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Biomedical subjects

O Capirci

Publications and source records attributed to O Capirci.

8 recordsLinked to original sources

Lexical production in children with Williams syndrome: spontaneous use of gesture in a naming task.

This study investigates lexical organization and lexical retrieval in children with Williams syndrome (WS), by examining both naming accuracy and accompanying use of gestures in a picture-naming task. Ten children with the genetic disorder of Williams syndrome (age range: 9.5-12.9) were compared with 20 typically developing children, 10 matched for chronological age (CA) and 10 for mental-age (MA). Lexical production was measured by administering the Boston Naming test (BNT). Older typically developing children performed significantly better than the other two groups. No differences in accuracy were found between the children with WS and the typically developing children matched for mental-age. The overall distribution of error types displayed by children with WS indicate that the lexical-semantic organization is similar to that of typically developing children. However, compared to controls, the WS group produced more iconic gestures during the task, in patterns that suggest the existence of specific word-finding difficulties in these children. Results are discussed within the framework of recent theories on the role of gesture in speech production.

Adolescent↗

Serum NGF levels in children and adolescents with either Williams syndrome or Down syndrome.

The neurotrophin nerve growth factor (NGF) is a major regulator of peripheral and central nervous system development. Serum NGF was measured in normally developing control children (n=26) and in individuals affected by congenital syndromes associated with learning disability: either Williams syndrome (WS; n=12) or Down syndrome (DS; n=21). Participants were assessed at three distinct developmental stages: early childhood (2 to 6 years), childhood (8 to 12 years), and adolescence (14 to 20 years). A sample was taken only once from each individual. Serum NGF levels were markedly higher in participants with WS, than DS and control participants. In addition, different developmental profiles emerged in the three groups: while in normally developing individuals NGF levels were higher in early childhood than later on, children with WS showed constantly elevated NGF levels. When compared to control participants, those with DS showed lower NGF levels only during early childhood. Neuropsychological assessment confirmed previously reported differences among the three groups in the development of linguistic/cognitive abilities. Some features of individuals with WS, such as hyperacusis and hypertension, could be related to high-circulating NGF levels.

Adolescent↗

Drawing abilities in Williams syndrome: a case study.

Children with Williams syndrome (WS) have been reported to exhibit an unusual cognitive profile characterized by marked preservation of linguistic abilities and poor visuospatial abilities against a backdrop of generalized mental retardation. Much of the data documenting this profile come from studies of older children and adults with WS. Very few studies have reported findings from the preschool and early school-age period. As a result, little is known about the early development of cognitive processes in children with WS. Capirci, Sabbadini, and Volterra (1996) reported data from a longitudinal case study of early language development in a young child with WS. This article presents the longitudinal profile of visuospatial abilities in this same child. Data on copying and free drawing collected over a period extending from late preschool to early school age are reported. It is clear from these data that this child does indeed exhibit deficits in visuospatial abilities. Her performance clearly improved with age, but deficits persist.

Child↗

[Williams syndrome].

Williams syndrome (WS) is a rare (2-5/100,000) genetic human disorder characterised by a typical facies and mental retardation with a deficit in the visuospatial cognitive function and a relative preservation of linguistic abilities in general, and spoken language in particular. This syndrome also includes morphological anomalies, metabolic functional impairments, and likely deficits in the pattern of brain ontogenesis. The genetic basis of WS, recently identified, are presented. A cognitive profile of the WS individuals is defined and compared to Down syndrome (DS) and autism cognitive profiles. Neuroanatomical features of WS, including a reduction in brain volume, preservation of cerebellum and frontal lobes, and a reduction of posterior cortical systems, are described. The possible role of NGF (nerve growth factor)--a neurotrophin involved in the development of brain cholinergic systems and the associated behavioural functions--in the aetiology of the typical mental retardation of WS patients, is critically discussed. Future research avenues, including the identification of potential neurobiological markers in order to precociously diagnose this syndrome, are reviewed.

Biomarkers↗

Teaching sign language to hearing children as a possible factor in cognitive enhancement.

We describe an educational experience designed to teach Italian Sign Language (LIS) to a group of hearing children. The hypothesis underlying this experience was that learning a visual-gestural language such as LIS may improve children's attentional abilities, visual discrimination, and spatial memory. To examine this hypothesis, we conducted two studies. The first involved an educational experience lasting two years with a group of hearing children attending a Sign Language class from first to second grade. The Raven PM 47 TEST was administered at the beginning and at the end of each school year to children attending the LIS classes and to a control group of children enrolled in the same school but not exposed to LIS. The second study involved an educational experience in first grade. The Raven PM 47 and Corsi's block tapping tests were administered at the beginning and at the end of the school year to the children attending the LIS classes, to children enrolled in the same school but at tending an English class, and to children not exposed to a second language. We found that in both studies the LIS group performed better than the other groups. These results suggest that learning a sign language may lead to a cognitive advancement in hearing children.

Journal Article↗

[Williams syndrome: a window to the development of cognitive and neural processes].

Williams syndrome (SW) is a rare (2-5/100,000) genetic human disorder characterised by a typical facies and mental retardation with a deficit in the visuo-spatial cognitive function and a relative preservation of linguistic abilities. This syndrome also includes morphological anomalies and metabolic-functional impairments, likely deficits in the pattern of brain ontogenesis. Neuropsychological and somatic features of the SW individuals are illustrated, and the correspondent genetic bases, recently identified, are presented. The possible role of NGF (nerve growth factor), a particular neurotrophin involved in the development of brain cholinergic system and the associated behavioural functions, in the aetiology of the typical mental retardation of SW patients, is critically discussed. Prospect of researches, including the identification of potential neurobiological markers and the definition of appropriate cognitive profiles of the SW, in order to precociously diagnose this syndrome, and a more thorough investigation of factors affecting phenotypic expression of this genetically determined pathological condition, are reviewed.

Adolescent↗

Linguistic abilities in Italian children with Williams syndrome.

Recent studies on subjects with Williams syndrome (WS) have revealed a particular facility for language, rarely observed in other mental retarded populations, inspiring much belief in the independence of language from cognition. Lexical and morphosyntactic abilities of 17 Italian WS individuals, between 4.10 and 15.3 years of age, were evaluated both in comprehension and production and compared with those of normally developing Italian children. WS subjects look similar to normal controls in lexical comprehension, but they appear to perform more poorly in grammatical comprehension. Furthermore they look deviant from normals in some morphosyntactic aspects of their production. They perform better than normal controls only with respect to phonological fluency, when semantic aspects are not involved. Our data show very little evidence for a dissociation between language and cognition.

Adolescent↗