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Executive Function in Young Males with Klinefelter (XXY) Syndrome with and without Comorbid Attention-Deficit/Hyperactivity Disorder

Published online by Cambridge University Press:  22 March 2011

Nancy Raitano Lee ,
Gregory L. Wallace ,
Liv S. Clasen ,
Rhoshel K. Lenroot ,
Jonathan D. Blumenthal ,
Samantha L. White ,
Mark J. Celano  and
Jay N. Giedd
Nancy Raitano Lee*
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Gregory L. Wallace
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Liv S. Clasen
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Rhoshel K. Lenroot
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Jonathan D. Blumenthal
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Samantha L. White
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Mark J. Celano
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
Jay N. Giedd
Affiliation:
Child Psychiatry Branch, National Institute of Mental Health, NIH, Bethesda, Maryland
*
Correspondence and reprint requests to: Nancy Raitano Lee, National Institute of Mental Health; NIH, Bldg. 10, 4C110, MSC-1367, 10 Center Drive, Bethesda, MD 20892-1600. E-mail: [email protected]
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Abstract

Deficits in executive function (EF) are reported to occur in individuals with Klinefelter syndrome (XXY). The degree of impairment, if any, is variable and the nature of these deficits has not been clearly elucidated in young males. In this report, we (a) examine EF skills using multiple tasks in a non-clinic referred group of youth with XXY, (b) describe the extent of EF weaknesses in XXY when this group is compared with typical males of a similar SES or typical males with similar verbal abilities, and (c) evaluate the contribution of comorbid attention-deficit/hyperactivity disorder (ADHD) to EF skills. The sample included 27 males with XXY (ages 9–25), 27 typically developing age- and vocabulary-matched males, and 22 age- and socioeconomic status-matched males. EF tasks included Verbal Fluency, the Trail Making Test, and the CANTAB Spatial Working Memory and Stockings of Cambridge tasks. Mixed model analysis of variance was used to compare the groups on EF tasks and revealed a main effect of group but no group by task interaction. Overall, the XXY group performed less well than both control groups, but performance did not differ significantly as a function of task. ADHD comorbidity in males with XXY was related to poorer EF skills. (JINS, 2011, 17, 522–530)

Keywords

Sex chromosomesNeuropsychologyCognitionAttention Deficit Hyperactivity DisorderWorking memoryProblem solvingXXY Klinefelter syndromeXY Typically developing malesSES Socioeconomic statusEF Executive functionCANTAB Cambridge Neuropsychological Test Automated BatterySS Standard Score
Type
Research Articles
Information
Journal of the International Neuropsychological Society , Volume 17 , Issue 3 , May 2011 , pp. 522 - 530
Copyright
Copyright © The International Neuropsychological Society 2011

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Footnotes

Conflict of Interest Statement: No conflicts declared.

References

REFERENCES

Barkley, R.A. (1997). Behavioral inhibition, sustained attention, and executive functions: Constructing a unifying theory of ADHD. Psychological Bulletin, 121, 65–94.Google Scholar
Bedard, A.C., Martinussen, R., Ickowicz, A., Tannock, R. (2004). Methylphenidate improves visual-spatial memory in children with attention-deficit/hyperactivity disorder. Journal of the American Academy of Child and Adolescent Psychiatry, 43, 260–268.CrossRefGoogle ScholarPubMed
Bender, B.G., Linden, M.G., Harmon, R.J. (2001). Neuropsychological and functional cognitive skills of 35 unselected adults with sex chromosome abnormalities. American Journal of Medical Genetics, 102, 309–313.CrossRefGoogle ScholarPubMed
Bender, B.G., Linden, M., Robinson, A. (1991). Cognitive and academic skills in children with sex chromosome abnormalities. Reading and Writing, 3, 315–327.CrossRefGoogle Scholar
Bender, B.G., Linden, M.G., Robinson, A. (1993). Neuropsychological impairment in 42 adolescents with sex chromosome abnormalities. American Journal of Medical Genetics, 48, 169–173.CrossRefGoogle ScholarPubMed
Bender, B.G., Puck, M.H., Salbenblatt, J.A., Robinson, A. (1986). Dyslexia in 47, XXY boys identified at birth. Behavior Genetics, 16, 343–354.CrossRefGoogle ScholarPubMed
Benjamini, Y., Hochberg, Y. (1995). Controlling the false discovery rate: A practical and powerful approach to multiple testing. Journal of the Royal Statistical Society, 57, 289–300.Google Scholar
Blair, C., Razza, R.P. (2007). Relating effortful control, executive function, and false belief understanding to emerging math and literacy ability in kindergarten. Child Development, 78, 647–663.CrossRefGoogle ScholarPubMed
Boada, R., Janusz, J., Hutaff-Lee, C., Tartaglia, N. (2009). The cognitive phenotype in Klinefelter syndrome: A review of the literature including genetic and hormonal factors. Developmental Disabilities Research Reviews, 15, 284–294.CrossRefGoogle ScholarPubMed
Bojesen, A., Juul, S., Gravholt, C.H. (2003). Prenatal and postnatal prevalence of Klinefelter syndrome: A national registry study. Journal of Clinical Endocrinology and Metabolism, 88, 622–626.CrossRefGoogle ScholarPubMed
Boone, K.B., Swerdloff, R.S., Miller, B.L., Geschwind, D.H., Razani, J., Lee, A., Paul, L. (2001). Neuropsychological profiles of adults with Klinefelter syndrome. Journal of the International Neuropsychological Society, 7, 446–456.CrossRefGoogle ScholarPubMed
Bruining, H., Swaab, H., Kas, M., van Engeland, H. (2009). Psychiatric characteristics in a self-selected sample of boys with Klinefelter syndrome. Pediatrics, 123, e865–e870.CrossRefGoogle Scholar
Cambridge Cognition Limited. (1999). Cambridge neuropsychological test automated battery. Cambridge: Cambridge Cognition.Google Scholar
Cohen, J. (1988). Statistical power analysis for the behavioral sciences (2nd ed.). Hillsdale, NJ: Erlbaum.Google Scholar
Deary, I.J., Pattie, A., Wilson, V., Whalley, L.J. (2005). The cognitive cost of being a twin: Two whole-population surveys. Twin Research and Human Genetics, 8, 376–383.CrossRefGoogle ScholarPubMed
DeLisi, L.E., Maurizio, A.M., Svetina, C., Ardekani, B., Szulc, K., Nierenberg, J., Harvey, P.D. (2005). Klinefelter's syndrome (XXY) as a genetic model for psychotic disorders. American Journal of Medical Genetics Part B, 135, 15–23.CrossRefGoogle Scholar
Dennis, M., Francis, D.J., Cirino, P.T., Schachar, R., Barnes, M.A., Fletcher, J.M. (2009). Why IQ is not a covariate in cognitive studies of neurodevelopmental disorders. Journal of the International Neuropsychological Society, 15, 331–343.CrossRefGoogle Scholar
Diamond, A., Barnett, W.S., Thomas, J., Munro, S. (2007). Preschool program improves cognitive control. Science, 318, 1387–1388.Google Scholar
Fales, C.L., Knowlton, B.I., Holyoak, K.J., Geschwind, D.H., Swerdloff, R.S., Gonzalo, I.G. (2003). Working memory and relational reasoning in Klinefelter syndrome. Journal of the International Neuropsychological Society, 9, 839–846.Google Scholar
Gaddes, W.H., Crockett, D.J. (1975). The Spreen-Benton aphasia tests, normative data as a measure of normal language development. Brain and Language, 2, 257–280.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, 107–116.3.0.CO;2-2>CrossRefGoogle ScholarPubMed
Giedd, J.N., Clasen, L.S., Wallace, G.L., Lenroot, R.K., Lerch, J.P., Wells, E.M., Samango-Sprouse, C. (2007). XXY (Klinefelter syndrome): A pediatric quantitative brain magnetic resonance imaging case-control study. Pediatrics, 119, e232–e240.CrossRefGoogle ScholarPubMed
Giedd, J.N., Lalonde, F.M., Celano, M.J., White, S.L., Wallace, G.L., Lee, N.R., Lenroot, R.K. (2009). Anatomical brain magnetic resonance imaging of typically developing children and adolescents. Journal of the American Academy of Child and Adolescent Psychiatry, 48, 465–470.Google Scholar
Graham, J.M. Jr., Bashir, A.S., Stark, R.E., Silbert, A., Walzer, S. (1988). Oral and written language abilities of XXY boys: Implications for anticipatory guidance. Pediatrics, 81, 795–806.CrossRefGoogle ScholarPubMed
Halperin, J.M., Healey, J.M., Zeitchik, E., Ludman, W.L., Weinstein, L. (1989). Developmental aspects of linguistic and mnestic abilities in normal children. Journal of Clinical and Experimental Neuropsychology, 11, 518–528.CrossRefGoogle ScholarPubMed
Hollingshead, A.B., Redlich, F.C. (1958). Social class and mental illness. New York: Wiley.CrossRefGoogle Scholar
Hyde, J.S. (1990). Meta-analysis and the psychology of gender differences. Journal of Women in Culture and Society, 16, 55–73.Google Scholar
Kaufman, J.B., Brent, D., Rao, U., Ryan, N. (1996). Kiddie-Sads-Present and Lifetime Version (K-SADS-PL).Google Scholar
Lezak, M.D., Howieson, D.N., Loring, D.W. (2004). Neuropsychological assessment (4th ed.). New York: Oxford University Press.Google Scholar
Miyake, A., Friedman, N.P., Emerson, M.J., Witzki, A.H., Howerter, A., Wager, T.D. (2000). The unity and diversity of executive functions and their contributions to complex “Frontal Lobe” tasks: A latent variable analysis. Cognitive Psychology, 41, 49–100.CrossRefGoogle ScholarPubMed
Netley, C., Rovet, J. (1984). Hemispheric lateralization in 47, XXY Klinefelter's syndrome boys. Brain and Cognition, 3, 10–18.CrossRefGoogle ScholarPubMed
Nielsen, J., Wohlert, M. (1990). Sex chromosome abnormalities found among 34,910 newborn children: Results from a 13-year incidence study in Arhus, Denmark. Birth Defects Original Articles Series, 26, 209–223.Google Scholar
Pennington, B.F., Bender, B.G., Puck, M., Salbenblatt, J., Robinson, A. (1982). Learning disabilities in children with sex chromosome anomalies. Child Development, 53, 1182–1192.Google Scholar
Pennington, B.F., Ozonoff, S. (1996). Executive functions and developmental psychopathology. Journal of Child Psychology and Psychiatry, 37, 51–87.CrossRefGoogle ScholarPubMed
Ratcliffe, S.G., Butler, G.E., Jones, M. (1990). Edinburgh study of growth and development of children with sex chromosome abnormalities. IV. Birth Defects Original Articles Series, 26, 1–44.Google ScholarPubMed
Ratcliffe, S.G., Masera, N., Pan, H., McKie, M. (1994). Head circumference and IQ of children with sex chromosome abnormalities. Developmental Medicine and Child Neurology, 36, 533–544.CrossRefGoogle ScholarPubMed
Ross, J.L., Roeltgen, D.P., Stefanatos, G., Benecke, R., Zeger, M.P., Kushner, H., Zinn, A.R. (2008). Cognitive and motor development during childhood in boys with Klinefelter syndrome. American Journal of Medical Genetics A, 146, 708–719.CrossRefGoogle Scholar
Ross, J.L., Zeger, M.P., Kushner, H., Zinn, A.R., Roeltgen, D.P. (2009). An extra X or Y chromosome: Contrasting the cognitive and motor phenotypes in childhood in boys with 47, XYY syndrome or 47. XXY Klinefelter syndrome. Developmental Disabilities Research Reviews, 15, 309–317.CrossRefGoogle ScholarPubMed
Rovet, J., Netley, C., Bailey, J., Keenan, M., Stewart, D. (1995). Intelligence and achievement in children with extra X aneuploidy: A longitudinal perspective. American Journal of Medical Genetics, 60, 356–363.CrossRefGoogle ScholarPubMed
Rovet, J., Netley, C., Keenan, M., Bailey, J., Stewart, D. (1996). The psychoeducational profile of boys with Klinefelter syndrome. Journal of Learning Disabilities, 29, 180–196.CrossRefGoogle ScholarPubMed
Spreen, O., Gaddes, W.H. (1969). Developmental norms for 15 neuropsychological tests age 6 to 15. Cortex, 5, 170–191.CrossRefGoogle ScholarPubMed
Steinman, K., Ross, J., Lai, S., Reiss, A., Hoeft, F. (2009). Structural and functional neuroimaging in Klinefelter (47,XXY) syndrome: A review of the literature and preliminary results from a functional magnetic resonance imaging study of language. Developmental Disabilities Research Reviews, 15, 295–308.Google Scholar
Stewart, D.A., Bailey, J., Netley, C.T., Rovet, J.F., Park, M.E. (1986). Growth and development of midadolescent children with X and Y chromosome aneuploidy: The Toronto study. In S.P. Ratcliffe (Ed.), Sex chromosome aneuploidy: Follow-up studies (Vol. 22, pp. 119–182). New York: Alan R. Liss.Google Scholar
Temple, C.M., Sanfilippo, P.M. (2003). Executive skills in Klinefelter's syndrome. Neuropsychologia, 41, 1547–1559.CrossRefGoogle ScholarPubMed
Theilgaard, A. (1984). A psychological study of the personalities of XYY- and XXY-men. Acta Psychiatrica Scandinavica, 69, 1–133.Google Scholar
Tombaugh, T.N., Kozak, J., Rees, L. (1999). Normative data stratified by age and education for two measures of verbal fluency: FAS and animal naming. Archives of Clinical Neuropsychology, 14, 167–177.Google ScholarPubMed
Tombaugh, T.N., Rees, L., McIntyre, N. (1998). Normative data for the Trail Making Test. Personal communication cited in Spreen and Strauss. A compendium of neuropsychological tests: Administration, norms and commentary (2nd ed.). New York: Oxford University Press.Google Scholar
van Rijn, S., Aleman, A., Swaab, H., Vink, M., Sommer, I., Kahn, R.S. (2008). Effects of an extra X chromosome on language lateralization: An fMRI study with Klinefelter men (47,XXY). Schizophrenia Reseach, 101, 17–25.CrossRefGoogle Scholar
van Rijn, S., Swaab, H., Aleman, A., Kahn, R.S. (2006). X Chromosomal effects on social cognitive processing and emotion regulation: A study with Klinefelter men (47,XXY). Schizophrenia Research, 84, 194–203.CrossRefGoogle Scholar
Warwick, M.M., Doody, G.A., Lawrie, S.M., Kestelman, J.N., Best, J.J., Johnstone, E.C. (1999). Volumetric magnetic resonance imaging study of the brain in subjects with sex chromosome aneuploidies. Journal of Neurology, Neurosurgery, and Psychiatry, 66, 628–632.Google Scholar
Wechsler, D. (1999). Wechsler abbreviated scale of intelligence. San Antonio, TX: Psychological Corporation/Harcourt Brace Jovanovich.Google Scholar
Webbink, D., Posthuma, D., Boomsma, D.I., de Geus, E.J.C., Visscher, P.M. (2008). Do twins have lower cognitive ability than singletons? Intelligence, 36, 539–547.CrossRefGoogle Scholar
Weiss, E.M., Kemmler, G., Deisenhammer, E.A., Fleischhacker, W.W., Delazer, M. (2003). Sex differences in cognitive functions. Personality and Individual Differences, 35, 863–875.CrossRefGoogle Scholar