Skip to main content

Advertisement

Log in

Emotion Recognition and Visual-Scan Paths in Fragile X Syndrome

  • Original Paper
  • Published:
Journal of Autism and Developmental Disorders Aims and scope Submit manuscript

Abstract

This study investigated emotion recognition abilities and visual scanning of emotional faces in 16 Fragile X syndrome (FXS) individuals compared to 16 chronological-age and 16 mental-age matched controls. The relationships between emotion recognition, visual scan-paths and symptoms of social anxiety, schizotypy and autism were also explored. Results indicated that, compared to both control groups, the FXS group displayed specific emotion recognition deficits for angry and neutral (but not happy or fearful) facial expressions. Despite these evident emotion recognition deficits, the visual scanning of emotional faces was found to be at developmentally appropriate levels in the FXS group. Significant relationships were also observed between visual scan-paths, emotion recognition performance and symptomology in the FXS group.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Notes

  1. ‘Normal’ was included rather than ‘neutral’ on the written response list due to a large number of young controls reading neutral as normal during piloting. Verbal task instructions, however, were given to all participants prior to beginning the emotion recognition task explaining the four choices as: happy, scared, angry, and neutrala normal or blank face with no expression.

  2. 65 % of all participants (FXS: 68.8 %; CA-matched controls: 56.3 %; MA-matched controls: 73.3 %, p = 0.536) failed to correctly label this image as neutral; over 90 % of errors judged the face to be angry.

  3. The analysis was conducted with males excluded and the pattern of results was similar. The only modification to results was that the difference between the FXS and CA-matched controls for sensitivity in recognizing neutral expressions now failed to reach significance (p = 0.101).

  4. Descriptive analyses revealed one outlier in each of the CA and MA matched groups. Analyses were performed with both the outliers removed and retained. The pattern of results was the same for both analyses; as such these data points were retained in the analysis.

References

  • Adolphs, R., Gosselin, F., Buchanan, T. W., Tranel, D., Schyns, P., & Damasio, A. R. (2005). A mechanism for impaired fear recognition after amygdala damage. Nature, 433(7021), 68–72.

    Article  PubMed  Google Scholar 

  • Adolphs, R., Sears, L., & Piven, J. (2001). Abnormal processing of social information from faces in autism. Journal of Cognitive Neuroscience, 13(2), 232–240.

    Article  PubMed  Google Scholar 

  • Agnew, S., & Powell, M. (2004). The effect of intellectual disability on children’s recall of an event across different question types. Law and Human Behavior, 28(3), 273–294.

    Article  PubMed  Google Scholar 

  • American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders: DSM-IV-TR. Washington, DC: American Psychiatric Association.

    Google Scholar 

  • Baron-Cohen, S., & Cross, G. (1992). Reading the eyes: Evidence for the role of perception in the development of a theory of mind. Mind and Language, 6, 173–186.

    Google Scholar 

  • Beidel, D. C., Turner, S. M., & Morris, T. L. (1998). The social phobia and anxiety inventory for children. New York: Multi-Health Systems.

    Google Scholar 

  • Berry-Kravis, E., & Potanos, K. (2004). Psychopharmacology in Fragile X syndrome—present and future. Mental Retardation and Developmental Disabilities Research Reviews, 10(1), 42–48.

    Article  PubMed  Google Scholar 

  • Birbaumer, N., Grodd, W., Diedrich, O., Klose, U., Erb, M., Lotze, M., et al. (1998). fMRI reveals amygdala activation to human faces in social phobia. NeuroReport, 9(6), 1223–1226.

    Article  PubMed  Google Scholar 

  • Bregman, J., Leckman, J. F., & Ort, S. I. (1988). Fragile X syndrome: Genetic predisposition to psychopathology. Journal of Autism and Developmental Disorders, 18(3), 343–355.

    Article  PubMed  Google Scholar 

  • Calder, A. J., & Jansen, J. (2005). Configural coding of facial expressions: The impact of inversion and photographic negative. Visual Cognition, 12(3), 495–518.

    Article  Google Scholar 

  • Calvo, M. G., & Marrero, H. (2009). Visual search of emotional faces: The role of affective content and featural distinctiveness. Cognition and Emotion, 23(4), 782–806. doi:10.1080/02699930802151654.

    Article  Google Scholar 

  • Cath, D. C., Ran, N., Smit, J. H., van Balkom, A. J., & Comijs, H. C. (2008). Symptom overlap between autism spectrum disorder, generalized social anxiety disorder and obsessive-compulsive disorder in adults: A preliminary case-controlled study. Psychopathology, 41(2), 101–110. doi:10.1159/000111555.

    Article  PubMed  Google Scholar 

  • Celani, G., Battacchi, M. W., & Arcidiacono, L. (1999). The understanding of the emotional meaning of facial expressions in people with autism. Journal of Autism and Developmental Disorders, 29(1), 57–66.

    Article  PubMed  Google Scholar 

  • Claridge, G., & Broks, P. (1984). Schizotypy and hemispheric function: I. Theoretical considerations and the measurement of schizotypy. Personality and Individual Differences, 5, 633–648.

    Article  Google Scholar 

  • Cohen, I. L., Sudhalter, V., Pfadt, A., Jenkins, E. C., & Brown, T. W. (1991). Why are autism and the Fragile-X syndrome associated? Conceptual and methodological issues. American Journal of Human Genetics, 48, 195–202.

    PubMed  Google Scholar 

  • Cohen, I. L., Vietze, P. M., Sudhalter, V., Jenkins, E. C., & Brown, T. W. (1989). Parent-child dyadic gaze patterns in Fragile X males and in non-Fragile X males with autistic disorder. Journal of Child Psychology and Psychiatry, 30, 845–856.

    Article  PubMed  Google Scholar 

  • Cornish, K., Kogan, C., Turk, J., Manly, T., James, N., Mills, A., et al. (2005). The emerging Fragile X premutation phenotype: Evidence from the domain of social cognition. Brain and Cognition, 57(1), 53–60.

    Article  PubMed  Google Scholar 

  • Cornish, K., Munir, F., & Wilding, J. (2001). A neuropsychological and behavioural profile of attention deficits in fragile X syndrome. Revista De Neurologia, 33(Suppl. 1), s24–s29.

    Google Scholar 

  • Cyhlarova, E., & Claridge, G. (2005). Development of a version of the Schizotypy Traits Questionnaire (STA) for screening children. Schizophrenia Research, 80(2–3), 253–261. doi:10.1016/j.schres.2005.07.037.

    Article  PubMed  Google Scholar 

  • Dalton, K. M., Holsen, L., Abbeduto, L., & Davidson, R. J. (2008). Brain function and gaze fixation during facial-emotion processing in Fragile X and autism. Autism Research, 1(4), 231–239. doi:10.1002/aur.32.

    Article  PubMed  Google Scholar 

  • Dalton, K. M., Nacewicz, B. M., Johnstone, T., Schaefer, H. S., Gernsbacher, M. A., Goldsmith, H. H., et al. (2005). Gaze fixation and the neural circuitry of face processing in autism. Nature Neuroscience, 8(4), 519–526. doi:10.1038/nn1421.

    PubMed  Google Scholar 

  • David, A. S., & Cutting, J. C. (1990). Affect, affective disorder and schizophrenia. A neuropsychological investigation of right hemisphere function. The British Journal of Psychiatry: The Journal of Mental Science, 156, 491–495.

    Article  Google Scholar 

  • Durand, K., Gallay, M., Seigneuric, A., Robichon, F., & Baudouin, J. Y. (2007). The development of facial emotion recognition: The role of configural information. Journal of Experimental Child Psychology, 97(1), 14–27. doi:10.1016/j.jecp.2006.12.001.

    Article  PubMed  Google Scholar 

  • Edwards, J., Jackson, H. J., & Pattison, P. E. (2002). Emotion recognition via facial expression and affective prosody in schizophrenia: A methodological review. Clinical Psychology Review, 22(6), 789–832.

    Article  PubMed  Google Scholar 

  • Ekman, P., & Friesen, W. V. (2003). Unmasking the face: A guide to recognizing emotions from facial expressions. Los Altos: Malor Books.

    Google Scholar 

  • Farah, M. J., Wilson, K. D., Drain, M., & Tanaka, J. N. (1998). What is “special” about face perception? Psychological Review, 105(3), 482–498.

    Article  PubMed  Google Scholar 

  • Farzin, F., Rivera, S. M., & Hessl, D. (2009). Brief report: Visual processing of faces in individuals with Fragile X syndrome: An eye tracking study. Journal of Autism & Developmental Disorders (Vol. 39, pp. 946-952): Springer Science & Business Media B.V.

  • Farzin, F., Scaggs, F., Hervey, C., Berry-Kravis, E., & Hessl, D. (2011). Reliability of eye tracking and pupillometry measures in individuals with Fragile X syndrome. Journal of Autism and Developmental Disorders, 41, 1515–1522.

    Article  PubMed  Google Scholar 

  • Franke, P., Maier, W., Hautzinger, M., Weiffenbach, O., Gansicke, M., Iwers, B., et al. (1996). Fragile-X carrier females: Evidence for a distinct psychopathological phenotype. American Journal of Medical Genetics, 64, 334–339.

    Article  PubMed  Google Scholar 

  • Frankland, P. W., Wang, Y., Rosner, B., Shimizu, T., Balleine, B. W., Dykens, E. M., et al. (2004). Sensorimotor gating abnormalities in young males with fragile X syndrome and Fmr1-knockout mice. Molecular Psychiatry, 9(4), 417–425.

    Article  PubMed  Google Scholar 

  • Fryns, J. P., Jacobs, J., Kleczkowska, A. A., & Van den Berghe, H. (1984). The psychological profile of the Fragile X syndrome. Clinical Genetics, 25, 131–134.

    Article  PubMed  Google Scholar 

  • Fukushima, J., Hatta, T., & Fukushima, K. (2000). Development of voluntary control of saccadic eye movements: I. Age-related changes in normal children. Brain & Development, 22(3), 173–180.

    Article  Google Scholar 

  • Garcia-Nonell, C., Rigau-Ratera, E., Harris, S., Hessl, D., Ono, M. Y., Tartaglia, N., et al. (2008). Secondary medical diagnosis in Fragile X syndrome with and without autism spectrum disorder. American Journal of Medical Genetics, 146A, 1911–1916.

    Article  PubMed  Google Scholar 

  • Garner, C., Callias, M., & Turk, J. (1999). Executive function and theory of mind performance of boys with fragile-X syndrome. Journal of Intellectual Disability Research, 43, 466–474.

    Article  PubMed  Google Scholar 

  • Gothelf, D., Furfaro, J. A., Hoeft, F., Eckert, M. A., Hall, S. S., O’Hara, R., et al. (2008). Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Annals of Neurology, 63(1), 40–51.

    Article  PubMed  Google Scholar 

  • Green, M., Williams, L. M., & Davidson, D. (2003). Visual scanpaths to threat-related faces in deluded schizophrenia. Psychiatry Research, 119(3), 271–285. doi:10.1016/s0165-1781(03)00129-x.

    Article  PubMed  Google Scholar 

  • Haas, B. W., Barnea-Goraly, N., Lightbody, A. A., Patnaik, S. S., Hoeft, F., Hazlett, H., et al. (2009). Early white-matter abnormalities of the ventral frontostriatal pathway in fragile X syndrome. Developmental Medicine and Child Neurology, 51(8), 593–599.

    Article  PubMed  Google Scholar 

  • Hacker, M. J., & Ratcliff, R. (1979). A revised table of d' for M-alternative forced choice. Perception and Psychophysics, 26, 168–170.

    Article  Google Scholar 

  • Hagan, C. C., Hoeft, F., Mackey, A., Mobbs, D., & Reiss, A. L. (2008). Aberrant neural function during emotion attribution in female subjects with fragile X syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 47(12), 1443–1454.

    Article  PubMed  Google Scholar 

  • Hagerman, R. (2002). The physical and behavioral phenotype. In R. Hagerman & P. Hagerman (Eds.), Fragile X syndrome: Diagnosis, treatment and research (3rd ed., pp. 3–109). Baltimore: The John Hopkins University Press.

    Google Scholar 

  • Hagerman, R. J., Jackson, A. W., Levitas, A., Rimland, B., & Braden, M. (1986). An analysis of autism in fifty males with the Fragile X syndrome. American Journal of Medical Genetics, 23, 359–374.

    Article  PubMed  Google Scholar 

  • Hatton, D. D., Hooper, S. R., Bailey, D. B., Skinner, M. L., Sullivan, K. M., & Wheeler, A. (2002). Problem behavior in boys with fragile X syndrome. American Journal of Medical Genetics, 108(2), 105–116.

    Article  PubMed  Google Scholar 

  • Hatton, D. D., Sideris, J., Skinner, M., Mankowski, J., Bailey, D. B., Roberts, J., et al. (2006). Autistic behavior in children with fragile X syndrome: Prevalence, stability, and the impact of FMRP. American Journal of Medical Genetics Part A, 140A(17), 1804–1813.

    Article  PubMed  Google Scholar 

  • Hayhoe, M., & Ballard, D. (2005). Eye movements in natural behavior. Trends in Cognitive Sciences, 9(4), 188–194.

    Article  PubMed  Google Scholar 

  • Hazlett, H. C., Poe, M. D., Lightbody, A. A., Gerig, G., MacFall, J. R., Ross, A. K., et al. (2009). Teasing apart the heterogeneity of autism: Same behavior, different brains in toddlers with Fragile X syndrome and autism. Journal of Neurodevelopmental Disorders, 1(1), 81–90.

    Article  PubMed  Google Scholar 

  • Hessl, D., Dyer-Friedman, J., Glaser, B., Wisbeck, J., Barajas, R. G., Taylor, A., et al. (2001). The influence of environmental and genetic factors on behavior problems and autistic symptoms in boys and girls with fragile X syndrome. Pediatrics, 108(5), e88. doi:10.1542/peds.108.5.e88.

  • Hessl, D., Rivera, S., Koldewyn, K., Cordeiro, L., Adams, J., Tassone, F., et al. (2007). Amygdala dysfunction in men with the fragile X premutation. Brain, 130(2), 404–416.

    Article  PubMed  Google Scholar 

  • Hessl, D., Tassone, F., Cordeiro, L., Koldewyn, K., McCormick, C., Green, C., et al. (2008). Brief report: Aggression and stereotypic behavior in males with fragile X syndrome—moderating secondary genes in a “single gene” disorder. Journal of Autism and Developmental Disorders, 38(1), 184–189. doi:10.1007/s10803-007-0365-5.

    Article  PubMed  Google Scholar 

  • Hessl, D., Wang, J. M., Schneider, A., Koldewyn, K., Le, L., Iwahashi, C. K., et al. (2011). Decreased Fragile X mental retardation protein expression underlies amygdala dysfunction in carriers of the Fragile X premutation. Biological Psychiatry, 70(9), 859–865.

    Article  PubMed  Google Scholar 

  • Hobson, R. P. (1991). Methodological issues for experiments on autistic individuals’ perception and understanding of emotion. Journal of Child Psychology and Psychiatry and Allied Disciplines, 32(7), 1135–1158.

    Article  Google Scholar 

  • Hodapp, R. M., Burack, J. A., & Zigler, E. (Eds.). (1990). Issues in the developmental approach to mental retardation. New York: Cambridge University Press.

    Google Scholar 

  • Holsen, L. M., Dalton, K. M., Johnstone, T., & Davidson, R. J. (2008). Prefrontal social cognition network dysfunction underlying face encoding and social anxiety in fragile X syndrome. Neuroimage, 43(3), 592–604. doi:10.1016/j.neuroimage.2008.08.009.

    Article  PubMed  Google Scholar 

  • Horley, K., Williams, K. R., Gonsalvez, C., & Gordon, E. (2003). Social phobics do not see eye to eye: A visual scanpath study of emotional expression processing. Journal of Anxiety Disorders, 17, 33–44.

    Article  PubMed  Google Scholar 

  • Horley, K., Williams, L. M., Gonsalvez, C., & Gordon, E. (2004). Face to face: Visual scanpath evidence for abnormal processing of facial expressions in social phobia. Psychiatry Research, 127, 43–53.

    Article  PubMed  Google Scholar 

  • Huber, K. (2007). Fragile X syndrome: Molecular mechanisms of cognitive dysfunction. American Journal of Psychiatry, 164(4), 556.

    Google Scholar 

  • Humphreys, K., Minshew, N. J., Leonard, G. L., & Behrmann, M. (2007). A fine-grain analysis of facial expression processing in high-functioning adults with autism. Neuropsychologia, 45(4), 685–695.

    Article  PubMed  Google Scholar 

  • Kaufmann, W. E., Cortell, R., Kau, C. S. M., Bukelis, I., Tierney, E., Gray, R. M., et al. (2004). Autism spectrum disorder in fragile X syndrome: Communication, social interaction, and specific behaviors. American Journal of Medical Genetics Part A, 129A(3), 225–234.

    Article  PubMed  Google Scholar 

  • Kendler, K. S., McGuire, M., Gruenberg, A. M., O’Hare, A., Spellman, M., & Walsh, D. (1993). The Roscommon family study. I. Methods, diagnosis of probands, and risk of schizophrenia in relatives. Archives of General Psychiatry, 50(7), 527–540.

    Article  PubMed  Google Scholar 

  • Kerby, D. S., & Dawson, B. L. (1994). Autistic features, personality, and adaptive behavior in males with the fragile X syndrome and no autism. American Journal on Mental Retardation, 98(4), 455–462.

    PubMed  Google Scholar 

  • Klin, A., Jones, W., Schultz, R., Volkmar, F., & Cohen, D. (2002). Visual fixation patterns during viewing of naturalistic social situations as predictors of social competence in individuals with autism. Archives of General Psychiatry, 59, 809–816.

    Article  PubMed  Google Scholar 

  • Krug, D., Arick, J., & Almond, P. (1993). Autism screening instrument for educational planning (2nd ed.). Austin: PRO-ED.

    Google Scholar 

  • Kuusikko, S., Happsamo, H., Jansson-Verkasalo, E., Hurtig, T., Mattila, M. L., Ebeling, H., et al. (2009). Emotion recognition in children and adolescents with autism spectrum disorders. Journal of Autism and Developmental Disorders, 39, 938–945.

    Article  PubMed  Google Scholar 

  • Leonard, L. B. (1998). Children with specific language impairment. Cambridge, MA: MIT Press.

    Google Scholar 

  • Lesniak-Karpiak, K., Mazzocco, M. M. M., & Ross, J. L. (2003). Behavioral assessment of social anxiety in females with Turner or fragile X syndrome. Journal of Autism and Developmental Disorders, 33(1), 55–67.

    Article  PubMed  Google Scholar 

  • Levy, Y., Pluber, H., & Bentin, S. (2011). Covert processing of facial expressions by people with Williams syndrome. Cortex, 47(1), 23–34. doi:10.1016/j.cortex.2009.09.011.

    Article  PubMed  Google Scholar 

  • Lewis, P., Abbeduto, L., Murphy, M., Richmond, E., Giles, N., Bruno, L., et al. (2006). Cognitive, language and social-cognitive skills of individuals with fragile X syndrome with and without autism. Journal of Intellectual Disability Research, 50, 532–545.

    Article  PubMed  Google Scholar 

  • Lightbody, A. A., & Reiss, A. L. (2009). Gene, brain, and behavior relationships in fragile X syndrome: Evidence from neuroimaging studies. Developmental Disabilities Research Reviews, 15(4), 343–352.

    Article  PubMed  Google Scholar 

  • Loughland, C. M., Williams, L. M., & Harris, A. W. F. (2004). Visual scanpath dysfunction in first degree relatives of schizophrenia probands: Evidence for a vulnerability marker? Schizophrenia Research, 67, 11–21.

    Article  PubMed  Google Scholar 

  • MacMillan, N. A., & Creelman, C. D. (1991). Detection theory: A user’s guide. New York: Cambridge University Press.

    Google Scholar 

  • Marsh, P. (2008). Impaired emotion perception in ADHD and first-episode schizophrenia: How are they different?. Germany: VDM Publishing.

    Google Scholar 

  • Marsh, P. J., & Williams, L. M. (2006). ADHD and schizophrenia phenomenology: Visual scanpaths to emotional faces as a potential psychophysiological marker? Neuroscience and Biobehavioral Reviews, 30(5), 651–665. doi:10.1016/j.neubiorev.2005.11.004.

    Article  PubMed  Google Scholar 

  • Mason, O., Claridge, G., & Jackson, M. (1995). New scales for the assessment of schizotypy. Personality and Individual Differences, 18(1), 7–13.

    Article  Google Scholar 

  • Mazzocco, M. M. M. (2000). Advances in research on the fragile X syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 6(2), 96–106.

    Article  PubMed  Google Scholar 

  • Mazzocco, M. M. M. (2001). Math learning disability and MD subtypes: Evidence from studies of Turner syndrome, Fragile X syndrome, and Neurofibromatosis Type 1. Journal of Learning Disabilities, 34(6), 520–533.

    Article  PubMed  Google Scholar 

  • Mazzocco, M. M. M., Pennington, B. F., & Hagerman, R. (1994). Social cognition skills among females with Fragile X. Journal of Autism and Developmental Disorders, 24(4), 473–484.

    Article  PubMed  Google Scholar 

  • Mazzocco, M. M. M., & Reiss, A. L. (1999). A behavioral neurogenetics approach to understanding the Fragile X syndrome. In H. Tager-Flusberg (Ed.), Neurodevelopmental disorders (pp. 43–64). Cambridge: MIT Press.

    Google Scholar 

  • Meyer-Lindenberg, A., Hariri, A. R., Munoz, K. E., Mervis, C. B., Mattay, V. S., Morris, C. A., et al. (2005). Neural correlates of genetically abnormal social cognition in Williams syndrome. Nature Neuroscience, 8(8), 991–993. doi:10.1038/nn1494.

    Article  PubMed  Google Scholar 

  • Mikhailova, E. S., Vladimirova, T. V., Iznak, A. F., Tsusulkovskaya, E. J., & Sushko, N. V. (1996). Abnormal recognition of facial expression of emotions in depressed patients with major depression disorder and schizotypal personality disorder. Biological Psychiatry, 40(8), 697–705. doi:10.1016/0006-3223(96)00032-7.

    Article  PubMed  Google Scholar 

  • Mineur, Y. S., Huynh, L. X., & Crusio, W. E. (2006). Social behavior deficits in the Fmr1 mutant mouse. Behavioural Brain Research, 168(1), 172–175.

    Article  PubMed  Google Scholar 

  • Morton, J., & Johnson, M. H. (1991). CONSPEC and CONLERN: A two-process theory of infant face recognition. Psychological Review, 98(2), 164–181.

    Article  PubMed  Google Scholar 

  • Ozonoff, S., Pennington, B. F., & Rogers, S. J. (1990). Are there emotion perception deficits in young autistic children? Journal of Child Psychology and Psychiatry and Allied Disciplines, 31(3), 343–361.

    Article  Google Scholar 

  • Palermo, R., & Coltheart, M. (2004). Photographs of facial expression: Accuracy, response times, and ratings of intensity. Behavior Research Methods Instruments & Computers, 36(4), 634–638.

    Article  Google Scholar 

  • 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(4), 249–261.

    Article  PubMed  Google Scholar 

  • Pennington, B. F., & Bennetto, L. (1998). A neuropsychology of mental retardation. In J. A. Burack, R. M. hodapp, & E. Zigler (Eds.), Handbook of mental retardation and development (pp. 80–114). Cambridge: Cambridge University Press.

    Google Scholar 

  • Pimperton, H., Pellicano, E., Jeffery, L., & Rhodes, G. (2009). The role of higher level adaptive coding mechanisms in the development of face recognition. Journal of Experimental Child Psychology, 104(2), 229–238. doi:10.1016/j.jecp.2009.05.009.

    Article  PubMed  Google Scholar 

  • Plesa Skwerer, L., Borum, L., Verbalis, A., Schofield, C., Crawford, N., Ciciolla, L., et al. (2009). Autonomic responses to dynamic displays of facial expression in adolescents and adults with Williams syndrome. Scan, 4, 93–100.

    PubMed  Google Scholar 

  • Poreh, A. M., Whitman, R. D., Weber, M., & Ross, T. (1994). Facial recognition in hypothetically schizotypic college students. The role of generalized poor performance. The Journal of Nervous and Mental Disease, 182(9), 503–507.

    Article  PubMed  Google Scholar 

  • Porter, M. A., Coltheart, M., & Langdon, R. (2007). The neuropsychological basis of hypersociability in Williams and Down syndrome. Neuropsychologia, 45(12), 2839–2849. doi:10.1016/j.neuropsychologia.2007.05.006.

    Article  PubMed  Google Scholar 

  • Porter, M. A., Shaw, T. A., & Marsh, P. J. (2010). An unusual attraction to the eyes in Williams-Beuren syndrome: A manipulation of facial affect while measuring face scanpaths. Cognitive Neuropsychiatry, 15(6), 505–530. doi:10.1080/13546801003644486.

    Article  PubMed  Google Scholar 

  • Raine, A. (1991). The SPQ: A scale for the assessment of schizotypal personality based on DSM-III-R criteria. Schizophrenia Bulletin, 17(4), 555–564.

    Article  PubMed  Google Scholar 

  • Rapee, R. M., & Heimberg, R. G. (1997). A cognitive-behavioral model of anxiety in social phobia. Behaviour Research and Therapy, 35(8), 741–756.

    Article  PubMed  Google Scholar 

  • Rawlings, D., & MacFarlane, C. (1994). A multidimensional schizotypal traits questionnaire for young adolescents. Personality and Individual Differences, 17(4), 489–496.

    Article  Google Scholar 

  • Riby, D. M., & Hancock, P. J. (2008). Viewing it differently: Social scene perception in Williams syndrome and autism. Neuropsychologia, 46(11), 2855–2860. doi:10.1016/j.neuropsychologia.2008.05.003.

    Article  PubMed  Google Scholar 

  • Roberts, J., Mazzocco, M. M. M., Murphy, M. M., & Hoehn-Saric, R. (2008). Arousal modulation in females with fragile X or Turner syndrome. Journal of Autism and Developmental Disorders, 38, 20–27.

    Article  PubMed  Google Scholar 

  • Rogers, S. J., Wehner, E. A., & Hagerman, R. (2001). The behavioral phenotype in Fragile X: Symptoms of autism in very young children with Fragile X syndrome, idiopathic autism, and other developmental disorders. Journal of Developmental and Behavioral Pediatrics, 22(6), 409–417.

    Article  PubMed  Google Scholar 

  • Rothman, K. J. (1990). No adjustments are needed for multiple comparisons. Epidemiology, 1(1), 43–46.

    Article  PubMed  Google Scholar 

  • Rump, K. M., Giovannelli, J. L., Minshew, N., & Strauss, M. (2009). The development of emotion recognition in individuals with autism. Child Development, 80(5), 1434–1447.

    Article  PubMed  Google Scholar 

  • Santos, A., Silva, C., Rosset, D., & Deruelle, C. (2010). Just another face in the crowd: Evidence for decreased detection of angry faces in children with Williams syndrome. Neuropsychologia, 48, 1071–1078.

    Article  PubMed  Google Scholar 

  • Sattler, J. M. (Ed.). (2001). Assessment of children: Cognitive applications. San Diego: Jerome M. Sattler.

    Google Scholar 

  • Sherman, S. L., Marsteller, F., Abramowitz, A. J., Scott, E., Leslie, M., & Bregman, J. (2002). Cognitive and behavioral performance among FMR1 high-repeat allele carriers surveyed from special education classes. American Journal of Medical Genetics, 114(4), 458–465.

    Article  PubMed  Google Scholar 

  • Simon, E. W., & Finucane, B. M. (1996). Facial emotion identification in males with fragile X syndrome. American Journal of Medical GeneticsSeminars in Medical Genetics, 67(1), 77-80.

    Google Scholar 

  • Simonian, S. J., Beidel, D. C., Turner, S. M., Berkes, J. L., & Long, J. H. (2001). Recognition of facial affect by children and adolescents with social phobia. Child Psychiatry and Human Development, 32(2), 137–145.

    Article  PubMed  Google Scholar 

  • Sobesky, W., Hull, C., & Hagerman, R. (1994). Symptoms of schizotypal personality disorder in Fragile X women. Journal of the American Academy of Child & Adolescent Psychiatry, 33(2), 247–255.

    Google Scholar 

  • Stein, M. B., Goldin, P. R., Sareen, J., Zorrilla, L. T. E., & Brown, G. G. (2002). Increased amygdala activation to angry and contemptuous faces in generalized social phobia. Archives of General Psychiatry, 59(11), 1027–1034. doi:10.1001/archpsyc.59.11.1027.

    Article  PubMed  Google Scholar 

  • Sudhalter, V., & Belser, R. C. (2001). Conversational characteristics of children with Fragile X syndrome: Tangential language. American Journal on Mental Retardation, 106(5), 389–400.

    Article  PubMed  Google Scholar 

  • Suvrathan, A., & Chattarji, S. (2011). Fragile X syndrome and the amygdala. Current Opinions in Neurobiology, 21(3), 509–515.

    Article  Google Scholar 

  • Suvrathan, A., Hoeffer, C. A., Wong, H., Klann, E., & Chattarji, S. (2010). Characterization and reversal of synaptic defects in the amgydala in a mouse model of Fragile X syndrome. Proceedings of the National academy of Sciences of the United States of America, 107(25), 11591–11596.

    Article  PubMed  Google Scholar 

  • Tamm, L., Menon, V., Johnston, C. K., Hessl, D. R., & Reiss, A. L. (2002). fMRI study of cognitive interference processing in females with fragile X syndrome. Journal of Cognitive Neuroscience, 14(2), 160–171.

    Article  PubMed  Google Scholar 

  • Tanaka, J., & Farah, M. J. (1993). Parts and wholes in face recognition. Quarterly Journal of Experimental Psychology, 46, 225–245.

    PubMed  Google Scholar 

  • Tassone, F., Hagerman, R. J., Ikle, D. N., Dyer, P. N., Lampe, M., Willemsen, R., et al. (1999). FMRP expression as a potential prognostic indicator in Fragile X syndrome. American Journal of Medical Genetics, 84, 250–261.

    Article  PubMed  Google Scholar 

  • Tassone, F., Hagerman, R. J., Taylor, A. K., Gane, L. W., Godfrey, T. E., & Hagerman, P. J. (2000). Elevated Levels of FMR1 mRNA in carrier males: A new mechanism of involvement in the Fragile-X syndrome. American Journal of Human Genetics, 66(1), 6–15.

    Article  PubMed  Google Scholar 

  • The Psychological Corporation. (1999). WASI manual. San Antonio: The Psychological Corporation.

    Google Scholar 

  • Thomas, L. A., De Bellis, M. D., Graham, R., & LaBar, K. S. (2007). Development of emotional facial recognition in late childhood and adolescence. Developmental Science, 10(5), 547–558. doi:10.1111/j.1467-7687.2007.00614.x.

    Article  PubMed  Google Scholar 

  • Torgersen, S., Kringlen, E., & Cramer, V. (2001). The prevalence of personality disorders in a community sample. Archives of General Psychiatry, 58(6), 590–596.

    Article  PubMed  Google Scholar 

  • Tsiouris, J. A., & Brown, W. T. (2004). Neuropsychiatric symptoms of Fragile X syndrome: Pathophysiology and pharmacotherapy. CNS Drugs, 18, 687–703.

    Article  PubMed  Google Scholar 

  • Turk, J., & Cornish, K. (1998). Face recognition and emotion perception in boys with fragile-X syndrome. Journal of Intellectual Disability Research, 42(6), 490–499.

    Article  PubMed  Google Scholar 

  • Turk, J., & Graham, P. (1997). Fragile X syndrome, autism and autistic features. Autism, 1(2), 175–197.

    Article  Google Scholar 

  • Turner, S. M., Beidel, D. C., & Dancu, C. V. (1996a). Social phobia and anxiety inventory: Manual. Toronto: Multi-Health Systems Inc.

    Google Scholar 

  • Turner, G., Webb, T., & Robinson, H. (1996b). Prevalence of Fragile X syndrome. American Journal of Medical Genetics, 64, 196–197.

    Article  PubMed  Google Scholar 

  • Veljaca, K. A., & Rapee, R. M. (1998). Detection of negative and positive audience behaviours by socially anxious subjects. Behaviour Research and Therapy, 36(3), 311–321.

    Article  PubMed  Google Scholar 

  • Verkerk, A. J., Pierettl, M., Sutcliffe, J. S., Fu, Y. H., Kuhl, D. P., Pizzuti, A., et al. (1991). Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in Fragile X syndrome. Cell, 65, 905–914.

    Article  PubMed  Google Scholar 

  • Watson, C., Hoeft, F., Garrett, A. S., Hall, S. S., & Reiss, A. L. (2008). Aberrant brain activation during gaze processing in boys with Fragile X syndrome. Archives of General Psychiatry, 65(11), 1315–1323.

    Article  PubMed  Google Scholar 

  • Whiteside, J. A. (1974). Eye movements of children, adults, and elderly persons during inspection of dot patterns. Journal of Experimental Child Psychology, 18(2), 313–332. doi:10.1016/0022-0965(74)90111-8.

    Article  PubMed  Google Scholar 

  • Willis, M. L., Palermo, R., Burke, D., McGrillen, K., & Miller, L. (2010). Orbitofrontal cortex lesions result in abnormal social judgements to emotional faces. Neuropsychologia, 48, 2182–2187.

    Article  PubMed  Google Scholar 

  • Wilson, C. E., Palermo, R., & Brock, J. (2012). Visual scan paths and recognition of facial identity in autism spectrum disorder and typical development. PLoS ONE, 7(5), e37681. doi:10.1371/journal.pone.0037681.

    Article  PubMed  Google Scholar 

  • Wishart, J. G., Cebula, K. R., Willis, D. S., & Pitcairn, T. K. (2007). Understanding of facial expressions of emotion by children with intellectual disabilities of differing aetiology. Journal of Intellectual Disability Research, 51(7), 551–563.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We would like to thank all the participants and their families for their time and enthusiasm. Thanks also to the Fragile X Association of Australia and Hunter Genetics at Hunter New England Health for their continued support of this research. We would also like to acknowledge Robyn Langdon and Samantha Baggott for their helpful suggestions on earlier drafts of this paper, as well as Alan Taylor for his statistical expertise.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Tracey A. Shaw.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shaw, T.A., Porter, M.A. Emotion Recognition and Visual-Scan Paths in Fragile X Syndrome. J Autism Dev Disord 43, 1119–1139 (2013). https://doi.org/10.1007/s10803-012-1654-1

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10803-012-1654-1

Keywords

Navigation