Behavioral phenotype in adults with Prader-Willi syndrome

Margje Sinnema*, Stewart L. Einfeld, Constance T. R. M. Schrander-Stumpel, Marian A. Maaskant, Harm Boer, Leopold M. G. Curfs

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

49 Citations (Web of Science)

Abstract

Prader-Willi syndrome (PWS) is characterized by temper tantrums, impulsivity, mood fluctuations, difficulty with change in routine, skinpicking, stubbornness and aggression. Many studies on behavior in PWS are limited by sample size, age range, a lack of genetically confirmed diagnosis of PWS and inconsistent assessment of behavior. The aim of this study was to explore systematically the relation between behavioral problems and age groups, genetic subtypes and BMI categories in an adult PWS population. Participants were contacted via the Dutch Prader-Willi Parent Association and through physicians specialized in persons with ID. Behaviors were studied using the Developmental Behavior Checklist for Adults (DBC-A). The forms were completed by the main caregivers of 98 adults with a genetically confirmed diagnosis of PWS. Differences between age groups were statistically significant (ANOVA, p = 0.03). DBC-A total scores were higher in the consecutive age groups, with the most behavioral problems in the oldest age groups. Differences between genetic subtypes were also statistically significant (ANOVA, p
Original languageEnglish
Pages (from-to)604-612
JournalResearch in Developmental Disabilities
Volume32
Issue number2
DOIs
Publication statusPublished - 2011

Keywords

  • Prader-Willi syndrome
  • Behavior problems
  • Developmental Behavior Checklist
  • Genetic subtypes
  • Ageing

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