Victoria Cole

Attention across modalities as a longitudinal predictor of early outcomes: the case of fragile X syndrome.

BACKGROUND   Fragile X syndrome (FXS) is an early diagnosed monogenic disorder, associated with a striking pattern of cognitive/attentional difficulties and a high risk of poor behavioural outcomes. FXS therefore represents an ideal model disorder to study prospectively the impact of early attention deficits on behaviour. METHODS   Thirty-seven boys with FXS aged 4-10 years and 74 typically developing (TD) boys took part. Study 1 was designed to assess visual and auditory attention at two time-points, 1 year apart. Study 2 investigated attention to multimodal information. Both tested attention markers as longitudinal predictors of risk for poor behaviour in FXS. RESULTS   Children with FXS attended less well than mental-age matched TD boys and experienced greater difficulties with auditory compared to visual stimuli. In addition, unlike TD children, they did not benefit from multimodal information. Attention markers were significant predictors of later behavioural difficulties in boys with FXS. CONCLUSIONS   Findings demonstrate, for the first time, greater difficulties with auditory attention and atypical processing of multimodal information, in addition to pervasive global attentional difficulties in boys with FXS. Attention predicted outcomes longitudinally, underscoring the need to dissect what drives differing developmental trajectories for individual children within a seemingly homogeneous group.

Does Attention Constrain Developmental Trajectories in Fragile X Syndrome? A 3-Year Prospective Longitudinal Study.

Basic attentional processes and their impact on developmental trajectories in fragile X syndrome were assessed in a 3-year prospective study. Although fragile X syndrome is a monogenic X-linked disorder, there is striking variability in outcomes even in young boys with the condition. Attention is a key factor constraining interactions with the environment, so it is a perfect candidate to predict trajectories in cognitive and behavioral outcomes. In this study, 48 boys with fragile X syndrome were assessed 3 times over 24 months. Although nonverbal IQ declined, there were significant improvements in nonverbal growth scores and in cognitive attention. In contrast, behavioral difficulties (i.e., autistic symptomatology, hyperactivity-inattention) remained stable over this time frame. Attentional markers in the visual and auditory modalities predicted intellectual abilities and classroom behavior, whereas auditory markers alone predicted autistic symptomatology.

Perceptual grouping and distance estimates in typical and atypical development: Comparing performance across perception, drawing and construction tasks

Perceptual grouping is a pre-attentive process which serves to group local elements into global wholes, based on shared properties. One effect of perceptual grouping is to distort the ability to estimate the distance between two elements. In this study, biases in distance estimates, caused by four types of perceptual grouping, were measured across three tasks, a perception, a drawing and a construction task in both typical development (TD: Experiment 1) and in individuals with Williams syndrome (WS: Experiment 2). In Experiment 1, perceptual grouping distorted distance estimates across all three tasks. Interestingly, the effect of grouping by luminance was in the opposite direction to the effects of the remaining grouping types. We relate this to differences in the ability to inhibit perceptual grouping effects on distance estimates. Additive distorting influences were also observed in the drawing and the construction task, which are explained in terms of the points of reference employed in each task. Experiment 2 demonstrated that the above distortion effects are also observed in WS. Given the known deficit in the ability to use perceptual grouping in WS, this suggests a dissociation between the pre-attentive influence of and the attentive deployment of perceptual grouping in WS. The typical distortion in relation to drawing and construction points towards the presence of some typical location coding strategies in WS. The performance of the WS group differed from the TD participants on two counts. First, the pattern of overall distance estimates (averaged across interior and exterior distances) across the four perceptual grouping types, differed between groups. Second, the distorting influence of perceptual grouping was strongest for grouping by shape similarity in WS, which contrasts to a strength in grouping by proximity observed in the TD participants.

Face processing in Williams syndrome is already atypical in infancy

Face processing is a crucial socio-cognitive ability. Is it acquired progressively or does it constitute an innately-specified, face-processing module? The latter would be supported if some individuals with seriously impaired intelligence nonetheless showed intact face-processing abilities. Some theorists claim that Williams syndrome (WS) provides such evidence since, despite IQs in the 50s, adolescents/adults with WS score in the normal range on standardized face-processing tests. Others argue that atypical neural and cognitive processes underlie WS face-processing proficiencies. But what about infants with WS? Do they start with typical face-processing abilities, with atypicality developing later, or are atypicalities already evident in infancy? We used an infant familiarization/novelty design and compared infants with WS to typically developing controls as well as to a group of infants with Down syndrome matched on both mental and chronological age. Participants were familiarized with a schematic face, after which they saw a novel face in which either the features (eye shape) were changed or just the configuration of the original features. Configural changes were processed successfully by controls, but not by infants with WS who were only sensitive to featural changes and who showed syndrome-specific profiles different from infants with the other neurodevelopmental disorder. Our findings indicate that theorists can no longer use the case of WS to support claims that evolution has endowed the human brain with an independent face-processing module.

A longitudinal study of perceptual grouping by proximity, luminance and shape in infants at two, four and six months

Grouping by luminance and shape similarity has previously been demonstrated in neonates and at 4 months, respectively. By contrast, grouping by proximity has hitherto not been investigated in infancy. This is also the first study to chart the developmental emergence of perceptual grouping longitudinally. Sixty-one infants were presented with a matrix of local stimuli grouped horizontally or vertically by luminance, shape or proximity at 2, 4, and 6 months. Infants were exposed to each set of stimuli for three presentation durations. Grouping was demonstrated for luminance similarity at the earliest testing age, 2 months, by shape similarity at 4 months, but was not observed for grouping by proximity. Grouping by shape similarity showed a distinctive pattern of grouping ability across exposure durations, which reflected familiarity preferences followed by novelty preferences. This remained stable across age. No link was found between the emergence of perceptual grouping ability and the exposure duration required to elicit grouping. We conclude by stressing the importance of longitudinal studies of infant development in furthering our understanding of human cognition, rather than relying on assumptions from the adult endstate.

Do behavioural inattention and hyperactivity exacerbate cognitive difficulties associated with autistic symptoms? Longitudinal profiles in fragile X syndrome

Abstract Developmental trajectories of autism spectrum disorder (ASD) or attention deficit and hyperactivity disorder (ADHD) comorbidity in genetically determined neurodevelopmental disorders are not well understood. The objective of the current longitudinal study was to explore symptomology and comorbidity in boys with fragile X syndrome focusing on their profiles of autistic and inattentive behaviours in performance on the Leiter R Performance Scale, a non-verbal IQ measure. Of the 46 children, 10 were classified as having combined ASD and ADHD symptoms, 14 met the criterion for autism alone, 10 met the criterion for ADHD alone, and 12 had no symptoms for either. Results indicated that changes in cognitive profiles over time were similar for all groups and there was a slight improvement in growth scores suggesting developmental change. In terms of ASD and ADHD symptomology, we also found stable differences across groups over time. Collectively, these findings highlight the pressing need to explore symptomology longitudinally across disorders at high risk from comorbid ASD and ADHD diagnoses.

The Development of Perceptual Grouping in Infants with Williams Syndrome

Perceptual grouping by luminance similarity and by proximity was investigated in infants with Williams syndrome (WS) aged between 6 and 36 months (visit 1, N=29). WS infants who were still under 36 months old, 8 months later, repeated the testing procedure (visit 2, N=15). Performance was compared to typically developing (TD) infants aged from 2 to 20 months (N=63). Consistent with the literature, TD participants showed grouping by luminance at the youngest testing age, 2 months. Grouping by proximity had not previous been charted in typical development: this study showed grouping by proximity at 8 months. Infants with WS could group by luminance. Developmental progression of the WS group showed some similarities to typical development, although further investigation is required to further address this in more depth. In contrast, infants with WS were not able to group by proximity. This pattern of emergence and development of grouping abilities is considered in relation to the pattern of grouping abilities observed in adults with WS.

Erratum to: KCTU randomisation and IMP management system

The online version of the original article can be found under doi:10.1186/1745-6215-14-S1-P63.