Investigation of the relationship between electrodermal and behavioural responses to executive tasks in Prader-Willi syndrome: An event-related experiment.

Background: Recent work suggests that maladaptive behaviors in genetic developmental disorders may emerge from autonomic dysfunctions impacting higher order executive functions. In Prader-Willi syndrome (PWS), executive functions are not well understood and investigations of possible underlying causes at the autonomic level are lacking.

Aims: This study aimed at clarifying the status of inhibition and working memory updating functions in PWS and searched for sympathetic signatures as well as to examine their links with executive performance.

A model to characterize psychopathological features in adults with Prader-Willi syndrome.

High prevalence of behavioral and psychiatric disorders in adults with Prader-Willi Syndrome (PWS) has been reported in last few years. However, data are confusing and often contradictory. In this article, we propose a model to achieve a better understanding of the psychopathological features in adults with PWS.

Executive functions and Prader-Willi syndrome: global deficit linked with intellectual level and syndrome-specific associations.

The aim of this study was to support the growing evidence suggesting that Prader-Willi Syndrome (PWS) might present with an impairment of executive functions (EFs) and to investigate whether this impairment is specific to patients with PWS or due to their intellectual disability (ID). Six tasks were administered to assess EFs (inhibition, switching, updating, cognitive estimation, and planning) to 17 patients with PWS and 17 age-matched healthy individuals. Performance was significantly impaired in the PWS group on all EFs and after controlling for IQ level, intergroup differences remained only for switching and cognitive estimation.

Medical, psychological and social features in a large cohort of adults with Prader-Willi syndrome: experience from a dedicated centre in France.

Background: Prader-Willi syndrome (PWS) is a developmental genetic disorder characterised by a variable expression of medical, cognitive and behavioural symptoms. In adulthood, the prevalence and severity of these symptoms determine the quality of life of the affected persons. Because of their rare disease condition, data on health and social problems in adults with PWS are scarce.

Behavioral profile of adults with Prader-Willi syndrome: correlations with individual and environmental variables.

Background: Maladaptive behavior has been reported as a phenotypical feature in Prader-Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause.