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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.

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. (Contains 5 tables and 1 footnote.)