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Impaired Cognitive Function in Women with Congenital Adrenal Hyperplasia

University Departments of Growth and Reproduction (T.H.J., K.M.M.) and Clinical Genetics (M.S.), Rigshospitalet, DK-2100 Copenhagen, Denmark; Department of Health Psychology (C.P.L.R., E.L.M.), Institute of Public Health, University of Copenhagen, DK-2100 Copenhagen, Denmark; and The Kinsey Institute for Research in Sex, Gender, and Reproduction (J.M.R.), Indiana University, Bloomington, Indiana 47405

Address all correspondence and requests for reprints to: Katharina M. Main, University Department of Growth and Reproduction, GR-5064, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark. E-mail: katharina.main{at}rh.hosp.dk.

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.

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