General intelligence and cognitive profile in women with congenital adrenal hyperplasia (CAH) (original) (raw)
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Early hormonal influences on cognitive functioning in congenital adrenal hyperplasia
Developmental Psychology, 1986
A battery of cognitive tests and a measure of early childhood activities were administered to 17 females and 8 males with congenital adrenal hyperplasia (CAH), an autosomal recessive disorder associated with elevated prenatal adrenal androgen levels. CAH females, as compared with unaffected female relatives, showed significantly enhanced performance on three tests of spatial ability-Hidden Patterns, Card Rotations, and Mental Rotations; no reliable differences in spatial ability were observed between male patients and controls. On the Early Life Activities Questionnaire (ELAQ), male patient and control groups did not differ significantly on any activity scales. In contrast, CAH females, relative to female control subjects, showed significantly lower frequencies of participation in activities involving verbal expression and a trend toward greater participation in spatial manipulation activities. Nevertheless, differences between female patient and control groups in early childhood activities did not account for observed differences in spatial ability, given the absence of a significant correlation between the spatial manipulation activity scale and spatial ability. These results are consistent with an effect of pre-and perinatal androgenizing hormones on the development of spatial ability. Sex hormone exposure during critical periods of development and central nervous system differentiation influences consequent patterns of sexually dimorphic behavior and neuroendocrine function. The organizational and/or sensitizing action of sex hormones on mating and non-mating behaviors has been documented in a variety of animals (
Early androgen exposure modulates spatial cognition in congenital adrenal hyperplasia (CAH)
Psychoneuroendocrinology, 2008
Major questions remain about the exact role of hormones in cognition. Furthermore, the extent to which early perturbation in steroid function affects human brain development continues to be a wide open area of research. Congenital Adrenal Hyperplasia (CAH), a genetic disorder of steroid dysfunction characterized in part by in utero over-production of testosterone, was used as a natural model for addressing this question. Here, CAH (n=54, mean age = 17.53, 31 female) patients were compared to healthy age-and sex-matched individuals (n=55, mean age = 19.02, 22 female) on a virtual equivalent of the Morris Water Maze task , an established measure of sex differences in spatial cognition in rodents. Findings revealed that females with CAH with the most severe form of the disease and expected highest level of in utero exposure to androgens were found to perform similarly to both healthy males and CAH males, whereas strong sex differences were apparent in milder forms of the disorder and in controls. Moreover, advanced bone age, an indicator of long-term childhood exposure to testosterone was correlated with improved performance. The results indicate that individuals exposed to both excess androgens prenatally and prolonged exposure during childhood may manifest long-lasting changes in cognitive function. Such finding suggests a pivotal role of hormonal function on brain development in humans, mirroring results from the animal literature.
Hormones, context, and “Brain Gender”: A review of evidence from congenital adrenal hyperplasia
Social Science & Medicine, 2012
Brain organization theory suggests that steroid hormones during fetal development permanently organize the brain for gender, including patterns of sexuality, cognition, temperament, and interests that differ by sex. This widely-accepted theory has important implications for health, ranging from medical management of infants with intersex conditions to suggested etiologies for sex differences in autism, depression, and other mental health problems. Studies of genetic females with congenital adrenal hyperplasia (CAH), in which high prenatal androgens have been linked to both atypical genitals and "masculine" patterns of gender and sexuality, are particularly important. Based on a comprehensive review of research on CAH, this article demonstrates that such studies have neglected four broad categories of variables that plausibly affect psychosexual development: 1) physiological effects of CAH, including complex disruption of steroid hormones from early development onwards; 2) intensive medical intervention and surveillance, which many women with CAH describe as traumatic; 3) direct effects of genital morphology on sexuality (versus indirect effects that "masculine" genitals may have on gender socialization); and 4) expectations of masculinization that likely affect both the development and evaluation of gender and sexuality in CAH. Complex and iterative interactions among postnatal biological variables, medical interventions, and social context provide a more plausible explanation for atypicalities in psychology and behavior that have been reported for genetic females with CAH than the conventional explanation that early androgens have "masculinized" their brains.
Psychoneuroendocrinology, 2015
Girls and women with classical congenital adrenal hyperplasia (CAH) experience elevated androgens prenatally and show increased male-typical development for certain behaviors. Further, individuals with CAH receive glucocorticoid (GC) treatment postnatally, and this GC treatment could have negative cognitive consequences. We investigated two alternative hypotheses, that: (a) early androgen exposure in females with CAH masculinizes (improves) spatial perception and quantitative abilities at which males typically outperform females, or (b) CAH is associated with performance decrements in these domains, perhaps due to reduced short-term-memory (STM). Adolescent and adult individuals with CAH (40 female and 29 male) were compared with relative controls (29 female and 30 male) on spatial perception and quantitative abilities as well as on Digit Span (DS) to assess STM and on Vocabulary to assess general intelligence. Females with CAH did not perform better (more male-typical) on spatial perception or quantitative abilities than *
Archives of Sexual Behavior, 2008
Hormonal manipulations indicate that early androgens organize sex differences in spatial ability in laboratory rats. In humans, spatial ability is also sexually dimorphic, and information about the effects of prenatal androgens on spatial ability can be obtained from studies of congenital adrenal hyperplasia (CAH) and the ratio of the second and fourth finger lengths (2D:4D). CAH is characterized by prenatal overproduction of adrenal androgens and several lines of evidence suggest that 2D:4D reflects prenatal androgen exposure. Some studies have found that these proxy measures of prenatal androgens predict spatial ability, others have found no significant relationship, and yet others have obtained results in the opposite direction. In light of these mixed findings, we conducted meta-analyses of published literature and unpublished results to determine if, across studies, either of these indicators of prenatal androgens predicts performance on spatial tasks that show a male advantage. In addition, we applied a trim and fill analysis to the data in search of asymmetry that might be an indication of publication bias. Results indicated that females with CAH perform better on these spatial tasks, and CAH males perform worse, than do controls. Little or no relationship exists between 2D:4D and spatial ability. Implications for possible hormonal contributions and the developmental timing of sex differences in spatial cognition are discussed.
Cognitive functions in children with congenital adrenal hyperplasia
Archives of Endocrinology and Metabolism
Objective: There is controversy regarding cognitive function in patients with congenital adrenal hyperplasia (CAH). This study is aimed at the assessment of cognitive functions in children with CAH, and their relation to hydrocortisone (HC) therapy and testosterone levels. Subjects and methods: Thirty children with CAH due to 21 hydroxylase deficiency were compared with twenty age-and sexmatched healthy controls. HC daily and cumulative doses were calculated, the socioeconomic standard was assessed, and free testosterone was measured. Cognitive function assessment was performed using the Wechsler Intelligence Scale-Revised for Children and Adults (WISC), the Benton Visual Retention Test, and the Wisconsin Card Sorting Test (WCST). Results: The mean age (SD) of patients was 10.22 (3.17) years [11 males (36.7%), 19 females (63.3%)]. Mean (SD) HC dose was 15.78 (4.36) mg/ m 2 /day. Mean (SD) cumulative HC dose 44,689. 9 (26,892.02) mg. Patients had significantly lower scores in all domains of the WISC test, performed significantly worse in some components of the Benton Visual Retention Test, as well as in the Wisconsin Card Sorting Test. There was no significant difference in cognitive performance when patients were subdivided according to daily HC dose (< 10, 10-15, > 15 mg/m 2 /day). A positive correlation existed between cumulative HC dose and worse results of the Benton test. No correlation existed between free testosterone and any of the three tests. Conclusion: Patients with CAH are at risk of some cognitive impairment. Hydrocortisone therapy may be implicated. This study highlights the need to assess cognitive functions in CAH.
Personality and congenital adrenal hyperplasia: Possible effects of prenatal androgen exposure
Hormones and Behavior, 2009
Influences of early androgen exposure on personality were investigated. Participants were either exposed to abnormal levels of androgens prenatally due to congenital adrenal hyperplasia (CAH, 40 females, 29 males), or were unaffected relative controls (29 females, 30 males). Compared to female controls, females with CAH were less tender-minded (p b .001; 16 Personality Factor Inventory (16PF)), and reported greater physical aggression (p = .03; Reinisch Aggression Inventory) and less interest in infants (p b .001; Melson's Questionnaire), but did not differ in dominance (16PF). Males with CAH did not differ from male controls in interest in infants but were less dominant (p = .008), and more tender-minded (p = .033) and reported reduced physical aggression (p = .025). Thus, both males and females with CAH showed alteration in three of the four constructs assessed. Prenatal androgen exposure may shift some, but not all, personality characteristics in the male-typical direction in females. It may also be associated with a decrease in some aspects of male-typical personality development in males, although personality differences in males with CAH could relate to illness.
Neuropsychological Functioning in Girls with Premature Adrenarche
Journal of the International Neuropsychological Society, 2012
Contemporary research indicates that brain development occurs during childhood and into early adulthood, particularly in certain regions. A critical question is whether premature or atypical hormone exposures impact brain development (e.g., structure) or function (e.g., neuropsychological functioning). The current study enrolled 40 girls (aged 6-8 years) diagnosed with premature adrenarche (PA) and a comparison group of 36 girls with on-time maturation. It was hypothesized that girls with PA would demonstrate lower IQ and performance on several neuropsychological tasks. The potential for a sexually dimorphic neuropsychological profile in PA was also explored. No significant univariate or multivariate group differences emerged for any neuropsychological instrument. However, effect size confidence intervals contained medium-sized group differences at the subscale level. On-time girls performed better on verbal, working memory, and visuospatial tasks. Girls with PA showed improved attention, but not a sexually dimorphic profile. These results, though preliminary, suggest that premature maturation may influence neuropsychological functioning.