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

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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.

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References

  • Baron-Cohen, S., Tager-Flusberg, H., & Cohen, D. J. (2000). Understanding other minds (2nd ed.). New York: Oxford University Press.

    Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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 

  • 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 

  • 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 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • Gauthier, I., & Nelson, C. A. (2001). The development of face expertise. Current Opinion in Neurobiology, 11(2), 219–224.

    Article  PubMed  Google Scholar 

  • Gosch, A., & Pankau, R. (1994). Social-emotional and behavioural adjustment in children with Williams-Beuren syndrome. American Journal of Medical Genetics, 52, 291–296.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • Johnson, M. H., & Morton, J. (1991). Biology and cognitive development. The case of face recognition. Oxford: Blackwell.

    Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • Martinez, A. M., & Benavente, R. (1998). The AR Face Database. CVC Technical Report, #24 June 1998.

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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 

  • Preus, M. (1984). The Williams syndrome: Objective definition and diagnosis. Clinical Genetics, 25(5), 422–428.

    Article  PubMed  Google Scholar 

  • Rhodes, G., Brake, S., & Atkinson, A. (1993). What’s lost in inverted faces? Cognition, 47, 25–27.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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 

  • 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.

    Article  Google Scholar 

  • Tager-Flusberg, H., Pleasa-Skwerer, D., Faja, S., & Joseph, R. M. (2003). People with Williams syndrome process faces holistically. Cognition, 89, 11–24.

    Article  PubMed  Google Scholar 

  • Udwin, O., & Yule, W. (1991). A cognitive and behavioural phenotype in Williams syndrome. Journal of Clinical and Experimental Neuropsychology, 13, 232–244.

    Article  PubMed  Google Scholar 

  • Wechsler, D. (1996). Manual for intelligence scale for children (3rd ed.). New York: The Psychological Corporation.

    Google Scholar 

  • Wechsler, D. (1997). Wechsler adult intelligence scale (3rd ed.). New York: The Psychological Corporation.

    Google Scholar 

Download references

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.

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Authors and Affiliations

  1. Centre National de Recherche Scientifique, Mediterranean Institute of Cognitive Neurosciences, 31 Chemin Joseph Aiguier, 13402, Marseille cedex 20, France

    Andreia Santos, Delphine Rosset & Christine Deruelle

  2. Centre Ressources Autisme, Hopital Ste Marguerite, Marseille, France

    Delphine Rosset

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  1. Andreia Santos
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  2. Delphine Rosset
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  3. Christine Deruelle
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Correspondence to Christine Deruelle.

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Santos, A., Rosset, D. & Deruelle, C. Human Versus Non-Human Face Processing: Evidence from Williams Syndrome. J Autism Dev Disord 39, 1552–1559 (2009). https://doi.org/10.1007/s10803-009-0789-1

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