Hypogonadotropic Hypogonadism With Anosmia—Kallmann's Syndrome

Clinical observations, hormonal measurements, and olfactory tests have been performed in six patients (five male, one female) with hypogonadotropic hypogonadism and anosmia—Kallmann's syndrome. All were deficient in gonadotropins and all had an obvious decrease in their primary and secondary sex characteristics. All were verified as anosmic. One patient had a craniotomy, and agenesis of the olfactory bulbs was identified. Although the five male patients had normal basal 17-hydroxycorticosteroid (17-OHCS) levels, administration of metyrapone in two of them failed to lead to the predicted rise in these levels, suggesting a defective pituitary reserve. A disorder of hypothalamic dysfunction is suggested, involving the control of releasing factors with hypogonadism and anosmia as the clinical markers. Our experience suggests that the syndrome is not rare and that all hypogonadal patients should be questioned about their sense of smell.