Implicit Learning in Children and Adults With Williams Syndrome

In comparison to explicit learning, implicit learning is hypothesized to be a phylogenetically older form of learning that is important in early developmental processes (e.g., natural language acquisition, socialization) and relatively impervious to individual differences in age and IQ. We examined implicit learning in a group of children and adults (9-49 years of age) with Williams syndrome (WS) and in a comparison group of typically developing individuals matched for chronological age. Participants were tested in an artificial-grammar learning paradigm and in a rotor-pursuit task. For both groups, implicit learning was largely independent of age. Both groups showed evidence of implicit learning but the comparison group outperformed the WS group on both tasks. Performance advantages for the comparison group were no longer significant when group differences in working memory or nonverbal intelligence were held constant.     

Implicit Learning in Children and Adults With Williams Syndrome

In comparison to explicit learning, implicit learning is hypothesized to be a phylogenetically older form of learning that is important in early developmental processes (e.g., natural language acquisition, socialization) and relatively impervious to individual differences in age and IQ. We examined implicit learning in a group of children and adults (9-49 years of age) with Williams syndrome (WS) and in a comparison group of typically developing individuals matched for chronological age. Participants were tested in an artificial-grammar learning paradigm and in a rotor-pursuit task. For both groups, implicit learning was largely independent of age. Both groups showed evidence of implicit learning but the comparison group outperformed the WS group on both tasks. Performance advantages for the comparison group were no longer significant when group differences in working memory or nonverbal intelligence were held constant.     

The Roles of Verbal Short-Term Memory and Working Memory in the Acquisition of Grammar by Children With Williams Syndrome

The roles of verbal short-term and working memory were examined in a sample of children with Williams syndrome (mean chronological age 10 years, 2 months) and a sample of grammar-matched children who are developing normally. Forward digit span, nonword repetition, and backward span were all found to be correlated with receptive grammatical ability in the sample of children with Williams syndrome, but not in the sample of children who are developing normally. The relation between working memory, as measured by backward digit span, and grammatical ability was found to be significantly stronger in children with Williams syndrome than in the control group. This finding highlights the possibility that children with Williams syndrome may rely on their working memory to a greater extent than children who are developing normally to learn grammar. Hierarchical regression analyses indicated receptive vocabulary may mediate the relations among forward digit span, backward digit span, and grammatical ability for the children with Williams syndrome. Phonological short-term memory, however, contributed independently to grammatical ability even after receptive vocabulary was taken into account.     

The Roles of Verbal Short-Term Memory and Working Memory in the Acquisition of Grammar by Children With Williams Syndrome

The roles of verbal short-term and working memory were examined in a sample of children with Williams syndrome (mean chronological age 10 years, 2 months) and a sample of grammar-matched children who are developing normally. Forward digit span, nonword repetition, and backward span were all found to be correlated with receptive grammatical ability in the sample of children with Williams syndrome, but not in the sample of children who are developing normally. The relation between working memory, as measured by backward digit span, and grammatical ability was found to be significantly stronger in children with Williams syndrome than in the control group. This finding highlights the possibility that children with Williams syndrome may rely on their working memory to a greater extent than children who are developing normally to learn grammar. Hierarchical regression analyses indicated receptive vocabulary may mediate the relations among forward digit span, backward digit span, and grammatical ability for the children with Williams syndrome. Phonological short-term memory, however, contributed independently to grammatical ability even after receptive vocabulary was taken into account.     

Morphological Abilities of Hebrew-Speaking Adolescents With Williams Syndrome

In recent years research has focused on the exact nature of the linguistic skills that individuals with Williams syndrome (WS) exhibit. This work has resulted in controversial positions, with an increasing number of studies casting doubt on previous claims of superior linguistic competence for individuals with WS. This study investigated morphosyntactic knowledge in Hebrew-speaking adolescents with WS. The participants' performance was compared to 2 groups of typically developing mental age-matched controls. Participants and controls were tested on experimental tasks designed to investigate knowledge of morphology. The findings suggest that individuals with WS have good control over the basic consonantal root structure of Hebrew words. However, rather poor performance was evident on other morphological paradigms. We conclude that there is little evidence from Hebrew to support a selective preservation of grammatical competence in individuals with WS.     

Morphological Abilities of Hebrew-Speaking Adolescents With Williams Syndrome

In recent years research has focused on the exact nature of the linguistic skills that individuals with Williams syndrome (WS) exhibit. This work has resulted in controversial positions, with an increasing number of studies casting doubt on previous claims of superior linguistic competence for individuals with WS. This study investigated morphosyntactic knowledge in Hebrew-speaking adolescents with WS. The participants' performance was compared to 2 groups of typically developing mental age-matched controls. Participants and controls were tested on experimental tasks designed to investigate knowledge of morphology. The findings suggest that individuals with WS have good control over the basic consonantal root structure of Hebrew words. However, rather poor performance was evident on other morphological paradigms. We conclude that there is little evidence from Hebrew to support a selective preservation of grammatical competence in individuals with WS.     

Early Linguistic Abilities of Italian Children With Williams Syndrome

Previous studies of linguistic and memory abilities in Italian-speaking children with Williams syndrome (WS) and Down syndrome (DS) are briefly reviewed. New data on linguistic performance of 6 Italian children with WS between 3 and 6 years of age are presented and compared with data on linguistic performance of 6 children with DS selected from a larger sample and matched for chronological age and vocabulary size and of 6 typically developing (TD) younger children matched for mental age and vocabulary size. The language measures also included a parent report of early phrase structure, a naming test, and a sentence repetition task. Analyses revealed that the 3 groups of children were at the same productive vocabulary level, but showed different patterns in sentence production and repetition. Children with WS produced more complete sentences, similar to TD children at the same vocabulary size, whereas children with DS produced more telegraphic and incomplete sentences. The difference between children with DS and those with WS was more marked on the repetition task, suggesting that phonological short-term memory may play a greater role when sentence production is measured through repetition. In addition, qualitative analysis of errors produced in the repetition test revealed interesting differences among the 3 groups. These results from younger children confirm and extend previous findings with older children and adolescents with WS. They further suggest that the apparently spared linguistic abilities of children with WS could emerge as an artifact of comparisons made to children with DS, whose sentence production competence is more compromised relative to other verbal and nonverbal abilities.     

Early Linguistic Abilities of Italian Children With Williams Syndrome

Previous studies of linguistic and memory abilities in Italian-speaking children with Williams syndrome (WS) and Down syndrome (DS) are briefly reviewed. New data on linguistic performance of 6 Italian children with WS between 3 and 6 years of age are presented and compared with data on linguistic performance of 6 children with DS selected from a larger sample and matched for chronological age and vocabulary size and of 6 typically developing (TD) younger children matched for mental age and vocabulary size. The language measures also included a parent report of early phrase structure, a naming test, and a sentence repetition task. Analyses revealed that the 3 groups of children were at the same productive vocabulary level, but showed different patterns in sentence production and repetition. Children with WS produced more complete sentences, similar to TD children at the same vocabulary size, whereas children with DS produced more telegraphic and incomplete sentences. The difference between children with DS and those with WS was more marked on the repetition task, suggesting that phonological short-term memory may play a greater role when sentence production is measured through repetition. In addition, qualitative analysis of errors produced in the repetition test revealed interesting differences among the 3 groups. These results from younger children confirm and extend previous findings with older children and adolescents with WS. They further suggest that the apparently spared linguistic abilities of children with WS could emerge as an artifact of comparisons made to children with DS, whose sentence production competence is more compromised relative to other verbal and nonverbal abilities.     

Anxiety, Fears, and Phobias in Persons With Williams Syndrome

Although much research has focused on the cognitive-linguistic profile associated with Williams syndrome, studies have yet to follow up on preliminary observations suggesting increased anxiety and fears in persons with this disorder. To this aim, Study 1 compared fears in 120 participants with Williams syndrome to 70 appropriately matched persons with mental retardation of mixed etiologies. Study 2 assessed differences in parent versus child reports of fears in 36 Williams syndrome and 24 comparison group parent-child dyads. In Study 3, rates of phobia and other anxiety disorders were assessed in standardized psychiatric interviews with the parents of 51 individuals with Williams syndrome. Relative to their counterparts, persons with Williams syndrome had significantly more fears as well as a wider range of frequently occurring fears, as reported by either parents or participants themselves. Children in both groups reported more fears than their parents. Whereas generalized and anticipatory anxiety were found in 51% to 60% of the sample with Williams syndrome, specific phobia was more prevalent, with 96% showing persistent and marked fears and 84% avoiding their fears or enduring them with distress. The feasibility of cognitive-behavioral treatments for phobia is discussed, as are implications for future research.     

Anxiety,Fears, and Phobias in Persons With Williams Syndrome

Although much research has focused on the cognitive-linguistic profile associated with Williams syndrome, studies have yet to follow up on preliminary observations suggesting increased anxiety and fears in persons with this disorder. To this aim, Study 1 compared fears in 120 participants with Williams syndrome to 70 appropriately matched persons with mental retardation of mixed etiologies. Study 2 assessed differences in parent versus child reports of fears in 36 Williams syndrome and 24 comparison group parent-child dyads. In Study 3, rates of phobia and other anxiety disorders were assessed in standardized psychiatric interviews with the parents of 51 individuals with Williams syndrome. Relative to their counterparts, persons with Williams syndrome had significantly more fears as well as a wider range of frequently occurring fears, as reported by either parents or participants themselves. Children in both groups reported more fears than their parents. Whereas generalized and anticipatory anxiety were found in 51% to 60% of the sample with Williams syndrome, specific phobia was more prevalent, with 96% showing persistent and marked fears and 84% avoiding their fears or enduring them with distress. The feasibility of cognitive-behavioral treatments for phobia is discussed, as are implications for future research.     

Systemic Inflammation in Older Adults With Asthma-COPD Overlap Syndrome

Purpose

The role of systemic inflammation on asthma-COPD overlap syndrome is unknown. This study aimed to examine systemic inflammation in asthma-COPD overlap syndrome, and to identify associations between clinical characteristics and inflammatory mediators in asthma-COPD overlap syndrome.

Methods

In 108 adults older than 55 years comprising healthy controls (n=29), asthma (n=16), COPD (n=21) and asthma-COPD overlap syndrome (n=42), serum high sensitivity C-reactive protein and Interleukin 6 (IL-6) were assayed. Spirometry, induced sputum, quality of life, comorbidities and medications were assessed, and their associations with asthma-COPD overlap syndrome were analyzed using logistic regression. Associations between systemic inflammatory mediators and clinical characteristics were tested in multivariate linear regression models.

Results

Patients with asthma-COPD overlap syndrome had significantly elevated IL-6 levels compared with healthy controls and asthmatics. Age, comorbidity index and IL-6 level were independently associated with asthma-COPD overlap syndrome. FEV1% predicted was inversely associated with IL-6 level, and cardiovascular disease was associated with an increased IL-6 level. Systemic markers were not associated with airway inflammation.

Conclusions

Systemic inflammation is commonly present in asthma-COPD overlap syndrome, and asthma-COPD overlap syndrome resembled COPD in terms of systemic inflammation. IL-6 is a pivotal inflammatory mediator that may be involved in airflow obstruction and cardiovascular disease and may be an independent treatment target.     

Short-Term Memory in Young Adults With Spastic Diplegic Cerebral Palsy

We examined short-term memory activation processes and task-relevant, task-irrelevant, and dynamic inhibitory processes in a group of young adults with hypoxic-ischaemic prenatal spastic diplegic cerebral palsy (HI-CP, N?=?27) with group-level and person-oriented methods of data analysis. Performance was compared with age, sex, and education of a matched control group (N?=?135). We found that HI-CP is accompanied with low level of correct answers and high levels of task-relevant and task-irrelevant intrusions and repetitions on the Memory with Pause and Homogeneous Interference short-term memory tasks. Person-oriented analyses revealed several different configurations of dysfunction in the HI-CP group.     

Functional Alterations in Order Short-Term Memory Networks in Adults With Dyslexia

Dyslexia is characterized not only by reading impairment but also by short-term memory (STM) deficits, and this particularly for the retention of serial order information. Here, we explored the functional neural correlates associated with serial order STM performance of adults with dyslexia for verbal and visual STM tasks. Relative to a group of age-matched controls, the dyslexic group showed abnormal activation in a network associated with order STM encompassing the right intraparietal and superior frontal sulcus, and this for both verbal and visual order STM conditions. This study highlights long-lasting alterations in non-language neural substrates and processes in dyslexia.     

Familial Williams Syndrome

Characteristic features of the Williams syndrome were observed in a mother and her presumably dizygotic twin daughters, suggesting either an X-linked dominant, autosomal dominant or multifactorial inheritance in this pedigree. The Williams syndrome generally has been thought to have a sporadic occurrence. The rare familial occurrence, however, suggests a possible heterogeneity in its etiology and also points out the importance of careful examination of the other family members for proper genetic counseling.     

Cognitive Heterogeneity in Williams Syndrome

This study used the Woodcock-Johnson Tests of Cognitive Ability-Revised to investigate a wide range of cognitive abilities in people with Williams syndrome (WS). It involved a comparatively large sample of 31 people with WS, but took a case-series approach. The study addressed the widespread claims of a characteristic "WS cognitive profile" by looking for heterogeneity rather than homogeneity. People with WS showed a variety of preserved (significantly above mental age [MA]), expected (at MA), and significantly impaired (significantly below MA) levels of functioning. Such results provide clear evidence for heterogeneity in cognitive functions within WS. We found the most homogeneity on a test of phonological processing and a test of phonological short-term memory, with half of the WS sample performing at MA levels on these tests. Interestingly, no WS individual showed a weakness on a test of nonverbal reasoning, and only one WS individual showed a weakness on a test of verbal comprehension. In addition, we found that strengths on analysis-synthesis and verbal analogies occurred only for WS individuals with an MA less than 5.5 years (our sample median MA); people with an MA greater than 5.5 years performed at MA level on these 2 tests. Results also provided preliminary evidence for distinct subgroups of WS people based on their cognitive strengths and weaknesses on a broad range of cognitive functions. On the basis of the findings, caution should be made in declaring a single cognitive profile that is characteristic of all individuals with WS. Just as there is heterogeneity in genetic and physical anomalies within WS, not all WS individuals share the same cognitive strengths and weaknesses. Also, not all WS individuals show the profile of a strength in verbal abilities and a weakness in spatial functions.     

Attentional Characteristics of Infants and Toddlers With Williams Syndrome During Triadic Interactions

Two studies were conducted to consider the looking behavior of infants and toddlers with Williams syndrome (WS). In Study 1, the looking behavior of a 10-month-old girl with WS during play sessions with her mother and with a stranger was compared to that of 2 groups of infants who were developing normally (ND), 1 matched for chronological age and the other for developmental age. The infant with WS spent more than twice as much time looking at her mother as the infants in either contrast group did. She also spent twice as much time looking at the stranger. In addition, during 78% of this time, her gaze at the stranger was coded as extremely intense. Looks of this intensity were virtually never made by the ND infants. In Study 2, the looking behavior of 31 individuals with WS ages 8 to 43 months during a genetics evaluation was compared to that of 319 control children in the same age range (242 with developmental delay due to causes other than WS). Twenty-three of the 25 participants with WS aged 33 months or younger demonstrated extended and intense looking at the geneticist. In contrast, none of the control participants looked extensively or intently at the geneticist. Findings are discussed in the context of previous research on arousal and focused attention during normal development and on temperament and personality of older children and adults with WS. It is argued that the unusual looking patterns evidenced by infants and toddlers with WS presage the unusual temperament and personality of older individuals with WS, and the possibility of a genetic basis for these behaviors is addressed.     

Attentional Characteristics of Infants and Toddlers With Williams Syndrome During Triadic Interactions

Two studies were conducted to consider the looking behavior of infants and toddlers with Williams syndrome (WS). In Study 1, the looking behavior of a 10-month-old girl with WS during play sessions with her mother and with a stranger was compared to that of 2 groups of infants who were developing normally (ND), 1 matched for chronological age and the other for developmental age. The infant with WS spent more than twice as much time looking at her mother as the infants in either contrast group did. She also spent twice as much time looking at the stranger. In addition, during 78% of this time, her gaze at the stranger was coded as extremely intense. Looks of this intensity were virtually never made by the ND infants. In Study 2, the looking behavior of 31 individuals with WS ages 8 to 43 months during a genetics evaluation was compared to that of 319 control children in the same age range (242 with developmental delay due to causes other than WS). Twenty-three of the 25 participants with WS aged 33 months or younger demonstrated extended and intense looking at the geneticist. In contrast, none of the control participants looked extensively or intently at the geneticist. Findings are discussed in the context of previous research on arousal and focused attention during normal development and on temperament and personality of older children and adults with WS. It is argued that the unusual looking patterns evidenced by infants and toddlers with WS presage the unusual temperament and personality of older individuals with WS, and the possibility of a genetic basis for these behaviors is addressed.     

Objects, Motions, and Paths: Spatial Language in Children With Williams Syndrome

The acquisition of spatial language is often assumed to be built upon an early-emerging system of nonlinguistic spatial knowledge. We tested this relationship by examining spatial language in children with Williams syndrome (WS), a rare genetic disorder that gives rise to severe nonlinguistic spatial deficits together with relatively spared language. Twelve children with WS, 12 normally developing mental-age matched children, and 12 normal adults described 80 videotaped motion events. Children with WS showed substantial control over key linguistic components of the motion event, including appropriate semantic and syntactic encoding of Figure and Ground objects, Manner of Motion, and Path. The expression of Path, although surprisingly spared, was more fragile among children with WS in contexts plausibly related to their nonlinguistic spatial deficit. The results show strong preservation of the formal aspects of spatial-linguistic knowledge and suggest that the nonlinguistic spatial deficits shown by children with WS have, at most, limited effects on their spatial language. These findings have implications for the relationship between spatial language and other aspects of spatial cognition.     

Objects,Motions, and Paths: Spatial Language in Children With Williams Syndrome

The acquisition of spatial language is often assumed to be built upon an early-emerging system of nonlinguistic spatial knowledge. We tested this relationship by examining spatial language in children with Williams syndrome (WS), a rare genetic disorder that gives rise to severe nonlinguistic spatial deficits together with relatively spared language. Twelve children with WS, 12 normally developing mental-age matched children, and 12 normal adults described 80 videotaped motion events. Children with WS showed substantial control over key linguistic components of the motion event, including appropriate semantic and syntactic encoding of Figure and Ground objects, Manner of Motion, and Path. The expression of Path, although surprisingly spared, was more fragile among children with WS in contexts plausibly related to their nonlinguistic spatial deficit. The results show strong preservation of the formal aspects of spatial-linguistic knowledge and suggest that the nonlinguistic spatial deficits shown by children with WS have, at most, limited effects on their spatial language. These findings have implications for the relationship between spatial language and other aspects of spatial cognition.