Assessment & Research

Intellectual abilities in a large sample of children with Velo-Cardio-Facial Syndrome: an update.

De Smedt et al. (2007) · Journal of intellectual disability research : JIDR 2007
★ The Verdict

In VCFS, expect IQ around 73 and watch for lower scores when the deletion is inherited from a parent.

✓ Read this if BCBAs writing cognitive-academic plans for kids with 22q11DS in school or clinic settings.
✗ Skip if Clinicians who only serve adults or clients without genetic syndromes.

01Research in Context

01

What this study did

The team tested IQ in 103 children with Velo-Cardio-Facial Syndrome.

They also asked whether the gene deletion came from a parent or happened on its own.

02

What they found

The average IQ was 73, in the mild disability range.

Kids who inherited the deletion from a parent scored lower than kids with a new deletion.

03

How this fits with other research

Duijff et al. (2012) looked at five-year-olds with the same syndrome and found visual-perception gaps, not motor issues, pulled IQ down.

Van Aken et al. (2010) showed that even when IQ is matched, children with 22q11DS still move their hands more slowly on tracking tasks.

Lancioni et al. (2000) used the same parent-vs-new comparison in Prader-Willi syndrome and also saw genetic subtype change verbal IQ.

04

Why it matters

When you see a child with VCFS, plan for IQ near 70 and check if the deletion is familial; those kids may need heavier academic supports. Add a quick visual-perception screen, because that slice of IQ can be trained.

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→ Action — try this Monday

Open the last VCFS report; if it says "familial deletion," add extra visual-perception and verbal comprehension goals to the plan.

02At a glance

Intervention
not applicable
Design
case series
Sample size
103
Population
other
Finding
not reported

03Original abstract

BACKGROUND: Learning disabilities are one of the most consistently reported features in Velo-Cardio-Facial Syndrome (VCFS). Earlier reports on IQ in children with VCFS were, however, limited by small sample sizes and ascertainment biases. The aim of the present study was therefore to replicate these earlier findings and to investigate intellectual abilities in a large sample of children with VCFS. In addition, we aimed to identify factors that may contribute to within-syndrome variability in cognitive performance, such as the mode of inheritance of the deletion, sex, the presence of a heart defect and psychiatric morbidity. METHOD: IQ data of 103 children with VCFS (56 males, 47 females) were collected. Psychiatric diagnosis was additionally recorded. RESULTS: Children with VCFS had a mean full-scale IQ (FSIQ) of 73.48 (range: 50-109). There were no effects of sex, presence of a heart defect and psychiatric condition on intellectual profile. Inheritance of the deletion affected cognitive performance in VCFS, with children with familial deletions having significant lower FSIQ than children with a de novo deletion. CONCLUSIONS: Learning disabilities are very common in children with VCFS, although marked within syndrome variability is noted. One factor contributing to this variability seems to be the mode of inheritance of the deletion.

Journal of intellectual disability research : JIDR, 2007 · doi:10.1111/j.1365-2788.2007.00955.x