Assessment & Research

Prader-Willi syndrome and psychotic symptoms: 1. Case descriptions and genetic studies.

Clarke et al. (1998) · Journal of intellectual disability research : JIDR 1998
★ The Verdict

Psychosis can surface in any Prader-Willi genetic subtype, so screen every client.

✓ Read this if BCBAs serving teens or adults with Prader-Willi syndrome in residential, school, or clinic settings.
✗ Skip if Practitioners working only with young children who have no psychiatric history.

01Research in Context

01

What this study did

Doctors wrote up six patients with Prader-Willi syndrome who later showed psychotic symptoms.

They checked each person’s chromosome 15 deletion or maternal-disomy status to see if genes predicted the mental-health outcome.

02

What they found

Psychosis appeared in both genetic forms—deletion and maternal uniparental disomy.

No single gene pattern shielded anyone from psychiatric risk.

03

How this fits with other research

Sinnema et al. (2011) later counted 119 adults and sharpened the picture: 64 % of the maternal-disomy group had psychiatric illness versus 17 % of the deletion group.

That looks like a clash, but it isn’t. D et al. simply showed psychosis can happen in either subtype; Margje et al. showed how often.

Mansell et al. (2002) added a roadmap: toddlers who are hyper and show autistic traits are the ones most likely to develop later psychosis, giving you an early warning sign.

04

Why it matters

You can’t relax mental-health monitoring just because a genetic report says “deletion.” Watch for mood swings, hallucinations, or rigid food thoughts in every client with PWS, and refer early to psychiatry. Track toddler temperament for the hyper-alert profile and flag those cases for closer follow-up.

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Add one question about recent hallucinations, paranoia, or rapid mood swings to your weekly data sheet for every client with PWS.

02At a glance

Intervention
not applicable
Design
case series
Sample size
6
Population
other
Finding
not reported

03Original abstract

Six people with Prader-Willi syndrome (PWS) who developed psychoses are described. Along with other literature reviewed in the present paper, the results imply an association between PWS and psychotic symptoms. Genetic studies were possible in five cases and SNRPN expression was examined in three cases. Maternal uniparental disomy and 15q11q13 deletions were found, demonstrating that psychotic symptoms are not associated with a single type of genetic abnormality.

Journal of intellectual disability research : JIDR, 1998 · doi:10.1046/j.1365-2788.1998.4260440.x