Behavior Genetics

, Volume 10, Issue 1, pp 31–41 | Cite as

Language and cognitive development in 47, XXX females followed since birth

  • B. Pennington
  • M. Puck
  • A. Robinson


In this report, data are presented on language and cognitive development in an unselected group of eleven 47, XXX females, followed since birth, who are now 6–14 years old. The results of the Yale Developmental Exam (at 2 years) and the Illinois Test of Psycholinguistic Abilities (ITPA) (at 4–6 years) show an early delay in language development. Those girls who presently have serious language and learning problems were significantly delayed in first walking or talking, whereas the relatively unaffected girls were not. Results of the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) at 4 years of age and the Wechsler Intelligence Scale for Children (WISC or WISC-R) at 8 years of age are similar and show a generalized depression of both verbal and nonverbal abilities. Thus, unlike 45, X females or 47, XXY males, triple-X females do not have a specifically nonverbal or verbal cognitive deficit.

Key Words

47, XXX 45, X sex chromosome aneuploidy cognitive development language development learning disabilities WPPSI WISC WISC-R ITPA YDE psycholinguistics 


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  1. Barr, M. L., Sergovich, F. R., Carr, D. H., and Shaver, E. L. (1969). The triplo-X female: An appraisal based on a study of 12 cases and a review of the literature.Can. Med. Assoc. J. 101:247–258.Google Scholar
  2. Bayley, N. (1969).Bayley Scales of Infant Development, Psychological Corp., New York.Google Scholar
  3. Cohen, J. (1959). The factorial structure of the WISC at ages 7–6, 10–6, and 13–6,J. Consult. Psychol. 23:285–299.Google Scholar
  4. Eller, E., Frankenburg, W., Puck, M., and Robinson, A. (1971). Prognosis in newborn infants with X-chromosomal abnormalities.Pediatrics 47:681–688.Google Scholar
  5. Epstein, C. H. (1979). Chromosome imbalance in mouse and man. Paper presented at the Western Society for Pediatric Research, Carmel Calif., February.Google Scholar
  6. Fujita, H., Yushida, Y., Tanigawa, Y., Yamauto, K., and Sakamoto, Y. (1972). A survey of sex chromosome anomalies among normal children and mental defectives.Jap. J. Hum. Genet. 16:198–204.Google Scholar
  7. Garron, D. D. (1977). Intelligence among persons with Turner's syndrome.Behav. Genet. 7(2):105–127.Google Scholar
  8. Hollingshead, A. B., and Redlich, M. (1953). Social stratification and psychiatric disorder.Am. Sociol. Rev. 18:163–169.Google Scholar
  9. Hook, E. B. (1978). Extra sex chromosomes and human behavior. The nature of the evidence regarding XYY, XXY, XXYY, and XXX genotypes. In Vallet, H. L., and Porter, I. H. (eds.),Genetic Aspects of Sexual Differentiation, Academic Press, New York.Google Scholar
  10. Jacobs, P. A. (1979). The incidence and etiology of sex chromosome abnormalities in man. In Robinson, A., Lubs, H., and Bergsma, D. (eds.),Sex Chromosome Aneuploidy: Prospective Studies in Children, National Foundation-March of Dimes, Birth Defects: Original Article Series, Vol. XV, No. 1, Alan R. Liss Inc., New York, pp. 3–14.Google Scholar
  11. Jacobs, P. A., Baikie, A. G., Court Brown, W. M., MacGregor, T. N., MacLean, N., and Harnden, D. G. (1959). Evidence for the existence of the human “super female.”Lancet 2:423–425.Google Scholar
  12. Kirk, S. A., and Kirk, W. D. (1974).Psycholinguistic Learning Disabilities: Diagnosis and Remediation, University of Illinois Press, Chicago.Google Scholar
  13. Kohn, G., Winter, J. S. D., and Mellman, W. J. (1968). Trisomy X in three children.J. Pediatr. 72:248–252.Google Scholar
  14. Morley, M. D., (1965).The Development and Disorders of Speech in Childhood, 2nd ed, E. & S Livingstone, Edinburgh.Google Scholar
  15. Olanders, S. (1967). Double Barr bodies in women in mental hospitals.Br. J. Psychiatr. 113:1097–1099.Google Scholar
  16. Polani, P. E. (1977). Abnormal sex chromosomes, behaviour and mental disorder. In Tanner, J. M. (ed.),Developments in Psychiatric Research, Hoddler and Staughton, Ltd., London, pp. 89–128.Google Scholar
  17. Puck, M., Tennes, K., Frankenburg, W., Bryant, K., and Robinson, A. (1975). Early childhood development of four boys with 47,XXY karyotype.Clin. Genet. 7:8–20.Google Scholar
  18. Robinson, A., and Puck, T. (1967). Studies on chromosomal nondisjunction in man. II.Am. J. Hum. Genet. 19:112–129.Google Scholar
  19. Robinson, A., Lubs, H. A., Nielsen, J., and Sorensen, K. (1979). Summary of clinical findings: Profiles of children with 47,XXY, 47,XXX, and 47,XYY karyotypes. In Robinson, A., Lubs, H., and Bergsma, D. (eds.),Sex Chromosome Aneuploidy: Prospective Studies in Children, National Foundation-March of Dimes, Birth Defects: Original Article Series, Vol. XV, No. 1, Alan R. Liss, Inc., New York, pp. 261–266.Google Scholar
  20. Siegel, S. (1956).Non-parametric Statistics for the Behavioral Sciences, McGraw-Hill, New York.Google Scholar
  21. Telfer, M. A., Richardson, C. E., Helmken, J., and Smith, G. F. (1970). Divergent phenotypes among 48,XXXX and 47,XXX females.Am. J. Hum. Genet. 22:326–335.Google Scholar
  22. Tennes, K., Puck, M., Bryant, K., Frankenburg, W., and Robinson, A. (1975). A developmental study of girls with trisomy X.Am. J. Hum. Genet. 27(1):71–80.Google Scholar

Copyright information

© Plenum Publishing Corporation 1980

Authors and Affiliations

  • B. Pennington
    • 1
  • M. Puck
    • 1
  • A. Robinson
    • 1
  1. 1.National Jewish Hospital and Research Center/National Asthma Center and University of Colorado Health Sciences CenterDenver

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