Language and Cognition in Two Children with Williams Syndrome

1989 ◽  
Vol 32 (3) ◽  
pp. 489-500 ◽  
Author(s):  
Donna Thal ◽  
Elizabeth Bates ◽  
Ursula Bellugi
Keyword(s):  
Language Acquisition ◽  
Spatial Cognition ◽  
Williams Syndrome ◽  
Cognitive Profile ◽  
Single Word ◽  
Normal Children ◽  
Linguistic Performance ◽  
Visual Spatial ◽  
Language And Cognition

In this study, aspects of language, single-gesture retrieval, combinatorial play, and visual-spatial cognition are reported in two children with Williams syndrome who were in the single-word stage of language acquisition. Williams syndrome children are of particular interest because, when older (10–15 years), they display an unusual profile of abilities that may include superior linguistic performance compared with other aspects of cognition. The two children in the present study are compared to a group of normal children in the single-word stage of language acquisition with a mean age of 14.8 months, a group of single-word stage children who are 6–18 months delayed, and a group of older normal children with a mean age of 23.5 months. Results are compatible with the unusual cognitive profile displayed by older Williams syndrome children. We discuss the possibility that this profile involves mechanisms that extend beyond the boundaries of language proper.

scholarly journals Learning and Using Abstract Words: Evidence from Clinical Populations

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Maria Luisa Lorusso ◽  
Michele Burigo ◽  
Alessandro Tavano ◽  
Anna Milani ◽  
Sara Martelli ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Williams Syndrome ◽  
Cognitive Profile ◽  
Typically Developing ◽  
Abstract Concepts ◽  
Visual Spatial ◽  
Abstract Words ◽  
Concrete Concepts ◽  
Concrete Words

It has been shown that abstract concepts are more difficult to process and are acquired later than concrete concepts. We analysed the percentage of concrete words in the narrative lexicon of individuals with Williams Syndrome (WS) as compared to individuals with Down Syndrome (DS) and typically developing (TD) peers. The cognitive profile of WS is characterized by visual-spatial difficulties, while DS presents with predominant impairments in linguistic abilities. We predicted that if linguistic abilities are crucial to the development and use of an abstract vocabulary, DS participants should display a higher concreteness index than both Williams Syndrome and typically developing individuals. Results confirm this prediction, thus supporting the hypothesis of a crucial role of linguistic processes in abstract language acquisition. Correlation analyses suggest that a maturational link exists between the level of abstractness in narrative production and syntactic comprehension.

scholarly journals Studying individual differences in the social environment to better understand language learning and processing

2016 ◽  
Vol 2 (s1) ◽  
Author(s):  
Shiri Lev-Ari
Keyword(s):  
Second Language ◽  
Individual Differences ◽  
Second Language Acquisition ◽  
Language Acquisition ◽  
Language Learning ◽  
Social Environment ◽  
Native Speakers ◽  
Linguistic Performance ◽  
The Social

AbstractPeople learn language from their social environment. Therefore, individual differences in the input that their social environment provides could influence their linguistic performance. Nevertheless, investigation of the role of individual differences in input on performance has been mostly restricted to first and second language acquisition. In this paper I argue that individual differences in input can influence linguistic performance even in adult native speakers. Specifically, differences in input can affect performance by influencing people’s knowledgebase, by modulating their processing manner, and by shaping expectations. Therefore, studying the role that individual differences in input play can improve our understanding of how language is learned, processed and represented.

Word learning in a special population: do individuals with Williams syndrome obey lexical constraints?

1997 ◽  
Vol 24 (3) ◽  
pp. 737-765 ◽  
Author(s):  
TASSOS STEVENS ◽  
ANNETTE KARMILOFF-SMITH
Keyword(s):  
Williams Syndrome ◽  
Word Learning ◽  
Neurodevelopmental Disorder ◽  
Fast Mapping ◽  
Mutual Exclusivity ◽  
Vocabulary Size ◽  
Normal Children ◽  
New Words ◽  
Adolescents And Adults ◽  
Lexical Constraints

Williams syndrome (WS), a rare neurodevelopmental disorder, is of special interest to developmental psycholinguists because of its uneven linguistico-cognitive profile of abilities and deficits. One proficiency manifest in WS adolescents and adults is an unusually large vocabulary despite serious deficits in other domains. In this paper, rather than focus on vocabulary size, we explore the processes underlying vocabulary acquisition, i.e. how new words are learned. A WS group was compared to groups of normal MA-matched controls in the range 3–9 years in four different experiments testing for constraints on word learning. We show that in construing the meaning of new words, normal children at all ages display fast mapping and abide by the constraints tested: mutual exclusivity, whole object and taxonomic. By contrast, while the WS group showed fast mapping and the mutual exclusivity constraint, they did not abide by the whole object or taxonomic constraints. This suggests that measuring only the size of WS vocabulary can distort conclusions about the normalcy of WS language. Our study shows that despite equivalent behaviour (i.e. vocabulary test age), the processes underlying how vocabulary is acquired in WS follow a somewhat different path from that of normal children and that the atypically developing brain is not necessarily a window on normal development.

Modality and Mood in First Language Acquisition

2015 ◽  
Author(s):  
Maya Hickmann ◽  
Dominique Bassano
Keyword(s):  
Language Acquisition ◽  
Developmental Process ◽  
First Language ◽  
Epistemic Modality ◽  
Linguistic Variation ◽  
First Language Acquisition ◽  
Acquisition Sequence ◽  
Phases Of Development ◽  
Language And Cognition

This chapter aims to provide a large overview of research focusing on the development of modality and mood during first language acquisition. This overview synthesizes results concerning both early and later phases of development, within and across a large number of languages, and including some more peripheral categories, such as evidentials and tense–aspect markings. Results recurrently show the earlier acquisition of agent-oriented modality as compared to epistemic modality. However, cross-linguistic variation has raised some questions about this acquisition sequence, suggesting that language-specific properties may partially impact timing during acquisition. In addition, findings about later phases show a long developmental process whereby children gradually come to master complex semantic and pragmatic modal distinctions. The discussion highlights the contribution of these conclusions to current theoretical debates, such as the role of input factors and the relation between language and cognition during ontogenesis.

Neuropsychological profile of Italians with Williams syndrome: An example of a dissociation between language and cognition?

2004 ◽  
Vol 10 (6) ◽  
pp. 862-876 ◽  
Author(s):  
STEFANO VICARI ◽  
ELISABETH BATES ◽  
MARIA CRISTINA CASELLI ◽  
PATRIZIO PASQUALETTI ◽  
CHIARA GAGLIARDI ◽  
...  
Keyword(s):  
Williams Syndrome ◽  
Digit Span ◽  
Older Children ◽  
Final State ◽  
Cross Sectional ◽  
Spatial Span ◽  
Mental Age ◽  
Visual Spatial ◽  
Neuropsychological Profile ◽  
Construction Tasks

Important claims have been made regarding the contrasting profiles of linguistic and cognitive performance observed in two genetically based syndromes, Williams syndrome (WS) and Down syndrome (DS). Earlier studies suggested a double dissociation, with language better preserved than nonverbal cognition in children and adults with WS, and an opposite profile in children and adults with DS. More recent studies show that this initial characterization was too simple, and that qualitatively different patterns of deficit observed within both language and visual–spatial cognition, in both groups. In the present study, large samples of children and adolescents with WS and age-matched DS are compared with typically developing (TD) controls matched to WS in mental age, on receptive and expressive lexical and grammatical abilities, semantic and phonological fluency, digit span and nonverbal visual–spatial span, and on 2 visual–spatial construction tasks. Study 1 confirmed distinct profiles of sparing and impairment for the 2 groups, within as well as between language and nonlinguistic domains, even after IQ variations were controlled. In Study 2 we compared performance of the children, adolescents and young adults with DS and WS included in the first study, divided on the basis of the chronological age of the participants (under 8 years; over 12 years). Although it is important to stress that these are cross-sectional rather than longitudinal data, the results demonstrated that the profile of younger children is different in respect to those of the older children; initial states of the system cannot be inferred by the final state. Possible neural substrates for these profiles and trajectories are discussed. (JINS, 2004, 10, 862–876.)

scholarly journals Cognitive Profile in Williams Syndrome: A Case Study

2005 ◽  
Vol 51 (101) ◽  
pp. 143-153 ◽  
Author(s):  
Óscar F. Gonçalves ◽  
Montserrat F. Prieto ◽  
Adriana Sampaio ◽  
Avelina Pérez ◽  
Margarida Henriques ◽  
...  
Keyword(s):  
Williams Syndrome ◽  
Cognitive Profile

VI. Genome Structure and Cognitive Map of Williams Syndrome

2000 ◽  
Vol 12 (supplement 1) ◽  
pp. 89-107 ◽  
Author(s):  
Julie R. Korenberg ◽  
Xiao-Ning Chen ◽  
Hamao Hirota ◽  
Zona Lai ◽  
Ursula Bellugi ◽  
...  
Keyword(s):  
Williams Syndrome ◽  
Large Scale ◽  
Hot Spot ◽  
Genome Structure ◽  
Single Copy ◽  
Human Cognition ◽  
Chromosome Band ◽  
Gene Marker ◽  
Artificial Chromosomes ◽  
Visual Spatial

Williams syndrome (WMS) is a most compelling model of human cognition, of human genome organization, and of evolution. Due to a deletion in chromosome band 7q11.23, subjects have cardiovascular, connective tissue, and neurode-velopmental deficits. Given the striking peaks and valleys in neurocognition including deficits in visual-spatial and global processing, preserved language and face processing, hypersociability, and heightened affect, the goal of this work has been to identify the genes that are responsible, the cause of the deletion, and its origin in primate evolution. To do this, we have generated an integrated physical, genetic, and transcriptional map of the WMS and flanking regions using multicolor metaphase and interphase fluorescence in situ hybridization (FISH) of bacterial artificial chromosomes (BACs) and P1 artificial chromosomes (PACs), BAC end sequencing, PCR gene marker and microsatellite, large-scale sequencing, cDNA library, and database analyses. The results indicate the genomic organization of the WMS region as two nested duplicated regions flanking a largely single-copy region. There are at least two common deletion breakpoints, one in the centromeric and at least two in the telomeric repeated regions. Clones anchoring the unique to the repeated regions are defined along with three new pseudogene families. Primate studies indicate an evolutionary hot spot for chromosomal inversion in the WMS region. A cognitive phenotypic map of WMS is presented, which combines previous data with five further WMS subjects and three atypical WMS subjects with deletions; two larger (deleted for D7S489L) and one smaller, deleted for genes telomeric to FZD9, through LIMK1, but not WSCR1 or telomeric. The results establish regions and consequent gene candidates for WMS features including mental retardation, hypersociability, and facial features. The approach provides the basis for defining pathways linking genetic underpinnings with the neuroanatomical, functional, and behavioral consequences that result in human cognition.

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