Cognitive impairment in adolescents and adults with congenital adrenal hyperplasia

Abstract

Summary: Objective: Impaired cognition has been reported in patients with congenital adrenal hyperplasia ( CAH ), although the findings have been conflicting. It has been hypothesized that the major causes of the deficits are prenatal hormonal imbalances and/or excessive glucocorticoid treatment. Design: An observational study investigating cognition in patients with CAH. Patients: A total of 55 patients with CAH and 58 control subjects from the general population, aged 16–33 years. Nine CAH subjects had been treated prenatally with dexamethasone. Setting: Singel research institute. Measurements: Standardized neuropsychological tests (Wechsler Scales and Stroop Interference Test) and questionnaires (Barkley Deficit in Executive Functioning Scale) were used. Results: Compared to controls, patients with CAH had impaired performance in tests measuring verbal working memory ( P = .024), visual‐spatial working memory ( P = .005 and P = .003) and inhibition ( P = .002). In measures of fluid intelligence/nonverbal logical reasoning, males with CAH performed poorer than control males ( P = .033). Patients with salt‐wasting CAH performed equally compared to patients with simple virilizing CAH . However, patients with a null genotype performed poorer than patients with a non‐null genotype and significantly worse on fluid intelligence/nonverbal logical reasoning ( P = .042). Prenatally‐treated women performed worse on most cognitive measures than women with CAH not treated prenatally. Conclusions: Patients with CAH had normal psychometric intelligence but impaired executive functions compared with population controls. A null CAH genotype was associated with poorer general cognitive capacity.