Abstract

Classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency requires life-long glucocorticoid (GC) replacement therapy. Previous studies on general cognitive ability in patients with CAH have been conflicting, and the majority focused on intelligence in adult patients. Some studies have reported normal intelligence in both women and men with CAH (1), whereas others have noted impaired intelligence (2, 3) and memory deficit (4) in children and adults with CAH. A recent investigation demonstrated that patients with CAH have impaired spatial perception and diminished quantitative abilities, most probably due to altered executive functioning (5-7). On the contrary, others have reported superior performance on spatial tests in women with CAH (8). In addition to the effects of postnatal GC, an excess of prenatal androgens may be associated with permanent changes in brain structures, organization, or function (9). Prenatal treatment with dexamethasone (DEX) may also affect cognitive functions, given that poorer cognitive abilities were shown in DEX-treated women with CAH compared with women with CAH who received no prenatal DEX therapy (7).

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