The Personality and Psycho‐sexual Development of Boys With 47 Xxy Chromosome Constitution

Bancroft, John; Axworthy, David; Ratcliffe, S G

doi:10.1111/j.1469-7610.1982.tb00061.x

Cited by 60 publications

(38 citation statements)

References 8 publications

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“…Population-based studies show that men with KS are more likely to complain of decreased sexuality and reduced sexual satisfaction, to engage in homosexual activities, and to exhibit impaired male gender identity (2,39,48). Difficulty relating with male peers, social isolation and, in the extreme, clinical autism can occur (2,15).…”

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confidence: 99%

Genetic, hormonal, and metabolomic influences on social behavior and sex preference of XXY mice

Liu

Erkkilä

Lue

et al. 2010

American Journal of Physiology-Endocrinology and Metabolism

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Lee PW, Swerdloff RS. Genetic, hormonal, and metabolomic influences on social behavior and sex preference of XXY mice. Am J Physiol Endocrinol Metab 299: E446-E455, 2010. First published June 22, 2010; doi:10.1152/ajpendo.00085.2010.-XXY men (Klinefelter syndrome) are testosterone deficient, socially isolated, exhibit impaired gender identity, and may experience more homosexual behaviors. Here, we characterize social behaviors in a validated XXY mouse model to understand mechanisms. Sociability and gender preference were assessed by three-chambered choice tasks before and after castration and after testosterone replacement. Metabolomic activities of brain and blood were quantified through fractional synthesis rates of palmitate and ribose (GC-MS). XXY mice exhibit greater sociability than XY littermates, particularly for male mice. The differences in sociability disappear after matching androgen exposure. Intact XXY, compared with XY, mice prefer male mice odors when the alternatives are ovariectomized female mice odors, but they prefer estrous over male mice odors, suggesting that preference for male mice may be due to social, not sexual, cues. Castration followed by testosterone treatment essentially remove these preferences. Fractional synthesis rates of palmitate are higher in the hypothalamus, amygdala, and hippocampus of XXY compared with XY mice but not with ribose in these brain regions or palmitate in blood. Androgen ablation in XY mice increases fractional synthesis rates of fatty acids in the brain to levels indistinguishable from those in XXY mice. We conclude that intact XXY mice exhibit increased sociability, differences in gender preference for mice and their odors are due to social rather than sexual cues and, these differences are mostly related to androgen deficiency rather than genetics. Specific metabolic changes in brain lipids, which are also regulated by androgens, are observed in brain regions that are involved in these behaviors.aneuploidy; Klinefelter syndrome; testosterone; X-inactivation; brain lipids KLINEFELTER SYNDROME (KS) was first described in 1942 and its chromosomal etiology identified in 1959 (11, 16). It is the most frequent sex chromosome aneuploidy in human males, with an incidence of 1 in 500 male conceptions and 1 in 800 -1,000 live male births (3,22,34). KS patients are males with an extra X chromosome (XXY), and the syndrome is typified by reproductive dysfunction such as androgen deficiency and associated sexual dysfunction, small testicular size, gynecomastia, delayed onset of puberty, infertility, and tall stature as well as nonreproductive characteristics, including dyslexia, slowed learning, and social isolation (17,40,42). KS males also exhibit higher rates of attention deficit hyperactivity disorder (AD/HD) and psychiatric diseases such as schizophrenia and depression. These clinical and behavioral features of KS could be explained as genetic and/or hormonal (testosterone deficiency). Others have also suggested that KS is a useful model to dissect the genetic ba...

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confidence: 99%

Genetic, hormonal, and metabolomic influences on social behavior and sex preference of XXY mice

Liu

Erkkilä

Lue

et al. 2010

American Journal of Physiology-Endocrinology and Metabolism

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“…Boys with SCA were rated as less inhibited in interaction with adults than boys in the clinical sample, and their scores were similar to norms. Boys with SCA have been described as having problems in relationships with peers (Bancroft et al 1982), and in some cases, problems with social responsivity (Cordeiro et al 2012). Because boys with SCA may experience problems relating with peers, they may be more oriented towards adults.…”

Section: Discussionmentioning

confidence: 99%

“…Boys with SCA have generally been described as more timid, less confident, and as having more problems relating to peers than controls (Bancroft et al 1982;Bender et al 1995;Cordeiro et al 2012). A temperamental style close to BI was described as typical among 108 American boys with SCA aged 4 to 15 years .…”

Section: Introductionmentioning

confidence: 99%

“…The social functioning of boys with SCA can be characterized by shyness, withdrawal, and anxiousness, and difficulties with social responsiveness have been found across karyotypes (e.g., Bancroft et al 1982;Cordeiro et al 2012;Ross et al 2012). High BI may be particularly problematic for boys, due to social expectations that boys should be more assertive, active, physical, and risk-willing than girls.…”

Section: Introductionmentioning

confidence: 99%

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Behavioral Inhibition in Boys with Sex Chromosome Aneuploidies Compared to a Clinical Sample

Fjermestad

Wahlborg

Stokke³

et al. 2017

Adv Neurodev Disord

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Sex chromosome aneuploidies (SCA) in boys involve physiological, cognitive, and socioemotional challenges. Internalizing problems in boys with SCA are underexamined. We examined behavioral inhibition (BI) in boys with SCA, compared to a clinical sample. BI is a temperamental style characterized by shyness, withdrawal, and cautiousness, and represents increased risk for internalizing problems. Parents (76% mothers) completed the Behavioral Inhibition Questionnaire (BIQ; Bishop et al. 2003), which comprises total BI and BI in six specific domains. Parents of 25 boys with SCA participated (boys' M age = 11.7 years, SD = 4.5, range 2-18 years), including boys with karyotypes 47,XXY, 47,XYY, 48,XXYY, and 48,XXXY. We compared their BI to 100 boys referred to mental health clinics and treated for anxiety (M age = 11.7 years, SD = 2.3, range 7-17 years), and to norms from 307 Australian boys aged >5 years. Total BI in boys with SCA was at the same level as clinic-referred boys (effect size difference d = 0.02), and higher than norms (d = 0.81). Boys with SCA were significantly more inhibited in physical situations than clinicreferred boys (p = .007; d = 0.71). Differences were small to negligible for BI domains involving peer, unfamiliar adults, and performance situations (all d ≤ 0.34). In conclusion, boys with SCA seem to be as behaviorally inhibited as boys treated for anxiety problems in mental health clinics. Inhibition in physical domains may be a particular challenge for boys with SCA.

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“…An increased incidence of anxiety, depression, neurosis, psychosis and substance abuse is reported in adolescents with 47,XXY . The language difficulty experienced by these males possibly contributes to the challenges in behavioral and social domains (Bancroft et al 1982, Jimenez 1991.…”

Section: Psychological Aspectsmentioning

confidence: 99%

Sex Chromosome Aneuploidies

Demaliaj¹,

Cerekja²,

Piazze³

2012

Aneuploidy in Health and Disease

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The Personality and Psycho‐sexual Development of Boys With 47 Xxy Chromosome Constitution

Cited by 60 publications

References 8 publications

Genetic, hormonal, and metabolomic influences on social behavior and sex preference of XXY mice

Genetic, hormonal, and metabolomic influences on social behavior and sex preference of XXY mice

Behavioral Inhibition in Boys with Sex Chromosome Aneuploidies Compared to a Clinical Sample

Sex Chromosome Aneuploidies

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