Vogl T J, Stemmler J, Heye B, Schopohl J, Danek A, Bergman C, Balzer J O, Felix R
Department of Radiology, Free University of Berlin, Clinic Rudolf Virchow, Germany.
Radiology. 1994 Apr;191(1):53-7. doi: 10.1148/radiology.191.1.8134597.
To identify morphologic differences between Kallman syndrome (KS) and idiopathic hypogonadotropic hypogonadism (IHH) and establish a role for magnetic resonance (MR) imaging in these disorders.
Twenty-eight patients were compared with 10 eugonal male volunteers. Eighteen patients had KS (hypogonadotropic hypogonadism with anosmia) and 10 had IHH. All participants underwent hormone analysis, a sniff-bottle smell test, and gadolinium-enhanced MR imaging. Changes in the hypothalamic-hypophyseal region and the rhinencephalon were evaluated.
MR imaging revealed intracranial morphologic changes in all patients on plain T1-weighted sections. Seventeen patients with KS demonstrated aplasia of an olfactory bulb; one olfactory sulcus was absent in six, rudimentary in four, and normal in eight. Olfactory bulbs were present in all 10 IHH patients and three showed one slightly hypoplastic bulb. Ten patients with KS and three with IHH showed an enlarged paranasal sinus system. Further MR findings were similar.
MR imaging demonstrates abnormalities of the rhinencephalon present in KS patients and occasionally absent in IHH patients.
识别卡尔曼综合征（KS）与特发性低促性腺激素性性腺功能减退症（IHH）之间的形态学差异，并确定磁共振（MR）成像在这些疾病中的作用。
将28例患者与10名性腺功能正常的男性志愿者进行比较。18例患者患有KS（伴有嗅觉缺失的低促性腺激素性性腺功能减退症），10例患有IHH。所有参与者均接受了激素分析、嗅瓶嗅觉测试和钆增强MR成像。评估下丘脑 - 垂体区域和嗅脑的变化。
MR成像在所有患者的T1加权平扫图像上均显示出颅内形态学改变。17例KS患者表现为嗅球发育不全；6例患者一侧嗅沟缺如，4例发育不全，8例正常。所有10例IHH患者的嗅球均存在，3例显示一侧嗅球轻度发育不良。10例KS患者和3例IHH患者显示鼻窦系统增大。进一步的MR表现相似。
MR成像显示KS患者存在嗅脑异常，而IHH患者偶尔不存在。
