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A Disorder of Olfactory and Hypothalamic Function

James L. Males, MD; John L. Townsend, MD; Robert A. Schneider, MD

Arch Intern Med. 1973;131(4):501-507.

Abstract
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.

Author Affiliations
Oklahoma City

From the Division of Endocrinology of the Department of Medicine, University of Oklahoma, Health Sciences Center, College of Medicine, Oklahoma City. Dr. Males is now with the 97th General USA Hospital, Frankfurt, Germany.

Footnotes
Received for publication July 20, 1972; accepted Aug 28.

Reprint requests to University of Oklahoma Health Sciences Center, PO Box 26901, Oklahoma City 73190 (Dr. Schneider).

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