School of Psychology, Macquarie University, North Ryde, NSW, 2109, Australia.
School of Psychology, Macquarie University, North Ryde, NSW, 2109, Australia.
Neuropsychologia. 2022 Oct 10;175:108366. doi: 10.1016/j.neuropsychologia.2022.108366. Epub 2022 Sep 8.
Individuals with Williams Syndrome (WS) or Downs Syndrome (DS) are often described as hypersociable, friendly and overly trusting of others. This hypersociability is a major concern for parents/caregivers due to the associated increased risk of exploitation and victimisation. Two brain regions - the amygdala and the orbitofrontal cortex (OFC) - have been implicated in driving this hypersociability in WS, and in the general population and have associations with emotional evaluation, threat detection and social motivation. However, there has been little neuroimaging research on this topic, especially in DS, to date. The aim of the present study was to investigate the potential neuroanatomical and neuropsychological correlates of hypersociability in WS and DS. Twelve individuals with WS (M = 22 years of age) and eleven individuals with DS (M = 26 years of age) completed a neuropsychological battery of executive functioning and social measures, including informant ratings on an ecologically measure of social approach. Clinical groups and twelve typically developing controls (M = 23 years) underwent a magnetic resonance imaging scan to investigate volumetric differences in the OFC and the amygdala. As expected, WS individuals displayed the highest overall social approach, especially in relation to need to approach strangers and drive to interact with strangers, as well as inappropriate/overfriendly behaviours. Both groups rated similarly in terms of social trust and unconditional positive regard. Emotion recognition abilities were similar across groups, with the DS group displaying some difficulties with negative emotions (especially anger). Inhibition and flexibility were similarly impaired across WS and DS. Compared to neurotypical controls, the DS group showed increased amygdala volumes bilaterally, while the WS group showed an enlarged right medial OFC. Approach ratings were significantly correlated with left amygdala and medial and left lateral OFC volumes in WS, and with these same regions bilaterally in DS. Results provide potential biological explanations for the hypersociability seen in WS and DS. Future research should focus on other potential neural correlates, as well as potential genetic and hormonal contributions to approach.
患有威廉姆斯综合征（WS）或唐氏综合征（DS）的个体通常被描述为过于社交、友好和过度信任他人。这种过度社交能力是父母/照顾者的主要关注点，因为这与剥削和受害的风险增加有关。两个大脑区域——杏仁核和眶额皮层（OFC）——被认为与 WS 中的这种过度社交能力以及普通人群中的情绪评估、威胁检测和社交动机有关。然而，迄今为止，针对这一主题的神经影像学研究很少，尤其是在 DS 中。本研究旨在探讨 WS 和 DS 中过度社交能力的潜在神经解剖学和神经心理学相关性。12 名 WS 个体（M=22 岁）和 11 名 DS 个体（M=26 岁）完成了执行功能和社会措施的神经心理学测试，包括对社交接近度的生态测量的信息提供者评分。临床组和 12 名典型发育对照组（M=23 岁）接受了磁共振成像扫描，以研究 OFC 和杏仁核的体积差异。正如预期的那样，WS 个体表现出最高的整体社交接近度，尤其是在与接近陌生人的需求和与陌生人互动的驱动力以及不适当/过于友好的行为方面。两组在社交信任和无条件积极关注方面的评分相似。情绪识别能力在各组之间相似，DS 组在识别负面情绪（尤其是愤怒）方面存在一些困难。抑制和灵活性在 WS 和 DS 中均有相似程度的损伤。与神经典型对照组相比，DS 组双侧杏仁核体积增加，而 WS 组右侧内侧 OFC 增大。在 WS 中，接近评分与左侧杏仁核和内侧及左侧外侧 OFC 体积显著相关，而在 DS 中，双侧均与这些区域相关。研究结果为 WS 和 DS 中所见的过度社交能力提供了潜在的生物学解释。未来的研究应重点关注其他潜在的神经相关性，以及对接近的潜在遗传和激素贡献。
