Obsessive-compulsive and psychotic-like behaviors across PWS subtypes: Developmental considerations

Funding Summary

Dr. Evans has previously developed a well-validated measure of rigidity and obsessive-compulsive behaviors, as well as behaviors associated with psychosis.  Here, he will work with the Global PWS Registry team to recruit families to complete these assessments three times over a year and see if they are well-suited for the PWS population.  These studies are expected to help identify those at highest risk for challenging behaviors, using a well characterized assessment. 

Lay Abstract

People with Prader-Willi syndrome (PWS) may exhibit a wide range of behaviors including rigid, obsessive-compulsive behaviors, temper tantrums, impulsivity, and unusual thoughts and behaviors. Many of the behaviors associated with PWS change over time, and some behaviors vary depending on the specific genetic subtype of PWS that a person has. In this study, we are trying to get a better understanding of the early risk factors for certain behavioral challenges. The information from this study will help parents and clinicians develop individualized interventions, early in development. Caregivers in the PWS registry will be asked to complete a series of questionnaires. One questionnaire addresses rigid, repetitive, obsessive-compulsive behaviors. The second measure focuses on psychosis-like behaviors such as cognitive disorganization, unusual patterns of thinking and perceiving, and social anxiety. We will also ask caregivers to complete two other brief questionnaires assessing behavioral strengths and challenges. We will compare the different PWS genetic subtypes (paternal deletions [Types I and II] and maternal uniparental disomy) on these measures. We will also examine how these behaviors differ across several age groups (early and middle childhood, adolescence, and adulthood). Because each person is different, it is important to understand how behaviors change over time within each individual. With this in mind, we will follow-up with the families two other times over the course of one year. Individual “growth charts” will be created for each person which will be useful information for developing personalized clinical interventions. In the long term, the information from this study will help identify the earliest signs of challenging behaviors so that they can be addressed as soon as possible. In future work, we hope to continue following up with the families every 6 months on these same measures, so that we can get an even better understanding of how the behaviors change over time. Understanding early risk factors and how they may be impacted by one’s genetic status and personal developmental trajectory will be helpful for caregivers and clinicians as we develop individualized treatment plans for each person.