Skip to main content
Log in

Social Functioning Among Girls with Fragile X or Turner Syndrome and Their Sisters

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

Abstract

Social behaviors among two genetically homogeneous groups—girls with fragile X (fraX) or Turner syndrome (TS)—were examined to address the role of family environment versus biological determinants of social dysfunction in girls with these disorders. Using a sibling pair design, girls with fraX or TS were compared with their own sisters on measures of IQ and social functioning. The 8 girls with fraX and the 9 girls with TS had lower FSIQ scores and higher ratings of social and attention problems relative to their own sisters. Girls with fraX also had higher ratings of withdrawn behaviors, relative to their own sisters. The unaffected sisters were not rated as demonstrating any difficulties in these areas, relative to controls. Correlations between problem ratings and FSIQ were not statistically significant. Although these preliminary findings do not indicate a lack of familial impact on social development in girls with either disorder, the results provide preliminary evidence that social dysfunction reported for girls with fraX or TS cannot be attributed solely, nor primarily, to global aspects of the family environment.

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.

Similar content being viewed by others

REFERENCES

  • Achenbach, T. M., & Edelbrock, C. S. (1983). Manual for the Child Behavior Checklist and Revised Child Behavior Profile. Burlington: University of Vermont, Department of Psychiatry.

    Google Scholar 

  • Alexander, D., Ehrardt, A. A., & Money, J. (1966). Defective figure drawing, geometric and human, in Turner's syndrome. Journal of Nervous and Mental Disorders 142, 161–167.

    Google Scholar 

  • Baum, D., Duffelmay, F., & Geelan, M. (1988). Resource teacher perceptions of the prevalence of social dysfunction among students with learning disabilities. Journal of Learning Disabilities 21, 380–381.

    PubMed  Google Scholar 

  • Bender, B. G., Linden, M. G., & Robinson, A. (1987). Environment and developmental risk in children with sex chromosome abnormalities. Journal of the American Academy of Child and Adolescent Psychiatry, 26, 499–503.

    PubMed  Google Scholar 

  • Brainard, S. S., Schreiner, R. A., & Hagerman, R. J. (1991). Cognitive profiles of the carrier fragile X woman. American Journal of Medical Genetics 38, 505–508.

    PubMed  Google Scholar 

  • Denckla, M. (1983). The neuropsychology of social-emotional learning disabilities. Archives of Neurology, 40, 717–724.

    Google Scholar 

  • Dorn, M. B., Mazzocco, M. M. M., & Hagerman, R. J. (1994). Behavioral and psychiatric disorders in adult male carriers of fragile X. Journal of the American Academy of Child and Adolescent Psychiatry 33(2), 256–264.

    PubMed  Google Scholar 

  • Downey, G., & Coyne, J. (1990). Children of depressed parents: An integrative review. Psychological Bulletin, 108, 50–76.

    Article  PubMed  Google Scholar 

  • Forehand, R. (1993). Family psychopathology and child functioning. Journal of Child and Family Studies, 2(2), 79–85.

    Google Scholar 

  • Freund, L. S., & Reiss, A. L. (1991). Cognitive profiles associated with the fra(X) syndrome in males and females. American Journal of Medical Genetics, 38, 542–547.

    PubMed  Google Scholar 

  • Freund, L. S., Reiss, A. L., & Abrams, M. T. (1993). Psychiatric disorders associated with fragile X in the young female. Pediatrics 91(2), 321–329.

    PubMed  Google Scholar 

  • Freund, L. S., Reiss, A. L., Hagerman, R., & Vinogradov, S. (1992). Chromosome fragility and psychopathology in obligate female carriers of the fragile X chromosome. Archives of General Psychiatry, 49, 54–60.

    PubMed  Google Scholar 

  • Hagerman, R. J. (1996). Physical and behavioral phenotype. In R. J. Hagerman & A. C. Cronister (Eds.), Fragile X syndrome (2nd ed., pp. 3–87). Baltimore: Johns Hopkins University Press.

    Google Scholar 

  • Hagerman, R. J., & Sobesky, W. E. (1989). Psychopathology in fragile X syndrome. American Journal of Orthopsychiatry, 59, 142–152.

    PubMed  Google Scholar 

  • Kovar, C. G. (1993). The neurocognitive and psychosocial phenotype of fragile X syndrome in school age girls. Unpublished Masters Thesis, University of Denver.

  • Lachiewicz, A. M. (1992). Abnormal behaviors of young girls with fragile X syndrome. American Journal of Medical Genetics, 43, 72–77.

    PubMed  Google Scholar 

  • Lachiewicz, A. M., & Dawson, D. V. (1994). Behavior problems of young girls with fragile X syndrome: Factor scores on the Conners' Parent's Questionnaire. American Journal of Medical Genetics, 51, 364–369.

    PubMed  Google Scholar 

  • LaHood, B. J., & Bacon, G. E. (1985). Cognitive abilities of adolescent Turner's syndrome patients. Journal of Adolescent Health Care, 6, 358–364.

    PubMed  Google Scholar 

  • Lobato, D., Barbour, L., Hall, L. J., & Miller, C. T. (1987). Psychosocial characteristics of preschool siblings of handicapped and nonhandicapped children. Journal of Abnormal Child Psychology, 15, 329–338.

    PubMed  Google Scholar 

  • Mazzocco, M. M., Pennington, B. F., & Hagerman, R. J. (1993). The neurocognitive phenotype of female carriers of fragile X: Additional evidence for specificity. Journal of Developmental and Behavioral Pediatrics, 14, 328–335.

    PubMed  Google Scholar 

  • McCauley, E. (1990). Psychosocial and emotional aspects of Turner syndrome. In D. B. Berch & B. G. Bender (Eds.), Sex chromosome abnormalities and human behavior (pp. 78–99). Boulder, CO: Westview.

    Google Scholar 

  • McCauley, E., Ito, J., & Key, T. (1986). Psychosocial functioning in girls with Turner's syndrome and short stature: Social skills, behavior problems, and self-concept. Journal of the American Academy of Child and Adolescent Psychiatry, 25, 105–112.

    Google Scholar 

  • McCauley, E., Kay, T., Ito, J., & Treder, R. (1987). The Turner syndrome: Cognitive deficits, affective discrimination, and behavior problems. Child Development, 58, 464–473.

    PubMed  Google Scholar 

  • Miezejeski, C. M., Jenkins, E. C., Hill, A. L., Wisniewski, K., French, J. H., & Brown, W. T. (1986). A profile of cognitive deficit in females from fragile X families. Neuropsychologia, 24, 405–409.

    PubMed  Google Scholar 

  • Pennington, B. F., Heaton, R. K., Karzmar, P., Pendleton, M. G., Lehman, R., & Schucard, D. W. (1985). The neuropsychological phenotype in Turner syndrome. Cortex, 21, 391–404.

    PubMed  Google Scholar 

  • Plomin, R. (1995). Genetics and children's experiences in the family. Journal of Child Psychology and Psychiatry, 36, 33–68.

    PubMed  Google Scholar 

  • Reich, W., & Welner, Z. (1988). DICA-R-P DSM-III-R Version. Seattle: University of Washington University.

    Google Scholar 

  • Reiss, A. L., Freund, L., Abrams, M. T., Boehm, C., & Kazazian, H. (1993). Neurobehavioral effects of the fragile X premutation in adult women: A controlled study. American Journal of Human Genetics, 52, 884–894.

    PubMed  Google Scholar 

  • Rourke, B. (1989). Nonverbal Learning Disabilities: The Syndrome and the Model. New York: Guilford Press.

    Google Scholar 

  • Rousseau, F., Heitz, D., Tarleton, J., MacPherson, J., Malmgren, H., Dahl, N., Barnicoat, A., Mathew, C., Mornet, E., Tejada, I., Maddalena, A., Spiegel, R., Schinzel, A., Marcos, J. A. G., Schwartz, C., & Mandel, J. L. (1994). A multicenter study on genotype-phenotype correlations in the fragile X syndrome, using direct diagnosis with probe StB 12.3: The first 2,253 cases. American Journal of Human Genetics, 55, 225–237.

    PubMed  Google Scholar 

  • Rovet, J. F. (1993). The psychoeducational characteristics of children with Turner syndrome. Journal of Learning Disabilities, 26, 333–341.

    PubMed  Google Scholar 

  • Sonis, W. A., Levine Ross, J., Blue, J., Cutler, G. B., Loriaux, P. L., & Klein, R. P. (1983,). Hyperactivity and Turner's syndrome. Paper presented at the American Academy of Child Psychiatry Meetings, San Francisco.

  • Sparrow, S. S., Balla, D. A., & Cicchetti, H. V. (1984). Vineland Adaptive Behavior Scales-Interview Edition Survey Form Manual. Circle Pines, MN: American Guidance Service.

    Google Scholar 

  • Verkerk, A. J., Pieretti, M., Sutcliffe, J. S., Fu, Y. H., Kuhl, D. P., Pizzuti, A., Reiner, O., Richards, S., Victoria, M. F., Fuping Zhang, M. F. V., Eussen, B. E., van Ommen, G. J. B., Blonden, L. A. J., Riggins, G. J., Chastain, J. L., Kunst, C. B., Galjaard, H., Caskey, C. T., Nelson, D. L., Oostra, B. A., & Warren, S. T. (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.

    PubMed  Google Scholar 

  • Voeller, K. K. S. (1991). Social-emotional learning disabilities. Psychiatric Annals, 21, 735–741.

    Google Scholar 

  • Weschler, D. (1974). WISC-R Manual: Wechsler Intelligence Scale for Children—Revised. New York: Psychological Corp.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mazzocco, M.M.M., Baumgardner, T., Freund, L.S. et al. Social Functioning Among Girls with Fragile X or Turner Syndrome and Their Sisters. J Autism Dev Disord 28, 509–517 (1998). https://doi.org/10.1023/A:1026000111467

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1023/A:1026000111467

Navigation