Department of Psychology, Boston University, 64 Cummington Street #126C, Boston, MA, 02115, USA,
J Neurodev Disord. 2011 Dec;3(4):325-34. doi: 10.1007/s11689-011-9100-9. Epub 2011 Nov 12.
People with Williams syndrome (WS) have been consistently described as showing heightened sociability, gregariousness, and interest in people, in conjunction with an uneven cognitive profile and mild to moderate intellectual or learning disability. To explore the mechanisms underlying this unusual social-behavioral phenotype, we investigated whether individuals with WS show an atypical appraisal style and autonomic responsiveness to emotionally laden images with social or nonsocial content. Adolescents and adults with WS were compared to chronological age-matched and nonverbal mental age-matched groups in their responses to positive and negative images with or without social content, using measures of self-selected viewing time (SSVT), autonomic arousal reflected in pupil dilation measures, and likeability ratings. The participants with WS looked significantly longer at the social images compared to images without social content and had reduced arousal to the negative social images compared to the control groups. In contrast to the comparison groups, the explicit ratings of likeability in the WS group did not correlate with their SSVT; instead, they reflected an appraisal style of more extreme ratings. This distinctive pattern of viewing interest, likeability ratings, and autonomic arousal to images with social content in the WS group suggests that their heightened social drive may be related to atypical functioning of reward-related brain systems reflected in SSVT and autonomic reactivity measures, but not in explicit ratings.
患有威廉姆斯综合征(WS)的人一直被描述为表现出高度的社交性、合群性和对人的兴趣,同时伴有认知能力不均衡和轻度至中度智力或学习障碍。为了探究这种不寻常的社会行为表型的机制,我们研究了 WS 患者是否表现出对具有社交或非社交内容的情感图片的非典型评价风格和自主反应。我们比较了 WS 患者与年龄匹配的和非语言性智力年龄匹配的青少年和成年人对有或没有社交内容的积极和消极图片的反应,使用自我选择观看时间(SSVT)、瞳孔扩张测量反映的自主唤醒以及喜好评分来衡量。与没有社交内容的图片相比,WS 患者观看社交图片的时间明显更长,与对照组相比,他们对负性社交图片的唤醒反应降低。与对照组不同的是,WS 组的明确喜好评分与他们的 SSVT 不相关;相反,他们反映出更极端的评价风格。这种对具有社交内容的图片的观看兴趣、喜好评分和自主唤醒的独特模式表明,他们强烈的社交驱力可能与反映在 SSVT 和自主反应测量中的与奖励相关的大脑系统的异常功能有关,但与明确的评分无关。