Congenital prosopagnosia: a preliminary report in the italian population

Author/s Maria Teresa Turano, Tessa Marzi, Maria Pia Viggiano
Publishing Year 2014 Issue 2013/4
Language Italian Pages 24 P. 381-404 File size 862 KB
DOI 10.3280/RIP2013-004003
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Congenital prosopagnosia (CP) is a lifelong deficit of face perception and recognition, not caused by brain damage and presumably present from birth. In the first phase of this study, face recognition abilities of 300 subjects were evaluated by using an integrated methodological approach, that involved the employment of a questionnaire, specifically designed for the self-assessment and an objective test, widely used for diagnostic purposes - the Cambridge Face Memory Test (CFMT, Duchaine and Nakayama , 2006). According to the score, we selected 12 potentially prosopagnosics individuals that subsequently underwent the CFMT. Three subjects presented congenital prosopagnosia; thus a preliminary prevalence rate was estimated. In the second phase of the study, the three subjects were tested with some behavioral experiments to explore different mechanisms of perceptual and memory face processing, compared to a healthy control group. The battery included: a task to test the face inversion effect; a recognition test of Italian famous faces; a task to test the familiarity for unknown faces with the classic paradigm Old-New; and finally, two recognition tasks involving non-face stimuli. Our results showed a specific deficit for faces, confirmed by a weaker memory face trace, and the absence of the classic inversion that may indicate an impairment of holistic process. All in all, our results show for the first time the presence of CP in the Italian population and suggest the important use of an integrated methodological approach for the selection of the clinical sample for future studies on CP in the Italian population.

Keywords: Congenital Prosopagnosia, face recognition, holistic processing, face inversion effect.

  1. de Gelder, B., & Rouw, R. (2000). Structural encoding precludes recognition of face parts in prosopagnosia. Cognitive Neuropsychology, 17, 89-102. DOI: 10.1080/026432900380508
  2. Avidan, G., Tanzer, M., Behrmann, M. (2011). Impaired holistic processing in congenital prosopagnosia. Neuropsychologia, 49, 2541-52. DOI: 10.1016/j.neuropsychologia.2011.05.002
  3. Behrmann, M., Avidan, G., Marotta, J.J., Kimchi, R. (2005). Detailed Exploration of Face-related Processing in Congenital Prosopagnosia: 1. Behavioral Findings. Journal of Cognitive Neuroscience, 17, 1130-1149. DOI: 10.1162/0898929054475154
  4. Behrmann, M., Avidan, G. (2005). Congenital prosopagnosia: faceblind from birth. Trends in Cognitive Sciences, 9,180-7. DOI: 10.1016/j.tics.2005.02.011
  5. Behrmann, M., Avidan, G., Gao, F., Black, S. (2007). Structural imaging reveals anatomical alterations in inferotemporal cortex in congenital prosopagnosia. Cerebral Cortex, 17, 2354-63. DOI: 10.1093/cercor/bhl144
  6. Benton, A.L., Sivan, A.B., Hamsher, K., Varney, N.R., Spreen, O. (1983) Contribution to Neuropsychological Assessment. New York: Oxford University Press.
  7. Bowles, D.C., McKone, E., Dawel, A., Duchaine, B., Palermo, R., Schmalzl, L., Rivolta, D., Wilson, E., & Yovel., G. (2009). Diagnosis prosopagnosia: Effect of ageing, sex, and participant-stimulus ethnic match on the Cambridge Face Memory Test and Cambridge Face Perception Test. Cognitive Neuropsychology, 26, 423-55. DOI: 10.1080/02643290903343149
  8. Carbon, C.C., Gruter, T., Weber, J.E., & Lueschow, A. (2007). Faces as objects of non-expertise: processing of thatcherised faces in congenital prosopagnosia. Perception, 36, 1635-45. DOI: 10.1068/p5467
  9. Crawford, J.R., & Howell, D.C. (1998). Comparing an individual’s test score against norms derived from small samples. The Clinical Neuropsychologist, 12, 482-486. DOI: 10.1076/clin.12.4.482.7241
  10. Crawford, J.R., & Garthwaite, P.H. (2002). Investigation of the single case in neuropsychology: confidence limits on the abnormality of test scores and test score differences. Neuropsychologia, 40, 1196-208. DOI: 10.1016/S0028-3932(01)00224-X
  11. de Haan, E.H., & Campbell, R. (1991). A fifteen year follow-up of a case of developmental prosopagnosia. Cortex, 27, 489-509. DOI: 10.1016/S0010-9452(13)80001-9
  12. Dinkelacker, V., Gruter, M., Klaver, P., Gruter, T., Specht, K., Weis, S., Kennerknecht, I., Elger, C.E., & Fernandez, G. (2011). Congenital prosopagnosia: multistage anatomical and functional deficits in face processing circuitry. Journal of Neurology, 258, 770-82. DOI: 10.1007/s00415-010-5828-5
  13. Duchaine, B.C., Nieminen-von Wendt, T., New, J., & Kulomaki, T. (2003). Dissociations of visual recognition in a developmental agnosic: evidence for separate developmental processes. Neurocase, 9, 380-9. DOI: 10.1076/neur.9.5.380.16556
  14. Duchaine, B., & Nakayama, K. (2004). Developmental prosopagnosia and the Benton Facial Recognition Test. Neurology, 62(1),1219-1220. DOI: 10.1212/01.WNL.0000118297.03161.B3
  15. Duchaine, B., Nakayama, K. (2005). Dissociations of face and object recognition in developmental prosopagnosia. Journal of Cognitive Neuroscience, 17, 249-261. DOI: 10.1162/0898929053124857
  16. Duchaine, B., & Nakayama, K. (2006). The Cambridge face memory test: Results for neurologically intact individuals and an investigation of its validity using inverted face stimuli and prosopagnosic participants. Neuropsychologia, 44, 576-585. DOI: 10.1016/j.neuropsychologia.2005.07.001
  17. Duchaine, B., Germine, L., & Nakayama, K. (2007). Family resemblance: Ten family members. with prosopagnosia and within-class object agnosia. Cognitive Neuropsychology, 24, 419-430. DOI: 10.1080/02643290701380491
  18. Duchaine, B., Murray, H., Turner, M., White, S., & Garrido, L. (2010). Normal social cognition in developmental prosopagnosia. Cognitive Neuropsychology, 25, 1-15. DOI: 10.1080/02643291003616145
  19. Duchaine, B. (2011). Developmental prosopagnosia. In: Handbook of Face Perception (Eds: Calder, Rhodes, Haxby, & Johnson), Oxford University Press.
  20. Gauthier, I., Tarr, M.J., Anderson, A.W., Skudlarski, P., & Gore, J.C. (1999). Activation of the middle fusiform 'face area' increases with expertise in recognizing novel objects. Nature Neuroscience, 2(6), 568-73. DOI: 10.1038/9224
  21. Gauthier, I., & Tarr, M.J. (1997). Becoming a “Greeble” expert: exploring mechanisms for face recognition. Vision Research, 37, 1673-82. DOI: 10.1016/S0042-6989(96)00286-6
  22. Garrido, L., Duchaine, B., & Nakayama, K. (2008). Face detection in normal and prosopagnosic individuals. Journal of Neuropsychology, 2, 219-240. DOI: 10.1348/174866407X246843
  23. Garrido, L., Furl, N., Draganski, B., Weiskopf, N., Stevens, J., Tan, G.C., Driver, J., Dolan, R.J., & Duchaine, B. (2009). Voxel-based morphometry reveals reduced grey matter volume in the temporal cortex of developmental prosopagnosics. Brain, 132, 3443-55. DOI: 10.1093/brain/awp271
  24. Grossi, D., Soricelli, A., Ponari, M., Salvatore, E., Quarantelli, M., Prinster, A., & Trojano, L. (2012). Structural connectivity in a single case of progressive prosopagnosia: The role of the right inferior longitudinal fasciculus. Cortex. DOI: 10.1016/j.cortex.2012.09.010
  25. Gruter, M., Gruter, T., Bell, V., Horst, J., Laskowski, W., & Sperling, K. (2007). Hereditary prosopagnosia: The first case series. Cortex, 43, 734-749. DOI: 10.1016/S0010-9452(08)70502-1
  26. Gruter, T., Gruter, M., & Carbon, C.C. (2008). Neural and genetic foundations of face recognition and prosopagnosia. Journal of neuropsychology, 2, 79-97. DOI: 10.1348/174866407X231001
  27. Humphreys, K., Avidan, G., & Behrmann, M. (2007). A detailed investigation of facial expression processing in congenital prosopagnosia as compared to acquired prosopagnosia. Experimental Brain Research, 176, 356-73. DOI: 10.1007/s00221-006-0621-5
  28. Kennerknecht, I., Gruter, T., Welling, B., Wentzek, S., Horst, J., Edwards, S., & Gruter, M. (2006). First report of prevalence of non-syndromic hereditary prosopagnosia (HPA). American journal of medical genetics. Part A, 140, 1617-22. DOI: 10.1002/ajmg.a.31343
  29. Kennerknecht, I., Plumpe, N., Edwards, S., & Raman, R. (2007). Hereditary prosopagnosia (hpa): the first report outside the caucasian population. Journal of Human Genetics, 52, 230-236. DOI: 10.1007/s10038-006-0101-6
  30. Kennerknecht, I., Ho, N.Y., & Wong, V.C.N. (2008). Prevalence of hereditary prosopagnosia (hpa) in hong kong chinese population. American journal of medical genetics. Part A, 146A, 2863-70. DOI: 10.1002/ajmg.a.32552
  31. Le Grand, R., Cooper, P.A., Mondloch, C.J., Lewis, T.L., Sagiv, N., de Gelder, B., & Maurer, D. (2007). What aspects of face processing are impaired in developmental prosopagnosia? Brain and Cognition, 61, 139-58. DOI: 10.1016/j.bandc.2005.11.005
  32. Lobmaier, J.S., Boelte, J., Mast, F.W., & Dobel, C. (2010). Configural and featural processing in humans with congenital prosopagnosia. Advances in Cognitive Psychology, 6, 23-34. DOI: 10.2478/v10053-008-0074-4
  33. Lundqvist, D., Flykt, A., & Ohman, A. (1998). The Karolinska Directed Emotional Faces - KDEF. Karolisnka Institut.
  34. Nishimura, M., Doyle, J., Humphreys, K., & Behrmann, M. (2010). Probing the face-space of individuals with prosopagnosia. Neuropsychologia, 48, 1828-41. DOI: 10.1016/j.neuropsychologia.2010.03.007
  35. Nunn, J.A., Postma, P., & Pearson, R. (2001). Developmental prosopagnosia: should it be taken at face value? Neurocase, 7, 15-27. DOI: 10.1093/neucas/7.1.15
  36. Palermo, R., Rivolta, D., Wilsonc, C.E., & Jeffery, L. (2011). Adaptive face space coding in congenital prosopagnosia: Typical figural aftereffects but abnormal identity aftereffects. Neuropsychologia, 49, 3801-12. DOI: 10.1016/j.neuropsychologia.2011.09.039
  37. Richler, J.J., Cheung, O.S., & Gauthier, I. (2011). Beliefs alter holistic face processing ... if response bias is not taken into account. Journal of Vision, 11, 17, DOI: 10.1167/11.13.17
  38. Rizzo, S., Venneri, A., & Papagno, C. (2002). Famous face recognition and naming test: A normative study. Neurological Sciences, 23(4), 153–159. DOI: 10.1007/s100720200056
  39. Rossion, B. (2008). Constraining the cortical face network by neuroimaging studies of acquired prosopagnosia. NeuroImage, 40, 423-426. DOI: 10.1016/j.-neuroimage.2007.10.047
  40. Schneider, W., Eschman, A., & Zuccolotto, A. (2002). E-Prime User’s Guide. Pittsburgh: Psychology Software Tools, Inc.
  41. Stollhoff, R., Jost, J., Elze, T., & Kennerknecht, I. (2011). Deficits in long-term recognition memory reveal dissociated subtypes in congenital prosopagnosia. PLoS One, 25, 6(1), 15702. DOI: 10.1371/journal.pone.0015702
  42. Susilo, T., McKone, E., Dennett, H., Darke, H., Palermo, R., Hall, A., Pidcock, M., Dawel, A., Jeffery, L., Wilson, C.E., & Rhodes, G. (2010). Face recognition impairments despite normal holistic processing and face space coding:
  43. evidence from a case of developmental prosopagnosia. Cognitive Neuropsychology, 27, 636-64. DOI: 10.1080/02643294.2011.613372
  44. Tree, J.J., & Wilkie, J. (2010). Face and object imagery in congenital prosopagnosia: A case series. Cortex, 46, 1189-1198. DOI: 10.1016/j.cortex.2010.03.005
  45. Wilmer, J.B., Germine, L., Chabris, C.F., Chatterjee, G., Williams, M., Loken, E., Nakayama, K., & Duchaine, B. (2010). Human face recognition ability is highly heritable. Proceedings of the National Academy of Sciences, 107, 5238-5241. DOI: 10.1073/pnas.0913053107
  46. Yardley. L., McDermott, L., Pisarski, S., Duchaine, B., & Nakayama, K. (2008). Psychosocial consequence of developmental prosopagnosia: a problem of recognition. Journal of Psychosomatic Research, 65, 445-451. DOI: 10.1016/j.jpsychores.2008.03.013
  47. Yin, R. (1969). Looking at upside down faces. Journal of Experimental Psychology, 81, 141-145. DOI: 10.1037/h0027474

Maria Teresa Turano, Tessa Marzi, Maria Pia Viggiano, Prosopagnosia congenita: uno studio preliminare nella popolazione italiana in "RICERCHE DI PSICOLOGIA " 4/2013, pp 381-404, DOI: 10.3280/RIP2013-004003