Social perception in children with fetal alcohol spectrum disorder

Although the profile of social cognitive difficulties is well recognized in children with certain neurodevelopmental disorders such as autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD), this profile is not as well established in other clinical pediatric populations. The objective of the present study is to examine patterns of social perception in children with fetal alcohol spectrum disorder (FASD) compared to typically-developing (TD) control children. A total of 56 children between 8 and 12 years of age—35 with FASD and 21 TD—completed the Reading the Mind in the Eyes Task – Children’s Version (RMET-C). The RMET-C accuracy scores were compared between groups and also by item difficulty and emotional valence. The relation between cognitive functioning, age, FASD severity, and RMET-C performance was also investigated. The children in the FASD group did not perform as well as the children in the TD group on the RMET-C Total score and Easy items, as well as the Positive, Negative, and Neutral emotional valence items. When age and IQ were investigated, there was a significant effect of age on the Positive items in the TD group, with scores increasing with age. With regard to FASD severity, children with alcohol-related neurodevelopmental disorder were outperformed by children with full/partial fetal alcohol syndrome on the Positive and Negative items. Overall, these results further the understanding of the social cognitive profile in children with FASD and how this profile relates to other childhood-onset neurodevelopmental disorders.     

Psychopathology and behavior problems in children and adolescents with Williams syndrome: Distinctive relationships with cognition

Several studies have documented the high prevalence of psychopathology and behavior problems in Williams syndrome (WS). However, the links between cognitive development and such symptoms need further clarification. Our study aims to expand current knowledge on levels of behavior problems and its links to cognition in a sample of Brazilian individuals with WS. A total of 25 children and adolescents with WS and their parents participated in this study. The participants’ IQs were assessed with the Wechsler Scales of Intelligence (for children or adults) and parental reports of psychopathology/behavior problems were collected using the Child Behavior Checklist (CBCL). The presence of clinically significant attention problems was a main feature in our sample of children and adolescents with WS. In the children, higher IQ scores were found to be significantly associated with less externalizing problems, while in the adolescents cognitive abilities were found to be associated with less internalizing symptoms. These results provide further insight into the links between psychopathology and behavior problems and cognitive abilities in WS, and suggest the need to take age into consideration when analyzing such relationships.     

Phonological storage and executive function deficits in children with mathematics difficulties

Children with mathematics difficulties suffer from working memory deficits. This study investigated the deficit profile of phonological storage and executive functions in working memory among children with mathematics difficulties. Based on multiple instruments and two assessment points, 68 children were screened out of 805 fifth graders. Of these 68 children, 18 were classified as children with only mathematics difficulties (MD), 20 were classified as children with mathematics and reading difficulties (MDRD), and 30 were typically developing (TD) peers matched on age and general ability. Measures for phonological storage, dual-task performance, inhibition, and updating of verbal and numerical materials were administered individually. Results showed that compared with the TD group, children with MD exhibited storage and inhibition deficits specific to numerical information and dual-task deficits of both verbal and numerical information, whereas children with MDRD showed extensive deficits on phonological storage and executive functions on both verbal and numerical tasks. Moreover, executive function deficits were not confined to phonological storage deficits. Implications of the findings for the working memory deficit profile and working memory training among children with mathematics difficulties were discussed.     

Unfamiliar face recognition in children with autistic spectrum disorders

We investigated unfamiliar face recognition in low‐functioning children with autistic spectrum disorders (ASD) using a ‘part‐of‐face’ method. This method has not previously been used for unfamiliar faces with this population. The ‘part‐of‐face’ procedure provides measures of both face recognition accuracy and of processing style. We compared the performance of the children with ASD with three control groups: children with developmental delay (DD), typically developing (TD) children matched for verbal cognitive ability and TD children matched for chronological age (CA). Compared to the DD group, the ASD group showed similar processing in recognition accuracy and processing style. Compared to the TD children, the ASD group did not show the same level of accuracy as controls of the same CA, instead showing similar performance to younger TD children. However, as both children with ASD and DD showed the same performance, no ASD‐specific deficit was found. Copyright © 2009 John Wiley & Sons, Ltd.     

Brief report: Response inhibition and processing speed in children with motor difficulties and developmental coordination disorder

A previous study reported that children with poor motor skills, classified as having motor difficulties (MD) or Developmental Coordination Disorder (DCD), produced more errors in a motor response inhibition task compared to typically developing (TD) children but did not differ in verbal inhibition errors. The present study investigated whether these groups differed in the length of time they took to respond in order to achieve these levels of accuracy, and whether any differences in response speed could be explained by generally slow information processing in children with poor motor skills. Timing data from the Verbal Inhibition Motor Inhibition test were analyzed to identify differences in performance between the groups on verbal and motor inhibition, as well as on processing speed measures from standardized batteries. Although children with MD and DCD produced more errors in the motor inhibition task than TD children, the current analyses found that they did not take longer to complete the task. Children with DCD were slower at inhibiting verbal responses than TD children, while the MD group seemed to perform at an intermediate level between the other groups in terms of verbal inhibition speed. Slow processing speed did not account for these group differences. Results extended previous research into response inhibition in children with poor motor skills by explicitly comparing motor and verbal responses, and suggesting that slow performance, even when accurate, may be attributable to an inefficient way of inhibiting responses, rather than slow information processing speed per se.     

Delineation of a spatial working memory profile using a non-verbal eye-tracking paradigm in young children with autism and Williams syndrome

Working memory deficits profoundly inhibit children’s ability to learn. While deficits have been identified in disorders such as autism spectrum disorder (ASD) and Williams syndrome (WS), findings are equivocal, and very little is known about the nature of these deficits early in development. A major barrier to advances in this area is the availability of tasks suitable for young children with neurodevelopmental disorders who experience difficulties with following verbal instructions or who are distressed by formal testing demands. To address these issues, a novel eye-tracking paradigm was designed based on an adaptation of the classic A not B paradigm in order to examine the early foundations of spatial working memory capabilities in 26 developmentally delayed preschool children with ASD, 18 age- and IQ-matched children with WS, and 19 age-matched typically-developing (TD) children. The results revealed evidence that foundational spatial working memory performance in ASD and WS was comparable with that of TD children. Performance was associated with intellectual ability in the ASD and TD groups, but not in the WS group. Performance was not associated with adaptive behavior in any group. These findings are discussed in the context of previous research that has been largely limited to older and substantially less developmentally delayed children with these neurodevelopmental disorders.     

The role of verbal and nonverbal memory in the Family Pictures Subtest: Data from children with specific language impairment

This study examined the contribution of verbal and visual memory to performance on the Family Pictures subtest of the Children’s Memory Scale. This subtest purports to assess declarative memory functioning in the visual/nonverbal domain. A total of 115 nine-year-old children participated in this study. Fifty-eight had specific language impairment (SLI), whilst the remaining 57 were typically developing (TD), with no history of language difficulties. Results showed that the children with SLI, who had intact declarative memory for visual but not verbal information, obtained significantly lower scores on the Family Pictures subtest when compared to the TD group. Regression analyses revealed that across the entire sample, individual differences on the Family Pictures subtest was best predicted by a measure of verbal working memory. These results question whether the Family Pictures subtest can be considered a measure of visual memory in pediatric populations. These results have implications for the interpretation of scores on this subtest regarding the nature of the types of neurocognitive difficulties children may exhibit.     

A cross‐syndrome study of the differential effects of sleep on declarative memory consolidation in children with neurodevelopmental disorders

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.     

Cross-linguistic assessment of verbal production from a narrative task in Williams Syndrome

Williams Syndrome (WS) is a disorder caused by a delection in chromosome 7, with a cognitive profile characterised by mild to moderate mental deficiency and difficulties in visual-spatial processing, which contrasts with a relative preservation of linguistic functions and narrative production. In this study, verbal performance was analysed in two groups of participants (N = 3), Portuguese and Spanish, genetically diagnosed with WS. Scores were low in Verbal IQ, as in Performance IQ. Narrative performance showed low coherence with respect to structure, process, and content. WS verbal performance was affected and there was no difference between both groups. We could not find any dissociation between cognitive functioning and linguistic production.     

How do individuals with Williams syndrome learn a route in a real‐world environment?

Individuals with Williams syndrome (WS) show a specific deficit in visuo‐spatial abilities. This finding, however, derives mainly from performance on small‐scale laboratory‐based tasks. This study investigated large‐scale route learning in individuals with WS and two matched control groups (moderate learning difficulty group [MLD], typically developing group [TD]). In a non‐labelling and a labelling (verbal information provided along the route) condition, participants were guided along one of two unfamiliar 1‐km routes with 20 junctions, and then retraced the route themselves (two trials). The WS participants performed less well than the other groups, but given verbal information and repeated experience they learnt nearly all of the turns along the route. The extent of improvement in route knowledge (correct turns) in WS was comparable to that of the control groups. Relational knowledge (correctly identifying spatial relationships between landmarks), compared with the TD group, remained poor for both the WS and the MLD group. Assessment of the relationship between performance on the large‐scale route‐learning task and that on three small‐scale tasks (maze learning, perspective taking, map use) showed no relationship for the TD controls, and only a few non‐specific associations in the MLD and WS groups.     

Neuropsychological characteristics of children with the 22q11 Deletion Syndrome: a descriptive analysis.

Previous reports of cognitive functioning in children with the 22q11 Deletion Syndrome have reported marked variability in IQ and achievement subtest scores. Studies have begun to explore neuropsychological function in 22q11 DS however results are inconsistent and the profile incomplete. We assessed 40 children ages 5-12 with 22q11 DS. Consistent with past results, visual-spatial memory was significantly lower than verbal memory. Differentially lowered scores were found only in visual attention, working memory and motor function. Contrary with some past results quantitative, verbal ability, and visual spatial memory scores were within 1 SD from the standardization sample mean. Motor behavior, not typically discussed with regard to 22q11 DS school-age children, may be critical to incorporate in neurocognitive studies of children with 22q11 DS. Implications of these findings are considered with regard to past results.     

Assessment of the global intelligence and selective cognitive capacities in preterm 8-year-old children

The aim of this study was to assess various cognitive abilities such as attention, IQ, reasoning, and memory related to academic achievement in 8- and 9-year-old preterm children. A total of 141 children were assessed. The preterm group (=37 weeks) comprised 63 children and was compared to 78 full-term children. Attention was evaluated using the d2 Selective Attention test, and the IQ by the L-M form of the Stanford-Binet Intelligence Scale, establishing a profile of abilities: perception, memory, comprehension, reasoning, and verbal fluency. Significant differences in IQ were found between the preterm and full-term children. Of the cognitive abilities assessed, the only significant differences were found in verbal fluency, with preterm boys showing lower verbal fluency scores than full-term children. In conclusion, all preterm groups have attention ability similar to that of full-term children. However, preterm children obtain lower scores in intelligence measures. In addition, preterm boys have verbal fluency difficulties. Taking into account the increase in preterm births, suitable intervention programs must be planned to attend the difficulties found.     

Basic number processing in children with specific learning disorders: Comorbidity of reading and mathematics disorders

As well as being the hallmark of mathematics disorders, deficits in number processing have also been reported for individuals with reading disorders. The aim of the present study was to investigate separately the components of numerical processing affected in reading and mathematical disorders within the framework of the Triple Code Model. Children with reading disorders (RD), mathematics disorders (MD), comorbid deficits (RD + MD), and typically developing children (TD) were tested on verbal, visual-verbal, and nonverbal number tasks. As expected, children with MD were impaired across a broad range of numerical tasks. In contrast, children with RD were impaired in (visual-)verbal number tasks but showed age-appropriate performance in nonverbal number skills, suggesting their impairments were domain specific and related to their reading difficulties. The comorbid group showed an additive profile of the impairments of the two single-deficit groups. Performance in speeded verbal number tasks was related to rapid automatized naming, a measure of visual-verbal access in the RD but not in the MD group. The results indicate that deficits in number skills are due to different underlying cognitive deficits in children with RD compared to children with MD: a phonological deficit in RD and a deficit in processing numerosities in MD.     

Fading-figure tracing in Williams syndrome

Williams syndrome (WS) is a neurodevelopmental disorder characterized by severe impairment of visuospatial abilities. Figure-drawing abilities, which are thought to reflect visuospatial abilities, have yet to be fully investigated in WS. The purpose of the present study was to clarify whether drawing abilities differ between WS individuals and typically developing children (TD). We compared the performance of two groups of subjects (WS, mean age 16 years; TD, 5-6 years of age) using a fading-figure tracing task that requires subjects to trace a target figure that is gradually disappearing from a PC screen. Although the TD group exhibited clearly improved performance with long fading time, the WS group did not. Moreover, the TD group exhibited poor performance for figures with more than six angles, regardless of the figure type (e.g. closed or open), whereas the WS group exhibited generally poor performance for figures with more than five angles but relatively preserved performance for open figures. These findings indicate that a combination of decreased visuospatial span associated with incomplete development of visual scanning and disproportionate development of global processing may cause drawing disabilities in WS.     

Working Memory and Cognitive Skills in Individuals with Down Syndrome

This work is aimed at analyzing working memory (WM) components and their relationships with other cognitive processes in individuals with Down syndrome (DS). Particular attention is given to examine whether a verbal WM deficit is due to difficulties in verbal abilities often showed by individuals with DS, or whether it is a deficit per se. A group of 20 individuals with DS was compared to a group of 20 typically developing (TD) children matched on vocabulary comprehension and to a group of 20 TD children matched on general verbal intelligence. The groups received a battery of 3 verbal and 3 visuospatial WM tasks requiring different degrees of control, and tests assessing verbal abilities (WPPSI verbal scale, PPVT), nonverbal skills (WPPSI performance scale), and logical thinking (LO). The results revealed that individuals with DS have deficits in both central executive (control) and verbal components of the WM system, and the latter one is independent of the general verbal abilities deficit. The data suggest that the development of central executive proceeds at a slower rate in individuals with DS and differently from TD children with comparable verbal abilities. The performance of individuals with DS on high-control WM tasks requires additional general resources that are strictly linked to intelligence.     

Effects of enthusiastic and non-enthusiastic voice in praise on the behavior of children with autism and typically developing children

Behavioral intervention manuals for children with autism spectrum disorder (ASD) commonly recommend that praise should be delivered in an enthusiastic tone of voice. Only a few studies, however, have explicitly tested this assumption, and results have been mixed. This study therefore compared the effects of enthusiastic and non-enthusiastic tone of voice in praise on the behavior of children with ASD. We also examined how typically developing (TD) children responded to enthusiastic and non-enthusiastic praise. Participants were 21 children with ASD matched on developmental age with the chronological age of 20 TD children. The effects of enthusiastic and non-enthusiastic praise were assessed using an application on a tablet computer designed to isolate tone of voice as a variable in vocally delivered praise. Two buttons produced the same six praise statements, one with an enthusiastic tone of voice and one with a neutral tone of voice. Results showed that the children with ASD, on average, allocated more responding to the square on the tablet computer producing enthusiastic praise as compared to the square producing non-enthusiastic praise. In addition, higher rates of responding to non-enthusiastic praise correlated positively with higher IQ, suggesting that non-enthusiastic praise was more effective for children with ASD with higher cognitive scores. The TD children, in contrast, did not allocate more responding to either the squares, suggesting that tone of voice in praise was not an important variable for the behavior of TD children.     

The development of route learning in Down syndrome,Williams syndrome and typical development: investigations with virtual environments

The ability to navigate new environments has a significant impact on the daily life and independence of people with learning difficulties. The aims of this study were to investigate the development of route learning in Down syndrome (N = 50), Williams syndrome (N = 19), and typically developing children between 5 and 11 years old (N = 108); to investigate use of landmarks; and to relate cognitive functions to route‐learning ability in these groups. Overall, measures of attention and long‐term memory were strongly associated with route learning, even once non‐verbal ability was controlled for. All of the groups, including 5‐ to 6‐year‐old TD children, demonstrated the ability to make use of all landmark types to aid route learning; those near junctions, those further from junctions, and also distant landmarks (e.g. church spire, radio mast). Individuals with WS performed better than a matched subset of TD children on more difficult routes; we suggest that this is supported by relatively strong visual feature recognition in the disorder. Participants with DS who had relatively high levels of non‐verbal ability performed at a similar level to TD participants.     

Patterns of intellectual ability in children with verbal deficits

Patterns of intellectual ability were examined in 81 children with verbal deficits identified by a Low Verbal/High Performance WISC profile. The results of verbal and nonverbal tests of intellectual functioning were factor-analyzed, and three groups of children were defined based on patterns of factor scores. Group 1 consisted of children with a Specific Language Disability (SLD) but good Abstract Reasoning ability, while Group 2 included SLD children with good Sequencing-Memory skills. Group 3 children displayed a General Language Disability (GLD) with deficits in both abstract reasoning and sequencing memory. The intellectual patterns were related to cognitive interpretations and found to have educational implications, with Group 1 children reading adequately, Group 2 children showing somewhat poorer reading skills, and Group 3 children reading very poorly. These findings emphasize the importance of identifying subgroups of children with verbal deficits and demonstrate the feasibility of extracting relatively specific cognitive information from global measures of intelligence. The results question the appropriateness of applying traditional assumptions regarding cognitive organization derived from studies of normal children to atypical groups of children.     

Time-based prospective memory difficulties in children with ADHD and the role of time perception and working memory

Time-based prospective memory (PM) is the ability to remember to perform an intended action at a given time in the future. It is a competence that is crucial for effective performance in everyday life and may be one of the main causes of problems for individuals who have difficulty in planning and organizing their life, such as children with attention deficit/hyperactivity disorder (ADHD). This study systematically examines different aspects of time-based PM performance in a task that involves taking an action at a given future time in a group of 23 children with ADHD who were compared with a matched group of typically-developing (TD) children. The children were asked to watch a cartoon and then answer a questionnaire about its content (ongoing task). They were also asked to press a key every 2 minutes while watching the cartoon (PM task). The relationships of time perception and verbal working memory with PM performance were examined by administering appropriate tasks. The results showed that the children with ADHD were less accurate than the TD children in the PM task and exhibited less strategic time-monitoring behavior. Time perception was found to predict PM accuracy, whereas working memory was mainly involved in time-monitoring behavior, but this applied more to the TD group than to the ADHD group, suggesting that children with ADHD are less able to use their cognitive resources when meeting a PM request.     

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study

In this study both the matching and developmental trajectories approaches were used to clarify questions that remain open in the literature on facial emotion recognition in Williams syndrome (WS) and Down syndrome (DS). The matching approach showed that individuals with WS or DS exhibit neither proficiency for the expression of happiness nor specific impairments for negative emotions. Instead, they present the same pattern of emotion recognition as typically developing (TD) individuals. Thus, the better performance on the recognition of positive compared to negative emotions usually reported in WS and DS is not specific of these populations but seems to represent a typical pattern. Prior studies based on the matching approach suggested that the development of facial emotion recognition is delayed in WS and atypical in DS. Nevertheless, and even though performance levels were lower in DS than in WS, the developmental trajectories approach used in this study evidenced that not only individuals with DS but also those with WS present atypical development in facial emotion recognition. Unlike in the TD participants, where developmental changes were observed along with age, in the WS and DS groups, the development of facial emotion recognition was static. Both individuals with WS and those with DS reached an early maximum developmental level due to cognitive constraints.