Memory and learning in children with 22q11.2 deletion syndrome: evidence for ventral and dorsal stream disruption?

This study examined memory functioning in children and adolescents with 22q11.2 Deletion Syndrome (DS; velocardiofacial syndrome). An overall verbal better than nonverbal memory pattern was evident on the Test of Memory and Learning (TOMAL), with children with 22q11.2 DS performing significantly below their siblings and children with low average IQ but similar to children with autism on facial memory. Children with 22q11 DS also performed significantly below their siblings on tests of verbal working memory. Children with autism performed significantly poorer than the siblings of children with 22q11.2 DS only on their recall of stories. Delayed recall was significantly poorer in children with 22q11.2 DS and children with autism, compared to sibling controls. Although there were no significant group differences on tests of multiple trial verbal or visual learning, a relative weakness was noted with multiple trial visual learning in children with 22q11.2 DS and their siblings, suggesting that an alternative or interactive factor other than the deletion may account for the relatively better verbal compared to nonverbal memory abilities. Deficits in facial memory in children with both 22q11.2 DS and autism suggest disruptions in ventral temporal pathways such as between fusiform gyrus and parahippocampal/hippocampal regions whereas deficits in verbal working memory in children with 22q11.2 DS implicates dorsolateral prefrontal regions, both intimating aberrant white matter pathways.     

Selective and sustained attention in children with spina bifida myelomeningocele

This study examined memory functioning in children and adolescents with 22q11.2 Deletion Syndrome (DS; velocardiofacial syndrome). An overall verbal better than nonverbal memory pattern was evident on the Test of Memory and Learning (TOMAL), with children with 22q11.2 DS performing significantly below their siblings and children with low average IQ but similar to children with autism on facial memory. Children with 22q11 DS also performed significantly below their siblings on tests of verbal working memory. Children with autism performed significantly poorer than the siblings of children with 22q11.2 DS only on their recall of stories. Delayed recall was significantly poorer in children with 22q11.2 DS and children with autism, compared to sibling controls. Although there were no significant group differences on tests of multiple trial verbal or visual learning, a relative weakness was noted with multiple trial visual learning in children with 22q11.2 DS and their siblings, suggesting that an alternative or interactive factor other than the deletion may account for the relatively better verbal compared to nonverbal memory abilities. Deficits in facial memory in children with both 22q11.2 DS and autism suggest disruptions in ventral temporal pathways such as between fusiform gyrus and parahippocampal/hippocampal regions whereas deficits in verbal working memory in children with 22q11.2 DS implicates dorsolateral prefrontal regions, both intimating aberrant white matter pathways.     

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.     

Developmental dyscalculia and basic numerical capacities: a study of 8-9-year-old students

Thirty-one 8- and 9-year-old children selected for dyscalculia, reading difficulties or both, were compared to controls on a range of basic number processing tasks. Children with dyscalculia only had impaired performance on the tasks despite high-average performance on tests of IQ, vocabulary and working memory tasks. Children with reading disability were mildly impaired only on tasks that involved articulation, while children with both disorders showed a pattern of numerical disability similar to that of the dyscalculic group, with no special features consequent on their reading or language deficits. We conclude that dyscalculia is the result of specific disabilities in basic numerical processing, rather than the consequence of deficits in other cognitive abilities.     

Defective number module or impaired access? Numerical magnitude processing in first graders with mathematical difficulties

This study examined numerical magnitude processing in first graders with severe and mild forms of mathematical difficulties, children with mathematics learning disabilities (MLD) and children with low achievement (LA) in mathematics, respectively. In total, 20 children with MLD, 21 children with LA, and 41 regular achievers completed a numerical magnitude comparison task and an approximate addition task, which were presented in a symbolic and a nonsymbolic (dot arrays) format. Children with MLD and LA were impaired on tasks that involved the access of numerical magnitude information from symbolic representations, with the LA children showing a less severe performance pattern than children with MLD. They showed no deficits in accessing magnitude from underlying nonsymbolic magnitude representations. Our findings indicate that this performance pattern occurs in children from first grade onward and generalizes beyond numerical magnitude comparison tasks. These findings shed light on the types of intervention that may help children who struggle with learning mathematics.     

Domain specific attentional impairments in children with chromosome 22q11.2 deletion syndrome

One of the defining cognitive characteristics of the chromosome 22q deletion syndrome (DS22q11.2) is visuospatial processing impairments. The purpose of this study was to investigate and extend the specific attentional profile of children with this disorder using both an object-based attention task and an inhibition of return task. A group of children with the disorder was compared in these tasks with a group of age-matched typically developing children. The children with DS22q11.2 demonstrated impaired spatially based orienting which is consistent with previous findings in this group. Strikingly, the children with DS22q11.2 also demonstrated an improved ability to use object-based cues, relative to the typically developing group. Finally, the children with DS22q11.2 demonstrated an intact inhibition of return system, however, it appears to be delayed developmentally.     

Basic number processing deficits in ADHD: a broad examination of elementary and complex number processing skills in 9- to 12-year-old children with ADHD-C

ADHD (attention-deficit hyperactivity disorder) and academic difficulties are frequently associated, but to date this link is poorly understood. In order to explore which components of number processing and calculation skills may be disturbed in children with ADHD we presented a series of respective tasks to 9- to 12-year-old children with ADHD-combined type and matched children without ADHD (of any type) without concomitant dyscalculia and/or dyslexia. Overall, group differences were not significant regarding overlearned and explicitly trained simple and complex calculation skills. More basic number processing skills are - for instance - the ability to compare one-digit numbers according to their magnitude (so-called magnitude comparison), to count or to transcode numbers, i.e. to write down an Arabic number '21' in verbal dictation. Significant differences favouring control children in basic number processing skills were obtained in a number comparison task and in a dot enumeration task. Importantly, our results cannot be explained by group differences regarding specific working memory and executive function components. Thus, number processing skills and in particular the processing of numerical magnitude should be investigated in children diagnosed with ADHD even when no comorbid learning disabilities are reported.     

Symbolic magnitude processing in elementary school children: A group administered paper-and-pencil measure (SYMP Test)

The ability to compare symbolic numerical magnitudes correlates with children’s concurrent and future mathematics achievement. We developed and evaluated a quick timed paper-and-pencil measure that can easily be used, for example in large-scale research, in which children have to cross out the numerically larger of two Arabic one- and two-digit numbers (SYMP Test). We investigated performance on this test in 1,588 primary school children (Grades 1–6) and examined in each grade its associations with mathematics achievement. The SYMP Test had satisfactory test-retest reliability. The SYMP Test showed significant and stable correlations with mathematics achievement for both one-digit and two-digit comparison, across all grades. This replicates the previously observed association between symbolic numerical magnitude processing and mathematics achievement, but extends it by showing that the association is observed in all grades in primary education and occurs for single- as well as multi-digit processing. Children with mathematical learning difficulties performed significantly lower on one-digit comparison and two-digit comparison in all grades. This all suggests satisfactory construct and criterion-related validity of the SYMP Test, which can be used in research, when performing large-scale (intervention) studies, and by practitioners, as screening measure to identify children at risk for mathematical difficulties or dyscalculia.     

发展性计算障碍儿童的数量转换缺陷

本研究拟考察发展性计算障碍儿童的认知缺陷成因。实验1要求被试在三种形式(点/点,数/数,点/数)下进行数量比较,实验2仅将点集替换为汉字数字词。结果表明障碍组和正常组在数/数、点/数和汉字/汉字比较任务上的成绩存在显著差异,而在点/点和汉字/汉字比较上没有差异。据此推论,计算障碍儿童符号加工能力受到损伤,符号与非符号数量转换能力存在缺陷,但非符号加工能力和不同符号间数量转换没有缺陷,支持语义提取缺陷假设。     

How does the severity of a learning disability affect working memory performance?

Working memory performance was examined in children aged 11-12 years who had borderline, mild, and moderate learning disabilities. Comparisons with children of average abilities were used to determine whether those with more severe learning disabilities had greater impairments in working memory. Seven measures of working memory span were used to assess temporary phonological short-term storage (digit span, word span), temporary visuo-spatial short-term storage (pattern span, spatial span), and temporary short-term storage with additional processing, or central executive, demands (listening span. odd one out span, reverse digit span). Children with mild and moderate learning disabilities were impaired on all measures of working memory compared to children of average abilities. Children with borderline learning disabilities were just as good as children with average abilities on visuo-spatial and complex span tasks, but showed an impairment on phonological span tasks. Children with moderate learning disabilities were indistinguishable from children with mild learning disabilities on simple span tasks, but were significantly poorer than the mild group on the more demanding complex span tasks. For the group as a whole, working memory was strongly related to mental age.     

How does the severity of a learning disability affect working memory performance?

Working memory performance was examined in children aged 11-12 years who had borderline, mild, and moderate learning disabilities. Comparisons with children of average abilities were used to determine whether those with more severe learning disabilities had greater impairments in working memory. Seven measures of working memory span were used to assess temporary phonological short-term storage (digit span, word span), temporary visuo-spatial short-term storage (pattern span, spatial span), and temporary short-term storage with additional processing, or central executive, demands (listening span, odd one out span, reverse digit span). Children with mild and moderate learning disabilities were impaired on all measures of working memory compared to children of average abilities. Children with borderline learning disabilities were just as good as children with average abilities on visuo-spatial and complex span tasks, but showed an impairment on phonological span tasks. Children with moderate learning disabilities were indistinguishable from children with mild learning disabilities on simple span tasks, but were significantly poorer than the mild group on the more demanding complex span tasks. For the group as a whole, working memory was strongly related to mental age.     

Dyslexia and dyscalculia: Two learning disorders with different cognitive profiles

This study tests the hypothesis that dyslexia and dyscalculia are associated with two largely independent cognitive deficits, namely a phonological deficit in the case of dyslexia and a deficit in the number module in the case of dyscalculia. In four groups of 8- to 10-year-olds (42 control, 21 dyslexic, 20 dyscalculic, and 26 dyslexic/dyscalculic), phonological awareness, phonological and visual-spatial short-term and working memory, naming speed, and basic number processing skills were assessed. A phonological deficit was found for both dyslexic groups, irrespective of additional arithmetic deficits, but not for the dyscalculia-only group. In contrast, deficits in processing of symbolic and nonsymbolic magnitudes were observed in both groups of dyscalculic children, irrespective of additional reading difficulties, but not in the dyslexia-only group. Cognitive deficits in the comorbid dyslexia/dyscalculia group were additive; that is, they resulted from the combination of two learning disorders. These findings suggest that dyslexia and dyscalculia have separable cognitive profiles, namely a phonological deficit in the case of dyslexia and a deficient number module in the case of dyscalculia.     

Working memory deficits in developmental dyscalculia: The importance of serial order

Although a number of studies suggests a link between working memory (WM) storage capacity of short-term memory and calculation abilities, the nature of verbal WM deficits in children with developmental dyscalculia (DD) remains poorly understood. We explored verbal WM capacity in DD by focusing on the distinction between memory for item information (the items to be retained) and memory for order information (the order of the items within a list). We hypothesized that WM for order could be specifically related to impaired numerical abilities given that recent studies suggest close interactions between the representation of order information in WM and ordinal numerical processing. We investigated item and order WM abilities as well as basic numerical processing abilities in 16 children with DD (age: 8–11 years) and 16 typically developing children matched on age, IQ, and reading abilities. The DD group performed significantly poorer than controls in the order WM condition but not in the item WM condition. In addition, the DD group performed significantly slower than the control group on a numerical order judgment task. The present results show significantly reduced serial order WM abilities in DD coupled with less efficient numerical ordinal processing abilities, reflecting more general difficulties in explicit processing of ordinal information.     

Developmental trajectory of number acuity reveals a severe impairment in developmental dyscalculia

Developmental dyscalculia is a learning disability that affects the acquisition of knowledge about numbers and arithmetic. It is widely assumed that numeracy is rooted on the “number sense”, a core ability to grasp numerical quantities that humans share with other animals and deploy spontaneously at birth. To probe the links between number sense and dyscalculia, we used a psychophysical test to measure the Weber fraction for the numerosity of sets of dots, hereafter called number acuity. We show that number acuity improves with age in typically developing children. In dyscalculics, numerical acuity is severely impaired, with 10-year-old dyscalculics scoring at the level of 5-year-old normally developing children. Moreover, the severity of the number acuity impairment predicts the defective performance on tasks involving the manipulation of symbolic numbers. These results establish for the first time a clear association between dyscalculia and impaired “number sense”, and they may open up new horizons for the early diagnosis and rehabilitation of mathematical learning deficits.     

Executive functioning and its relation to ASD and ADHD symptomatology in 22q11.2 deletion syndrome*

Children with 22q11.2 deletion syndrome (22q11DS; velo-cardio-facial-syndrome) are at risk for the developmental disorders, attention deficit hyperactivity disorder (ADHD) and autism spectrum disorder (ASD). In this study, the relation between executive functioning (EF) and the severity of ADHD and ASD symptoms is examined, since EF is known to be important in relation to emotional and behavioral problems. The participants consist of 58 children (38 females) with a mean age of 13.5 years (SD 2.6). Standardized assessment was used to evaluate the severity of ASD and ADHD symptomatology. The major aspects of EF, i.e., cognitive flexibility, inhibition, sustained attention, distractibility, working memory and reaction speed, were evaluated. The profile of EF in 22q11DS was found to be characterized by weaker performance compared to the norms on all subdomains of EF. Poor cognitive flexibility and inhibition, as well as high distractibility, were found to be related to more severe ASD symptoms, while poor quality of sustained attention and high distractibility were found to be related to more severe ADHD symptoms. It is concluded that children with 22q11DS experience impairments in EF, and that the degree of impairment on specific EF subdomains is related to the severity of ASD and/or ADHD symptomatology. These results may help in defining the mediating role of neurocognitive dysfunctions in the development of social and behavioral problems in 22q11DS.     

Working memory deficit in children with mathematical difficulties: a general or specific deficit?

This study examined whether children with mathematical difficulties (MDs) or comorbid mathematical and reading difficulties have a working memory deficit and whether the hypothesized working memory deficit includes the whole working memory system or only specific components. In the study, 31 10-year-olds with MDs and 37 10-year-olds with both mathematical and reading difficulties were compared with 47 age-matched and 50 younger controls (9-year-olds) on a number of working memory tasks. Compared with the age-matched controls, both groups of children with MDs performed worse on tasks tapping the central executive (e.g., visual matrix span) and the phonological loop (e.g., word span). More important, the MD group performed worse than the younger controls on the counting span task, whereas the group with comorbid mathematical and reading difficulties performed worse on the counting span task and the visual matrix span task. These findings provide support for the assumption that children with MDs have a working memory deficit. More specifically, children with MDs have a central executive deficit connected to concurrent processing and storage of numerical and visual information.     

Basic numerical skills in children with mathematics learning disabilities: a comparison of symbolic vs non-symbolic number magnitude processing

Forty-five children with mathematics learning disabilities, with and without comorbid reading disabilities, were compared to 45 normally achieving peers in tasks assessing basic numerical skills. Children with mathematics disabilities were only impaired when comparing Arabic digits (i.e., symbolic number magnitude) but not when comparing collections (i.e., non-symbolic number magnitude). Moreover, they automatically processed number magnitude when comparing the physical size of Arabic digits in an Stroop paradigm adapted for processing speed differences. Finally, no evidence was found for differential patterns of performance between MD and MD/RD children in these tasks. These findings suggest that children with mathematics learning disabilities have difficulty in accessing number magnitude from symbols rather than in processing numerosity per se.     

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.     

发展性计算障碍儿童的数认知

发展性计算障碍作为一种特异性学习障碍,影响儿童的数学成就与数学学习观念,因而引起了多学科研究的关注和重视。文章分析了发展性计算障碍领域在数认知方面的已有研究,揭示了发展性计算障碍的数认知缺陷及内部差异,从一般认知加工障碍与数字加工模块障碍两个角度阐述了发展性计算障碍的可能成因,最后对当前研究进行了小结与展望,认为数认知缺陷的复杂关系、亚类型特征、成因之间的相互作用与机制还需进一步研究     

分散注意条件下学优生与学困生视空间工作记忆的比较研究

通过控制干扰刺激量 ,研究分散注意对学优生和学困生视空间WM的影响。实验一的结果表明 :在不同干扰条件下 ,学困生视空间WM均比学优生差 ;干扰任务不仅影响学困生视空间WM的加工能力 ,也影响他们的存储能力。这说明学困生不能有效抑制外界干扰可能是他们视空间WM存在缺陷的主要原因之一。实验二的结果表明 :视空间WM有缺陷是各类学困生普遍存在的问题 ;对于学优生来说 ,不同类型学优生视空间WM不同 ,语文学优比数学学优的学生的视空间WM能力更强 ,主要表现在加工功能上。