MAGNETIC-RESONANCE-IMAGING IN THE CONGENITAL ADRENAL-HYPERPLASIA POPULATION - INCREASED FREQUENCY OF WHITE-MATTER ABNORMALITIES AND TEMPORAL-LOBE ATROPHY

Congenital adrenal hyperplasia results from an adrenal enzyme deficien cy, that causes an underproduction of glucocorticoids and sometimes mi neralocorticoids and a resultant overproduction of androgens, until tr eatment with replacement glucocorticoids is instituted. The goal of th is study was to determine the frequency and etiology of white-matter c hanges and temporal lobe atrophy demonstrable on magnetic resonance im aging (MRI) in a group of children and young adults with congenital ad renal hyperplasia. About one third of the patients evidenced white-mat ter abnormalities or temporal lobe atrophy. All patients, except one w ith a known stroke, had normal neurologic examinations. Exposure to ex cess exogenous glucocorticoids in the process of being treated for con genital adrenal hyperplasia is the most theoretically appealing explan ation for these MRI findings. However, the relationship of MRI finding s to treatment status (over- versus under-suppressed) does not run in clear parallel.