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Author: Deborah M Riby (46)


Nov
2017

Anxiety is a prevalent mental health issue for individuals with Williams syndrome (WS). Relatively little is known about the developmental course of anxiety, or how it links with core features of WS, namely social and executive functioning (EF). In this study, parent-reports of anxiety were compared across a 4-year period (N = 17), and links between anxiety, social and EF were explored from concurrent parent-reports (N = 26).

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Jul
2017

Paying attention is a critical first step toward learning. For children in primary school classrooms there can be many things to attend to other than the focus of a lesson, such as visual displays on classroom walls. The aim of this study was to use eye-tracking techniques to explore the impact of visual displays on attention and learning for children.

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Dec
1969

The primary aim of the current study was to employ event-related potentials (ERPs) methodology to disentangle the mechanisms related to inhibitory control in older adults with Williams syndrome (WS). Eleven older adults with WS (mean age 42), 16 typically developing adults (mean age 42) and 13 typically developing children (mean age 12) participated in the study. ERPs were recorded during a three-stimulus visual oddball task, during which participants were required to make a response to a rare target stimulus embedded in a train of frequent non-target stimuli.

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Dec
1969

To examine patterns of energy drink consumption by children and young people, attitudes towards these drinks, and any associations with health or other outcomes.
Rapid evidence assessment and narrative synthesis.
9 electronic bibliographic databases, reference lists of relevant studies and searches of the internet.

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Nov
2017

Despite well-documented attention deficits in children with intellectual and developmental disabilities (IDD), distinctions across types of attention problems and their association with academic attainment has not been fully explored. This study examines visual attention capacities and inattentive/hyperactive behaviours in 77 children aged 4 to 11 years with IDD and elevated behavioural attention difficulties. Children with autism spectrum disorder (ASD; n = 23), Down syndrome (DS; n = 22), and non-specific intellectual disability (NSID; n = 32) completed computerized visual search and vigilance paradigms.

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Oct
2016


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Mar
2017

Sensory atypicalities are a common feature of autism spectrum disorder (ASD). To date, the relationship between sensory atypicalities in dyads of children with ASD and their parents has not been investigated. Exploring these relationships can contribute to an understanding of how phenotypic profiles may be inherited, and the extent to which familial factors might contribute towards children's sensory profiles and constitute an aspect of the broader autism phenotype (BAP).

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Dec
1969

Personal space refers to a protective barrier that we strive to maintain around our body. We examined personal space regulation in young people with Williams syndrome (WS) and their typically developing, chronological age-matched peers using a parent report questionnaire and a stop-distance paradigm. Individuals with WS were reported by their parents to be more likely to violate the personal space of others, and indeed they maintained a shorter interpersonal distance in the stop-distance paradigm.

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May
2017

The present study compared the course of parent-report and actigraphy-derived sleep profiles over a 1-year period, in school-age children with autism spectrum disorder and typically developing children. The Children's Sleep Habits Questionnaire and 14 nights of actigraphy were used to assess sleep profiles. Parents also completed the Spence Children's Anxiety Scale, the Social Worries Questionnaire and the Bedtime Routines Questionnaire.

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Dec
2015

Interpersonal distance regulation is crucial for successful social interactions. We investigated personal space awareness in Williams syndrome (WS) and autism spectrum disorder (ASD) compared to typical development. Parents reported that individuals with WS and ASD were significantly more likely than those developing typically to invade the personal space of others.

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Dec
1969

Existing eye-tracking literature has shown that both adults and children with autism spectrum disorders (ASD) show fewer and slower fixations on faces. Despite this reduced saliency and processing of other faces, recognition of their own face is reported to be more "typical" in nature. This study uses eye-tracking to explore the typicality of gaze patterns when children with ASD attend their own faces compared to other familiar and unfamiliar faces.

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Jul
2014

Previous research exploring declarative memory in Williams syndrome (WS) has revealed impairment in the processing of episodic information accompanied by a relative strength in semantic ability. The aim of the current study was to extend this literature by examining how relatively spared semantic memory may support episodic remembering. Using a level of processing paradigm, older adults with WS (aged 35-61 years) were compared to typical adults of the same chronological age and typically developing children matched for verbal ability.

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Dec
2013

The neurodevelopmental disorder Williams syndrome (WS) has been associated with a social phenotype of hypersociability, non-social anxiety and an unusual attraction to faces. The current study uses eye tracking to explore attention allocation to emotionally expressive faces. Eye gaze and behavioural measures of anxiety and social reciprocity were investigated in adolescents and adults with WS when compared to typically developing individuals of comparable verbal mental age (VMA) and chronological age (CA).

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May
2014

The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore the profiles of social behaviour and anxiety across a broad age range of individuals with the disorder (n = 59, ages 6-36 years).

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Dec
2013

Individuals with the neuro-developmental disorder Williams syndrome (WS) are characterised by a combination of features which makes this group vulnerable socially, including mild-moderate cognitive difficulties, pro-social drive, and indiscriminate trust. The purpose of this study was to explore a key socio-communicative skill in individuals with WS, namely, mental state recognition abilities. We explored this skill in a detailed way by looking at how well individuals with WS recognise complex everyday mental states, and how they allocate their attention while making these judgements.

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Nov
2013

Research exploring cognitive processing associated with Williams Syndrome (WS) has suggested that executive functioning deficits exist across the developmental spectrum. Such executive functions include problem solving, planning, dividing attention and inhibiting responses. Within a framework of executive functions, the aim of the current study was to explore attentional lapse and inhibition skills in older adults with WS (n=20; aged 36-61 yr) and consider the implications of deficits within this group.

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May
2013

From a young age the typical development of social functioning relies upon the allocation of attention to socially relevant information, which in turn allows experience at processing such information and thus enhances social cognition. As such, research has attempted to identify the developmental processes that are derailed in some neuro-developmental disorders that impact upon social functioning. Williams syndrome (WS) and autism are disorders of development that are characterized by atypical yet divergent social phenotypes and atypicalities of attention to people.

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Mar
2013

The developmental disorder of Williams syndrome (WS) is associated with an overfriendly personality type, including an increased tendency to approach strangers. This atypical social approach behaviour (SAB) has been linked to two potential theories: the amygdala hypothesis and the frontal lobe hypothesis. The current study aimed to investigate heterogeneity of SAB in WS by exploring whether subgroups of SAB profiles could be identified using cluster analytic techniques.

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Jan
2013

During face-to-face interactions typically developing individuals use gaze aversion (GA), away from their questioner, when thinking. GA is also used when individuals with autism (ASD) and Williams syndrome (WS) are thinking during question-answer interactions. We investigated GA strategies during face-to-face social style interactions with familiar and unfamiliar interlocutors.

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Nov
2013

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.

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Mar
2013

Recent research suggests that older adults have difficulties with aspects of configural face processing. The present study examined whether age-related declines in sensitivity to configural face information are dependent on the face region in which configural changes occur.
Younger and older adults completed a face-matching task that required the detection of configural manipulations to either the eye or the mouth regions of target faces.

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Feb
2013

This study explored the relationship between sensory processing abnormalities and repetitive behaviours in children with Williams Syndrome (WS; n = 21). This is a novel investigation bringing together two clinical phenomena for the first time in this neuro-developmental disorder. Parents completed the Sensory Profile (Short Form; Dunn in The sensory profile manual.

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May
2012

This research compared the abilities of children and adults to perceive an optimal tempo for pieces of music. Participants heard eight melodies played at a range of tempi and made a 2AFC of "too fast" or "too slow" for each presentation. Children (aged between 5 to 11 years) and adults (aged between 17 to 54 years) showed the same variation in perceived optimal tempi across melodies.

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Mar
2012

Visual communication cues facilitate interpersonal communication. It is important that we look at faces to retrieve and subsequently process such cues. It is also important that we sometimes look away from faces as they increase cognitive load that may interfere with online processing.

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Dec
1969

The human face is a powerful elicitor of emotion, which induces autonomic nervous system responses. In this study, we explored physiological arousal and reactivity to affective facial displays shown in person and through video-mediated communication. We compared measures of physiological arousal and reactivity in typically developing individuals and those with the developmental disorders Williams syndrome (WS) and autism spectrum disorder (ASD).

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Apr
2012

  During face-to-face questioning, typically developing children and adults use gaze aversion (GA), away from their questioner, when thinking. GA increases with question difficulty and improves the accuracy of responses. This is the first study to investigate whether individuals with autism spectrum disorder (ASD; associated with reduced sociability and atypical face gaze) and Williams syndrome (WS; associated with hypersociability and atypical face gaze) use GA to manage cognitive load during face-to-face interactions.

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Nov
2011

The present study investigated verbal and spatial working memory (WM) functioning in individuals with the neuro-developmental disorder Williams syndrome (WS) using WM component tasks. While there is strong evidence of WM impairments in WS, previous research has focused on short-term memory and has neglected assessment of executive components of WM. There is a particular lack of consensus concerning the profile of verbal WM functioning in WS.

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Apr
2012

Individuals with autism have difficulties interpreting face cues that contribute to deficits of social communication. When faces need to be processed for meaning they fail to capture and hold the attention of individuals with autism. In the current study we illustrate that faces fail to capture attention in a typical manner even when they are non-functional to task completion.

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Feb
2012

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.

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Dec
1969

Williams syndrome.

Adv Child Dev Behav 2010 ;39:163-209
Deborah M Riby, Melanie A Porter

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Sep
2011

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.

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Jan
2011

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.

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Jun
2010

The Williams syndrome (WS) social phenotype is characterised by a high level of social engagement, heightened empathy and prolonged attention to people's faces. These behaviours appear in contradiction to research reporting problems recognising and interpreting basic emotions and more complex mental states from other people. The current task involved dynamic (moving) face stimuli of an actor depicting complex mental states (e.

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Dec
2009

A developmental study is presented in which participants must detect the Thatcher illusion in order to match unfamiliar faces on identity. 114 participants between 6 and 67 years of age completed a matching task whereby face pairs were presented upright or under inversion. At all ages, participants were more accurate matching upright than inverted faces.

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Apr
2010


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Apr
2010

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).

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Nov
2009

. During face-to-face questioning typically developing children and adults use gaze aversion (GA), away from their questioner, when thinking. GA increases with question difficulty and improves the accuracy of responses.

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Mar
2008

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.

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Jul
2009

In working memory (WM) span tests participants have to maintain to-be-remembered information while processing other, potentially distracting, information. Previous studies have shown that WM span scores are greater when span lists start with a long processing task and end with a short processing task than when these processing tasks are presented in the reverse order (e.g.

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Mar
2009

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.

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Jan
2009

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.

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Sep
2008

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.

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Nov
2008

Interventions aimed at improving glucose regulatory mechanisms have been suggested as a possible source of cognitive enhancement in the elderly. In particular, previous research has identified episodic memory as a target for facilitation after either moderate increases in glycaemia (after a glucose drink) or after improvements in glucose regulation. The present study aimed to extend this research by examining the joint effects of glucose ingestion and glucose regulation on cognition.

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Jan
2008

Familiar and unfamiliar face perception is typically dissociated by the relative use of internal and external face features. The Williams syndrome (WS) social phenotype emphasises hypersociability, with an interest in interacting with people irrespective of familiarity. The aim is to explore whether unfamiliar face processing is characterised by the typical dissociation between internal and external features in WS, or whether the social stimulus drive towards strangers is linked to atypicalities of unfamiliar face processing.

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Sep
2007

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.

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Dec
2006

In two experiments we examined whether the allocation of attention in natural scene viewing is influenced by the gaze cues (head and eye direction) of an individual appearing in the scene. Each experiment employed a variant of the flicker paradigm in which alternating versions of a scene and a modified version of that scene were separated by a brief blank field. In Experiment 1, participants were able to detect the change made to the scene sooner when an individual appearing in the scene was gazing at the changing object than when the individual was absent, gazing straight ahead, or gazing at a nonchanging object.

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