Visuospatial bias in line bisection in Williams syndrome.

BACKGROUND: Recently, a study using the subjective straight-ahead task showed that individuals with Williams syndrome (WS) present a bias in the representation of body perception. The aim of the present study is to examine the horizontal midline body representation in WS participants using the bisection line task, which is an important benchmark for an egocentric frame of reference. METHOD: Fifteen WS participants (mean age = 21.7 ± 9.5 years) were compared with two typical development control groups: one composed of 15 participants matched on chronological age and one composed of 15 children matched on mental age. The task consisted of dividing each line in a series of 18 lines into two equal halves by drawing a vertical mark with a pencil in the centre of the line. RESULTS: Individuals with WS presented a significant leftward bias in comparison to mental age and chronological age groups. CONCLUSIONS: The leftward deviation in WS could be linked to the body representation bias and difficulties in the development of the egocentric reference system. An early detection of such deviation should help in the development of targeted interventions for WS individuals to improve visual-spatial skills and learning.

Verbal short-term memory shows a specific association with receptive but not productive vocabulary measures in Down syndrome.

BACKGROUND: Verbal short-term memory (STM) capacity has been considered to support vocabulary learning in typical children and adults, but evidence for this link is inconsistent for studies in individuals with Down syndrome (DS). The aim of this study was explore the role of processing demands on the association between verbal STM and vocabulary measures in DS, by comparing receptive vocabulary measures with high STM processing demands to productive vocabulary measures with low STM processing demands. METHOD: Forty-seven adults with Down syndrome were administered receptive vocabulary and productive vocabulary tasks, as well as measures of verbal STM abilities and intellectual efficiency. RESULTS: Bayesian regression analyses showed that verbal STM abilities were strongly and specifically associated with receptive vocabulary measures but not productive lexical abilities after controlling for intellectual efficiency, and this is despite the fact that vocabulary abilities as measured by receptive and productive vocabulary tasks were closely associated. CONCLUSIONS: In Down syndrome, verbal STM abilities may be predictive of specific task demands associated with receptive vocabulary tasks rather than of vocabulary development per se.

How Flexible is the Use of Egocentric Versus Allocentric Frame of Reference in the Williams Syndrome Population?

OBJECTIVE: This study examined the spontaneous use of allocentric and egocentric frames of reference and their flexible use as a function of instructions. METHOD: The computerized spatial reference task created by Heiz and Barisnikov (2015) was used. Participants had to choose a frame of reference according to three types of instructions: spontaneous, allocentric and egocentric. The performances of 16 Williams Syndrome participants between 10 and 41 years were compared to those of two control groups (chronological age and non-verbal intellectual ability). RESULTS: The majority of Williams Syndrome participants did not show a preference for a particular frame of reference. When explicitly inviting participants to use an allocentric frame of reference, all three groups showed an increased use of the allocentric frame of reference. At the same time, an important heterogeneity of type of frame of reference used by Williams Syndrome participants was observed. CONCLUSION: Results demonstrate that despite difficulties in the spontaneous use of allocentric and egocentric frames of reference, some Williams Syndrome participants show flexibility in the use of an allocentric frame of reference when an explicit instruction is provided.

Where is the 'subjective straight ahead' in Williams syndrome?

BACKGROUND: Individuals with Williams Syndrome (WS) are known to have particular difficulties when performing visuo-spatial tasks, which could be related to their difficulties in using a specific reference system to determine spatial relations. The aim of the present study was to assess the internal representation of the body's sagittal plane, which is an important benchmark for an egocentric frame of reference. METHOD: The results of 18 WS individuals (mean age = 20.5 ± 9.2 years) on the subjective straight ahead (SSA) task were compared with those of two healthy control groups composed of 36 participants matched on chronological age matched on chronological age (CA) and 30 young children matched on non-verbal intellectual ability (YC). RESULTS: Individuals with WS showed a significant left deviation on the SSA body's sagittal plane representation compared with the chronological age control group and a marginal left deviation compared with the young children control group. A comparison with the objective SA (0°) showed a significant leftward deviation in the WS group but not in the two control groups. CONCLUSIONS: Individuals with WS showed a significant leftward deviation in the SSA task. This bias of the body's longitudinal axe representation could have a negative impact on the use of an egocentric reference system, which could be the cause for their difficulties in defining spatial relations (e.g. location and orientation) necessary for performing spatial tasks.

Visual-motor integration, visual perception and motor coordination in a population with Williams syndrome and in typically developing children.

BACKGROUND: Williams syndrome (WS) is characterised by severe deficits in visual-spatial abilities in contrast to relatively well-developed language abilities. There is very limited knowledge about visual-motor integration (VMI) in people with WS. METHOD: Twenty-six participants with WS aged 6 to 41 years were assessed with all three tests of the Beery-VMI test, namely the VMI test, the visual perception test (VP) and the motor coordination test (MC). Their results were compared with those of 154 typically developing children (TD) aged 4 to 12. RESULTS: No influence of age on the three tested abilities was found amongst the participants with WS in comparison with the TD children. The participants with WS scored similarly to the 5-year-old TD children in all three tasks; their scores on the VMI correlated with the results on the VP and MC tests, which were similar to those of the TD children. Finally, the scores on the non-verbal intelligence test (Raven Coloured Progressive Matrices) were highly predictive of the scores in the VMI and VP tests and partially explain the variance in the MC scores. CONCLUSIONS: The present study is the first to use all three tasks of the Beery-VMI test. For the TD children, the performances on the three subtests did not show the same developmental trajectory. In contrast, the participants with WS did not show the same developmental trajectory. The participants with WS exhibited poor performances on all tasks with scores comparable with the 5-year-old TD children. As high correlations between these abilities were observed, improving VP and MC could help the development of VMI, which in turn could improve visual-spatial abilities in individuals with WS.

[Detection of executive function disorders with a standard neurodevelopmental follow-up of premature children]. / Suivi neurodéveloppemental à 5 ans des extrêmes prématurés et détection des difficultés sur le plan des fonctions exécutives.

INTRODUCTION: A significant proportion of prematurely born children encounter behavioral difficulties, such as attention deficit or hyperactivity, which could be due to executive function disorders. AIMS: To examine whether the standard neurodevelopmental assessment offered to premature children in Switzerland recognizes executive function disorders. METHODS: The study population consisted of 49 children born before 29 weeks of gestation who were examined between 5 and 6 years of age with a standard assessment, with additional items to assess executive functioning. Children with severe neurodevelopmental impairment were excluded (mental retardation, cerebral palsy, autism). Standard assessment consisted in the Kaufman Assessment Battery for Children (K-ABC), which comprises three subscales: sequential processes (analysis of sequential information), simultaneous processes (global analysis of visual information), and composite mental processes (CMP) (result of the other two scales), as well as a behavioral evaluation using the standardized Strengths and Difficulties Questionnaire (SDQ). Executive functioning was assessed with tasks evaluating visual attention, divided attention, and digit memory as well as with a specialized questionnaire, the Behavior Rating Index of Executive Functions (BRIEF), which evaluates several aspects of executive function (regulation, attention, flexibility, working memory, etc). RESULTS: Children were divided according to their results on the three K-ABC scales (< or >85), and the different neuropsychological tasks assessing executive function were compared between the groups. The CMP did not differentiate children with executive difficulties, whereas a score < 85 on the sequential processes was significantly associated with worse visual and divided attention. There was a strong correlation between the SDQ and the BRIEF questionnaires. For both questionnaires, children receiving psychotherapy had significantly higher results. Children who presented behavioral problems assessed with the SDQ presented significantly higher scores on the BRIEF. CONCLUSION: A detailed analysis of the standard neurodevelopmental assessment allows the identification of executive function disorders in premature children. Children who performed below 85 on the sequential processes of the K-ABC had significantly more attentional difficulties on the neuropsychological tasks and therefore have to be recognized and carefully followed. Emotional regulation had a strong correlation with behavioral difficulties, which were suitably assessed with the SDQ, recognized by the families, and treated.

[Development of executive functions in preterm children]. / Développement des fonctions exécutives de l'enfant prématuré.

The rate of children born prematurely has increased considerably in the last few decades, and their developmental outcome remains of great concern. The literature on the impact of prematurity has reported a wide range of cognitive and behavioral problems that may be related to deficits in executive function (EF) skills. EF refers to a series of high-level processes (selective attention, inhibition, set shifting, working memory, planning, goal setting) that develop throughout childhood and adolescence and play an important role in cognitive and social development as well as in school achievement. EF skills have been linked to the prefrontal cortex, as well as to other neural networks and brain regions including the basal ganglia and cerebellum. This paper focuses on studies related to the development of EF and social behavior in children born preterm. The preschool period is a critical time to perform neuropsychological assessment in addition to IQ testing, and to detect the child's specific needs in order to adapt effective intervention to enhance the development of executive processes in these high-risk children.

Emotional reactivity at 12 months in very preterm infants born at <29 weeks of gestation.

The present study evaluated the socio-emotional development of very preterm born infants at 12 months corrected age. Forty-one infants born very preterm (<29 weeks of gestation) were compared to 22 infants born full term on a standardized behavioral assessment and a parental temperament questionnaire, both measuring emotional reactivity to joy, anger and fear, as well as sustained attention. The behavioral assessment showed that very preterm infants exhibited as much joy as full term infants during a joy-eliciting episode. However, they expressed a significantly higher reactivity in anger-eliciting situations and a reduced reactivity toward fear-eliciting situations. For all three emotion-eliciting situations, the preterm infants reacted with a higher level of motor activity. The preterm infants also exhibited a distinct attention pattern with a significantly higher initial attention level which declined rapidly throughout the episode. The questionnaire did not show any group differences. The clinical relevance of these results in terms of preliminary hallmarks of later behavioral difficulties such attention deficit/hyperactivity disorder are discussed as well as the inconsistencies observed between the questionnaire and the behavioral assessment.

Social reasoning skills in adults with Down syndrome: the role of language, executive functions and socio-emotional behaviour.

BACKGROUND: Although the prevalence of mental illness and behaviour problems is lower in adults with Down syndrome (DS) than in other populations with intellectual disabilities, they do present emotional and relational problems, as well as social integration difficulties. However, studies reporting on specific competences known to be central in developing appropriate social relationships (e.g. social reasoning, emotion processing, theory of mind) remain rare in the adult DS population and the mechanisms underlying these people's emotional and relational difficulties are unclear. METHOD: The present study investigated the ability to understand the appropriateness of others' social behaviour in 34 adults with DS, using the Social Resolution Task (SRT). Their results were compared with those of 34 typically developing (TD) children matched for gender and receptive vocabulary. The relationships among the SRT experimental task, cognitive competences (receptive and productive vocabulary, non-verbal reasoning, inhibition, selective attention) and a caregiver-rated measure of socio-emotional behaviour were examined in the DS group. RESULTS: The DS participants' global SRT scores did not differ from those of the controls. However, analyses of the SRT subscores revealed that the DS group identified significantly fewer inappropriate situations than the control group. Nevertheless, when they correctly identified the behaviour as inappropriate, they were as well as the controls to explain the rules underlying their responses. Regression analyses showed that receptive vocabulary and selective attention and a specific dimension of the socio-emotional profile (social relating skills) constituted the best predictors of the DS adults' performance on the SRT. CONCLUSIONS: The main findings show that the DS participants demonstrate relatively good social reasoning skills in comparison with TD children matched for verbal age. However, the two groups present distinctions in their response patterns, and the influence of cognitive variables on success on the SRT also appears different. While selective attention skills are found to be significant predictors for both groups, the influence of receptive vocabulary level is much stronger in the DS group. The implications of particular cognitive and socio-emotional factors for success on the SRT in this group are considered in more detail.

Attentional networks efficiency in preterm children.

Recent studies have reported specific executive and attentional deficits in preterm children. However, the majority of this research has used multidetermined tasks to assess these abilities, and the interpretation of the results lacks an explicit theoretical backdrop to better understand the origin of the difficulties observed. In the present study, we used the Child Attention Network Task (Child ANT; Rueda et al. 2004) to assess the efficiency of the alerting, orienting and executive control networks. We compared the performance of 25 preterm children (gestational age < or = 32 weeks) to 25 full-term children, all between 5(1/2) and 6(1/2) years of age. Results showed that, as compared to full-term children, preterm children were slower on all conditions of the Child ANT and had a specific deficit in executive control abilities. We also observed a significantly higher correlation between the orienting and executive control networks in the preterm group, suggesting less differentiation of these two networks in this population.

An investigation of verbal short-term memory and phonological processing in four children with Williams syndrome.

Abstract Although phonological processing is generally considered to be a proficiency in Williams syndrome (WS), there are very few studies which have extensively explored phonological processing abilities in WS. In this study, we re-assessed phonological processing in WS by exploring verbal STM and phonological awareness abilities in 4 children with WS (CA: 10-12 years) and two control groups, one matched for chronological age (CA) and the other matched for verbal mental age (VA). Our results confirm and extend previous claims of preserved phonological STM in WS by showing specifically preserved STM performance for non-words, compared to both VA and CA control groups. However, we observed that this was the case only for non-words where support of phonological and lexico-semantic knowledge was minimized, with reduced phonological and lexico-semantic effects on STM performance. Furthermore, a more direct assessment of phonological processing abilities through phonological awareness tasks showed impaired performance for the 4 WS children. Our data confirm that STM for non-words represents a real strength in WS but they do not support previous assumptions of a more general preservation of phonological processing abilities in WS. Implications for impaired and preserved cognitive processes underlying verbal STM and phonological awareness abilities in WS are discussed.