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Journal of Autism and Developmental Disorders

, Volume 39, Issue 11, pp 1552–1559 | Cite as

Human Versus Non-Human Face Processing: Evidence from Williams Syndrome

  • Andreia Santos
  • Delphine Rosset
  • Christine Deruelle
original paper

Abstract

Increased motivation towards social stimuli in Williams syndrome (WS) led us to hypothesize that a face’s human status would have greater impact than face’s orientation on WS’ face processing abilities. Twenty-nine individuals with WS were asked to categorize facial emotion expressions in real, human cartoon and non-human cartoon faces presented upright and inverted. When compared to both chronological and mental age-matched controls, WS participants were able to categorize emotions from human, but not from non-human faces. The use of different perceptual strategies to process human and non-human faces could not explain this dissociation. Rather, the findings suggest an increased sensitivity to socially relevant cues, such as human facial features, possibly related to the hallmark feature of WS—hypersociability.

Keywords

Facial expressions Cartoon faces Inversion effect Williams syndrome 

Notes

Acknowledgments

We are grateful to all the participants, their parents and the Regional Williams Syndrome Associations. Andreia Santos was supported by a grant from the FCT-MCTES (Fundação para a Ciência e a Tecnologia, Ministério Da Educação, Tecnologia e do Ensino Superior, SFRH/BD/18820/2004) to conduct this study. We are also grateful to Duncan Milne for his insightful comments on this manuscript.

References

  1. Baron-Cohen, S., Tager-Flusberg, H., & Cohen, D. J. (2000). Understanding other minds (2nd ed.). New York: Oxford University Press.Google Scholar
  2. Bayes, M., Magano, L. F., Rivera, N., Flores, R., & Perez-Jurado, L. A. (2003). Mutational mechanisms of Williams-Beuren syndrome deletions. American Journal of Human Genetics, 73(1), 131–151.PubMedCrossRefGoogle Scholar
  3. Bellugi, U., Lichtenberger, L., Jones, W., Lai, Z., & St. George, M. (2000). I. The neurocognitive profile of Williams syndrome: A complex pattern of strengths and weaknesses. Journal of Cognitive Neuroscience, 12(Suppl 1), 7–29.PubMedCrossRefGoogle Scholar
  4. Bellugi, U., Marks, S., Bihrle, A., & Sabo, H. (1988a). Dissociation between language and cognitive functions in Williams syndrome. In D. Bishop & K. Mogford (Eds.), Language development in exceptional circumstances (pp. 177–189). Hillsdale, NJ: Erlbaum.Google Scholar
  5. Bellugi, U., Sabo, H., & Vaid, J. (1988b). Spatial deficits in children with Williams syndrome. In J. Stiles-Davis, U. Kritchevshy, & U. Bellugi (Eds.), Spatial cognition: Brain bases and development (pp. 273–297). Hillsdale, New Jersey: Lawrence Erlbaum.Google Scholar
  6. Bellugi, U., Wang, P. P., & Jernigan, T. L. (1994). Williams syndrome: An unusual neuropsychological profile. In S. H. Broman & J. Grafman (Eds.), Atypical cognitive deficits in developmental disorders: Implications for brain function (pp. 23–56). Hillsdale, NJ: Lawrence Erlbaum Associates.Google Scholar
  7. Bukach, C. M., Gauthier, I., & Tarr, M. J. (2006). Beyond faces and modularity: The power of an expertise framework. Trends in Cognitive Sciences, 10(4), 159–166.PubMedCrossRefGoogle Scholar
  8. Deruelle, C., Mancini, J., Livet, M. O., Cassé-Perrot, C., & de Schonen, S. (1999). Configural and local processing of faces in children with Williams syndrome. Brain and Cognition, 41, 276–298.PubMedCrossRefGoogle Scholar
  9. Deruelle, C., Rondan, C., Mancini, J., & Livet, M. O. (2003). Exploring face processing in Williams syndrome. Cognition, Brain and Behaviour, 7(2), 157–172.Google Scholar
  10. Deruelle, C., Rondan, C., Mancini, J., & Livet, M. O. (2006). Do children with Williams syndrome fail to process visual configural information? Research in Developmental Disabilities, 27, 243–253.PubMedCrossRefGoogle Scholar
  11. Deruelle, C., & Santos, A. (2009). Happy, sad or angry? What strategies do children with Williams syndrome use to recognize facial expressions of emotion? Evolution Psychiatrique, 74, 55–63.CrossRefGoogle Scholar
  12. Dykens, E. M., & Rosner, B. A. (1999). Refining behavioral phenotypes: Personality-motivation in Williams and Prader-Willi syndromes. American Journal on Mental Retardation, 104, 158–169.PubMedCrossRefGoogle Scholar
  13. Farran, E. K., & Jarrold, C. (2003). Visuo-spatial cognition in Williams syndrome: Reviewing and accounting for the strengths and weaknesses in performance. Developmental Neuropsychology, 23, 173–200.PubMedCrossRefGoogle Scholar
  14. Farran, E. K., Jarrold, C., & Gathercole, S. E. (2003). Divided attention, selective attention and drawing: processing preferences in Williams syndrome are dependent on the task administered. Neuropsychologia, 41, 676–687.PubMedCrossRefGoogle Scholar
  15. Gagliardi, C., Frigerio, E., Burt, D. M., Cazzaniga, I., Perret, D. I., & Borgatti, R. (2003). Facial expression recognition in Williams syndrome. Neuropsychologia, 41, 733–738.PubMedCrossRefGoogle Scholar
  16. Gauthier, I., & Nelson, C. A. (2001). The development of face expertise. Current Opinion in Neurobiology, 11(2), 219–224.PubMedCrossRefGoogle Scholar
  17. Gosch, A., & Pankau, R. (1994). Social-emotional and behavioural adjustment in children with Williams-Beuren syndrome. American Journal of Medical Genetics, 52, 291–296.PubMedCrossRefGoogle Scholar
  18. Jarrold, C., Baddeley, A. D., & Hewes, A. K. (1998). Verbal and non-verbal abilities in Williams syndrome phenotype: Evidence for diverging developmental trajectories. Journal of Child Psychology and Psychiatry, 39, 511–523.PubMedCrossRefGoogle Scholar
  19. Järvinen-Pasley, A., Bellugi, U., Reilly, J., Mills, D. L., Galaburda, A., et al. (2008). Defining the social phenotype in Williams syndrome: A model for linking gene, the brain, and behavior. Development and Psychopathology, 20, 1–35.PubMedCrossRefGoogle Scholar
  20. Johnson, M. H., & Morton, J. (1991). Biology and cognitive development. The case of face recognition. Oxford: Blackwell.Google Scholar
  21. Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., et al. (2000). Hypersociability in Williams syndrome. Journal of Cognitive Neuroscience, 12(Suppl 1), 30–46.PubMedCrossRefGoogle Scholar
  22. Karmiloff-Smith, A., Klima, E., Bellugi, U., Grant, J., & Baron-Cohen., S. (1995). Is there a social module? Language, face-processing and theory of mind in Williams syndrome. Journal of Cognitive Neuroscience, 7, 196–208.CrossRefGoogle Scholar
  23. Karmiloff-Smith, A., Thomas, M., Annaz, D., Humphreys, K., Ewing, S., Brace, N., et al. (2004). Exploring the Williams syndrome face-processing debate: The importance of building developmental trajectories. Journal of Child Psychology and Psychiatry, 45(7), 1258–1274.PubMedCrossRefGoogle Scholar
  24. Korenberg, J. R., Chen, X. N., Hirota, H., Lai, Z., Bellugi, U., Burian, D., et al. (2000). IV. Genome structure and cognitive map of Williams syndrome. Journal of Cognitive Neuroscience, 12(Suppl. 1), 89–107.PubMedCrossRefGoogle Scholar
  25. Laing, E., Butterworth, G., Ansari, D., Gsödl, M., Longhi, E., Panagiotaki, G., et al. (2002). Atypical development of language and social communication in toddlers with Williams syndrome. Developmental Science, 5, 233–246.CrossRefGoogle Scholar
  26. Martens, M. A., Wilson, S. J., & Reutens, D. C. (2008). Williams syndrome: A critical review of the cognitive, behavioral, and neuroanatomical phenotype. Journal of Child Psychology and Psychiatry, 49(6), 576–608.PubMedCrossRefGoogle Scholar
  27. Martinez, A. M., & Benavente, R. (1998). The AR Face Database. CVC Technical Report, #24 June 1998.Google Scholar
  28. Mervis, C. B., Robinson, B., Bertrand, J., Morris, C. A., Klein-Tasman, B., & Armstrong, S. (2000). The Williams syndrome cognitive profile. Brain and Cognition, 44, 604–628.PubMedCrossRefGoogle Scholar
  29. Plesa-Skwerer, D., Faja, S., Schofield, C., Verballis, A., & Tager-Flusberg, H. (2006). Perceiving facial and vocal expressions of emotion in individuals with Williams syndrome. American Journal on Mental Retardation, 111(1), 15–26.PubMedCrossRefGoogle Scholar
  30. Pober, B. R., & Dyckens, E. M. (1996). Williams syndrome: An overview of medical, cognitive, and behavioural features. Child and Adolescent Psychiatric Clinics of North America, 5, 929–943.Google Scholar
  31. Preus, M. (1984). The Williams syndrome: Objective definition and diagnosis. Clinical Genetics, 25(5), 422–428.PubMedCrossRefGoogle Scholar
  32. Rhodes, G., Brake, S., & Atkinson, A. (1993). What’s lost in inverted faces? Cognition, 47, 25–27.PubMedCrossRefGoogle Scholar
  33. Rose, F. E., Lincoln, A. J., Lai, Z., Ene, M., Searcy, Y. M., & Bellugi, U. (2007). Orientation and affective expression effects on face recognition in Williams Syndrome and Autism. Journal of Autism and Developmental Disorders, 37(3), 513–522.PubMedCrossRefGoogle Scholar
  34. Rosset, D., Rondan, C., Da Fonseca, D., Santos, A., Assouline, B., & Deruelle, C. (2008). Typical emotion processing for cartoon but not for real faces in children with autistic spectrum disorders. Journal of Autism and Developmental Disorders, 38, 919–925.PubMedCrossRefGoogle Scholar
  35. Santos, A., & Deruelle, C. (2009). Verbal peaks and visual valleys in theory of mind ability in Williams syndrome. Journal of Autism and Developmental Disorders, 39(4), 651–659.PubMedCrossRefGoogle Scholar
  36. Santos, A., Milne, D., Rosset, D., & Deruelle, C. (2007). Challenging symmetry on mental retardation: Evidence from Williams syndrome. In E. B. Heinz (Ed.), Mental retardation research advances (pp. 147–174). NY: Nova Science Publishers.Google Scholar
  37. Santos, A., Rondan, C., Milne, D., Démonet, J.-F., & Deruelle, C. (2008). Social relevance boosts context processing in Williams syndrome. Developmental Neuropsychology, 33(4), 1–12.CrossRefGoogle Scholar
  38. Tager-Flusberg, H., Pleasa-Skwerer, D., Faja, S., & Joseph, R. M. (2003). People with Williams syndrome process faces holistically. Cognition, 89, 11–24.PubMedCrossRefGoogle Scholar
  39. Udwin, O., & Yule, W. (1991). A cognitive and behavioural phenotype in Williams syndrome. Journal of Clinical and Experimental Neuropsychology, 13, 232–244.PubMedCrossRefGoogle Scholar
  40. Wechsler, D. (1996). Manual for intelligence scale for children (3rd ed.). New York: The Psychological Corporation.Google Scholar
  41. Wechsler, D. (1997). Wechsler adult intelligence scale (3rd ed.). New York: The Psychological Corporation.Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Andreia Santos
    • 1
  • Delphine Rosset
    • 1
    • 2
  • Christine Deruelle
    • 1
  1. 1.Centre National de Recherche ScientifiqueMediterranean Institute of Cognitive NeurosciencesMarseille cedex 20France
  2. 2.Centre Ressources AutismeHopital Ste MargueriteMarseilleFrance

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