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Cognitive, behavioral, and adaptive functioning in fragile X and non-fragile X retarded men

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Abstract

The cognitive, behavioral, and adaptive functioning of 12 men with fragile X syndrome (aged 23 to 62 years) was systematically assessed and compared to two matched groups of retarded men without fragile X syndrome residing at the same institution. The fragile X group was largely indistinguishable from the camparison groups on the cognitive, behavioral, and adaptive measures. Fragile X patients were, however, significantly more likely to have achieved levels of adaptive functioning commensurate with their intellectual abilities. Fragile X subjects who had similarly affected siblings emerged as significantly higher-functioning in all areas than Fragile X subjects who did not have affected siblings. These findings are discussed with respect to future research.

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References

  • Arthur, G. (1952).Leiter international performance scale. Washington D.C.: Psychological Service.

    Google Scholar 

  • Beery, K., & Buktenica, N. (1967).The developmental test of visual-motor integration. Cleveland, Ohio: Modern Curriculum Press.

    Google Scholar 

  • Blomquist, H., Bohman, M., Edvinsson, S., Gilberg, C., Gustavson, K., Holmgren, G., & Wahlstrom, J., (1985). Frequency of the fragile X syndrome in infantile autism.Human Genetics, 27, 113–117.

    Google Scholar 

  • Brown, W., Friedman, E., Jenkins, E., Brocks, J., Wishiewski, K., Raguthu, S., & Franech, J. (1982). Association of fragile X syndrome with autism.Lancet, I. 100.

    Google Scholar 

  • Chudley, A. (1984). Behavior phenotype. In J. Optiz & G. Sutherland (Eds.), Conference report: International workshop on the fragile X syndrome and X-linked mental retardation.American Journal of Medical Genetics, 17, 45–50.

    Google Scholar 

  • Cruz, F. (1985). Fragile X syndrome.American Journal of Mental Deficiency, 90, 119–123.

    Google Scholar 

  • Grossman, H. (Ed.). (1983).Classification in mental retardation. Washington, D.C.: American Association on Mental Deficiency.

    Google Scholar 

  • Hagerman, R., McBogg, P., & Hagerman, P. (1983).The Fragile-X syndrome: History, diagnosis and treatment. Dillon, Colorado: Spectrum.

    Google Scholar 

  • Herbst, D. (1980). Nonspecific x-linked mental retardation 1: A review with information from 24 families.American Journal of Medical Genetics, 7, 443–460.

    Google Scholar 

  • Herbst, D., Dunn, H., Dill, F., Kalousek, D., & Krywanick, L. (1981). Further delineation of x-linked mental retardation.Human Genetics, 58, 366–372.

    Google Scholar 

  • Hodapp, R., & Zigler, E. (in press). Applying the developmental perspective to individuals with Down syndrome. In D. Cicchetti & D. Beechly (Eds.),Down syndrome: The developmental perspective. New York: Cambridge University Press.

  • Jacobs, P., Glover, T., Mayer, M., Fox, P., Gerrard, J., Dunn, H., & Herbst, D. (1980). X-linked mental retardation: A study of 7 families.American Journal of Medical Genetics, 7, 471–489.

    Google Scholar 

  • Kleinberg, J., & Gallingan, B., (1983). Effects of deinstitutionalization on adaptive behavior of mentally retarded adults.American Journal of mental Deficiency, 88, 21–27.

    Google Scholar 

  • Krug, D., Arick, J., & Almond, P. (1980).Autism Behavior Checklist. Portland, Oregon: ASIEP Educational Company.

    Google Scholar 

  • Landesman-Dwyer, S. (1981). Living in the community.American Journal of Mental Deficiency, 86, 223–234.

    Google Scholar 

  • Lejeune, J. (1982). Is the fragile X syndrome amenable to treatment?Lancet, 1, 273–274.

    Google Scholar 

  • Lubs, H., Watson, M., Breg, R., & Lujan, E. (1984). Restudy of the original marker X family.American Journal of Medical Genetics, 17, 133–145.

    Google Scholar 

  • Mattel, J., Mattel, M., Aumeras, C., auger, M., & Giraud, F. (1981), X-linked mental retardation with the fragile x: A study of 15 families.Human Genetics, 59, 281–289.

    Google Scholar 

  • McCarthy, D. (1970).McCarthy Scales of Children's Abilities, New York: Psychological Corporation.

    Google Scholar 

  • Neuhäuser, G., Zerbin-Ridin, E., Pfeiffer, R., & Lelar, H. (1969). Beobachturgen zum Problem des gesonlechtsyeburden-recessiven Schwaschsinns.Archiv für Psychiatrie und Nervenkrankleiten, 212, 207–224.

    Google Scholar 

  • Paul, R., Cohen, D., Breg, R., Watson, M., & Herman, S. (1984). Fragile X syndrome: Its relation to speech and language disorders.Journal of Speech and Hearing Disorders, 49, 328–332.

    Google Scholar 

  • Paul, R., Dykens, E., Leckman, J., Watson, M., Breg, R., & Cohen, D. (1987). A comparison of language characteristics of mentally retarded adults with fragile X syndrome and those with nonspecific mental retardation and autism.Journal of Autism and Developmental Disabilities, 17, 457–468.

    Google Scholar 

  • Peebles-Howard, P., Stoddard, G., & Mims. (1979). Familial X-linked mental retardation, verbal disability and marked X chromosomes.American Journal of Human Genetics, 31, 214–222.

    Google Scholar 

  • Sattler, J. (1974).Assessment of children's intelligence. Philadelphia: W.B. Saunders.

    Google Scholar 

  • Sparrow, S., Balla, D., & Cicchetti, D. (1984).Vineland Adaptive Behavior Scales. Circle Pines, Minnesota: American Guidance Service.

    Google Scholar 

  • Terman, L., & Merrill, M. (1973).Stanford-Binet Intelligence Scale. Boston: Houghton Mifflin.

    Google Scholar 

  • Theobald, T., & Hay, D. (1982, June 23–26).Behavior correlations of the fragile X syndrome. Paper presented at the Twelfth Annual Meeting of the Behavior Genetic Association, Fort Collins, Colorado.

  • Turner, G., Brookwell, R., Danial, A., Selikowitz, M., & Zilibowitz, M. (1980). Heterozygous expression of X-linked mental retardation and the X marker: fra (X) (q27).New England Journal of Medicine, 303, 662–664.

    Google Scholar 

  • Turner, G., & Jacobs, P. (1983). Marker X-linked mental retardation. In H. Harris & K. Hirschorn (Eds.),Advances in human genetics New York: Plenum.

    Google Scholar 

  • Watson, M., Leckman, J., Annex, B., Breg, R., Coles, D., Volkmar, F., Cohen, D., & Carter, C. (1984). Fragile X in a survey of 75 autistic males.New England Journal of medicine.

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We wish to express our sincere appreciation to the staff of the Southbury Training School for their friendly assistance and cooperation throughout this study: most especially to the cottage staff, Edward Benjamin, Richard Witham, Dr. Jean Gino, and Dr. Carol Carter. We also thank Dr. Robert Hodapp for his careful reading of this manuscript. To our research nurse, Sharon Ort, research associate Wendy Marans, and research assistants Deborah Soriano, Elaine Algiers, and Karen Beebe, we also express our appreciation. This research was supported by the NIMH Mental Health Clinical Research Center grant No. MR30929, the Children's Clinical Research Center grant No. RROO125, NICHD grant No. HP03008-18, the MacArthur Foundation, the Merck Foundation, and U.S. Public Health Service grant No. HD-11624.

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Dykens, E., Leckman, J., Paul, R. et al. Cognitive, behavioral, and adaptive functioning in fragile X and non-fragile X retarded men. J Autism Dev Disord 18, 41–52 (1988). https://doi.org/10.1007/BF02211817

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