Hostname: page-component-586b7cd67f-rdxmf Total loading time: 0 Render date: 2024-11-22T17:21:15.290Z Has data issue: false hasContentIssue false

Working memory and relational reasoning in Klinefelter syndrome

Published online by Cambridge University Press:  27 August 2003

Christina L. Fales*
Affiliation:
Department of Psychology, University of California, Los Angeles, California
Barbara J. Knowlton
Affiliation:
Department of Psychology, University of California, Los Angeles, California
Keith J. Holyoak
Affiliation:
Department of Psychology, University of California, Los Angeles, California
Daniel H. Geschwind
Affiliation:
Department of Neurology, University of California, Los Angeles, California
Ronald S. Swerdloff
Affiliation:
Harbor-UCLA Medical Center, Torrance, California
Irene Gaw Gonzalo
Affiliation:
Harbor-UCLA Medical Center, Torrance, California
*
Reprint requests to: Christina L. Fales, Department of Psychology, University of California—Los Angeles, 405 Hilgard Avenue, Los Angeles, CA 90095-1563. E-mail: [email protected]

Abstract

Klinefelter syndrome (KS) is a sex chromosome abnormality associated with male infertility and mild cognitive deficits. Individuals with KS have been reported to have impaired verbal ability, as well as deficits in executive function. To further understand the nature of their deficits, we assessed specific elements of frontal lobe function such as working memory and relational reasoning. Men with KS exhibited a deficit in a transitive inference task in which participants ordered a set of names based on a list of propositions about the relative heights of the people named. This deficit was present even for items in which the propositions were given in order, so a chaining strategy could be used. Men with KS are also impaired on the n-back task, which uses letters as stimuli. In contrast, these men performed as well as controls in nonverbal reasoning (Raven's Progressive Matrices). These results suggest that men with KS have intact nonverbal reasoning abilities, but that a difficulty in encoding verbal information into working memory may underlie their executive and linguistic impairments. (JINS, 2003, 9, 839–846.)

Type
Research Article
Copyright
Copyright © The International Neuropsychological Society 2003

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Baddeley, A.D. (1986). Working memory. Oxford, UK: Oxford University Press.Google ScholarPubMed
Bender, B.G., Linden, M.G., & Robinson, A. (1993). Neuropsychological impairment in 42 adolescents with sex chromosome abnormalities. American Journal of Medical Genetics, 48, 169173.10.1002/ajmg.1320480312CrossRefGoogle ScholarPubMed
Boone, K.B., Swerdloff, R.S., Miller, B.L., Geschwind, D.H., Razani, J., Lee, A., Gonzalo, I.G., Haddal, A., Rankin, K., Lu, P., & Paul, L. (2001). Neuropsychological profiles of adults with Klinefelter Syndrome. Journal of the International Neuropsychological Society, 7, 446456.10.1017/S1355617701744013CrossRefGoogle ScholarPubMed
Carpenter, P.A., Just, M.A., & Shell, P. (1990). What one intelligence test measures: A theoretical account of the processing in the Raven Progressive Matrices Test. Psychological Review, 97, 404431.CrossRefGoogle ScholarPubMed
Christoff, K., Prabhakaran, V., Dorfman, J., Zhao, Z., Kroger, J.K., Holyoak, K.J., & Gabrieli, J.D.E. (2001). Rostrolateral prefrontal cortex involvement in relational integration during reasoning. NeuroImage, 14, 11361149.10.1006/nimg.2001.0922CrossRefGoogle ScholarPubMed
Cohen, J.D., Forman, S.D., Braver, T.S., Casey, B.J., Servan-Schreiber, D., & Noll, D.C. (1994). Activation of the prefrontal cortex in a nonspatial working memory task with functional MRI. Human Brain Mapping, 1, 293304.CrossRefGoogle Scholar
Cohen, J.D., Perlstein, W.M., Braver, T.S., Nystrom, L.E., Noll, D.C., Jonides, J., & Smith, E.E. (1997). Temporal dynamics of brain activation during a working memory task. Nature, 386, 604608.10.1038/386604a0CrossRefGoogle ScholarPubMed
D'Esposito, M., Postle, B.R., & Rypma, B. (2000). Prefrontal cortical contributions to working memory: Evidence from event-related fMRI studies. Experimental Brain Research, 133, 311.10.1007/s002210000395CrossRefGoogle ScholarPubMed
Geschwind, D.H., Gregg, J., Boone, K.B., Karrim, J., Pawlikowska-Haddal, A., Rao, E., Ellison, J., Ciccodicola, A., D'Urso, M., Woods, R., Rappold, G.A., Swerdloff, R., & Nelson, S.F. (1998). Klinefelter's Syndrome as a model of anomalous laterality: Testing gene dosage in the X chromosome pseudoautosomal region using a DNA microarray. Developmental Genetics, 23, 215229.10.1002/(SICI)1520-6408(1998)23:3<215::AID-DVG7>3.0.CO;2-X3.0.CO;2-X>CrossRefGoogle ScholarPubMed
Geschwind, D.H., Boone, K.B., Miller, B.L., & Swerdloff, R.S. (2000). Neurobehavioral phenotype of Klinefelter Syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 6, 107116.10.1002/1098-2779(2000)6:2<107::AID-MRDD4>3.0.CO;2-23.0.CO;2-2>CrossRefGoogle ScholarPubMed
Graham, J., Bashir, A., Stark, R., Silbert, A., & Walzer, S. (1988). Oral and written language abilities of XXY boys: Implications for anticipatory guidance. Pediatrics, 81, 795806.Google ScholarPubMed
Halford, G.S. (1984). Can young children integrate premises in transitivity and serial order tasks? Cognitive Psychology, 16, 6593.10.1016/0010-0285(84)90004-5CrossRefGoogle Scholar
Halford, G.S., Wilson, W.H., & Phillips, S. (1998). Processing capacity defined by relational complexity: Implications for comparative, developmental, and cognitive psychology. Brain and Behavioral Sciences, 21, 823864.10.1017/S0140525X98001769CrossRefGoogle ScholarPubMed
Hummel, J.E. & Holyoak, K.J. (1997). Distributed representations of structure: A theory of analogical access and mapping. Psychological Review, 104, 427466.CrossRefGoogle Scholar
Kroger, J.K., Saab, F.W., Fales, C.L., Bookheimer, S.Y., Cohen, M.S., & Holyoak, K.J. (2002). Recruitment of anterior dorsolateral prefrontal cortex in human reasoning: A parametric study of relational complexity. Cerebral Cortex, 12, 477485.CrossRefGoogle ScholarPubMed
Mandoki, M.W., Sumner, G.S., Hoffman, R.P., & Riconda, D.L. (1991). A review of Klinefelter's Syndrome in children and adolescents. Journal of the American Academy of Child and Adolescent Psychiatry, 30, 167172.CrossRefGoogle ScholarPubMed
Netley, C. & Rovet, J. (1984). Hemispheric lateralization in 47, XXY Klinefelter's Syndrome boys. Brain and Cognition, 3, 1018.10.1016/0278-2626(84)90002-2CrossRefGoogle ScholarPubMed
Nielsen, J. & Sorensen, K. (1984). The importance of early diagnosis of Klinefelter's Syndrome. In Bandman, H-J. & Breit, R. (Eds.), Klinefelter's Syndrome (pp. 170187). Berlin, Germany: Springer-Verlag. 10.1007/978-3-642-69644-2_22CrossRefGoogle Scholar
Norman, D.A. & Shallice, T. (1986). Attention to action: Willed and automatic control of behavior. In Davidson, R.J., Schwarts, G.E., & Shapiro, D. (Eds.), Consciousness and self-regulation: Advances in research and theory, Vol. 4 (pp. 118). New York: Plenum Press.Google Scholar
Petrides, M., Alivisatos, B., Meyer, E., & Evans, A.C. (1993). Functional activation of the human frontal cortex during the performance of a verbal working memory task. Proceedings of the National Academy of Sciences, USA, 90, 878882.CrossRefGoogle Scholar
Prabhakaran, V., Smith, J.A.L., Desmond, J.E., Glover, G.H., & Gabrieli, J.D.E. (1997). Neural substrates of fluid reasoning: An fMRI study of neocortical activation during performance of the Raven's Progressive Matrices Test. Cognitive Psychology, 33, 4363.CrossRefGoogle ScholarPubMed
Raven, J.C. (1941). Standardization of progressive matrices, 1938. British Journal of Medical Psychology, 19, 137150.CrossRefGoogle Scholar
Robin, N. & Holyoak, K.J. (1995). Relational complexity and the functions of prefrontal cortex. In Gazzaniga, M.S. (Ed.), The cognitive neurosciences (pp. 987997). Cambridge, Massachusetts: MIT Press.Google Scholar
Robinson, A., Bender, B.G., & Borelli, J.B. (1986). Sex chromosomal aneuploidy: Prospective and longitudinal studies. Birth Defects Original Article Series, 22, 2371.Google ScholarPubMed
Ross, J.L., Roeltgen, D., Kushner, H., Wei, F., & Zinn, A.R. (2000a). The Turner syndrome-associated neurocognitive phenotype maps to distal Xp. American Journal of Human Genetics, 67, 672681.CrossRefGoogle Scholar
Ross, J.L., Zinn, A.R., & McCauley, E. (2000b). Neurodevelopmental and psychosocial aspects of Turner syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 3, 135141.3.0.CO;2-K>CrossRefGoogle Scholar
Rovet, J., Netley, C., Keenan, M., Bailey, J., & Stewart, D. (1996). The psychoeducational profile of boys with Klinefelter Syndrome. Journal of Learning Disabilities, 29, 180196.10.1177/002221949602900208CrossRefGoogle ScholarPubMed
Smith, E.E. & Jonides, J. (1998). Neuroimaging analyses of human working memory. Proceedings of the National Academy of Sciences, USA, 95, 1206112068.10.1073/pnas.95.20.12061CrossRefGoogle ScholarPubMed
Smith, E.E., Jonides, J., & Koeppe, R.A. (1996). Dissociating verbal and spatial working memory using PET. Cerebral Cortex, 6, 1120.10.1093/cercor/6.1.11CrossRefGoogle ScholarPubMed
Sorensen, K. (1992). Physical and mental development of adolescent males with Klinefelter Syndrome. Hormone Research, 37, (Suppl. 3), 5561.Google ScholarPubMed
Stewart, D.A., Bailey, J.D., Netley, C.T., Rovet, J., & Park, E. (1986). Growth and development from early to mid-adolescence of children with X and Y chromosome aneuploidy: The Toronto study. Birth Defects, 22, 119182.Google Scholar
Theilgaard, A. (1984). A psychological study of the personality of XYY- and XXY-men. Acta Psychiatrica Scandinavian Supplement, 69, (Suppl. 315), 1133.Google Scholar
Waltz, J.A., Knowlton, B.J., Holyoak, K.J., Boone, K.B., Mishkin, F.S., de Menezes Santos, M., Thomas, C.R., & Miller, B.L. (1999). A system for relational reasoning in human prefrontal cortex. Psychological Science, 10, 119125.CrossRefGoogle Scholar
Walzer, S., Graham, J.M., & Bashir, A. (1982). Preliminary observations on language and learning in XXY boys. In Stewart, D.A. (Ed.), Children with sex chromosome aneuploidy: Follow-up studies, Vol. 18, No. 4 (pp. 185192). New York: Alan R. Liss.Google Scholar