Assessment & Research

Behavioural and emotional disturbances in people with Prader-Willi Syndrome.

Steinhausen et al. (2004) · Journal of intellectual disability research : JIDR 2004
★ The Verdict

Rising BMI in teens and young adults with Prader-Willi syndrome is a red flag for escalating behavior problems.

✓ Read this if BCBAs writing plans for adolescents or adults with PWS in residential, day-program, or home settings.
✗ Skip if Clinicians who only serve preschoolers or clients without genetic obesity syndromes.

01Research in Context

01

What this study did

H-Hatton et al. (2004) asked parents to fill out a behavior checklist about their sons and daughters with Prader-Willi syndrome.

The group ranged from teens to young adults. The team looked at whether BMI, sex, IQ, or genetic subtype predicted more behavior trouble.

02

What they found

Parents reported more tantrums, mood swings, and compulsions as their kids got older.

Heavier BMI went hand-in-hand with worse behavior scores. Gender, IQ, and genetic subtype made no clear difference.

03

How this fits with other research

Fullana et al. (2007) later used matched groups and found the same climb in psychopathology, backing up the 2004 picture.

S-Heald et al. (2020) widened the lens to a whole country and showed over half of Irish people with PWS now carry a psychiatric diagnosis, extending the warning.

Matson et al. (1999) looked earlier and added sleep: daytime sleepiness also fuels behavior issues, so poor sleep may be one hidden driver behind the 2004 spike.

04

Why it matters

Expect behavior plans to lose power if weight creeps up during adolescence. Track BMI at every clinic visit and pair nutrition goals with behavior goals. Add a sleep screen—fixing apnea or sleep quality may calm daytime outbursts without extra drugs.

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Graph your client’s weight and behavior data on the same sheet—if both lines climb together, call the dietitian and sleep team today.

02At a glance

Intervention
not applicable
Design
case series
Sample size
58
Population
other
Finding
not reported

03Original abstract

BACKGROUND: The study of the behaviour profile in subjects with Prader-Willi Syndrome (PWS). METHODS: A total of fifty-eight 3- to 29-year-old subjects with PWS were studied using a standardized parent report of behavioural and emotional disturbances. RESULTS: There was an increase of behavioural and emotional disturbances for the adolescent and young adult age range, whereas gender and intelligence were not significant. Increasing body mass index (BMI) was also associated with more behavioural and emotional disturbances. There was no significant relation between genetic status and behavioural abnormalities. CONCLUSIONS: This systematic study supports single case observations of a heightened psychiatric vulnerability of adolescent and young adult PWS subjects.

Journal of intellectual disability research : JIDR, 2004 · doi:10.1111/j.1365-2788.2004.00582.x