 22q11DS follow a unique developmental trajectory. Cognitive deterioration is severe in some but does not appear to predict behavioral problems in early adolescence.

Research in developmental disabilities, 2013 · doi:10.1016/j.ridd.2013.06.001