Children with williams syndrome: Is there a single neuropsychological profile?

Pezzini, G.; Vicari, Stefano; Volterra, Virginia; Milani, L.; Ossella, Maria Teresa

doi:10.1080/87565649909540742

Cited by 80 publications

(67 citation statements)

References 22 publications

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“…Also different cognitive profi les were described in participants with comparable intellectual defi cits or even with the same aetiology (Vicari, Albertini & Caltagirone, 1992;Pezzini et al, 1999). All these observations seem to support a theoretical approach that considers intellectual disabilities not as a mere slowing of normal cognitive development, but as distinct, individual profi les, that can be qualitatively specifi ed.…”

Section: Introductionmentioning

confidence: 64%

“…Children with Williams syndrome often show marked impairment in certain visual-spatial abilities (especially praxic-constructive) and relative preservation of both productive and receptive language, at least concerning the phonological elements (Volterra, Capirci, Pezzini, Sabbadini & Vicari, 1996;Pezzini, Vicari, Volterra, Milani & Ossella, 1999). Also different cognitive profi les were described in participants with comparable intellectual defi cits or even with the same aetiology (Vicari, Albertini & Caltagirone, 1992;Pezzini et al, 1999).…”

Section: Introductionmentioning

confidence: 99%

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Implicit versus explicit memory function in children with Down and Williams syndrome

Vicari¹

2001

Downs Syndr. Res. Pract.

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-The present study was aimed at evaluating implicit memory processes in participants with Williams syndrome and comparing them to children with Down syndrome and to mental-age matched typically developing children. For this purpose, tests of verbal and visuo-perceptual explicit memory, verbal and visual repetition priming as well as procedural learning tasks were administered to 12 participants with Williams syndrome, 14 with Down syndrome and 32 typically developing children. Participants with Williams syndrome showed a level of repetition priming similar to that of mental-age typically developing controls. In contrast, children with Williams syndrome showed a reduced learning rate in the two procedural tasks. As regards children with Down syndrome, we document comparable implicit memory abilities. In contrast, regarding explicit memory, typically developing children performed better than individuals with Down syndrome. This fi nding is relevant for our knowledge about the qualitative aspects of the anomalous cognitive development in individuals with intellectual disabilities and the neurobiological substrate underlying this development.

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Section: Introductionmentioning

confidence: 64%

Section: Introductionmentioning

confidence: 99%

Implicit versus explicit memory function in children with Down and Williams syndrome

Vicari¹

2001

Downs Syndr. Res. Pract.

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“…Other methodological challenges include: the focus on group averages despite extensive cognitive heterogeneity in WS (Pezzini, Vicari, Voltera, Milani, & Ossella, 1999;Porter & Coltheart, 2005); the need to consider regression to the mean, the phenomenon whereby extreme scores are more likely to change over time (revert closer to the mean) than scores that are less extreme; and standard error of measurement (whether significant changes in scores are clinically meaningful or reflect statistical error).…”

Section: Other Methodological Challengesmentioning

confidence: 99%

A Longitudinal Study of Cognitive Abilities in Williams Syndrome

Porter

Dodd

2011

Developmental Neuropsychology

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“…They argue that the reduced frequency effect in WS might be the result of a rigid use of a phonological encoding strategy for both high and low frequency words and propose that people with WS might be "hyper-phonological". The authors relate this pattern of results to WS linguistic abilities where there is some evidence for a pattern of well-preserved phonology coupled with impairments in lexical-semantics and syntax (Capirci et al, 1996;Pezzini et al, 1999;.…”

Section: Introductionmentioning

confidence: 99%

“…There is also some evidence for differences in the processing of concrete and abstract items at the neurological level (Breedin et al, 1994;Weiss and Rappelsberger, 1996). Concrete words are thought to be 'richer' in terms of the number of features that define them (Plaut and Shallice, 1993) This 'richness' may explain concreteness effects in short-term memory. Recent studies have demonstrated that both normal children (Nation et al, 1999) and adults (Walker and Hulme, 1999) recall concrete words more easily than abstract words.…”

Section: Introductionmentioning

confidence: 99%

Love Is… AN ABSTRACT WORD: THE INFLUENCE OF LEXICAL SEMANTICS ON VERBAL SHORT-TERM MEMORY IN WILLIAMS SYNDROME

Laing

Grant²,

Thomas³

et al. 2005

Cortex

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Contact : lib-eprints@bbk.ac.uk Laing, E.; Grant, J.; Thomas, M.; Parmigiani, C.; Ewing, S.; KarmiloffSmith, A. (2005) Love is . . . an abstract word: the influence of phonological and semantic factors on verbal short-term memory in Williams syndrome -Cortex, 41(2), pp. 169-179 INTRODUCTIONWilliams syndrome (WS) is a rare genetic disorder resulting from a micro deletion on one copy of chromosome 7q11.23 (Ewart et al., 1993;Frangiskakis et al., 1996;Tassabehji et al., 1996; for up-to-date review, see Donnai and KarmiloffSmith, 2002). It is characterised by specific physical anomalies including facial dysmorphology, and renal and cardiovascular anomalies. Abnormalities are also found in brain volume (Jernigan et al., 1993), brain structure (Galaburda et al., 1994) and brain biochemistry (Rae et al., 1998).Williams syndrome is typically characterised by an uneven cognitive profile: despite low IQs (predominantly in the 50-65 range), language and face-processing capacities have been shown to be relative strengths while more serious deficits are found in visuo-spatial cognition, number, problemsolving and planning (Arnold et al., 1985;Udwin and Dennis, 1995;Bellugi et al., 1990). The surprising linguistic fluency of individuals with WS led initially to claims that language in WS is 'selectively preserved' (Bellugi et al., 1988). However, more recent studies have suggested that the WS language system is not only delayed but follows an atypical developmental pathway that impacts on all facets of language Singer-Harris et al., 1997;Thomas and Karmiloff-Smith, 2003).The present paper focuses on one particular aspect of the WS cognitive profile, verbal shortterm memory. Verbal short-term memory has been considered a relative strength in WS and has been shown to be at the level of mental age (Udwin and Yule, 1990) or even higher than mental age (Mervis et al., 1999). In contrast, spatial short-term memory is severely impaired. Wang and Bellugi (1994) compared short-term memory in individuals with Williams syndrome and DS and found that while the WS group performed better than the DS group on a verbal short-term memory task, the Down syndrome group outperformed the WS group on a test of visual-spatial memory. Jarrold et al. (1999) extended these findings by demonstrating that this pattern holds even when differences in verbal and non-verbal skills are taken into account. Vicari et al. (1996a) further examined the dissociation between verbal and visuo-spatial memory by considering both short-and long-term memory processing. As in previous studies, they found that when compared to typically developing controls, individuals with Williams syndrome had comparable verbal memory spans and significantly lower spatial memory. However, this pattern was not replicated in long-term memory, where verbal and spatial memory were both impaired. On the basis of these findings, Vicari et al. (1996a) argue for a further dissociation in WS memory, between normally developing verbal short-term memory and deficient long-term memory.Other ...

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Children with williams syndrome: Is there a single neuropsychological profile?

Cited by 80 publications

References 22 publications

Implicit versus explicit memory function in children with Down and Williams syndrome

Implicit versus explicit memory function in children with Down and Williams syndrome

A Longitudinal Study of Cognitive Abilities in Williams Syndrome

Love Is… AN ABSTRACT WORD: THE INFLUENCE OF LEXICAL SEMANTICS ON VERBAL SHORT-TERM MEMORY IN WILLIAMS SYNDROME

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