Journal of Autism and Developmental Disorders

, Volume 37, Issue 3, pp 513–522 | Cite as

Orientation and Affective Expression Effects on Face Recognition in Williams Syndrome and Autism

  • Fredric E. Rose
  • Alan J. Lincoln
  • Zona Lai
  • Michaela Ene
  • Yvonne M. Searcy
  • Ursula Bellugi
Original Paper


We sought to clarify the nature of the face processing strength commonly observed in individuals with Williams syndrome (WS) by comparing the face recognition ability of persons with WS to that of persons with autism and to healthy controls under three conditions: Upright faces with neutral expressions, upright faces with varying affective expressions, and inverted faces with neutral expressions. No differences were observed under the upright/neutral expression condition. However, the WS group was more accurate than the autism group when discriminating upright faces with varying affective expressions, whereas the opposite pattern emerged when discriminating inverted faces. We interpret these differences as a reflection of the contrasting social features of the two syndromes.


Autism Williams syndrome Emotion Affect Face processing Visual discrimination 



This work was supported, in part, by NIH grant PO1HD33113 Program Project awarded to U. Bellugi and by a seed money grant from the Alliant International University awarded to Alan J. Lincoln, Ph.D. We would like to acknowledge Julie R. Korenberg, MD, Ph.D., of Cedars-Sinai Medical Center and the University of California at Los Angeles for confirming the presence of the elastin deletion in our WS participants.


  1. Alvarez, T. D., & Neville, H. J. (1995). The development of face recognition continues into adulthood: An ERP study. Neuroscience Abstracts, 21, 2086.Google Scholar
  2. American Academy of Pediatrics. (2001). Health care supervision for children with Williams syndrome. Pediatrics, 107, 1190–1204.Google Scholar
  3. American Psychiatric Association (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Association.Google Scholar
  4. Atkinson, J., Anker, S., Braddick, O., Nokes, L., Mason, A., Braddick, F., et al. (2001). Visual and visuospatial development in young children with Williams syndrome. Developmental Medicine & Child Neurology, 43(5), 330–337.CrossRefGoogle Scholar
  5. Baird, C., Charman, T., Baron-Cohen, S., Cox, A., Swettenham, J., Wheelwright, S., & Drew, A. (2000). A screening instrument for autism at 18thmonths of age: A 6-year follow-up study. Journal of the American Academy of Child and Adolescent Psychiatry, 39, 694–702.PubMedCrossRefGoogle Scholar
  6. Baron-Cohen, S., Cox, A., Baird, G., Swettenham, J., Nightingale, N., Morgan, K., Drew, A., & Charman, T., (1996). Psychological markers in the detection of autism in infancy in a large population. British Journal of Psychiatry, 168, 158–163.PubMedGoogle Scholar
  7. Bartlett, J. C., & Searcy, J. (1993). Inversion and configuration of faces. Cognitive Psychology, 25, 281–316.PubMedCrossRefGoogle Scholar
  8. Bellugi, U., Lichtenberger, L., Jones, W., Lai, Z., & St. George, M. (2000). The Neurocognitive profile of Williams syndrome: A complex pattern of strengths and weaknesses. Journal of Cognitive Neuroscience, 12(supplement), 7–29.Google Scholar
  9. Bellugi, U., Wang, P. P., & Jernigan, T. L. (1994). Williams syndrome: An unusual neuropsychological profile. In: S. Broman, J. Grafman (Eds.), Atypical cognitive deficits in developmental disorders: Implications for brain function. Hillsdale NJ: Lawrence Erlbaum Associates, Inc.Google Scholar
  10. Benton, A. L., Hamsher, K. de S., Varney, N. R., & Spreen, O. (1983). Contributions to neuropsychological assessment. New York: Oxford University Press.Google Scholar
  11. Boucher, J., & Lewis, V. (1992). Unfamiliar face recognition in relatively able autistic children. Journal of Child Psychology and Psychiatry, 33, 843–859.PubMedCrossRefGoogle Scholar
  12. Bradshaw, J. L., & Wallace, G. (1971). Models for the processing and identification of faces. Perception and Psychophysics, 9, 443–448.Google Scholar
  13. Cohen, D.J., & Volkmar, F. R. (1997). Handbook of Autism and Pervasive Developmental Disorders (2nd ed.). New York: Wiley.Google Scholar
  14. Corsello, C. M. (2000). Recognition of faces and the individual with autism: Saliency of facial features. Dissertation Abstracts International, 61, 4-B.Google Scholar
  15. Crisco, J. J., Dobbs, J. M., & Mulhern, R. K. (1988). Cognitive processing of children with Williams syndrome. Developmental Medicine & Child Neurology, 30, 650–656.CrossRefGoogle Scholar
  16. Critchley, H., Daly, E., Phillips, M., Brammer, M., Bullmore, E., et al. (2000). Explicit and implicit neural mechanisms for processing of social information from facial expressions: A functional magnetic resonance imaging study. Human Brain Mapping, 9, 93–105.PubMedCrossRefGoogle Scholar
  17. Davies, S., Bishop, D., Manstead, A. S. R., & Tantam, D. (1994). Face perception in children autism and Asperger’s syndrome. Journal of Child Psychology and Psychiatry, 35, 1033–1057.PubMedCrossRefGoogle Scholar
  18. Deruelle, C., Mancini, J., Livet, M. O., Casse-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
  19. Diamond, R., & Carey, S. (1986). Why faces are and are not special: An effect of expertise. Journal of Experimental Psychology: General, 115, 107–117.CrossRefGoogle Scholar
  20. Elgar, K., & Campbell, R. (2001). Annotation: the cognitive neuroscience of face recognition: implications for developmental disorders. Journal of Child Psychology and Psychiatry, 42(6), 705–717.PubMedCrossRefGoogle Scholar
  21. Ellis, H. D. (1990). Developmental trends in face recognition. The Psychologist, 3, 114–119.Google Scholar
  22. Farah, M. J. (1996). Is face recognition “special”? Evidence from neuropsychology. Behavioural Brain Research, 76, 181–189.PubMedCrossRefGoogle Scholar
  23. Farah, M. J., Tanaka, J. N., & Drain, M. (1995). What causes the face inversion effect. Journal of Experimental Psychology: Human Perception and Performance, 21, 628–634.PubMedCrossRefGoogle Scholar
  24. Farah, M. J., Wilson, K. D., Drain, M., & Tanaka, J. N. (1998). What is “Special” about face perception? Psychological Review, 105, 482–498.PubMedCrossRefGoogle Scholar
  25. Galaburda, A. M., Bellugi, U. (2000). V. Multi-level analysis of cortical neuroanatomy in Williams syndrome. Journal of Cognitive Neuroscience, 12(supplement), 74–88.PubMedCrossRefGoogle Scholar
  26. Galaburda, A. M., Holinger, D. P., Bellugi, U., & Sherman, G. F. (2002). Williams syndrome: Neuronal size and neuronal packing density in primary visual cortex. Archives of Neurology, 59, 1461–1467.PubMedCrossRefGoogle Scholar
  27. Gauthier, I., Williams, P., Tarr, M. J., & Tanaka, J. (1998). Training ‘greeble’ experts: A framework for studying expert object recognition processes. Vision Research, 38, 2401–2428.PubMedCrossRefGoogle Scholar
  28. Gilliam, J. E. (1995). Gilliam Autism Rating Scale, examiner’s manual. Austin, TX: ProEd.Google Scholar
  29. Grelotti, D. J., Gauthier, I., & Schultz, R. T. (2002). Social interest and the development of cortical face specialization: What autism teaches us about face processing. Developmental Psychobiology, 40, 213–225.PubMedCrossRefGoogle Scholar
  30. Grice, S. J., Spratling, M. W., Karmiloff-Smith, A., Halit, H., Csibra, G., de Haan, M., Johnson, M. H. (2001). Disordered visual processing and oscillatory brain activity in autism and Williams syndrome. Neuroreport, 12(12), 2697–2700.PubMedCrossRefGoogle Scholar
  31. Happe, F. (1999). Understanding assets and deficits in autism: Why success is more interesting than failure. Psychologist, 12, 540–546.Google Scholar
  32. Hauck, M., Fein, D., Maltby, N., Waterhouse, L., & Feinstein, C. (1998). Memory for faces in children with autism. Child Neuropsychology, 4(3), 187–198.Google Scholar
  33. Haxby, J. V., Hoffman, E. A., & Gobbini, M. I. (2002). Human neural systems for face recognition and social communication. Biological Psychiatry, 51, 59–67.PubMedCrossRefGoogle Scholar
  34. Hobson, R. P., Ouston, J., & Lee, A. (1988). Emotion recognition in autism: Coordinating faces and voices. Psychological Medicine, 18, 911–923.PubMedCrossRefGoogle Scholar
  35. Jarrold, C., Baddeley, A. D., & Hewes, A. K. (1999). Genetically dissociated components of working memory: Evidence from Down’s and Williams syndrome. Neuropsychologia, 37, 637–651.PubMedCrossRefGoogle Scholar
  36. Jones, W., & Lai, Z. C. (1997). The relationship between intact face processing and impaired spatial cognition in Williams syndrome. International Behavioral Neuroscience Society Abstracts, 6, #P2–51, p. 59.Google Scholar
  37. Jones, W., Anderson, D., Reilly, J., & Bellugi, U. (1998). Emotional expression in infants and children with Williams syndrome: A relationship between temperament and genetics? Journal of the International Neuropsychological Society, 4, 56.Google Scholar
  38. Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., & Adolphs, R. (2000). Hypersociability in Williams syndrome. Journal of Cognitive Neuroscience, 10(supplement), 30–46.CrossRefGoogle Scholar
  39. Jones, W., Hickok, G., & Lai, Z. (1998). Does face processing rely on intact visual-spatial abilities? Evidence from Williams syndrome. Abstract, Cognitive neuroscience society 1998 annual meeting abstract program, 80, #67.Google Scholar
  40. Karmiloff-Smith, A. (1997). Crucial differences between developmental cognitive neuroscience and adult neuropsychology. Developmental Neuropsychology, 13, 513–524.CrossRefGoogle Scholar
  41. Klin, A., Sparrow, S. S., de Bildt, A., Cicchetti, D. V., Cohen, D. J., & Volkmar, F. R. (1999). A normed study of face recognition in autism and related disorders. Journal of Autism and Developmental Disorders, 29, 499–508.PubMedCrossRefGoogle Scholar
  42. Korenberg, J. R., Chen, X.-N., Hirota, H., Lai, Z., Bellugi, U., Burian, D., Roe, B., & Matsuoka, R. (2000). Geneome structure and cognitive map of Williams syndrome. Journal of the Cognitive Neuroscience Society, 12(Suppl), 89–107.Google Scholar
  43. Lai (1992). A neuropsychological test of cortical circuitry subserving affective processing in the developing human brain. Unpublished doctoral dissertation. University of Minnesota, Minneapolis.Google Scholar
  44. Le Couteur, A., Rutter, M., Lord, C., Rios, Robertson, Holdgrafer, & McLennan (1989). Autism diagnostic Interview: A standardized investigator-based instrument. Journal of Autism and Developmental Disorders, 19, 363–387.Google Scholar
  45. Maurer, D., LeGrand, R., & Mondloch, C. J. (2002). The many faces of configural processing. Trends in Cognitive Sciences, 6, 255–260.PubMedCrossRefGoogle Scholar
  46. Mills, D. L., Alvarez, T. D., St. George, M., Appelbaum, L. G., Bellugi, U., & Neville, H. (2000). Electrophysiological studies of face processing in Williams syndrome. Journal of Cognitive Neuroscience, 10(supplement), 47–64.Google Scholar
  47. Mobbs, D., Garrett, A. S., Menon, V., Rose, F. E., Bellugi, U., & Reiss, A. L. (2004). Anomalous brain activation during face and gaze processing in Williams syndrome. Neurology, 62(11), 2070–2076.PubMedGoogle Scholar
  48. Mondloch, C. J., Le Grand, R., & Maurer, D. (2002). Configural Face Processing develops more slowly than featural face processing. Perception, 31, 553–566.PubMedCrossRefGoogle Scholar
  49. Ozonoff, S., Pennington, B.F., & Rogers, S.J. (1990). Are there emotion perception deficits in young autistic children? Journal of Child Psychology and Psychiatry, 31(3), 343–361.PubMedCrossRefGoogle Scholar
  50. Pelphrey, K. A., Sasson, N. J., Reznick, J. S., Paul, G., Goldman, B. D., & Piven, J. (2002). Visual scanning of faces in autism. Journal of Autism and Developmental Disorders, 32, 249–261.PubMedCrossRefGoogle Scholar
  51. Phillips, W., Baron-Cohen, S., & Rutter, M. (1992). The role of eye contact in goal detection: evidence from normal infants and children with autism or mental handicap. Developmental Psychopathology, 4, 375–383.CrossRefGoogle Scholar
  52. Pierce, K., Muller, R.-A., Ambrose, J., Allen, G., & Courchesne, E. (2001). Face processing occurs outside the fusiform ‘face area’ in autism: evidence from functional MRI. Brain, 124, 2059–2073.PubMedCrossRefGoogle Scholar
  53. Pober, B. R., Dykens, E. M. (1996). Williams syndrome: An overview of medical, cognitive, and behavioral features. Child and Adolescent Psychiatric Clinics of North America, 5, 929–943.Google Scholar
  54. Reiss, A. L., Eliez, S., Schmitt, J. E., Straus, E., Lai, Z., Jones, W., et al. (2000). IV. Neuroanatomy of Williams syndrome: a high-resolution MRI study. Journal of Cognitive Neuroscience, 12(supplement), 65–73.PubMedCrossRefGoogle Scholar
  55. Rhodes, G. (1988). Looking at faces: First-order and second-order features as determinantas of facial appearance. Perception, 17, 43–63.PubMedCrossRefGoogle Scholar
  56. Rhodes, G., Brake, S., & Atkinson, A. P. (1993). What’s lost in inverted faces? Cognition, 47, 25–57.PubMedCrossRefGoogle Scholar
  57. Rossen, M. L., Jones, W., Wang, P. P., & Klima, E. S. (1995). Face processing: Remarkable sparing in Williams syndrome. Special Issue, Genetic Counseling, 6, 138–140.Google Scholar
  58. Schmitt, J. E., Watts, K., Eliez, S., Bellugi, U., Galaburda, A. M., & Reiss, A. L. (2002). Increased gyrification in Williams syndrome: Evidence using 3D MRI methods. Developmental Medicine & Child Neurology, 44, 292–295.CrossRefGoogle Scholar
  59. Senior, C., Barnes, J., Jenkins, R., Landau, S., Phillips, M. L., & David, A. S. (1999). Attribution of social dominance and maleness to schematic faces. Social Behavior and Personality, 27, 331–338.CrossRefGoogle Scholar
  60. Tager-Flusberg, H., Plesa-Skwerer, D., Faja, S., & Joseph, R. M. (2003). People with Williams syndrome process faces holistically. Cognition, 89, 11–24.PubMedCrossRefGoogle Scholar
  61. Tanaka, J. W., & Farah, M. (1993). Parts and wholes in face recognition. Quarterly Journal Of Experimental Psychology, 46(A), 225–245.Google Scholar
  62. Tarr, M. J., Gauthier, I. (2000). FFA: A flexible fusiform area for subordinate-level visual processing automatized by expertise [Review]. Nature Neuroscience, 3, 764–769.PubMedCrossRefGoogle Scholar
  63. Udwin, O., & Yule, W. (1991). A cognitive and behavioral phenotype in Williams syndrome. Journal of Clinical and Experimental Neuropsychology, 12(2), 232–244.Google Scholar
  64. Voeller, K. K. S. (1986). Right-hemisphere deficit syndrome in children. American Journal of Psychiatry, 143, 1004–1009.PubMedGoogle Scholar
  65. Wang, P. P., & Bellugi, U. (1994). Evidence from two genetic syndromes for a dissociation between verbal and visual-spatial short-term memory. Journal of Clinical & Experimental Neuropsychology, 16, 317–322.Google Scholar
  66. Weeks, S. J., & Hobson, R. P. (1987. The salience of facial expression for autistic children. Journal of Child Psychology and Psychiatry, 28, 137–151.PubMedCrossRefGoogle Scholar
  67. Yin, R. K. (1969). Looking at upside-down faces. Journal of Experimental Psychology, 81, 141–145.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • Fredric E. Rose
    • 1
  • Alan J. Lincoln
    • 1
    • 2
    • 3
  • Zona Lai
    • 1
  • Michaela Ene
    • 1
    • 2
    • 3
  • Yvonne M. Searcy
    • 1
  • Ursula Bellugi
    • 1
  1. 1.Laboratory for Cognitive NeuroscienceThe Salk Institute for Biological StudiesLa JollaUSA
  2. 2.Alliant International UniversitySan DiegoUSA
  3. 3.Center for Autism ResearchEvaluation and Service, Inc.San DiegoUSA

Personalised recommendations