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BACKGROUND: 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. METHODS: 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. RESULTS: 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. DISCUSSION: 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.

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The behavioral phenotype characteristic of Williams syndrome (WS) is marked by strong interest in social interaction, manifested in attention to human faces, empathy, approach behavior and social disinhibition, often coexisting with generalized anxiety. Despite their heightened social interest, people with WS show deficits in explicit emotion recognition tasks similar to those of people with other developmental disabilities. In the current study we explored whether individuals with WS show distinctive autonomic responsiveness to social-emotional information, using skin conductance response and heart rate measures. Autonomic activation was investigated in response to facial expressions of emotion in adolescents and adults with WS, compared to age-matched normal controls and to age-, IQ- and language-matched individuals with learning or intellectual disabilities (LID). Overall participants with WS were less electrodermally responsive to dynamically presented face stimuli than the age- and IQ-matched LID group, and showed more heart rate deceleration when viewing emotional faces than the controls. These findings, indicating hypoarousal but increased interest in response to the dynamic presentation of facial emotions in WS, are consistent with the behavioral profile of high approachability toward social stimuli in this population.

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BACKGROUND: Individuals with autism spectrum disorders (ASDs) show variability in their sensory behaviors. In this study we identified clusters of toddlers with ASDs who shared sensory profiles and examined differences in affective symptoms across these clusters. METHOD: Using cluster analysis 170 toddlers with ASDs were grouped based on parent rating of the Infant Toddler Sensory Profile (Dunn, 2002) under-responsivity, over-responsivity, and seeking scales. Affective symptoms were evaluated with the Infant Toddler Social Emotional Assessment (Carter & Briggs-Gowan, 2005). RESULTS: Three clusters were identified: (1) low frequency of sensory symptoms (n = 44); (2) high frequency of symptoms (n = 49); and (3) mixed (n = 77); high frequency of under-and over-responsivity and low frequency of seeking). Relative to the low frequency cluster, parents rated toddlers in the high frequency and mixed clusters (both characterized by high frequencies of sensory under- and over-responsivity) as higher on negative emotionality, depression, and anxiety symptoms. Sensory and affective differences among clusters remained after co-varying severity of ASD symptoms. CONCLUSIONS: Interdisciplinary assessments are recommended for toddlers with ASDs in order to identify the interplay of sensory and affective symptoms.

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Autism spectrum disorders (ASD) are common neurodevelopmental disorders that occur along a broad continuum of severity with impairments in social interactions, communication and behaviour. This review highlights recent advances in autism research that shed light on the causes of the disorder and that have implications for clinical practice. It focuses on (1) the rising prevalence of ASD with attention given to recent epidemiological studies, (2) important genetic discoveries that may affect clinical evaluation of children with ASD, (3) active areas of research in cognitive neuroscience that seek to explain the underlying mechanisms of a complex disorder and (4) important studies on clinical populations with implications for screening and early identification of infants and toddlers with ASD.

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One of the most active areas of current research in the field of developmental disorders is autism. Since the NIH State of the Science conference, held in 1995 (Bristol et al. [1996] J. Autism Dev. Disorders 26:121-154), funding opportunities for comprehensive research programs addressing genetic, neurobiological, and behavioral aspects of this complex disorder have grown exponentially. Although we are far from having a complete understanding of the causes and deficits that define autism, significant progress has been made over the past few years. In this review, we summarize recent developments across a number of different areas of research in the field of autism, including diagnosis; defining the phenotypic features in individuals with autism; genetic bases; and neurobiological deficits.

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In this paper we argue that there are two distinct components of a theory of mind: a social-cognitive and a social-perceptual component. Evidence for this proposal is presented from various sources, including studies of children with Williams syndrome, a rare genetic neurodevelopmental disorder. Earlier work has demonstrated that people with Williams syndrome appear to be spared in the social-perceptual component of a theory of mind. In this paper we present evidence that they are not spared in the social-cognitive component of theory of mind. Three experiments with young children with Williams syndrome were conducted. In each experiment the children with Williams syndrome were compared to age-, IQ-, and language-matched children with Prader-Willi syndrome, and children with non-specific mental retardation. The experiments used different measures of theory of mind ability, including false belief (Experiment 1), explanation of action (Experiment 2), and recognition of emotional expressions (Experiment 3). In none of these experiments did the children with Williams syndrome evidence superior performance compared to the control groups. The results from this and other studies on Williams syndrome support the view that the social-cognitive and social-perceptual components of a theory of mind are dissociable. In Williams syndrome only the latter components, which are linked to distinct neurobiological substrates, are spared.

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Second-order mental state attribution in a group of children with Williams syndrome was investigated. The children were compared to age, IQ, and language-matched groups of children with Prader-Willi syndrome or nonspecific mental retardation. Participants were given two trials of a second-order reasoning task. No significant differences between the Williams syndrome and Prader-Willi or mentally retarded groups on any of the test questions were found. Results contrast with the view that individuals with Williams syndrome have an intact theory of mind and suggest that in their attributions of second-order mental states, children with Williams syndrome perform no better than do other groups of children with mental retardation.

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This brief report summarizes a workshop that was held at the National Institutes of Health in April 1998. The goal of the workshop was to further the development of a definition for the phenotype of specific language impairment (SLI). The report includes a discussion of research recommendations that will refine our current views of the definition of the SLI phenotype and sets out priority areas that are in need of further study to help advance understanding of this complex language-based disorder.

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This paper surveys current research on the social and communicative impairments in autism. In diagnostic schemes, the criteria for identifying autism in these domains include overlapping features. One approach to interpreting this overlap is to consider that social and communicative impairments reflect the same underlying cognitive deficit, referred to as the 'theory of mind' hypothesis of autism. On this view autism involves primary difficulties in identifying mental states in other people, and in interpreting behavior and action in relation to a person's mental state. Studies on the relationship between social behavior, communicative functioning, and theory of mind in children with autism are reviewed, emphasizing the connections between these areas of impairment that are central to the definition of the autistic syndrome.

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This study tested the hypothesis that Williams syndrome, a rare genetic neurodevelopmental disorder with an unusual cognitive phenotype, involves spared abilities in the domain of understanding other minds. A group of retarded adults with Williams syndrome was compared to an age-, IQ-, and language-matched group of adults with Prader-Willi syndrome, another genetic disorder without the cognitive characteristics of Williams syndrome, and a group of age-matched normal adults, on a task that taps mentalizing ability. The task involved selecting the correct labels to match photographs of complex mental state expressions in the eye region of the face. The adults with Williams syndrome performed significantly better than the adults with Prader-Willi on this task, and about half the group performed in the same range as the normal adults. These findings are consistent with anecdotal evidence about Williams syndrome and provide evidence that mentalizing is a distinct cognitive domain. This spared cognitive capacity may be linked to the relative sparing of limbic-cerebellar neural substrate in Williams syndrome, which is also connected to cortico-frontal regions that are known to be involved in understanding complex mental states.

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Longitudinal videotape recordings of six young children with autism and six age- and language-matched children with Down syndrome in structured play with their mothers at home were coded for the focus of the child's visual attention for four bimonthly visits and for facial affect for two of the four visits. The main finding was that the children with autism showed reduced expression of positive affect in a familiar social context. The autistic group attended to the mother's face and the researchers only about half as much as the Down syndrome group, but these differences did not reach statistical significance. Compared to the Down syndrome group, the autistic group displayed a smaller proportion of their total positive affect toward the mother's face and toward the researcher, but only the latter group difference reached statistical significance. Although limited by the small sample size, these findings suggest that autistic children's known deficits in attention and affective responsiveness to others persist even in structured interactions with a familiar partner in the home.

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Sixty-eight 3-year-olds received a standard appearance-reality task along with either a trick task, in which the appearance question was placed in the context of a deceptive game, or a reduced information processing task, in which a dual object (e.g., a sponge-rock) was presented along with an object that matched the dual object's identity (a sponge) and one that matched the dual object's appearance (a rock). Children were more likely to pass either the trick or reduced information processing task and fail the standard than the reverse. Thus, 3-year-olds can grasp the distinction between appearance and reality (a) when their goal is to trick someone, which may prime them to think about the other's mental state, and (b) when they do not need to held conflicting object identities in mind at the same time.

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Compared the performance of autistic and mentally retarded subjects, all of whom had passed a standard first-order test of false belief, on a new second-order belief task. 12 autistic and 12 mentally retarded subjects, matched on verbal mental age (assessed by PPVT and a sentence comprehension subtest of the CELF) and full-scale IQ were given two trials of a second-order reasoning task which was significantly shorter and less complex than the standard task used in all previous research. The majority of subjects in both groups passed the new task, and were able to give appropriate justifications to their responses. No group differences were found in performance on the control or test questions. Findings are interpreted as evidence for the role of information processing factors rather than conceptual factors in performance on higher order theory of mind tasks.

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Investigated examples of naturalistic humor in a group of 6 young children with autism and 6 age- and language-matched children with Down syndrome, who were videotaped while interacting with their mothers in bimonthly 1-hour sessions over the course of 1 year. Humor episodes were analyzed on three dimensions: cognitive developmental, social, and intentionality. The autistic children produced significantly less humor overall and less humor involving nonverbal incongruity. The only jokes in the study were told by 2 of the children with Down syndrome. Results indicate that while children with autism can produce and appreciate humor to a limited extent in a naturalistic setting, they do so at a significantly reduced level compared to matched controls. Findings are discussed in relation to the social-cognitive deficits in autistic children, which are among the primary characteristics of the syndrome.

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The relationship between performance on a false belief task and a new task designed to elicit explanations of human action was examined in two experiments. In the first study normal preschoolers' performance on both tasks was significantly correlated. The second experiment included matched groups of autistic, mentally retarded, and older normal children. The autistic subjects were significantly worse than controls at offering mentalistic explanations. Performance on the false belief and the explanation of action tasks was highly correlated for the autistic subjects. These findings are discussed in terms of the relationships between language and theory of mind.

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This study investigated the production of different types of speech pauses and repairs in the story narratives produced by autistic, mentally retarded, and normal children, matched on verbal mental age. Ten children in each group were asked to tell the story depicted in a wordless picture book. The narratives were analyzed for frequency of grammatical (between phrase) and nongrammatical (within phrase) pauses, and for several measures of story length and complexity. The main results were that children with autism produced significantly fewer nongrammatical pauses, and that their nongrammatical pausing was correlated with measures of story length and complexity. These findings suggest that the stories told by the autistic children reflect reduced cognitive and communicative demand. The implications of this study for future research on the use of a variety of prosodic characteristics as measures of social cognitive deficit in autism are discussed.

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