Search

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., Methods and Procedures: The performance of thirty adults with PWS was compared to that of thirty healthy adults on two tasks assessing inhibition and working memory updating while electrodermal activity (EDA) was recorded., Outcomes and Results: PWS adults underperformed healthy adults in the inhibition and the working memory updating tasks and showed abnormal skin conductance responses. Distinct EDA have been found in PWS and healthy adults. Furthermore, while EDA reflected distinct cognitive processes, correlations between electrodermal and behavioural data were absent when examining the two groups separately., Conclusions and Implications: PWS is associated with a slight impairment of inhibition and a severe impairment of working memory updating. Furthermore, there are specific sympathetic autonomic signatures in PWS that do not present straightforward links with executive dysfunctions., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

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. In conclusion, PWS seems to be associated with a global impairment of EFs that appears to be closely linked with intellectual impairment but also with the PWS itself.

Introduction: The aim of the present study was to determine whether individuals with Prader-Willi syndrome (PWS) have impaired global executive functioning and whether this deficit is linked with intellectual disability. Another objective focussed on the variability in performance of intellectual quotient (IQ) and executive functions (EF) depending on the genotypic subtype. A final objective investigated whether the relationships between IQ and EF are different according to the genotypic subtype., Method: Twenty individuals with PWS and mild-to-moderate IQ (standard scores between 55 and 90, age range 19 and 49 years old, SD = 28.1) were administered an ecological battery of executive functioning (behavioural assessment of dysexecutive syndrome, BADS, adapted from Wilson et al. (1996) Behavioural Assessment of the Dysexecutive Syndrome. Thames Valley Test Company: Bury St Edmunds, UK.). The BADS contains six tests evaluating EF. The sample comprised 14 deletion subtype and six maternal uniparental disomy (m-UPD) subtype., Results: Behavioural assessment of dysexecutive syndrome scores were below the level of the standardized healthy populations of the battery and equivalent to those of the neuropathological standardized population. Most scores on EF tasks were relatively highly correlated with Full Scale and Verbal IQs but were not significant or moderately correlated with Performance IQ. Lastly, underlying differences were found in scores on two EF tasks (the Rule Shift Card and the Zoo Map subtests) between the deletion and m-UPD subtypes., Discussion: These data suggest a deficit of executive functioning in PWS that is linked more with verbal skills than performance skills. They also suggest that the impact on executive functioning may differ according to the genotype., (© 2013 John Wiley & Sons Ltd.)

Background: The present study investigated decision-making in patients with Prader-Willi syndrome (PWS) using the Soochow Gambling Task (SGT). The objective was to assess whether adults with PWS have impaired hot executive functions and whether the deficit is specific to PWS or linked to intellectual disabilities. Method: The SGT performance of 26 adults with PWS was compared to that of 26 healthy adults matched on chronological age, and to that of 26 adults with intellectual disabilities but without PWS, matched on intellectual quotient level and chronological age. Results: The PWS adults showed an absence of learning pattern over trials in the SGT, unlike the healthy individuals, and showed equivalent performances to adults with intellectual disabilities. Conclusions: The results suggest a deficit of hot executive functions in PWS, which is linked to intellectual disabilities and not specific to the syndrome.