Impaired Cognitive Function in Women with Congenital Adrenal Hyperplasia

• Published in: The journal of clinical endocrinology and metabolism 2006-04, Vol.91 (4), p.1376-1381
• Main Authors: Johannsen, Trine H., Ripa, Caroline P. L., Reinisch, June M., Schwartz, Marianne, Mortensen, Erik L., Main, Katharina M.
• Format: Article
• Language: English
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adrenal Hyperplasia, Congenital - complications; Adrenal Hyperplasia, Congenital - psychology; Adrenals. Adrenal axis. Renin-angiotensin system (diseases); Adult; Biological and medical sciences; Cognition Disorders - etiology; Cognition Disorders - psychology; Education; Endocrinopathies; Female; Follow-Up Studies; Fundamental and applied biological sciences. Psychology; Genotype; Hospitalization; Humans; Hypoglycemia - etiology; Hyponatremia - etiology; Intelligence Tests; Medical sciences; Middle Aged; Neuropsychological Tests; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Quality of Life; Treatment Outcome; Vertebrates: endocrinology
• ISSN: 0021-972X; 1945-7197
• DOI: 10.1210/jc.2005-1959

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.