Pediatric Genetics, Duke University Medical Center, Durham, North Carolina 27710, USA.
J Intellect Disabil Res. 2012 Sep;56(9):865-78. doi: 10.1111/j.1365-2788.2011.01477.x. Epub 2011 Aug 31.
Although distinctive neuropsychological impairments have been delineated in children with chromosome 22q11 deletion syndrome (22q11DS), social skills and social cognition remain less well-characterised.
To examine social skills and social cognition and their relationship with neuropsychological function/behaviour and psychiatric diagnoses in children with 22q11DS.
Sixty-six children with 22q11DS and 54 control participants underwent neuropsychological testing and were administered the Diagnostic Analysis of Non-Verbal Accuracy (DANVA) for face and auditory emotion recognition, a measure of social cognition: their parents/guardians were administered the Social Skills Rating System (SSRS) - parent version, Child Behavior Checklist (CBCL) - parent version and the Computerised Diagnostic Interview Schedule for Children (C-DISC).
The 22q11DS group exhibited significantly lower social skills total score and more problem social behaviours, lower neurocognitive functioning, higher rates of anxiety disorders and more internalising symptoms than the control group. Participants with 22q11DS also exhibited significant deficits in their ability to read facial expressions compared with the control group, but performed no differently than the control participants in the processing of emotions by tone of voice. Within the 22q11DS group, higher social competency was correlated with higher global assessment of functioning and parental socio-economic status. Social competency was worse in those with anxiety disorders, attention deficit hyperactivity disorder, more than two psychiatric diagnoses on the C-DISC and higher internalising symptoms. No significant correlations of SSRS scores were seen with IQ, executive functions, attention, or verbal learning and memory. No correlations were found between social cognition and social skill scores.
Our results indicate that social skills in children with 22q11DS are associated with behaviour/emotional functioning and not with neurocognition. Thus, treating the behaviour or emotional problems such as attention deficit hyperactivity disorder and anxiety disorders may provide a pathway for improving social skills in these children.
尽管染色体 22q11 缺失综合征（22q11DS）患儿存在明显的神经心理学缺陷，但社交技能和社会认知仍未得到充分描述。
研究 22q11DS 患儿的社交技能和社会认知及其与神经心理学功能/行为和精神科诊断的关系。
66 名 22q11DS 患儿和 54 名对照参与者接受神经心理学测试，并进行了面部和听觉情绪识别的非言语准确性诊断分析（DANVA），这是一种社会认知的测量方法：他们的父母/监护人接受了社交技能评定量表（SSRS）-父母版本、儿童行为检查表（CBCL）-父母版本和儿童计算机诊断访谈量表（C-DISC）。
22q11DS 组的社会技能总分和问题行为较多，神经认知功能较低，焦虑障碍发生率较高，内化症状较多，与对照组相比差异有统计学意义。与对照组相比，22q11DS 组参与者阅读面部表情的能力也明显较差，但在通过语调处理情绪方面与对照组参与者没有差异。在 22q11DS 组中，较高的社会能力与较高的总体功能评估和父母的社会经济地位相关。在患有焦虑障碍、注意缺陷多动障碍、C-DISC 上有两个以上精神科诊断和内化症状较高的患者中，社会能力更差。SSRS 评分与智商、执行功能、注意力或言语学习和记忆无显著相关性。也没有发现社会认知和社会技能评分之间的相关性。
我们的研究结果表明，22q11DS 患儿的社交技能与行为/情绪功能有关，而与神经认知无关。因此，治疗注意力缺陷多动障碍和焦虑障碍等行为或情绪问题可能为改善这些儿童的社交技能提供途径。
