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 Girls with fraX also had higher ratings of withdrawn behaviors, relative to their own sisters.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.
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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.
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.
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.
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.
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.
Denckla, M. (1983). The neuropsychology of social-emotional learning disabilities. Archives of Neurology, 40, 717–724.
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.
Downey, G., & Coyne, J. (1990). Children of depressed parents: An integrative review. Psychological Bulletin, 108, 50–76.
Forehand, R. (1993). Family psychopathology and child functioning. Journal of Child and Family Studies, 2(2), 79–85.
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.
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.
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.
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.
Hagerman, R. J., & Sobesky, W. E. (1989). Psychopathology in fragile X syndrome. American Journal of Orthopsychiatry, 59, 142–152.
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.
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.
LaHood, B. J., & Bacon, G. E. (1985). Cognitive abilities of adolescent Turner's syndrome patients. Journal of Adolescent Health Care, 6, 358–364.
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.
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.
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.
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.
McCauley, E., Kay, T., Ito, J., & Treder, R. (1987). The Turner syndrome: Cognitive deficits, affective discrimination, and behavior problems. Child Development, 58, 464–473.
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.
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.
Plomin, R. (1995). Genetics and children's experiences in the family. Journal of Child Psychology and Psychiatry, 36, 33–68.
Reich, W., & Welner, Z. (1988). DICA-R-P DSM-III-R Version. Seattle: University of Washington University.
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.
Rourke, B. (1989). Nonverbal Learning Disabilities: The Syndrome and the Model. New York: Guilford Press.
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.
Rovet, J. F. (1993). The psychoeducational characteristics of children with Turner syndrome. Journal of Learning Disabilities, 26, 333–341.
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.
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.
Voeller, K. K. S. (1991). Social-emotional learning disabilities. Psychiatric Annals, 21, 735–741.
Weschler, D. (1974). WISC-R Manual: Wechsler Intelligence Scale for Children—Revised. New York: Psychological Corp.
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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
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DOI: https://doi.org/10.1023/A:1026000111467