Idiopathic hypothalamic hypogonadism with polyostotic fibrous dysplasia: report of a case

A 48-year-old man with Albright's syndrome and hypogonadism is presented. Eunuchoid status, hypogenitalia and hypogonadotropinemia were associated with pathological fracture and deformity of bones. GnRH test revealed minimal LH response and delayed FSH peak. After pulsatile GnRH priming, the LH response to GnRH increased. Results of other provocative hypothalamic-pituitary tests were consistent with hypothalamic lesions. To the best of our knowledge, this is the first report of a male patient with Albright's syndrome and idiopathic hypothalamic hypogonadism. The hypothesis of endocrinopathy and its relation to other components of this syndrome were reviewed.