Assessment & Research

The neuropsychology of 22q11 deletion syndrome. A neuropsychiatric study of 100 individuals.

Niklasson et al. (2010) · Research in developmental disabilities 2010
★ The Verdict

In 22q11DS, visuo-motor and IQ delays are core, but poor planning signals added autism or ADHD and needs its own program.

✓ Read this if BCBAs assessing or treating children or adults with 22q11 deletion syndrome.
✗ Skip if Clinicians who work only with high-IQ, neurotypical clients.

01Research in Context

01

What this study did

Niklasson et al. (2010) mapped the minds of 100 people with 22q11 deletion syndrome.

They gave a full IQ battery and tests of visuo-motor skill, planning, and memory.

The goal was to see which problems come from the syndrome itself and which ride along with extra diagnoses like autism or ADHD.

02

What they found

Average IQ sat at 71. Visuo-motor gaps showed up in almost everyone.

Poor planning appeared only when autism or ADHD was also present.

In short, expect slow hands and low scores across the board, but target executive skills only when comorbid labels are in the file.

03

How this fits with other research

Van Den Heuvel et al. (2018) followed kids for two years and found social skills slid even when IQ stayed flat. Together the papers warn that cognitive scores can hide growing social trouble.

Wong et al. (2009) linked poor scores on a card-sort test to early signs of psychosis. Their finding joins Lena’s planning data to flag executive tasks as both assessment and early-warning tools.

Whitehouse et al. (2014) looked at adults with even lower IQs and saw more psychopathology. The work extends Lena’s map into older, more impaired clients and hints that IQ level may shape mental-health risk.

04

Why it matters

When you meet a client with 22q11DS, start with visuo-motor and IQ baselines. If autism or ADHD is also on the sheet, add executive-function probes and social-skills checks. Re-test social domains yearly even if IQ looks stable, and watch card-sort scores for early red flags of psychosis. These steps turn a single snapshot into a living treatment plan.

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

Run a quick card-sort or tower test; if scores are low and the client has ASD/ADHD, add an executive-function goal to the plan.

02At a glance

Intervention
not applicable
Design
case series
Sample size
100
Population
developmental delay, autism spectrum disorder, adhd
Finding
not reported

03Original abstract

The primary objective of this study was to study the impact of ASD/ADHD on general intellectual ability and profile, executive functions and visuo-motor skills in children and adults with 22q11 deletion syndrome (22q11DS). A secondary aim was to study if gender, age, heart disease, ASD, ADHD or ASD in combination with ADHD had an impact on general intellectual ability and profile. One hundred consecutively referred individuals aged 1-35 years with 22q11DS were given in-depth neuropsychological assessments. Mean full scale IQ was 71 with a normal distribution around this mean. Higher IQ for females than males, and a negative trend for IQ with higher age were found. Intellectual impairment, as well as visuo-motor dysfunction, was found to be related to 22q11DS per se and not to ASD/ADHD. In the area of executive function, the presence of ASD/ADHD predicted poor planning ability in the children in the study.

Research in developmental disabilities, 2010 · doi:10.1016/j.ridd.2009.09.001