Gender dysphoria in an adolescent diagnosed with Klinefelter syndrome over a follow-up period

Year 2018, Volume: 4 Issue: 1, 48 - 51, 04.01.2018

Sabide Duygu Tunas Gulser Dinc , Zeynep Goker , Ozden Sukran Uneri

https://doi.org/10.18621/eurj.313304

Cited By: 1

Abstract

Although genetic factors have been thought to be main
cause for gender dysphoria, its etiology is still not clearly understood. Klinefelter
syndrome is the most seen sex chromosomal disorder. In the literature, there
are fewer case reports in connection with Klinefelter syndrome and gender
dysphoria. Herein, we report a 16-year-old adolescent patient displaying gender
dysphoria features, who has revealed Klinefelter syndrome after genetic
examination, and has been treated with testosterone hormone, and his gender
dysphoria symptoms have disappeared. In our case, chromosomal anomaly and lower
levels of androgen could play a role in the etiopathogenesis of gender
dysphori. Bringing Klinefelter syndrome disorder to mind in gender
dysphoria cases, even it is a rare disorder, could positively affect the course
of the treatment as was in our case. The relation between psychiatric symptoms,
which can be seen in gender dysphoria, and testosterone does not known exactly.
Further studies, which are randomized-controlled ones, can help to better
understand the subject. 

Keywords

adolescent, gender dysphoria, Klinefelter syndrome, depression

References

• [1] Smith ES, Junger J, Derntl B, Habel U. The transsexual brain--A review of findings on the neural basis of transsexualism. Neurosci Biobehav Rev 2015;59:251-66.
• [2] Heylens G, De Cuypere G, Zucker KJ, Schelfaut C, Elaut E, Vanden Bossche H, et al. Gender identity disorder in twins: a review of the case report literature. J Sex Med 2012;9:751-7.
• [3] Green R. Family cooccurrence of ‘‘gender dysphoria’’: ten sibling or parent-child pairs. Arch Sex Behav 2000;29:499-507.
• [4] Haberman M, Hollingsworth F, Falek A, Michael RP. Gender identity confusion, schizophrenia and a 47 XYY karyotype: a case report. Psychoneuroendocrinology 1975;1:207-9.
• [5] Buhrich N, Barr R, Lam-Po-Tang PR. Two transsexuals with 47,XYY karyotype. Br J Psychiatry 1978;133:77-81.
• [6] Snaith RP, Penhale S, Horsfield P. Male-to-female transsexual with XYY karyotype. Lancet 1991;337:557-8.
• [7] Taneja N, Ammini AC, Mohapatra I, Saxena S, Kucheria K. A transsexual male with 47, XYY karyotype. Br J Psychiatry 1992;161:698-9.
• [8] Turan MT, Esel E, Dundar M, Candemir Z, Basturk M, Sofuoglu S, et al. Female-to-male transsexual with 47, XXX karyotype. Biol Psychiatry 2000;48:1116-7.
• [9] Mepham N, Bouman WP, Arcelus J, Hayter M, Wylie KR. People with gender dysphoria who self-prescribe cross-sex hormones: prevalence, sources, and side effects knowledge. J Sex Med 2014;11:2995-3001.
• [10] Khandelwal A, Agarwal A, Jiloha RC. A 47-XXY female with gender identity disorder. Arch Sex Behav 2010;39:1021-3.
• [11] Fisher AD, Castellini G, Casale H, Fanni E, Bandini E, Campone B, et al. Hypersexuality, paraphilic behaviors, and gender dysphoria in individuals with Klinefelter's syndrome. J Sex Med 2015;12:2413-24.
• [12] Kawahara K, Jono T, Nishi Y, Ushijima H, Ikeda M. Effects of testosterone therapy on bipolar disorder with Klinefelter syndrome. Gen Hosp Psychiatry 2015;37:192e1-e2.
• [13] American Psychiatric Association. Diagnostic and statistical manual of mental disorders (DSM-5). American Psychiatric Pub, 2013.
• [14] Bao AM, Swaab DF. Sexual differentiation of the human brain: relation to gender identity, sexual orientation and neuropsychiatric disorders. Front Neuroendocrinol 2011;32:214-26.
• [15] Lanfranco F, Kamischke A, Zitzmann M, Nieschlag E. Klinefelter's syndrome. Lancet 2004;364:273-83.
• [16] Kovacs D, Vassos E, Liu X, Sun X, Hu J, Breen G, et al. The androgen receptor gene polyglycine repeat polymorphism is associated with memory performance in healthy Chinese individuals. Psychoneuroendocrinology 2009;34:947-52.
• [17] Skakkebaek A, Bojesen A, Kristensen MK, Cohen A, Hougaard DM, Hertz JM, et al. Neuropsychology and brain morphology in Klinefelter syndrome-the impact of genetics. Andrology 2014;2:632-40.
• [18] Hare L, Bernard P, Sánchez FJ, Baird PN, Vilain E, Kennedy T, et al. Androgen receptor repeat length polymorphism associated with male-to-female transsexualism. Biol Psychiatry 2009;65:93-6.
• [19] Host C, Skakkebaek A, Groth KA, Bojesen A. The role of hypogonadism in Klinefelter syndrome. Asian J Androl 2014;16:185-91.
• [20] Samango-Sprouse CA, Sadeghin T, Mitchell FL, Dixon T, Stapleton E, Kingery M, et al. Positive effects of short course androgen therapy on the neurodevelopmental outcome in boys with 47,XXY syndrome at 36 and 72 months of age. Am J Med Genet A 2013;161A:501-8.
• [21] Nielsen J, Pelsen B, Sorensen K. Follow-up of 30 Klinefelter males treated with testosterone. Clin Genet 1988;33:262-9.
• [22] Rinieris PM, Malliaras DE, Batrinos ML, Stefanis CN. Testosterone treatment of depression in two patients with Klinefelter's syndrome. Am J Psychiatry 1979;136:986-8.