Hypothalamic-Pituitary Function in the Olfacto-Genital Syndrome

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Abstract
Three patients with Kallmann's syndrome, 1 hyposmic man, 24 yr old, and 2 anosmic women, 17 and 26 yr old, have had studies of hypothalamo-pituitary function. ACTH, TSH, and GH pituitary reserves were found normal when evaluated respectively by metyrapone test, TSH increase after the intravenous administration of 200 μg of TRH and GH response to insulin-induced hypoglycemia. The subcutaneous administration of 100 μg of synthetic LRH produced an 8- to 18-fold increase in plasma LH, and an increase of plasma FSH varying between 320% and 830%. These responses are of greater magnitude than those hitherto reported. Finally, pituitary secretory reserve of prolactin was found normal as evidenced by an increase of plasma prolactin levels of 250% after the intravenous administration of 200 or 400 μg of synthetic TRH. However, when 25 mg of chlorpromazine were administrated intramuscularly, the patient with hyposmia responded with a 450% increase of plasma prolactin while no increase was observed in the two cases with anosmia. The results obtained from these investigations in Kallmann's syndrome indicate the complete integrity of the adenohypophysis, and suggest that the hypogonadotropic hypogonadism is of hypothalamic origin. The possibility exists, at least in some cases, of a defect in the hypothalamic control of pituitary prolactin secretion.

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