Obsessions and Compulsions in Prader‐Willi Syndrome

Abstract

This study examines the nature, severity and correlates of non-food obsessions and compulsions in 91 people with Prader-Willi syndrome (PWS) aged 5-47 years (mean age = 19 years). Prominent symptoms, seen in 37-58% of the sample, included hoarding; ordering and arranging; concerns with symmetry and exactness; rewriting; and needs to tell, know or ask. A remarkably high proportion of participants had moderate to severe symptom severity ratings; 64% showed symptom-related distress, and 80% showed symptom-related adaptive impairment. The study also compared obsessive-compulsive symptoms in 43 adults with PWS to age- and sex-matched non-retarded adults with obsessive-compulsive disorder (OCD). The PWS and OCD groups showed similar levels of symptom severity and numbers of compulsions; they also showed more areas of symptom similarity than difference. Increased risks of OCD in persons with PWS are strongly indicated. Implications are discussed for pharmacotherapy, behavioral therapy and family support.

The Prevalence and Phenomenology of Repetitive Behavior in Genetic Syndromes

“…These findings are largely consistent with previous studies of repetitive behavior in Prader-Willi syndrome (Dykens & Kasari, 1997;Dykens et al, 1996;Steinhausen et al, 2002;Wigren & Hansen, 2003). However, whilst 61.3% of individuals within the Prader-Willi syndrome group scored above the clinical cut-off on the item referring to repetitive questioning, previously reported to be characteristic of the syndrome (Clarke, Boer, Chung, Sturmey & Deb, 1996) this behavior did not demonstrate high specificity within this group.…”

“…However, the specific topographies of behavior described within the syndrome include ordering, rituals and hoarding. These are very different to those reported within individuals with Obsessive Compulsive Disorder (Dykens et al, 1996), suggesting that the application of this term may not be entirely appropriate.…”

“…Similarly, studies of repetitive behavior in Cornelia de Lange syndrome have identified an association between repetitive behaviors and the presence of self-injury, self-restraint behaviors and hyperactivity (Hyman, Oliver & Hall, 2002; in review citation anonymised for blind review), suggesting that a deficit in behavior regulation might be a common underlying factor (Petty & Oliver, 2005). Recent research within Prader-Willi syndrome has demonstrated the presence of a short-term memory deficit in affected individuals (Dykens, Hodapp & Finucane 2000) and compromised capacity for attention switching (in review citation anonymised for blind review) and these specific cognitive deficits might account for the repetitive questioning and preference for routine, which is commonly reported in the syndrome (Dykens, Leckman & Cassidy, 1996). At the neurobiological level, study of mutant mouse models of a range of neurodevelopmental disorders has also revealed potential aetiological pathways for repetitive behavior.…”

“…The table highlights some of the difficulties that arise when this terminology is employed within the intellectual disability population. For example, the term 'Obsessive Compulsive Disorder' has been employed to describe repetitive behavior in Prader-Willi syndrome (Dykens et al, 1996). However, the specific topographies of behavior described within the syndrome include ordering, rituals and hoarding.…”

“…Studies of repetitive behavior in Prader-Willi syndrome indicate a lower prevalence of stereotyped behavior (Clarke & Boer, 1998) and a heightened prevalence of 'compulsive' behavior (Dykens et al, 1996;Dykens & Kasari, 1997). In Fragile X syndrome there is a heightened prevalence of both of these subtypes of repetitive behavior (Backes et al, 2000;Hagerman & Lampe, 1999;Mazzocco et al, 1998), suggesting a generalised heightened probability of repetitive behavior.…”