Williams syndrome: Reduced attention to other’s eyes in a hypersocial phenotype

Abstract

Williams syndrome (WS) is a rare genetic disorder which leads to high social motivation as well as intellectual disability and difficulties with social interaction. Attention to others’ eyes is crucial for social understanding. Individuals with WS are typically highly attentive to faces, but there is a lack of knowledge about how they attend to other’s eyes, and the mechanisms underlying potential alterations in behavior. It has been suggested that physiological hypo-arousal enables individuals with WS to maintain their gaze longer at other’s faces. The aim of this study was to better understand if there is atypical gaze behavior among individuals with WS which might underlie their reduced social understanding and related difficulties with social interaction. We examined the speed and likelihood of gaze shifts to and from other’s eyes in individuals with WS (n = 37; mean age 23 years), and controls (n = 167) in stratified age groups (7 months, 8-12 years, 13-17 years, adults). Peak saccadic velocity was studied as an index of arousal. Individuals with WS were less likely, and slower, to orient to the eyes compared with typically developing controls in all age groups from eight years of age but did not differ from 7-month-old infants. Peak saccadic velocity was reduced in WS, replicating previous results of hypo-arousal. The results were supported by both frequentist and Bayesian statistics. Despite the hyper-social behavioral phenotype, WS is associated with reduced attention to others’ eyes. This could contribute to the difficulties with complex social interaction observed in WS.

Disrupted Attention to Other’s Eyes is Linked to Symptoms of ADHD in Childhood

“…Rapid orienting to eyes is observed already in infancy and is believed to be driven by a largely subcortical network of brain regions sensitive to the coarse visual characteristics of eyes [14,41,42]. Disruptions at this stage have been linked to autism [43][44][45] (but see [46]) and Williams syndrome, a genetic disorder with early emerging social alterations [47]. In contrast, the latency to reorient from eyes is believed to be modulated by higher order cognitive processes including spatial attention and motivation.…”