Executive functions (EF) and focal attention have been identified as a weakness in the profile of 22q11.2 deletion syndrome (22q11DS). However, due to a high variety of tasks used across previous studies, it remains unclear whether impairments may be more pronounced for specific subdomains of EF and focal attention. Furthermore, age-related changes have only been examined in a few studies, so far only yielding a partial view of the overall developmental profile.

In a broad age range (8–35 years) composed of longitudinal data, 183 participants (103 diagnosed with 22q11DS) completed an extensive assessment of EF and attention. To get a more comprehensive overview of specific versus global impairments, several tasks were assessed within multiple domains.

Results suggest differential impairments and trajectories in specific EF subdomains. Specifically, our findings suggest that individuals with 22q11DS not only showed lower overall inhibition skills, but also that initiation skills developed at a slower pace compared to healthy controls. Results are less clear regarding cognitive flexibility, updating and focal attention, for which performance strongly depended on the tasks that was selected to assess the domain.

Findings confirm and extend knowledge on differential developmental patterns of EF and attention domains in 22q11DS. They further stress the necessity to administer extensive, multifaceted evaluations to gain a more reliable overview of patients’ cognitive profile.

执行功能 (EF) 和焦点注意力已被确定为 22q11.2 缺失综合征 (22q11DS) 特征的弱点。然而，由于先前研究中使用的任务种类繁多，尚不清楚对于 EF 和焦点注意力的特定子域是否会更明显的损伤。此外，仅在少数研究中检查了与年龄相关的变化，到目前为止仅对整体发展概况产生了部分看法。

在由纵向数据组成的广泛年龄范围（8-35 岁）中，183 名参与者（103 名被诊断患有 22q11DS）完成了对 EF 和注意力的广泛评估。为了更全面地了解特定损伤与全局损伤，我们在多个领域评估了几项任务。

结果表明特定 EF 子域中的不同损伤和轨迹。具体来说，我们的研究结果表明，与健康对照组相比，具有 22q11DS 的个体不仅表现出较低的整体抑制技能，而且起始技能的发展速度也较慢。关于认知灵活性、更新和焦点注意力的结果不太清楚，其表现在很大程度上取决于为评估领域而选择的任务。

研究结果证实并扩展了关于 22q11DS 中 EF 和注意域的差异发展模式的知识。他们进一步强调有必要进行广泛的、多方面的评估，以更可靠地了解患者的认知状况。