Journal of Autism and Developmental Disorders

, Volume 38, Issue 1, pp 20–27

Arousal Modulation in Females with Fragile X or Turner Syndrome

  • Jane Roberts
  • Michèle M. M. Mazzocco
  • Melissa M. Murphy
  • Rudolf Hoehn-Saric
Original Paper

Abstract

The present study was carried out to examine physiological arousal modulation (heart activity and skin conductance, across baseline and cognitive tasks, in females with fragile X or Turner syndrome and a comparison group of females with neither syndrome. Relative to the comparison group, for whom a greater increase in skin conductance was associated with poor arithmetic performance and less risk taking behavior, females with fragile X displayed a minimal increase in heart activity that was nevertheless associated with poor performance on mental arithmetic. In contrast, no arousal–cognitive performance relationship emerged for the group with Turner syndrome. Taken together, our findings suggest that distinct profiles of arousal modulation might be associated with cognitive deficits in these syndrome populations.

Keywords

Psychophysiology Arousal Cognition Fragile X syndrome Turner syndrome 

References

  1. Althaus, M., Mulder, L. J., Mulder, G., Aarnoudse, C. C., & Minderaa, R. B. (1999). Cardiac adaptivity to attention-demanding tasks in children with a pervasive developmental disorder not otherwise specified (PDD-NOS). Biological Psychiatry, 46, 799–809.PubMedCrossRefGoogle Scholar
  2. Bailey, D. B. Jr., Hatton, D. D., & Skinner, M. (1998). Early developmental trajectories of males with fragile X syndrome. American Journal on Mental Retardation, 1, 29–39.CrossRefGoogle Scholar
  3. Belser, R. C., & Sudhalter, V. (1995). Arousal difficulties in males with fragile X syndrome: A preliminary report. Developmental Brain Dysfunction, 8, 270–279.Google Scholar
  4. Cohen, I. L. (1995). Behavioral profiles of autistic and nonautistic fragile X males. Developmental Brain Dysfunction, 8, 252–269.Google Scholar
  5. Dykens, E. M. (2000). Psychopathology in children with intellectual disability. Journal of Child Psychology & Psychiatry, 41, 407–417.CrossRefGoogle Scholar
  6. Fowles, D. C., Christie, M. J., Edelberg, R., Grings, W. W., Lykken, D. T., & Venables, P. H. (1981). Publication recommendations for electrodermal measurements. Psychophysiology, 18, 232–239.PubMedCrossRefGoogle Scholar
  7. Hagerman, R. J. (2002). The physical and behavioral phenotype. In R. J. Hagerman & P. J. Hagerman (Eds.) Fragile X syndrome: Diagnosis, treatment, and research (3rd ed., pp. 206–248). Baltimore: Johns Hopkins University Press.Google Scholar
  8. Hansen, A. L., Johnsen, B. H., & Thayer, J. F. (2003). Vagal influence on working memory and attention. International Journal of Psychophysiology, 48, 263–74.PubMedCrossRefGoogle Scholar
  9. Hessl, D., Glaser, B., Dyer-Friedman, J., Blasey, C., Hastie, T., Gunnar, M., et al. (2002). Cortisol and behavior in fragile X syndrome. Psychoneuroendocinology, 27, 855–72.CrossRefGoogle Scholar
  10. Hook, E. B., & Warburton, D. (1983). The distribution of chromosomal genotypes associated with Turner’s syndrome: Livebirth prevalence rates and evidence for diminished fetal mortality and severity in genotypes associated with structural X abnormalities or mosaicism. Human Genetics, 64, 24–27.PubMedCrossRefGoogle Scholar
  11. Keysor, C. S., & Mazzocco, M. M. M. (2002). A developmental approach to understanding fragile X syndrome in females. Microscopy Research and Technique, 57, 179–186.PubMedCrossRefGoogle Scholar
  12. Keysor, C. S., Mazzocco, M. M., McLeod, D. R., & Hoehn-Saric, R. (2002). Physiological arousal in females with fragile X or Turner syndrome. Developmental Psychobiology, 41, 133–146.PubMedCrossRefGoogle Scholar
  13. Kwon, H., Menon, V., Eliez, S., Warsofsky, I. S., White, C. D., Dyer-Friedman, J., et al. (2001). Functional neuroanatomy of visuospatial working memory in fragile X syndrome: relation to behavioral and molecular measures. American Journal of Psychiatry, 158, 1040–1051.PubMedCrossRefGoogle Scholar
  14. Mazzocco, M. M. M. (2006). The cognitive phenotype of Turner syndrome: Specific learning disabilities. International Congress Series, 1298, 83–92.CrossRefGoogle Scholar
  15. McCauley, E., Feuillan, P., Kushner, H., & Ross, J. L. (2001). Psychosocial development in adolescents with Turner syndrome. Journal of Behavioral and Developmental Pediatrics, 22, 360–365.Google Scholar
  16. McLeod, D. R., Hoehn-Saric, R., Labib, A. S., & Greenblatt, D. J. (1988). Six weeks of diazepam treatment in normal women: Effects on psychomotor performance and psychophysiology. Journal of Clinical Psychopharmacology, 8, 83–99.PubMedCrossRefGoogle Scholar
  17. Miller, L. J., McIntosh, D. N., McGrath, J., Shyu, V., Lampe, M., Taylor, A. K., et al. (1999). Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report. American Journal of Medical Genetics, 4, 268–279.CrossRefGoogle Scholar
  18. Porges, S. W. (1985, April). Method and apparatus for evaluating rhythmic oscillations in aperiodic physiological response systems. U. S. Patent 451 0944.Google Scholar
  19. Reyes Del Paso, G. A., Gonzalez, E., & Hernandez, J. A. (2004). Baroreceptor sensitivity and effectiveness varies differentially as a function of cognitive-attentional demands. Biological Psychology, 67, 385–395.CrossRefGoogle Scholar
  20. Roberts, J. E., Boccia, M. L., Bailey, D. B., Hatton, D. H., & Skinner, M. (2001). Cardiovascular indices of psychophysiological arousal in boys with fragile X syndrome. Developmental Psychobiology, 39, 107–123.PubMedCrossRefGoogle Scholar
  21. Rousseau, F., Heitz, D., Tarleton, J., MacPherson, J., Malmgren, H., Dahl, N., et al. (1994). A multicenter study on genotype-phenotype correlations in the fragile X syndrome, using direct diagnosis with probe StB12.3: the first 2,253 cases. American Journal of Human Genetics, 55, 225–237.PubMedGoogle Scholar
  22. Rypman, B., Berger, J. S., Genova, H. M., Rebbechi, D., & D’Esposito, M. (2005). Dissociating age-related changed in cognitive strategy and neural efficiency using event-related fMRI. Cortex, 41, 582–594.Google Scholar
  23. Skuse, D. H., Morris, J. S., & Dolan, R. J. (2005). Functional dissociation of amygdala-modulated arousal and cognitive appraisal, in Turner syndrome. Brain, 128, 2084–2096.PubMedCrossRefGoogle Scholar
  24. Tamm, L., Menon, V., Johnston, C. K., Hessl, D. R., & Reiss, A. L. (2002). fMRI study of cognitive interference processing in females with fragile X syndrome. Journal of Cognitive Neuroscience, 14, 160–171.PubMedCrossRefGoogle Scholar
  25. Turner, G., Webb, T., Wake, S., & Robinson, H. (1996). Prevalence of the fragile X syndrome. American Journal of Medical Genetics, 64, 196–197.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Jane Roberts
    • 1
  • Michèle M. M. Mazzocco
    • 2
    • 3
    • 4
    • 5
  • Melissa M. Murphy
    • 2
    • 4
  • Rudolf Hoehn-Saric
    • 2
  1. 1.Frank Porter Graham Child Development InstituteUniversity of North Carolina at Chapel HillChapel HillUSA
  2. 2.Department of Psychiatry and Behavioral SciencesJohns Hopkins University School of MedicineBaltimoreUSA
  3. 3.Department of Population and Family Health SciencesJohns Hopkins University School of MedicineBaltimoreUSA
  4. 4.Kennedy Krieger InstituteBaltimoreUSA
  5. 5.MSDPBaltimoreUSA

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