Atypical development of language and social communication in toddlers with Williams syndrome

Abstract

Williams syndrome (WS) is a genetic disorder which results in an uneven cognitive profile. Despite superior language compared to other syndromes in the phenotypic outcome, toddlers with WS are as delayed in their language onset and early linguistic development as are toddlers with other syndromes. The cause of this delay in WS is as yet unknown. In a series of experiments, we examined whether atypical socio-interactive precursors to language could contribute to the explanation of the late language onset and atypical developmental pathways observed in WS. Experiment 1 showed that despite superficially good social skills, toddlers with WS were only proficient at dyadic interaction. They were impaired in triadic interaction, essential for the referential uses of language, and showed none of the correlations between socio-interactive markers and language seen in the typical controls. Experiment 2 focused on the comprehension and production of referential pointing. Again, the WS group was impaired, despite vocabulary levels higher than those of typically developing controls. Finally, Experiment 3 examined fine motor skills. The WS lack of pointing could not be explained in terms of motor impairments, since the WS toddlers were proficient at fine motor control, such as the pincer grip. Overall, our data indicate that the early stages of WS language follow an atypical pathway. The findings challenge the frequent claims in the literature that individuals with Williams syndrome have preserved linguistic and social skills.

Attention to Faces in Williams Syndrome

“…Brown et al (2003) also found evidence of problems planning saccades and shifting attention. Infants also show attention shifting difficulties during triadic interactions, relating attention skills to social cognition (see Laing et al 2002). It seems likely that problems modulating attention exist throughout the developmental spectrum and therefore it makes sense that they may also link to gaze behaviours associated with the disorder.…”

Verbal Peaks and Visual Valleys in Theory of Mind Ability in Williams Syndrome

“…Jones et al 2000), individuals with WS also have some social difficulties. For instance, they have difficulties in triadic social interaction (Laing et al 2002) and in making and sustaining friendships particularly with their peers (Gosch and Pankau 1994). Moreover, they have poor social judgement (Gosch and Pankau 1994) and often lack of social inhibition .…”

“…In fact, social behaviour in WS may also be maladapted and characterized by overfriendliness, oversensitivity and poor-peer relations (e.g. Laing et al 2002). Knowledge of the mechanisms implicated in this unusual social behaviour of WS suggests far more complexity than was originally envisioned.…”

Why neuroscience matters to cognitive neuropsychology

“…In typical development, infants are initially drawn to face-like visual stimuli, progressively coming to devote more discriminating attention to specifically human faces in contrast to other face-like stimuli as well as to other objects. Infants with WS share this preference for face-like stimuli, even showing a disproportionate interest in faces compared to that of normal controls (Mervis and Bertrand 1997;Bellugi et al 2000;Laing et al 2002). By contrast, this bias seems to be completely lacking in individuals with ASD.…”