Impaired cognitive function in women with congenital adrenal hyperplasia.

CONTEXT

Congenital adrenal hyperplasia (CAH) is a disorder with a wide spectrum of severity.

OBJECTIVE

The objective of this study was to investigate cognitive function in CAH women.

DESIGN

This was a case-control study.

SETTING

This study was conducted at a tertiary center for pediatric endocrinology at the University Hospital of Copenhagen.

PARTICIPANTS

Thirty-five Danish CAH women (age 17-51 yr) were included, and participation rate was 84%. Control women were recruited through the Danish Civil Registration System and matched on age and education.

MAIN OUTCOME MEASURES

An abbreviated form of the Wechsler Adult Intelligence Scale was used, i.e. full-scale intelligence quotient (IQ; five of 11 subtests), which included three of six verbal IQ subtests and two of five performance IQ subtests.

RESULTS

A significantly lower IQ was found in CAH patients compared with controls with respect to mean full-scale IQ (84.5 vs. 99.1; P < 0.001), mean verbal IQ (86.6 vs. 97.3; P < 0.001), and mean performance IQ (85.7 vs. 101.3; P < 0.001). The salt-wasting CAH group had lower IQ scores than the simple-virilizing CAH group, which reached significance for mean total IQ (81.2 vs. 92.8, P = 0.04) and mean verbal IQ (84.7 vs. 95.5, P = 0.05), and additionally, lower scores than the late-onset CAH group, which reached significance for performance IQ (mean 81.5 vs. 96.2, P = 0.02).

CONCLUSIONS

Impaired cognitive function was observed in patients with CAH, especially in salt-wasting CAH. These intriguing findings may reflect adverse effects of hyponatremic episodes, suboptimal postnatal hormone replacement therapy or prenatal adrenal androgen excess, and the potential psychosocial consequences of the disorder.