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

Abstract

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 ...

Perfil comunicativo de indivíduos com a síndrome de Williams-Beuren

“…Este estudo mostrou que a ininteligibilidade de fala foi um dos itens com diferença estatística significante entre os grupos, junto com a persistên-cia temática, que prejudicou tanto a ocorrência de iniciativas quanto a de respostas durante a conversação, corroborando a presença de prejuízos tanto no processamento semântico quanto fonológico para os indivíduos com a SWB (9)(10) . Destaca-se aqui a ocorrência de alterações fonológicas para os casos com a SWB com rebaixamento intelectual significativo, segundo desempenho nos testes psicométricos utilizados para este estudo.…”

“…A integridade do sistema fonológico, como parte das habilidades de linguagem nesta síndrome, se sustenta pelos estudos que mostraram desempenho semelhante entre os indivíduos com a SWB com o de indivíduos de mesma idade mental e com desenvolvimento de linguagem típi-co (7)(8) . Porém, outros estudos que também compararam o desempenho dos indivíduos com a síndrome a outros de mesma idade mental e com ausência de dificuldades de linguagem, mostraram que tanto o processamento semântico quanto o fonológico podem estar prejudicados na SWB (9)(10) . Já, no que diz respeito à característica de fala fluente, atribuída aos indivíduos com a SWB, autores discordaram desta qualidade quando compararam o desempenho desses indivíduos a outros de mesma idade mental, com desempenho lingüístico dentro dos padrões de normalidade e que identificaram a presença de prejuízos na fluência verbal decorrente de dificuldades na seleção lexical.…”

Memory Profiles of Down, Williams, and Fragile X Syndromes: Implications for Reading Development

“…77,90,92,112,156 Furthermore, phonological similarity, word length, frequency, lexicality, concreteness, primacy, and recency have the same effects on immediate verbal memory in young people with WS as in typically developing peers or peers who have ID. 147,152,157,158 In contrast, young people with WS perform much more poorly than typically developing children on visuospatial memory tasks such as the Corsi span task, whether matched on receptive vocabulary, nonverbal ability, or general mental age. 91,150,153,159 -163 However, this impairment seems to be related primarily to spatial as opposed to visual memory.…”

Symmetry of Cortical Folding Abnormalities in Williams Syndrome Revealed by Surface-Based Analyses

“…Relatively intact language with a rich vocabulary in the presence of generally impaired cognition is a hallmark of WS (Laing et al, 2005;Ypsilnati et al, 2005). WS patients characteristically exhibit heightened emotional responses to music (Levitin et al, 2004) and other sounds (Levitin et al, 2005).…”