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.

中文翻译：

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Prader-Willi 综合征成人计划能力缺陷的研究

背景

Prader-Willi 综合征 (PWS) 是一种复杂的发育性遗传疾病，与智力障碍和执行功能缺陷相关，导致组织混乱和个人自主性差。

宗旨

本研究旨在确定患有 PWS 的成年人在规划技能方面的障碍，与他们的智力障碍有关，以及食物强迫对其表现的影响。

方法和程序

来自执行不良综合征行为评估的动物园地图的修改版本被用于三组：一组患有 PWS 的成年人与两组按实际年龄匹配的组进行比较，一组具有典型发育 (TD)，一组具有智力障碍（ID）。

结果和结果

与 TD 成年人相比，患有 PWS 和 ID 的成年人都表现出增加的计划时间和较低的计划任务原始分数。与对照组相比，PWS 组的执行时间和错误数量更高。所有三组在非食物条件下仅在错误数量和原始分数方面表现更差。

结论和影响

PWS 成人的规划能力受损。结果还表明，智力水平对参与者的表现有影响。这些发现对于了解 PWS 患者日常生活的困难至关重要。