Assessment & Research

Psychopathology in adults with 22q11 deletion syndrome and moderate and severe intellectual disability.

Evers et al. (2014) · Journal of intellectual disability research : JIDR 2014
★ The Verdict

Adults with 22q11DS plus moderate or severe ID carry a high load of mental illness—watch for psychosis when you see cognitive decline.

✓ Read this if BCBAs working with adults who have 22q11 deletion syndrome and low IQ.
✗ Skip if Clinicians who serve only children or clients without genetic syndromes.

01Research in Context

01

What this study did

Doctors looked at adults who have 22q11 deletion syndrome and a low IQ.

They wanted to know how many of these adults also have mental health problems.

The team wrote down every diagnosis they found in the medical files.

02

What they found

Almost all of the adults had at least one mental health condition.

Psychosis showed up most often in people whose IQ had dropped over time.

The study says to watch for warning signs of psychosis in this group.

03

How this fits with other research

Niklasson et al. (2010) mapped the general 22q11DS mind, showing most people have an IQ near 71.

Whitehouse et al. (2014) zoomed in on the lower end of that range and found heavy psychiatric load.

Wong et al. (2009) already linked poor card-sort scores to early psychosis in teens with 22q11DS.

The new adult data match that pattern: worse thinking plus age equals higher psychosis risk.

Wilde et al. (2017) saw similar high rates of self-injury and aggression in adults with TSC plus ID.

Both rare-syndrome studies tell us to screen for serious behavior and mood issues.

04

Why it matters

If you serve adults with 22q11DS and IQ below 55, add a brief psychosis screen to every visit.

Ask about hallucinations, delusions, or sudden drops in daily skills.

Early flags let you refer for psychiatry before crisis hits.

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Add one question to your intake: “Any new trouble telling real from unreal?” and note if IQ has slipped.

02At a glance

Intervention
not applicable
Design
case series
Sample size
33
Population
intellectual disability, other
Finding
not reported

03Original abstract

BACKGROUND: 22q11 deletion syndrome (22q11DS) is associated with mild or borderline intellectual disability (ID). There are hardly any reports on subjects with 22q11DS with moderate or severe ID, and therefore its behavioural and psychiatric characteristics are unknown. METHOD: We describe behavioural and psychiatric characteristics of 33 adults with 22q11DS and a Full-Scale IQ (FSIQ) below 55. Participants were divided into two groups: one group having a FSIQ ≤ 55 caused by intellectual decline (n = 21) and one group with a FSIQ ≤ 55 who had always functioned at this level (n = 12). RESULTS: High scores on psychopathology sub-scales were found for both subgroups. 22q11DS patients with intellectual decline showed higher rates of co-morbid psychopathology, particularly psychosis. Furthermore, psychosis and intellectual decline were positive correlated. CONCLUSION: This is the first report addressing adult patients with 22q11DS and moderate to severe ID. Overall we found high levels of psychopathology with higher scores of psychopathology in the intellectual decline group. Life time psychosis seems to be related to deterioration.

Journal of intellectual disability research : JIDR, 2014 · doi:10.1111/jir.12117