Relationship between Aberrant Behavior Checklist (ABC) scores, hyperphagia, and quality of life in youth with Prader-Willi syndrome.

Background

Patients with Prader-Willi syndrome (PWS) can experience a complex array of symptoms including hyperphagia and behavioral disturbances. The Aberrant Behavior Checklist (ABC) is a standardized rating scale for the assessment of maladaptive behaviors with established use in populations with developmental disabilities. Our study aimed to describe ABC scores in youths with PWS and how they relate to changes in hyperphagia and quality of life with age.

Methods

Families seen at the Seattle Children's Hospital Prader-Willi syndrome (SCH PWS) clinic filled out questionnaires (ABC, PWS Hyperphagia Questionnaire [HQ], Pediatric Quality of Life [PQL]) as part of clinical care. A retrospective analysis was done of questionnaires completed between October 2014 and December 2025 for individuals between 5 and 21 years of age. Multivariate longitudinal growth models were fit to describe the mean and variance in all three repeated-measures outcomes across age.

Results

There was substantial individual variability for all outcomes. However, strong associations were noted between questionnaire scores. There were strong baseline associations across domains with higher ABC and hyperphagia severity associated with poorer quality of life. Strong slope-slope correlation was noted, particularly between ABC and HQ, and between HQ and PQL such that worsening hyperphagia was associated with worse behavior scores and lower quality of life.

Conclusion

Maladaptive behaviors, hyperphagia, and quality of life appear to be strongly linked in youth with PWS when measured with standardized domain-specific surveys. Further evaluation regarding the impact of hyperphagia and targeted treatment for hyperphagia on quality of life and maladaptive behavior are warranted.