Deborah M. Riby

Viewing it differently: Social scene perception in Williams syndrome and Autism

The genetic disorder Williams syndrome (WS) is associated with a propulsion towards social stimuli and interactions with people. In contrast, the neuro-developmental disorder autism is characterised by social withdrawal and lack of interest in socially relevant information. Using eye-tracking techniques we investigate how individuals with these two neuro-developmental disorders associated with distinct social characteristics view scenes containing people. The way individuals with these disorders view social stimuli may impact upon successful social interactions and communication. Whilst individuals with autism spend less time than is typical viewing people and faces in static pictures of social interactions, the opposite is apparent for those with WS whereby exaggerated fixations are prevalent towards the eyes. The results suggest more attention should be drawn towards understanding the implications of atypical social preferences in WS, in the same way that attention has been drawn to the social deficits associated with autism.

Do faces capture the attention of individuals with Williams syndrome or Autism? Evidence from tracking eye movements

The neuro-developmental disorders of Williams syndrome (WS) and autism can reveal key components of social cognition. Eye-tracking techniques were applied in two tasks exploring attention to pictures containing faces. Images were (i) scrambled pictures containing faces or (ii) pictures of scenes with embedded faces. Compared to individuals who were developing typically, participants with WS and autism showed atypicalities of gaze behaviour. Individuals with WS showed prolonged face gaze across tasks, relating to the typical WS social phenotype. Participants with autism exhibited reduced face gaze, linking to a lack of interest in socially relevant information. The findings are interpreted in terms of wider issues regarding socio-cognition and attention mechanisms.

Anxiety and Repetitive Behaviours in Autism Spectrum Disorders and Williams syndrome: A Cross-syndrome Comparison

Children with Autism Spectrum Disorder or Williams syndrome are vulnerable to anxiety. The factors that contribute to this risk remain unclear. This study compared anxiety in autism spectrum disorder and Williams Syndrome and examined the relationship between repetitive behaviours and anxiety. Thirty-four children with autism and twenty children with Williams Syndrome were assessed with measures of anxiety and repetitive behaviours. Children with autism had higher levels of anxiety. Within the autism sample higher levels of repetitive behaviours were associated with more anxiety. This was not replicated in the Williams Syndrome sample, indicating a differential role for restricted and repetitive behaviours in relation to anxiety. Understanding the links between repetitive behaviours and anxiety is essential for effective intervention.

Executive neuropsychological functioning in individuals with Williams syndrome

The present study investigated executive neuropsychological functioning in individuals with the neuro-developmental disorder Williams syndrome (WS) using a set of validated standardized neuropsychological tasks. Relatively few studies have examined frontal lobe related executive functions within the cognitive phenotype associated with the disorder. The present study compared participants with WS to typically developing participants who were individually matched for (1) chronological age and (2) verbal mental age (N=19 each group) on tasks of attention-set shifting, planning and working memory from the Cambridge Neuropsychological Test Automated Battery (CANTAB). To address the specificity of executive function impairment, non-executive tasks of delayed short-term memory and short-term memory span were also administered. Individuals with WS (mean age 18 years) showed impaired executive functioning on tasks of attention set-shifting, working memory, and planning. Non-executive deficits were also observed in short-term delayed memory and memory span. Neuropsychological impairments were correlated with a range of behavioural problems assessed using parent-rated Questionnaires. Overall, these findings point to the role of a range of executive function impairments in WS but further suggest that cognitive impairments extend beyond executive dysfunction.

The eyes or the mouth? Feature salience and unfamiliar face processing in Williams syndrome and autism

Using traditional face perception paradigms the current study explores unfamiliar face processing in two neurodevelopmental disorders. Previous research indicates that autism and Williams syndrome (WS) are both associated with atypical face processing strategies. The current research involves these groups in an exploration of feature salience for processing the eye and mouth regions of unfamiliar faces. The tasks specifically probe unfamiliar face matching by using (a) upper or lower face features, (b) the Thatcher illusion, and (c) featural and configural face modifications to the eye and mouth regions. Across tasks, individuals with WS mirror the typical pattern of performance, with greater accuracy for matching faces using the upper than using the lower features, susceptibility to the Thatcher illusion, and greater detection of eye than mouth modifications. Participants with autism show a generalized performance decrement alongside atypicalities, deficits for utilizing the eye region, and configural face cues to match unfamiliar faces. The results are discussed in terms of feature salience, structural encoding, and the phenotypes typically associated with these neurodevelopmental disorders.

Exploring face perception in disorders of development: evidence from Williams syndrome and autism

Individuals with Williams syndrome (WS) and autism are characterized by different social phenotypes but have been said to show similar atypicalities of face-processing style. Although the structural encoding of faces may be similarly atypical in these two developmental disorders, there are clear differences in overall face skills. The inclusion of both populations in the same study can address how the profile of face skills varies across disorders. The current paper explored the processing of identity, eye-gaze, lipreading, and expressions of emotion using the same participants across face domains. The tasks had previously been used to make claims of a modular structure to face perception in typical development. Participants with WS (N=15) and autism (N=20) could be dissociated from each other, and from individuals with general developmental delay, in the domains of eye-gaze and expression processing. Individuals with WS were stronger at these skills than individuals with autism. Even if the structural encoding of faces appears similarly atypical in these groups, the overall profile of face skills, as well as the underlying architecture of face perception, varies greatly. The research provides insights into typical and atypical models of face perception in WS and autism.

Attention-deficit/hyperactivity disorder and Williams syndrome: Shared behavioral and neuropsychological profiles

We compared verbally matched attention-deficit/hyperactivity disorder (ADHD), Williams syndrome (WS), and typically developing individuals (N = 19 each group) on behavioral symptoms (Conners ADHD rating scale) and neuropsychological functioning. Neuropsychological tasks included those that assessed short-term memory and executive functions from the CANTAB (Cambridge Neuropsychological Test Automated Battery) neuropsychological battery. Children with WS scored within the abnormal range and did not differ in severity from ADHD children on the Conners Oppositionality, Cognitive Problems/Inattention, Hyperactivity, and ADHD Index subscales. The WS and ADHD groups also showed similar patterns of neuropsychological functioning, particularly in working memory (WM) strategy use and delayed short-term memory (STM). The findings may have clinical implications for the management of individuals with WS, highlighting the potential significance of behavioral, educational, and pharmacological strategies and treatments known to be useful in the treatment of children with ADHD for individuals with WS.

Do Children with Williams Syndrome Really Have Good Vocabulary Knowledge? Methods for Comparing Cognitive and Linguistic Abilities in Developmental Disorders.

The comparison of cognitive and linguistic skills in individuals with developmental disorders is fraught with methodological and psychometric difficulties. In this paper, we illustrate some of these issues by comparing the receptive vocabulary knowledge and non‐verbal reasoning abilities of 41 children with Williams syndrome, a genetic disorder in which language abilities are often claimed to be relatively strong. Data from this group were compared with data from typically developing children, children with Down syndrome, and children with non‐specific learning difficulties using a number of approaches including comparison of age‐equivalent scores, matching, analysis of covariance, and regression‐based standardization. Across these analyses children with Williams syndrome consistently demonstrated relatively good receptive vocabulary knowledge, although this effect appeared strongest in the oldest children.

Attention to Faces in Williams Syndrome.

Williams syndrome (WS) is associated with distinct social behaviours. One component of the WS social phenotype is atypically prolonged face fixation. This behaviour co-exists with attention difficulties. Attention is multi-faceted and may impact on gaze behaviour in several ways. Four experiments assessed (i) attention capture by faces, (ii) interference from facial stimuli, (iii) face bias, and (iv) attention disengagement. Individuals with WS were compared to typically developing participants of comparable nonverbal ability and chronological age. The first three experiments revealed no atypicality of attention to faces in WS. However, in experiment 4 there was a suggestion that individuals with WS (compared to those developing typically) found it much more time consuming to disengage from faces than objects. The results are discussed in terms of attention abnormalities and possible face disengagement difficulties in WS.

Spontaneous attention to faces in Asperger syndrome using ecologically valid static stimuli

Previous eye tracking research on the allocation of attention to social information by individuals with autism spectrum disorders is equivocal and may be in part a consequence of variation in stimuli used between studies. The current study explored attention allocation to faces, and within faces, by individuals with Asperger syndrome using a range of static stimuli where faces were either viewed in isolation or viewed in the context of a social scene. Results showed that faces were viewed typically by the individuals with Asperger syndrome when presented in isolation, but attention to the eyes was significantly diminished in comparison to age and IQ-matched typical viewers when faces were viewed as part of social scenes. We show that when using static stimuli, there is evidence of atypicality for individuals with Asperger syndrome depending on the extent of social context. Our findings shed light on the previous explanations of gaze behaviour that have emphasised the role of movement in atypicalities of social attention in autism spectrum disorders and highlight the importance of consideration of the realistic portrayal of social information for future studies.