Daniela Plesa Skwerer

Attending preferentially to social information in the environment is important in developing socio-communicative skills and language. Research using eye tracking to explore how individuals with autism spectrum disorder deploy visual attention has increased exponentially in the past decade; however, studies have typically not included minimally verbal participants. In this study, we compared 37 minimally verbal children and adolescents with autism spectrum disorder with 34 age-matched verbally fluent individuals with autism spectrum disorder in how they viewed a brief video in which a young woman, surrounded by interesting objects, engages the viewer, and later reacts with expected or unexpected gaze-shifts toward the objects. While both groups spent comparable amounts of time looking at different parts of the scene and looked longer at the person than at the objects, the minimally verbal autism spectrum disorder group spent significantly less time looking at the person’s face during the episodes where gaze following—a precursor of joint attention—was critical for interpreting her behavior. Proportional looking-time toward key areas of interest in some episodes correlated with receptive language measures. These findings underscore the connections between social attention and the development of communicative abilities in autism spectrum disorder.

Despite many studies documenting the prevalence of various co-occurring psychiatric symptoms in children and adults with ASD, less is known about how these symptoms relate to subtypes defined by particular phenotypic features within the ASD population. We examined the severity and prevalence of comorbid symptoms of psychopathology, emotion dysregulation, and maladaptive behaviors, as well as adaptive functioning, in a group of 65 minimally verbal children (n = 33) and adolescents (n = 32) with ASD. On the Child and Adolescent Symptom Inventory (CASI-5), for all the symptom classifications except oppositional defiant disorder and conduct disorder, more participants in our sample showed elevated or clinically concerning severity scores relative to the general population. On the Emotion Dysregulation Inventory (EDI), the mean scores for Reactivity and Dysphoria factors in our sample were lower than in the autism calibration sample, which included a large number of inpatient youth with ASD. Overall, few differences were found between the children and adolescents within this severely impaired group of ASD individuals based on clinical cutoff scores on the CASI-5 and EDI factor scores. Psychiatric comorbidities and emotion dysregulation measures were not correlated with autism symptom severity or with measures of adaptive functioning, and were largely unrelated to IQ in our sample. The number of clinically significant psychiatric symptoms on the CASI-5 emerged as the main predictor of maladaptive behaviors. Findings suggest a wide range of co-occurring psychopathology and high degree of maladaptive behavior among minimally verbal children and adolescents with ASD, which are not directly attributable to autism symptom severity, intellectual disability or limitations in adaptive functioning.

Anecdotal and caregiver reports often highlight the sociability and empathy of children with Williams syndrome (WS), a genetically based neurodevelopmental disorder characterised by a distinctive, cognitive and social phenotype. Despite these characteristics, people with WS have many difficulties navigating the social world. In this study, we investigated whether the heightened social motivation and empathy demonstrated by children with WS lead to prosocial behaviours such as instrumental helping. We compared 2;8 to 5;8 year olds with WS to an age-matched and developmental quotient-matched group of children with Down syndrome (DS) and an age-matched group of typically developing children, in their responses to semi-structured naturalistic situations designed to elicit empathic and helping behaviours. Children with WS showed more empathic concern than both comparison groups towards a person in distress but did not differ from controls in their level of helping behaviour. Children in both the WS and DS groups consistently received higher ratings on empathy than on helpfulness, in contrast to the balanced profile shown by the typically developing children. Findings suggest that the heightened emotional responsivity displayed by children with WS or DS does not readily translate into other forms of socially competent behaviour. The complex relations between empathy and prosocial behaviours in typical and atypical development are discussed.

This research addresses the challenges of assessing receptive language abilities in minimally verbal children with autism spectrum disorder by comparing several adapted measurement tools: a standardized direct assessment of receptive vocabulary (i.e. Peabody Picture Vocabulary Test-4); caregiver report measures including scores on the Vineland-II Communication domain and a vocabulary questionnaire consisting of a list of words ranging from simple, developmentally early, to more advanced words expected to be understood by at least some older children and adolescents; an eye-tracking test of word comprehension, using a word–image pair matching paradigm similar to that often used in studies of infant language acquisition; and a computerized assessment using a touch screen for directly measuring word comprehension with the same stimuli used in the eye-tracking experiment. Results of this multiple-method approach revealed significant heterogeneity in receptive language abilities across participants and across assessment methods. Our findings underscore the need to find individualized approaches for capturing the potential for language comprehension of minimally verbal children with autism spectrum disorder who remain otherwise untestable, using several types of assessment that may include methods based on eye-tracking or touch-screen responding.

This chapter examines the social phenotype of Williams syndrome (WS). WS is a rare genetic disorder in which about 25 genes are missing from one copy of the long arm of chromosome 7. People with WS are strikingly social creatures. They are outgoing, gregarious, warm, and socially engaged with other people. Despite their mild to moderate levels of intellectual disability, children and adults with WS will approach new people they meet, strike up a conversation, and show intense interest in them. However, there is also a darker side to the social world of people with WS. First, the social engagement, especially in young children, can be so extreme that their fearlessness and willingness to accompany complete strangers pose real potential danger. And second, by middle childhood they experience difficulties forming friendships, have limited conversational and other discourse skills, and are vulnerable to teasing and social rejection.

Research on language in individuals with Williams syndrome (WS) has been fueled by persistent theoretical controversies for two decades. These shifted from initial focus on dissociations between language and cognition functions, to examining the paradox of socio-communicative impairments despite high sociability and relatively proficient expressive language. We investigated possible sources of communicative difficulties in WS in a collaborative referential communication game. Five- to thirteen-year-old children with WS were compared to verbal mental age- and to chronological age-matched typically developing children in their ability to consider different types of information to select a speaker's intended referent from an array of items. Significant group differences in attention deployment to object locations, and in the number and types of clarification requests, indicated the use of less efficient and less mature strategies for reference resolution in WS than expected based on mental age, despite learning effects similar to those of the comparison groups, shown as the game progressed.

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.