EVIDENCE FOR THE BAUMAN VARIANT IN KALLMANNS-SYNDROME

BACKGROUND AND OBJECTIVE Idiopathic hypogonadotrophic hypogonadism (IH H) is a condition of gonadotrophin releasing hormone (GnRH) deficiency . IHH associated with anosmia is Kallmann's syndrome. A variant has be en described by Bauman where a patient with Kallmann's syndrome appare ntly regained normal hypothalamo-pituitary function 2 years after the initial diagnosis. GnRH secretory activity can be assesed by measuring LH pulsatility. Our objective was to define the pattern of LH pulsati lity in men with IHH and Kallmann's syndrome compared with those of no rmal controls, and to determine whether there is evidence for a Bauman variant of Kallmann's syndrome. DESIGN Patients with IHH and Kallmann 's syndrome were recruited from the endocrine clinic. Long-term hormon e replacement therapy was discontinued. LH pulsatility was determined. PATIENTS Three men with IHH, 3 men with classical Kallmann's syndrome and 5 normal male volunteers. MEASUREMENTS Baseline serum FSH, LH and testosterone. Intensive blood sampling every 10 minutes for serum LH from 1000 to 1600 h during the day and 2200 to 0400 h during the night to measure LH pulsatility. RESULTS The volunteer group showed normal LH pulsatility. In the patient group, LH secretion was apulsatile in o ne, showed significantly diminished amplitude in four, and there was n ormal pulsatility in one patient which remained normal 5 months later. CONCLUSION Three patients with idiopathic hypogonadotrophic hypogonad ism and 2 with Kallmann's syndrome had variable degrees of GnRH defici ency. One patient with Kallmann's syndrome had apparently normal GnRH activity, which remained normal 5 months later. This patient appears t o have the Bauman variant of Kallmann's syndrome.