A profile of mental health and behaviour in Prader–Willi syndrome

Abstract

BackgroundPrader–Willi syndrome (PWS) is a neurogenetic syndrome with an associated behavioural phenotype and a high incidence of behaviours of concern and psychiatric co‐morbidity. These associated behaviours and co‐morbidities are not well addressed by existing interventions, and they impact significantly on affected individuals and their caregivers.MethodsWe undertook a national survey of the needs of individuals with PWS and their families in Ireland. In this paper, we report on the parent/caregiver‐reported mental health, behavioural and access to services.ResultsOver 50% of individuals with PWS in this survey had at least one reported psychiatric diagnosis, the most common diagnosis was anxiety. The most commonly reported behaviours in children were skin picking, repetitive questioning, difficulty transitioning and non‐compliance. The same four behaviours were reported by caregivers as being the most commonly occurring in adolescents and adults in addition to food‐seeking behaviours. Increased needs for mental health services were also reported by caregivers. Individuals with PWS had an average wait of 22 months for an appointment with a psychologist and 4 months for an appointment with a psychiatrist.ConclusionThis study highlighted high levels of psychiatric co‐morbidities and behavioural concerns in individuals with PWS in Ireland. The findings of this study suggest that there is an urgent need to provide specialist psychiatric and behavioural interventions to manage complex mental health and behavioural needs to better support individuals with PWS and reduce caregiver burden.

Suicidality in individuals with Prader-Willi syndrome: a review of registry survey data

“…In line with the previous reports [ 12 , 27 – 29 ], high rates of behavioral and psychiatric comorbidity were reported in this sample (Table 3 ). As in general population research, in this collection of individuals with PWS, those with a reported history of SI were more likely to have depression, anxiety, bipolar disorder, or psychosis and more likely to be receiving therapeutic or pharmacologic mental health treatments.…”

Characteristics and relationship between hyperphagia, anxiety, behavioral challenges and caregiver burden in Prader-Willi syndrome

“…Some studies in PWS did not appear to capture the challenge of anxiety in the PWS population [37,38]. Other studies have, however, found moderate to high levels of anxiety in PWS samples [3,7,39]. Different approaches in assessing anxiety were utilized in these studies that may explain the discrepancies between results.…”

“…Individuals with PWS present with central hypotonia and feeding problems in early infancy, followed during childhood and adulthood by intellectual and learning disabilities, maladaptive behaviors, hypogonadism and incomplete sexual development, short stature due to growth hormone deficiency and severe hyperphagia, which is a hallmark symptom of the syndrome. Emotional and behavioral challenges are prominent in PWS and include anxiety, repetitive behaviors, temper outbursts, and oppositional behavior [2][3][4][5][6][7]. As individuals with PWS reach adulthood, they are at high risk for developing psychiatric illness, including psychosis and major depression [8].…”

Behavioural change in Prader–Willi syndrome during COVID‐19 pandemic

“…Distinct behavioural traits are common, among them stubbornness, compulsions, temper outbursts and skin-picking (Whittington and Holland 2018). Repetitive questioning and difficulties in dealing with change are also characteristic and were described by caregivers as resulting in frequent misconduct (Feighan et al 2020). Furthermore, individuals with PWS seem to have a higher susceptibility to psychiatric disorders, mainly obsessive compulsive behaviours, depression, autism spectrum disorders and psychosis (Soni et al 2007;Soni et al 2008).…”