Cross-Cultural Studies of
Williams Syndrome
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1
California State University, United States
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2
The Salk Institute for Biological Studies, United States
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3
San Diego State University, United States
This work is concerned with ways in which children with Williams syndrome (WS), a rare neurodevelopmental disorder arising from a hemizygous deletion in chromosome band 7q11.23, are affected by social experiences of markedly differing cultures: the United States and Japan in one study, and France, Italy as well as the United States in another study. WS presents a compelling model for this investigation because its genetic phenotype is well defined and results in an uneven cognitive and social profile, including overt over-friendliness toward strangers. While a number of research groups have been studying the cognitive strengths and weaknesses of individuals with WS in various countries, there have been no studies to date that explore the social phenotype in WS across different cultures.
We describe two studies examining the ways in which social behavior in WS, stemming from specific genetic underpinnings, can be mediated by cultural expectations. In the first study, we conducted a crosscultural comparison using an instrument that measures aspects of sociability commonly found among people with WS (the Salk Institute Sociability Questionnaire). Quantitative analyses revealed a significant effect of diagnostic category in that in both countries (Japan and the U.S.), children with WS were rated as significantly higher in global sociability and more likely to approach strangers than were their normal counterparts. There was also an effect of culture, in that regardless of diagnostic category, both WS and normal children in Japan were rated lower than their counterparts in the U.S. In another cross-cultural study, we examined the use of language for social purposes, using a narrative task for individuals with WS in the U.S., Italy and France. We examined aspects of language structure (morphology and syntax) as well as the evaluative language, which refers to lexically conveyed affect used for social purposes (e.g., emphatics, intensifiers, character speech and sound effects). We found that WS were significantly higher than age matched normal controls in the use of language that conveyed affect and sociability. At the same time, there was also a significant effect of culture across groups, with the Italians exceeding the levels of evaluative language found in the U.S., and the French using significantly less social evaluation. We, thus, conclude that the excessively social phenotype of children with Williams syndrome, although markedly present across cultures, appears to vary in its intensity by culture. These are intriguing illustrations of the interactions between the effects of a genetic predisposition and the effects of different cultural experiences.
Conference:
12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008.
Presentation Type:
Oral Presentation
Topic:
SESSION 7: Social Cognition and Social Phenotype of Williams Syndrome
Citation:
Comfort
CZ,
Bellugi
U and
Reilly
J
(2009). Cross-Cultural Studies of
Williams Syndrome.
Conference Abstract:
12th International Professional Conference on Williams Syndrome.
doi: 10.3389/conf.neuro.09.2009.07.026
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Received:
30 Apr 2009;
Published Online:
30 Apr 2009.
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Correspondence:
C. Z Comfort, California State University, Long Beach, United States, ccomfort@csulb.edu