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Mental health outcomes in Prader-Willi Syndrome: An Australian cohort study
Emma K Baker1, Tayla Chellew2, Minh Bui3
1Diagnosis and Development, Murdoch Children's Research Institute, Royal Children's Hospital, Parkville, Victoria, Australia; Flinders Health and Medical Research Institute (Sleep Health), Flinders University, Bedford Park, South Australia, Australia.
Objective:
Prader-Willi syndrome (PWS) is an imprinting disorder associated with complex cognitive, physical, and behavioral features, as well as high rates of mental health conditions. Understanding mental health conditions in individuals with PWS and their association with behavioral characteristics is an essential step towards better supporting individuals with PWS.
Methods:
Thirty individuals with PWS (14 deletion, 16 non-deletion) aged 3-45 years participated in this study to examine associations between mental health conditions (Prader-Willi Syndrome Mental Health Questionnaire), intellectual functioning (Wechsler Intelligence Scales), autistic traits (Autism Diagnostic Observation Schedule-2), and challenging daytime behaviors (Prader-Willi Syndrome Behavior Questionnaire; Aberrant Behavior Checklist).
Results:
73% of individuals with PWS had at least one parent-reported mental health condition. Anxiety (65%) was the most common, followed by obsessive compulsive disorder (43%) and depression (40%). While reports of any mental health condition and depression were more common in individuals with a deletion compared to their non-deletion counterparts, these differences were primarily driven by the fact that the deletion group was significantly older than the non-deletion group. Those with PWS and mental health conditions had lower verbal IQ (p = .003), greater autistic traits (p < .001), greater food seeking (p = .004) and oppositional behaviors (p = .021), and increased maladaptive behaviors (p = .001) compared to those without a mental health condition.
Conclusion:
This study highlighted elevated rates of mental health conditions and additional cognitive and behavioral challenges among individuals with PWS, including in young children with PWS. Age-related factors were a primary factor relating to differences between molecular classes on mental health presentations, highlighting the need for earlier supports and interventions that may improve mental health prognoses in individuals with PWS.