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Longitudinal Changes in Cognitive and Adaptive Behavior Scores in Children and Adolescents with the Fragile X Mutation or Autism

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Abstract

Studies of the relationship between the fragile X (FRAXA) mutation and autism have been controversial. Although there are differences between the two populations, individuals with FRAXA and autism exhibit remarkably similar aberrant behavior patterns. We examined comparably aged children and adolescents with FRAXA or autism to determine whether longitudinal changes in cognitive ability and adaptive behavior were similar in the two groups. We found decreases in IQ scores in young children with FRAXA as well as in those with autism. Declines in IQ scores were steeper among children with FRAXA. Older children and adolescents with autism exhibit stable test-retest scores, whereas older children with FRAXA continue to show decreases. Comparable declines in adaptive behavior composite scores were observed in both groups, at all ages tested, and across all adaptive behavior domains.

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REFERENCES

  • Bailey, D. B., Jr., Hatton, D. D., Mesibov, G., Ament, N., & Skinner, M. (2000). Early development, temperment, and functional impairment in autism and fragile X syndrome. Journal of Autism and Developmental Disorders, 30, 49–59.

    PubMed  Google Scholar 

  • Baumgardner, T., Reiss, A. L., Freund, L., & Abrams, M. (1995). Specification of the neurobehavioral phenotype in males with fragile X syndrome. Pediatrics, 95, 744–752.

    PubMed  Google Scholar 

  • Blomquist, H. K., Bohman, M., Edvinsson, S. O., Gillberg, C., Gustavson, K. H., Holgren, G., & Wahlstrom, J. (1985). Frequency of the fragile X syndrome in infantile autism: A Swedish multicenter study. Clinical Genetics, 27, 113–117.

    PubMed  Google Scholar 

  • Brown, W. T., Greer, M. K., Aylward, E. H., Fisch, G. S., Wolf-Schein, E. G., Gross, A., Ritvo, E., Ruttenberg, B. A. Bentley, W., & Castells, S. (1986). Fragile X syndrome and autism: A multicenter study. American Journal of Medical Genetics, 23, 341–352.

    PubMed  Google Scholar 

  • Brown, W. T., Jenkins, E. C., Friedman, E., Brooks, J., Wisniewski, K., Raguthu, S., & French, J. (1982). Autism is associated with the fragile X syndrome. Journal of Autism and Developmental Disorders, 12, 303–307.

    PubMed  Google Scholar 

  • Carr, J. (1992). Longitudinal research in Down syndrome. In N. W. Bray (Ed.), International review of research in mental retardation (pp. 197–223). San Diego: Academic Press.

    Google Scholar 

  • Comery, T. A., Harris, J. B., Willems, P. J., Oostra, B. A., Irwin, S. A., Weiler, I. J., Greenough, W. T. (1997). Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits. Proceedings of the National Academy of Sciences of the U.S.A., 94, 5401–5404.

    Google Scholar 

  • Dykens, E. M., & Volkmar, F. R. (1997). Medical conditions associated with autism. In D. J. Cohen & F. R. Volkmar (Eds.), Handbook of autism and pervasive developmental disorders (2nd ed., pp. 388–410). New York: Wiley.

    Google Scholar 

  • Einfeld, S., Molony, H., & Hall W. (1989). Autism is not associated with the fragile X syndrome. American Journal of Medical Genetics, 34, 187–193.

    PubMed  Google Scholar 

  • Feinstein, C., & Reiss, A. L. (1998). Autism: The point of view from fragile X studies. Journal of Autism and Developmental Disorders, 28, 393–405.

    Google Scholar 

  • Fisch, G. S. (1992). Is autism associated with the fragile X syndrome? American Journal of Medical Genetics, 43, 47–55.

    PubMed  Google Scholar 

  • Fisch, G. S., Arinami, T., Foster-Iskenius, U., Fryns, J.-P., Curfs, L. M., Borghgraef, M., Howard-Peoples, P. N., Schwartz, C. E., Simensen, R. J., & Shapiro, L. R. (1991). Relationship between age and IQ among fragile X males: A multicenter study. American Journal of Medical Genetics, 38, 481–487.

    PubMed  Google Scholar 

  • Fisch, G. S., Carpenter, N., Holden, J. J. A., Howard-Peoples, P. N., Maddalena, A., Borghgraef, M., Steyaert, J., & Fryns, J.-P. (1999a). Longitudinal changes in cognitive and adaptive behavior in fragile X females: A prospective multicenter analysis. American Journal of Medical Genetics, 83, 308–312.

    PubMed  Google Scholar 

  • Fisch, G. S., Carpenter, N., Holden, J. J. A., Simensen, R., Howard-Peoples, P. N., Maddalena A., Pandya, A., & Nance, W. (1999b). Longitudinal assessment of adaptive and maladaptive behaviors in fragile X males: Growth, development and profiles. American Journal of Medical Genetics, 83, 308–312.

    PubMed  Google Scholar 

  • Fisch, G. S., Shapiro, L. R., Simensen, R. J., Schwartz, C. E., Fryns, J.-P., Borghgraef, M., Curfs, L. M., Howard-Peoples, P. N., Arinami, T., & Mavrou, A. (1992). Longitudinal changes in IQ among fragile X males: Clinical evidence for more than one mutation? American Journal of Medical Genetics, 43, 28–34.

    Google Scholar 

  • Fisch, G. S., Simensen, R., Tarleton, J., Chalifoux, M., Holden, J. J. A., Carpenter, N., Howard-Peoples, P. N., & Maddalena, A. (1996). Longitudinal study of cognitive abilities and adaptive behavior in fragile X males: A prospective multicenter analysis. American Journal of Medical Genetics, 64, 356–361.

    PubMed  Google Scholar 

  • Freeman, B. J., Del'Homme, M., Guthrie, D., & Zhang, F. (1999). Vineland Adaptive Behavior Scores as a function of age and initial IQ in 210 autisc children. Journal of Autism and Developmental Disorders, 29, 379–384.

    PubMed  Google Scholar 

  • Freeman, B. J., Rahbar, B., Ritvo, E. R., Bice, T. L., Yokota, B. A., & Ritvo, R. (1991). The stability of cognitive and behavioral parameters in autism: A twelve-year prospective study. Journal of the American Academy of Child and Adolescent Psychiatry, 30, 479–482.

    Google Scholar 

  • Freeman, B. J., Ritvo, E. R., Needleman, R., & Yokota, A. (1985). The stability of cognitive and linguistic parameters in autism: A five-year prospective study. Journal of the American Academy of Child and Adolescent Psychiatry, 24, 459–464.

    Google Scholar 

  • Hinton, V. J., Brown, W. T., Wisniewski, K., & Rudelli, R. D. (1991). Analysis of neocortex in three males with fragile X syndrome. American Journal of Medical Genetics, 41, 289–294.

    PubMed  Google Scholar 

  • Jacobson, J. W., & Ackerman, L. J. (1990). Differences in adaptive functioning among people with autism or mental retardation. Journal of Autism and Developmental Disorders, 20, 205–219.

    PubMed  Google Scholar 

  • Lord, C., Rutter, M., & Le Courteur, A. (1994). Autism Diagnostic Interview Revised: A revised version of a diagnostic interview for caregivers of individuals with possible pervasive developmental disorders. Journal of Autism and Developmental Disorders, 24, 659–685.

    Google Scholar 

  • Lord, C., & Schopler, E. (1989). The role of age at assessment, developmental level, and test in the stability of intelligence scores inn young autistic children. Journal of Autism and Developmental Disorders, 19, 483–499.

    PubMed  Google Scholar 

  • Loveland, K. A, & Kelly, M. L. (1988). Development of adaptive behavior in adolescents and young adults with autism and Down syndrome. American Journal on Mental Retardation, 93, 84–92.

    PubMed  Google Scholar 

  • Meryash, D. L., Szymanski, L. S., & Gerald, P. (1982). Infantile autism associated with the fragile X syndrome. Journal of Autism and Developmental Disorders, 12, 349–355.

    Google Scholar 

  • Naglieri, J. A., & Pfeiffer, S. I. (1983). Reliability and stability of the WISC-R for children with below average IQs. Educational and Psychological Research, 3, 203–208.

    Google Scholar 

  • Rogers, R. C., & Simensen, R. J. (1987). Fragile X syndrome: A common etiology of mental retardation. American Journal of Mental Deficiency, 91, 445–449.

    PubMed  Google Scholar 

  • Rudelli, R. D., Brown, W. T., Wisniewski, K., Jenkins, E. C., Laure-Kamionowska, M., Connell F., & Wisniewski, H. M. (1985). Adult Fragile X syndrome: Clinico-neuropathologic findings. Acta Neuorpathologica, 67, 289–295.

    Google Scholar 

  • Rumsey, J. M., Rapaport, J. L., & Sceery, W. R. (1985). Autistic children as adults: Psychiatric, social, and behavioral outcomes. Journal of the American Academy of Child and Adolescent Psychiatry, 24, 465–473.

    Google Scholar 

  • Schatz, J., & Hamdan-Allen, G. (1995). Effects of age and IQ on adaptive behavior domains for children with autism. Journal of Autism and Developmental Disorders, 25, 51–60.

    PubMed  Google Scholar 

  • Schopler, E., Reichler, R. J., & Renner, B. R. (1988). The Childhoood Autism Rating Scale (CARS). Los Angeles: Western Psychological Services.

    Google Scholar 

  • Sigman, M., Riskin, E., & Arbeile, S. (1999). Continuity and change in the social competence of children with autism, Down syndrome and developmental delays. Monographs of the Society for Research in Child Development, 64, 1–114.

    Google Scholar 

  • Silverstein, A. B. (1982). Note on the constancy of the IQ. American Journal of Mental Deficiency, 87, 227–228.

    PubMed  Google Scholar 

  • Sparrow, S. S., Ball, D. A., & Cicchetti, D. V. (1984). Vineland Adaptive Behavior Scales. Circle Pines, MN: American Guidance Service.

    Google Scholar 

  • Stone, W. L., Ousley, O. Y., Hepburn, S. L., Hogan, K. L., & Brown, C. S. (1999). Patterns of adaptive behavior in very young children with autism. American Journal of Mental Retardation, 104, 187–199.

    PubMed  Google Scholar 

  • Thorndike, R. L., Hagen, E. P., & Sattler, J. M. (1987). Technical manual, Stanford-Binet Intelligence Test: Fourth Edition. Chicago: Riverside Publishing.

    Google Scholar 

  • Venter, A., Lord, C., & Schopler, E. (1992). A follow-up study of high-functioning autistic children. Journal of Child Psychology and Psychiatry, 33, 489–507.

    PubMed  Google Scholar 

  • Venter, P. A., Op't Hof, J., Coezee, D. S., Van de Wa, H. C., & Retie, F. (1984). No marker X chromosome in autistic children. Human Genetics, 67, 107.

    PubMed  Google Scholar 

  • Weiler, I. J., Irwin, S. A., Klintsova, A. Y., Spencer, C. M., Brazelton, A. D., Miyashiro, K., Comery, T. A., Patel, B., Eberwine, J., & Greenough, W. T. (1997). Fragile X mental retardation protein is translated near synapses in response to neurotransmitter activation. Proceedings of the National Academy of Sciences of the U.S.A., 94, 5395–5400.

    Google Scholar 

  • Wishart, J. G. (1993). The development of learning difficulties in children with Down syndrome. Journal of Intellectual Disability Research, 37, 389–403.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. School of Medicine, Yale University, New Haven, Connecticut, 06520

    Gene S. Fisch

  2. Greenwood Genetics Center, Greenwood, South Carolina, 29646

    Richard J. Simensen & R. J. Schroer

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  1. Gene S. Fisch
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  2. Richard J. Simensen
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  3. R. J. Schroer
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Fisch, G.S., Simensen, R.J. & Schroer, R.J. Longitudinal Changes in Cognitive and Adaptive Behavior Scores in Children and Adolescents with the Fragile X Mutation or Autism. J Autism Dev Disord 32, 107–114 (2002). https://doi.org/10.1023/A:1014888505185

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