Prader-Willi syndrome in a large sample from Spain: general features, obesity and regular use of psychotropic medication.
Large Spanish survey shows behavior issues and psychotropic use are common in PWS, and weight climbs faster without growth-hormone therapy.
01Research in Context
What this study did
A Spanish team mailed surveys to families and clinics. They collected data on 177 people with Prader-Willi syndrome. The survey asked about weight, medicines, and behavior problems.
What they found
Three out of four people showed behavior trouble. Almost half of adults over 30 took psychotropic drugs. People without growth-hormone therapy were heavier.
How this fits with other research
S-Heald et al. (2020) saw the same pattern in Ireland. Both countries find high rates of mood and behavior issues.
H-Hatton et al. (2004) first linked heavier weight to worse behavior in a small case set. The new Spanish data confirm that link in a large national group.
Matson et al. (1999) warned that sleepiness fuels behavior problems. The 2024 study adds obesity and medicine use to the same risk picture.
Why it matters
When you assess a client with PWS, check three things at every visit: weight trend, sleep quality, and current medicines. If growth-hormone therapy stopped, flag weight early. Share the numbers with the medical team so they can adjust food plans or meds before crisis hits.
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02At a glance
03Original abstract
BACKGROUND: Prader-Willi syndrome (PWS), a genetically determined disorder, the most frequent cause of early onset obesity, is associated with physical and cognitive dysfunctions and behavioural disturbances; these disturbances are frequently treated with psychotropic medication. The aim of this cross-sectional study was to describe the characteristics of the first large national sample of persons with PWS in Spain and analyse the relationships of those characteristics with key demographic and clinical factors, particularly with obesity and the regular use of psychotropic medication. METHODS: Participants were recruited among all members of the Spanish Prader-Willi Association who agreed to take part in the study and fulfilled its inclusion criteria. Family and patient demographic features, family size and birth order, intelligence quotient (IQ), anthropometric measures, lifestyle habits, behavioural disturbances (with the Aberrant Behavior Checklist) and clinical data, as well as use of psychotropic drugs and their side effects (with the UKU scale), were collected in genetically confirmed cases of PWS. Bivariate and logistic regression analyses were used for determining the associations of demographic and clinical factors with both obesity and the regular use of psychotropic medication. RESULTS: The cohort included 177 participants (aged 6-48 years), that is, 90 (50.8%) males and 87 (49.2%) females. Behavioural disturbances were present in a range of 75% to 93% of participants; psychotropic medication was prescribed to 81 (45.8%) of them. Number of siblings showed a direct correlation with IQ, especially among males, and inappropriate speech was more intense in only-child females. Obesity was, in parallel, strongly associated with ascending age and with not being currently under growth hormone (GH) treatment. Participants taking any psychotropic medication were characterised by more frequent age ≥30 years, high level of hyperactivity and a psychiatric diagnosis. CONCLUSIONS: Characterisation of persons with PWS in Spain confirms their physical and behavioural phenotype and supports the long-term application of GH therapy and the rational use of psychotropic medication.
Journal of intellectual disability research : JIDR, 2024 · doi:10.1111/jir.13123