412 - Aberrant Behavior Checklist Scores in Youth with Prader-Willi Syndrome

Prader-Willi Syndrome (PWS) is a rare genetic disorder caused by the loss of expression of paternal genes on chromosome 15, resulting in an array of physical, cognitive, and behavioral disturbances. The Aberrant Behavior Checklist (ABC) is a standardized rating scale assessing maladaptive behaviors with established use in populations with developmental disabilities. Although used in the PWS population, changes in ABC scores and how they relate to hyperphagia (HP) and quality of life (QL) have not been analyzed.

Objective:

To describe ABC scores in youths with PWS, changes in scores over time, and how they relate to HP (measured by the Hyperphagia Questionnaire [HQ]) and QL (measured by Quality-of-Life questionnaire [QoL]).

Design/Methods:

A retrospective review of patients (5-22) seen at the SCH PWS clinic (2014-2022). Questionnaires obtained as part of clinical visits. Only completed checklists were analyzed.  Scores analyzed by age ranges (5-8, 9-12,13-16, 17-22) and described as mean +/- standard deviation (SD).  Multivariate multilevel models evaluated the change of ABC, HQ, and QoL scores over time. Variables assessed include sex at birth and genetic subtype (deletion [D] vs non-deletion [ND]).

Results: Analysis included 69 subjects (50 F/19 M; 24 D/40 ND/5 undetermined) with 322 completed ABC questionnaires.  ABC scores rose in early childhood and decreased during teen years. Average HQ and QoL scores did not change significantly over time. In D vs. ND subtypes, D subtypes showed less improvement in ABC, HQ, and QoL over time. The higher the total ABC score at 13 years, the more significant the improvement in ABC noted by 20 years. Higher ABC Total scores (higher maladaptive behaviors) were associated with lower QoL scores (worse QL) and a higher HQ (worse health) at 13 years. At age 13, higher QoL (better QL) was related to a lower HQ score (better health). HQ changes were negatively related to change in QoL (r = -0.841, p < .001) and in ABC Total (r = -0.696, p < .001) such that a slower decrease in HQ per year (worse health) corresponds with a slower increase in QoL scores (worse QL) and faster increase in ABC Total (higher maladaptive behaviors).

Conclusion(s):

Individuals with PWS and their families face many challenges related to maladaptive behavior. Our study is the first to describe changes in ABC scores in youth with PWS across ages to evaluate maladaptive behaviors and how they relate to HP and QL. This study suggests relationships among maladaptive behaviors, H and QL in individuals with PWS. The ABC, HQ, and QoL may be used together to assess clinical improvement with intervention.