Search

Low inhibitory control (IC) is sometimes associated with enhanced problem-solving amongst adults, yet for young children high IC is primarily framed as inherently better than low IC. Here, we explore associations between IC and performance on a novel problem-solving task, amongst 102 English 2- and 3-year-olds (Study 1) and 84 Swedish children, seen at 18-months and 4-years (Study 2). Generativity during problem-solving was negatively associated with IC, as measured by prohibition-compliance (Study 1, both ages, Study 2 longitudinally from 18-months). High parent-reported IC was associated with poorer overall problem-solving success, and greater perseveration (Study 1, 3-year-olds only). Benefits of high parent-reported IC on persistence could be accounted for by developmental level. No concurrent association was observed between problem-solving performance and IC as measured with a Delay-of-Gratification task (Study 2, concurrent associations at 4-years). We suggest that, for young children, high IC may confer burden on insight- and analytic-aspects of problem-solving.

Typically developing (TD) infants adapt to the social world in part by shifting the focus of their processing resources to the relevant aspects of a visual scene. Any impairment in visual orienting may therefore constrain learning and development in domains such as language. However, although something is known about visual orienting in infants at risk of autism, very little is known about it in infants/toddlers with other neurodevelopmental disorders. This is partly because previous studies focused on older children and rarely compared the children to "both" chronological age (CA)- and mental age (MA)-matched TD controls. Yet, if visual orienting is important for learning and development, then it is imperative to investigate it "early in development" and ascertain whether it relates to higher level cognitive functions such as language. We used eye-tracking technology to directly compare visual orienting in infants/toddlers with one of three neurodevelopmental disorders--Down syndrome (DS), fragile X syndrome (FXS) and Williams syndrome (WS)--matched on CA or MA to TD controls (~15 months). We also measured language ability using the Mullen Scales of Early Learning (MSEL). We found that the ability to "disengage" attention from a visual stimulus in order to shift it to another visual stimulus is related to language ability in infants/toddlers irrespective of group affiliation. We also found that, contrary to the literature, infants and toddlers with DS (but not WS) are slow at disengaging attention. Our data suggest that orienting attention constrains language development and is impaired in DS.

Background: Studies in Down syndrome (DS) and Williams syndrome (WS) have suggested that mathematical abilities are impaired. However, it is unclear which domain-general or domain-specific abilities impact on mathematical development in these developmental disorders. Method: The current study examined the foundations of mathematical development across participants with WS (n = 24) and DS (n = 26) compared to typically developing (TD) children (n = 26) in relation to domain-general (i.e., general intelligence and visuospatial abilities) and domain-specific abilities (non-symbolic and symbolic number abilities). Results: Developmental trajectories showed that mathematical abilities were delayed in line with overall mental age in DS and WS. Whilst visuospatial abilities predicted performance for DS and TD participants, this was not the case for the WS group, instead Approximate Number Sense abilities predicted mathematical development. Conclusions: These findings suggest that those with DS and WS may benefit from different mathematical intervention programmes.

Previous studies suggest that tasks dependent on the mental number line may be difficult for Williams Syndrome (WS) and Down Syndrome (DS) groups. However, few have directly assessed number line estimation in these groups. The current study assessed 28 WS, 25 DS and 25 typically developing (TD) participants in non-verbal intelligence, number familiarity, visuo-spatial skills and number line estimation. Group comparisons indicated no differences in number line estimation. However, the WS group displayed difficulties with visuo-spatial skills and the DS group displayed difficulties with number familiarity. Differential relationships between number line estimation and visuo-spatial/number familiarity skills were observed across groups. Data is discussed in the context of assessment of skills in neurodevelopmental disorders.

It has been reported that approximate number sense (ANS) task performance is impaired in individuals with Williams syndrome (WS) and Down syndrome (DS). Research with infants has suggested this impairment is caused by sticky fixation in WS and sustained attention deficits for those with DS. This study examined looking patterns of older children and adults with WS (n = 24) and DS (n = 23) during an ANS task compared to typically developing controls matched for chronological age and those matched for mental age. Results showed that, although there were no group differences, looking patterns changed with chronological age for both the WS and DS groups. Looking behaviour related to ANS performance only in the WS group. Implications for interventions are discussed.

Sleep plays a key role in the consolidation of newly acquired information and skills into long term memory. Children with Down syndrome (DS) and Williams syndrome (WS) frequently experience sleep problems, abnormal sleep architecture, and difficulties with learning; thus, we predicted that children from these clinical populations would demonstrate impairments in sleep-dependent memory consolidation relative to children with typical development (TD) on a cognitive procedural task: The Tower of Hanoi. Children with DS (n = 17), WS (n = 22) and TD (n = 34) completed the Tower of Hanoi task. They were trained on the task either in the morning or evening, then completed it again following counterbalanced retention intervals of daytime wake and night time sleep. Children with TD and with WS benefitted from sleep for enhanced memory consolidation and improved their performance on the task by reducing the number of moves taken to completion, and by making fewer rule violations. We did not find any large effects of sleep on learning in children with DS, suggesting that these children are not only delayed, but atypical in their learning strategies. Importantly, our findings have implications for educational strategies for all children, specifically considering circadian influences on new learning and the role of children's night time sleep as an aid to learning.

Abstract Background Down syndrome (DS) is associated with variable intellectual disability and multiple health and psychiatric comorbidities. The impact of such comorbidities on cognitive outcomes is unknown. We aimed to describe patterns of physical health and psychiatric comorbidity prevalence, and receptive language ability, in DS across the lifespan, and determine relationships with cognitive outcomes. Methods Detailed medical histories were collected and cognitive abilities measured using standardised tests for 602 individuals with DS from England and Wales (age range 3 months to 73 years). Differences in prevalence rates between age groups and between males and females were determined using chi-squared or Fisher’s exact tests. In adults, rates for psychiatric comorbidities were compared to expected population rates using standardised morbidity ratios (SMRs). Adapted ANCOVA functions were constructed to explore age and sex associations with receptive language ability across the lifespan, and regression analyses were performed to determine whether the presence of health comorbidities or physical phenotypes predicted cognitive abilities. Results Multiple comorbidities showed prevalence differences across the lifespan, though there were few sex differences. In adults, SMRs were increased in males and decreased in females with DS for schizophrenia, bipolar disorder, and anxiety. Further, SMRs were increased in both males and females with DS for dementia, autism, ADHD, and depression, with differences more pronounced in females for dementia and autism, and in males for depression. Across the lifespan, receptive language abilities increasingly deviated from age-typical levels, and males scored poorer than females. Only autism and epilepsy were associated with poorer cognitive ability in those aged 16–35 years, with no relationships for physical health comorbidities, including congenital heart defects. Conclusions Our results indicate the prevalence of multiple comorbidities varies across the lifespan in DS, and in adults, rates for psychiatric comorbidities show different patterns for males and females relative to expected population rates. Further, most health comorbidities are not associated with poorer cognitive outcomes in DS, apart from autism and epilepsy. It is essential for clinicians to consider such differences to provide appropriate care and treatment for those with DS and to provide prognostic information relating to cognitive outcomes in those with comorbidities.

Previous studies suggest that tasks dependent on the mental number line may be difficult for Williams Syndrome (WS) and Down Syndrome (DS) groups. However, few have directly assessed number line estimation in these groups. The current study assessed 28 WS, 25 DS and 25 typically developing (TD) participants in non-verbal intelligence, number familiarity, visuo-spatial skills and number line estimation. Group comparisons indicated no differences in number line estimation. However, the WS group displayed difficulties with visuo-spatial skills and the DS group displayed difficulties with number familiarity. Differential relationships between number line estimation and visuo-spatial/number familiarity skills were observed across groups. Data is discussed in the context of assessment of skills in neurodevelopmental disorders., Author(s): V. Simms [sup.1] , A. Karmiloff-Smith, E. Ranzato [sup.2] , J. Van Herwegen [sup.3] Author Affiliations: (1) grid.12641.30, 0000000105519715, School of Psychology, Ulster University, , Coleraine, UK (2) grid.15538.3a, [...]

It has been reported that approximate number sense (ANS) task performance is impaired in individuals with Williams syndrome (WS) and Down syndrome (DS). Research with infants has suggested this impairment is caused by sticky fixation in WS and sustained attention deficits for those with DS. This study examined looking patterns of older children and adults with WS (n = 24) and DS (n = 23) during an ANS task compared to typically developing controls matched for chronological age and those matched for mental age. Results showed that, although there were no group differences, looking patterns changed with chronological age for both the WS and DS groups. Looking behaviour related to ANS performance only in the WS group. Implications for interventions are discussed., Author(s): Jo Van Herwegen [sup.1] , Erica Ranzato [sup.2] , Annette Karmiloff-Smith, Victoria Simms [sup.3] Author Affiliations: (Aff1) 0000000121901201, grid.83440.3b, Department of Psychology and Human Development, UCL Institute of Education, [...]

Genetic syndrome groups at high risk of autism comorbidity, like Down syndrome and fragile X syndrome, have been presented as useful models for understanding risk and protective factors involved in the emergence of autistic traits. Yet despite reaching clinical thresholds, these 'syndromic' forms of autism appear to differ in significant ways from the idiopathic or 'non-syndromic' autism profile. We explore alternative mechanistic explanations for these differences and propose a developmental interpretation of syndromic autism that takes into account the character of the genetic disorder. This interpretation anticipates syndrome-specific autism phenotypes, since the neurocognitive and behavioural expression of the autism is coloured by syndromically defined atypicalities. To uncover the true nature of comorbidities and of autism per se, we argue that it is key to extend definitions of autism to include the perceptual and neurocognitive characteristics of the disorder and then apply this multilevel conceptualisation to the study of syndromic autism profiles.

In executing purposeful actions, adults select sufficient and necessary limbs. But infants often move goal-irrelevant limbs, suggesting a developmental process of motor specialization. Two experiments with 9- and 12-month-olds revealed gradual decreases in extraneous movements in non-acting limbs during unimanual actions. In Experiment 1, 9-month-olds produced more extraneous movements in the non-acting hand/arm and feet/legs than 12-month-olds. In Experiment 2, analysis of the spatiotemporal dynamics of infants' movements revealed developmental declines in the spatiotemporal coupling of movements between acting and non-acting arms. We also showed that the degree of specialization in infants' unimanual actions is associated with individual differences in motor experience and visual attention, indicating the experience-dependent and broad functional nature of these developmental changes. Our study provides important new insights into motor development: as in cognitive domains, motor behaviours are initially broadly tuned to their goal, becoming progressively specialized during the first year of life.

In order to understand how language abilities emerge in typically and atypically developing infants and toddlers, it is important to embrace complexity in development. In this paper, we describe evidence that early language development is an experience-dependent process, shaped by diverse, interconnected, interdependent developmental mechanisms, processes, and abilities (e.g. statistical learning, sampling, functional specialization, visual attention, social interaction, motor ability). We also present evidence from our studies on neurodevelopmental disorders (e.g. Down syndrome, fragile X syndrome, Williams syndrome) that variations in these factors significantly contribute to language delay. Finally, we discuss how embracing complexity, which involves integrating data from different domains and levels of description across developmental time, may lead to a better understanding of language development and, critically, lead to more effective interventions for cases when language develops atypically.

Sleep plays an active role in memory consolidation. Because children with Down syndrome (DS) and Williams syndrome (WS) experience significant problems with sleep and also with learning, we predicted that sleep-dependent memory consolidation would be impaired in these children when compared to typically developing (TD) children. This is the first study to provide a cross-syndrome comparison of sleep-dependent learning in school-aged children. Children with DS (n = 20) and WS (n = 22) and TD children (n = 33) were trained on the novel Animal Names task where they were taught pseudo-words as the personal names of ten farm and domestic animals, e.g. Basco the cat, with the aid of animal picture flashcards. They were retested following counterbalanced retention intervals of wake and sleep. Overall, TD children remembered significantly more words than both the DS and WS groups. In addition, their performance improved following night-time sleep, whereas performance over the wake retention interval remained stable, indicating an active role of sleep for memory consolidation. Task performance of children with DS did not significantly change following wake or sleep periods. However, children with DS who were initially trained in the morning continued to improve on the task at the following retests, so that performance on the final test was greater for children who had initially trained in the morning than those who trained in the evening. Children with WS improved on the task between training and the first retest, regardless of whether sleep or wake occurred during the retention interval. This suggests time-dependent rather than sleep-dependent learning in children with WS, or tiredness at the end of the first session and better performance once refreshed at the start of the second session, irrespective of the time of day. Contrary to expectations, sleep-dependent learning was not related to baseline level of performance. The findings have significant implications for educational strategies, and suggest that children with DS should be taught more important or difficult information in the morning when they are better able to learn, whilst children with WS should be allowed a time delay between learning phases to allow for time-dependent memory consolidation, and frequent breaks from learning so that they are refreshed and able to perform at their best.

This article outlines the "over-pruning hypothesis" of autism. The hypothesis originates in a neurocomputational model of the regressive sub-type (Thomas, Knowland & Karmiloff-Smith, 2011a, 2011b). Here we develop a more general version of the over-pruning hypothesis to address heterogeneity in the timing of manifestation of ASD, including new computer simulations which reconcile the different observed developmental trajectories (early onset, late onset, regression) via a single underlying atypical mechanism; and which show how unaffected siblings of individuals with ASD may differ from controls either by inheriting a milder version of the pathological mechanism or by co-inheriting the risk factors without the pathological mechanism. The proposed atypical mechanism involves overly aggressive synaptic pruning in infancy and early childhood, an exaggeration of a normal phase of brain development. We show how the hypothesis generates novel predictions that differ from existing theories of ASD including that (1) the first few months of development in ASD will be indistinguishable from typical, and (2) the earliest atypicalities in ASD will be sensory and motor rather than social. Both predictions gain cautious support from emerging longitudinal studies of infants at-risk of ASD. We review evidence consistent with the over-pruning hypothesis, its relation to other current theories (including C. Frith's under-pruning proposal; C. Frith, 2003, 2004), as well as inconsistent data and current limitations. The hypothesis situates causal accounts of ASD within a framework of protective and risk factors (Newschaffer et al., 2012); clarifies different versions of the broader autism phenotype (i.e. the implication of observed similarities between individuals with autism and their family members); and integrates data from multiple disciplines, including behavioural studies, neuroscience studies, genetics, and intervention studies.

In typical infants, the achievement of independent locomotion has a positive impact on the development of both small-scale and large-scale spatial cognition. Here we investigated whether this association between the motor and spatial domain: (1) persists into childhood and (2) is detrimental to the development of spatial cognition in individuals with motor deficits, namely, individuals with attention deficit hyperactivity disorder (ADHD) and individuals with Williams syndrome (WS). Despite evidence of a co-occurring motor impairment in many individuals with ADHD, little is known about the developmental consequences of this impairment. Individuals with WS demonstrate impaired motor and spatial competence, yet the relationship between these two impairments is unknown. Typically developing (TD) children (N = 71), individuals with ADHD (N = 51), and individuals with WS (N = 20) completed a battery of motor tasks, a measure of independent exploration, and a virtual reality spatial navigation task. Retrospective motor milestone data were collected for the ADHD and WS groups. Results demonstrated a relationship between fine motor ability and spatial navigation in the TD group, which could reflect the developmental impact of the ability to manually manipulate objects, on spatial knowledge. In contrast, no relationships between the motor and spatial domains were observed for the ADHD or WS groups. Indeed, while there was evidence of motor impairment in both groups, only the WS group demonstrated an impairment in large-scale spatial navigation. The motor-spatial relationship in the TD, but not the ADHD and WS groups, suggests that aspects of spatial cognition can develop via a developmental pathway which bypasses input from the motor domain.

Down syndrome (DS) is associated with intellectual disability and an ultra-high risk of developing dementia. Informant ratings are invaluable to assess abilities and related changes in adults with DS, particularly for those with more severe intellectual disabilities and/or cognitive decline. We previously developed the informant rated Cognitive Scale for Down Syndrome (CS-DS) to measure everyday cognitive abilities across memory, executive function, and language domains in adults with DS, finding CS-DS scores are a valid measure of general abilities, and are significantly lower for those with noticeable cognitive decline compared to those without decline. To further test the validity of the CS-DS in detecting changes associated with cognitive decline we collected longitudinal data across two time points, approximately 1.5–2 years apart, for 48 adults with DS aged 36 years and over. CS-DS total scores (78.83 ± 23.85 vs. 73.83 ± 25.35, p = 0.042) and executive function scores (46.40 ± 13.59 vs. 43.54 ± 13.60, p = 0.048) significantly decreased between the two time points, with scores in the memory domain trending towards a significant decrease (22.19 ± 8.03 vs. 20.81 ± 8.63, p = 0.064). Adults with noticeable cognitive decline at follow-up showed a trend to significantly greater change in total scores (7.81 ± 16.41 vs. 3.59 ± 16.79, p = 0.067) and significantly greater change in executive function scores (5.13 ± 9.22 vs. 1.72 ± 9.97, p = 0.028) compared to those without decline. Change in total scores showed significant correlations with change in scores from other informant measures of everyday adaptive abilities and symptoms associated with dementia, and participant assessment of general cognitive abilities (all p < 0.005), while change in memory scores (R2 = 0.28, p = 0.001) better predicted change in participant cognitive assessment scores than change in executive function (R2 = 0.15, p = 0.016) or language (R2 = 0.15, p = 0.018) scores. These results suggest informants may better detect changes in the executive function domain, while change in informant rated memory scores best predicts change in assessed cognitive ability. Alternatively, memory domain scores may be sensitive to changes across both early and late cognitive decline, whereas executive function domain scores are more sensitive to changes associated with later noticeable cognitive decline. Our results provide further support for the validity of the CS-DS to assess everyday cognitive abilities and to detect associated longitudinal changes in individuals with DS.