[The function of the hypothalamo-hypophyseal-gonadal system in patients with Friedreich's ataxia]

Both the decrease of reproductive adaptation and effect of reproductive compensation were revealed in 19 cases of Friedreich's ataxia (FA). Retardation of sexual development was observed, the degree of which correlated with the age of disease manifestation. By means of radioimmunoassay basal and stimulated secretion of hypophysial and peripheral sex hormones were examined in 6 males and 9 females (17-30-years). The gonadal hypofunction was observed as the consequence of hypothalamo-hypophysial insufficiency. The decrease of follicle stimulating hormone and prolactin levels was found. The conclusion was made that pathological process in FA involves hypothalamus that causes in turn insufficiency of norepinephrine and epinephrine hypothalamic neurotransmission.