A Case of Kallmann's Syndrome with Hypoplasia of Olfactory Bulb

후각구 형성부전을 동반한 칼만증후군 1례

Kallmann's syndrome has both a general and specific connotation in describing general condition of gonadotropin-releasing hormone (GnRH) deficiency or a particular cluster of anomalies associated with primary eunuchoidism. The familial occurrence of hypogonadotropic hypogonadism associated with anosmia, color blindness, synkinesia, and mental defect is the classic Kallmann's syndrome. Interestingly, anosmia, or lack of smell, was not found in the absence of gonadal deficiency in the original study of this disorder. This disorder was found on both sexes, but the male to female ratio was 11:1, and Kallmann's syndrome is more often listed under disorders of male hypogonadism for this reason. Gross anatomy has shown disorders of the olfactory bulbs associated with Kallmann's syndrome and it was demonstrated a failure of GnRH-containing cells to migrate from the olfactory placode to the hypothalamus and preoptic area. We have experienced a case of Kallmann's syndrome which showed a hypoplasia of olfactory bulb in MRI during the workup of primary amenorrheic patient. So we report this case with a brief review of literatures.