Abstract
Klinefelter's Syndrome was first described in 1942. However, many of the psychiatric, developmental, and neurologic manifestations were not recognized until much later. The present paper reviews the literature pertaining to these aspects of Klinefelter's Syndrome. The etiology, incidence, and prevalence of this syndrome are also discussed. The importance of early detection and therapeutic intervention to minimize psychiatric morbidity is discussed.
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Bancroft, J., Axworthy, D., and Ratcliffe, S. (1982). The personality and psychosexual development of boys with 47XXY chromosome constitution.J. Child Psychol. Psychiat. 23: 169–80.
Barker, T., and Black, W. (1976). Klinefelter Syndrome in a military population.Arch. Gen. Psychiatry 33: 607–10.
Becker, K. (1972). Clinical and therapeutic experiences with Klinefelter's Syndrome.Fertil. Steril., 23: 568–78.
Beumont, P., Bancroft, J., Beardwood, C.,et al. (1972). Behavioural changes after treatment with testosterone: Case report.Psychological Med. 2: 70–2.
Caldwell, P., and Smith, D. (1972). The XXY (Klinefelter's) Syndrome in childhood: Detection and treatment.J. Pediat. 80: 250–8.
Cameron, O., and Landau, S. (1980). Lithium carbonate treatment of mania associated with Klinefelter's Syndrome (Letter).JAMA: 243, 1712.
Campbell, M., Breuer, H., and Wolman, S. (1973). The interplay of biological and environmental factors in a preschool-age patient with Klinefelter's Syndrome.Pediat. 52: 29–37.
Caroff, S. (1978). Klinefelter's Syndrome and bipolar affective illness: A case report.Am. J. Psychiat. 153: 748–9.
Crandall, B., Carrel, R., and Sparkes, R. (1972). Chromosome findings in 700 children referred to a psychiatric clinic.J. Pediat. 80: 62–8.
Evans, D., and Crechlow, R., (1987). Carcinoma of the male breast and Klinefelter's Syndrome: Is there an association? CA 37: 246–51.
Forssman, H. (1970). The mental implications of sex chromosome aberrations.Brit. J. Psychiat. 117: 315–63.
Forssman, H., and Hambert, G. (1963). Incidence of Klinefelter's Syndrome among mental patients (Letter).Lancet i: 1327.
Funderburk, S., and Ferjo, N. (1978). Clinical observations in Klinefelter (47,XXY) Syndrome.J. Ment. Defic. Res. 22: 207–12.
Garvey, M., and Mutton, D. (1973). Sex chromosome aberrations and speech development.Arch. Dis. Child. 48: 937–41.
Hambert, G. (1964). Positive sex chromatin in men with epilepsy.Acta Med. Scand. 175: 663–5.
Hambert, G. (1966).Males with Positive Sex Chromatin, Munksgaard, Copenhagen.
Harkulich, J., Marchner, T., and Brown, E. (1979). Neurological, neuropsychological, and behavioral correlates of Klinefelter's Syndrome.J. Nerv. Ment. Dis. 167: 359–63.
Hunter, J. (1969). A controlled study of the psychopathology and physical measurements of Klinefelter's Syndrome.Brit. J. Psychiat. 115: 443–8.
Jablensky, A., Janota, I., and Shepherd, M. (1970). Neuropsychiatric illness and neuropathological findings in a case of Klinefelter's Syndrome.Psychol. Med. 1: 18–39.
Jacobs, P., and Strong, J. (1959). A case of human intersexuality having a possible XXY sex determining mechanism.Nature (London) 183: 302–3.
Johnson, O. (ed.) (1985).The 1986 Almanac, Houghton Mifflin, New York.
Kessler, S., and Moos, R. (1973). Behavioral aspects of chromosomal disorders.Annu. Rev. Med. 24: 89–102.
Klinefelter, H. Reifenstein, E., and Albright, F. (1942). Syndrome characterized by Gynecomastia, aspermatogenesis without A-Leydigism, and increased excretion of follicle-stimulating hormone.J. Clin. Endocrin. 2: 615–627.
Lesage, J., and Chouinard, G. (1978). Manic-depressive illness associated with Klinefelter's Syndrome and essential tremors.Am. J. Psych. 135: 59–61.
Maclean, N., Court Brown, W., Jacobs, P.et al. (1968). A survey of sex chromatin abnormalities in mental hospitals.J. Med. Genet. 5: 165–72.
McDanal, C, Finley, S., and Finley, W. (1983). Psychiatric disturbances in a 6-year-old boy with Klinefelter's Syndrome.S. Med. J. 76: 1068–9.
Muller, N., and Endres, M. (1987). An XX male with schizophrenia: A case of personality development and illness similar to that in XXY males.J. Clin. Psych. 48, 379–80.
Nichols, C., Heerema, N., Palmer, C.,et al (1987). Klinefelter's Syndrome associated with mediastinal germ cell neoplasms.J. Clin. Oncol. 5: 1290–4.
Nielsen, J., Bjamason, S., Friedrich, U.,et al. (1970). Klinefelter's Syndrome in children.J. Child Psychol. Psychiat. 11: 109–19.
Nielsen, J., Johnsen, S., and Sorensen, K. (1980). Follow-up 10 years later of 34 Klinefelter males with Karyotype 47,XXY and 16 hypogonadal males with karyotype 46,XY.Psychol. Med. 10: 345–52.
Pasqualini, R. (1957). Psychopathology of Klinefelter's Syndrome.Lancet 273: 164–7.
Paulsen, C., Gordon, D., and Carpenter, R. (1986). Klinefelter's Syndrome and its variants: A hormonal and chromosomal study.Rec. Prog. Horm. Res. 24: 321–53.
Petersdorf, R., Adams, R., Braunwald, E.,et al. (eds.) (1983).Harrison's Principles of Internal Medicine, (tenth edition), McGraw-Hill, New York.
Polani, P. (1969). Abnormal sex chromosomes and mental disorder.Nature (London) 223: 680–6.
Pomeroy, J. (1980). Klinefelter's Syndrome and schizophrenia.Brit. J. Psychiat. 136: 597–9.
Raboch, J. Mellan, J., and Starka, L., Klinefelter's Syndrome: Sexual development and activity.Arch. Sex. Dev. Act. 8: 333–9.
Raboch, J., and Nedoma, K. (1958). Sex chromatin and sexual behavior.Psychosom. Med. 20: 55–9.
Raboch, J., and Starka, L. (1973). Reported coital activity of men and levels of plasma testosterone.Arch. Sex. Behav. 2: 309–15.
Rinieris, P., Malliaras, D., Batrinos, M.et al. (1979). Testosterone treatment of depression in two patients with Klinefelter's Syndrome.Am. J. Psychiat. 136: 986–8.
Roy, A. (1981). Schizophrenia and Klinefelter's Syndrome.Can. J. Psychiat. 26: 262–4.
Schiavi, R., Theilgaard, A., Owen, D.,et al. (1988). Sex chromosome anomalies, hormones, and sexuality.Arch. Gen. Psychiatry 45: 19–24.
Sorensen, K., and Nielsen, J. (1977). Twenty psychotic males with Klinefelter's Syndrome.Acta Psychiat. Scand. 56: 249–55.
Sperber, M., Salomon, L., Collins, M.et al. (1972). Childhood schizophrenia and 47,XXY Klinefelter's Syndrome.Amer. J. Psychiat. 128: 1400–8.
Swanson, D., and Stipes, A. (1969). Psychiatric aspects of Klinefelter's Syndrome.Amer. J. Psychiat. 126: 814–22.
Tennes, K., Puck, M., Orfanakis, D.,et al. (1977). The early childhood development of 17 boys with sex chromosome anomalies: A prospective study.Pediat. 59: 574–83.
Wakeling, A. (1972). Comparative study of psychiatric patients with Klinefelter's Syndrome and hypogonadism.Psychol. Med. 2: 139–54.
Wu, F., Bancroft, J., Davidson, D.,et al. (1982). The behavioural effects of testosterone undecanoate in adult men with Klinefelter's Syndrome: A controlled study.Clin. Endocrinol. 16: 489–97.
Wegman, T., and Smith, D. (1963). Incidence of Klinefelter's Syndrome among juvenile delinquents and felons (Letter).Lancet i: 274.
Wyngaarden, J., and Smith, L. (eds.),Cecil Textbook of Medicine, (seventeenth edition), Saunders, Philadelphia.
Yeragani, V., and Hopkinson, G. (1981). Klinefelter's Syndrome with atypical presenting features.Can. J. Psychiat. 26: 567–8.
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Liester, M.B. Psychiatric aspects of Klinefelter's Syndrome. Journal of the Multihandicapped Person 2, 241–250 (1989). https://doi.org/10.1007/BF01100094
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DOI: https://doi.org/10.1007/BF01100094