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Background: The present study investigated the influence of emotional contexts on mental flexibility in adults with Prader-Willi syndrome (PWS) using a voluntary task-switching paradigm that was implemented with emotionally valenced pictures. The study aims were to assess whether adults with PWS have impaired switching abilities, whether the deficit is specific to PWS or linked to intellectual disabilities, and the influence of emotional contexts on performance., Method: The task-switching performance of 30 adults with PWS was compared with that of 30 healthy adults matched on chronological age, and to that of 30 adults with intellectual disabilities but without PWS, matched on intellectual quotient level and chronological age. Indicators of switching performance were switching cost and repetition bias. Emotional contexts were operationalised with positive, neutral and negative task-irrelevant pictures., Results: Adults with PWS showed a large increase in switching costs compared with the two control groups, and this effect did not vary across emotional contexts. More fine-tuned examination revealed subtle performance modulations: negative contexts tended to increase the repetition bias in all three groups while positive contexts slowed down global performance in PWS., Conclusions: The results confirmed previous studies, showing impaired switching abilities in PWS over and beyond the influence of intellectual level, but revealed no robust variations in switching deficits across emotional contexts., (© 2021 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

Background: Prader-Willi syndrome (PWS) is a complex developmental genetic disorder associated with intellectual disability and deficits in executive functions which result in disorganisation and poor personal autonomy., Aims: This study aimed to determine impairments in planning skills of adults with PWS, in relation with their intellectual disabilities, as well as the influence of food compulsions on their performance., Methods and Procedures: A modified version of the Zoo Map from the Behavioural Assessment of the Dysexecutive Syndrome was used in three groups: a group of adults with PWS in comparison with two groups both matched on chronological age, one with typical development (TD) and one with intellectual disability (ID)., Outcomes and Results: Compared to TD adults, both adults with PWS and ID showed increased planning time and lower raw scores on the planning task. The execution time and the number of errors were higher in the PWS group compared to the comparison groups. All three groups performed worse in the non-food condition only for number of errors and raw score., Conclusions and Implications: Planning abilities were impaired in PWS adults. Results also showed that intellectual level plays a role in participants' performance. These findings are essential to understand the difficulties of people with PWS daily life., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

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.)