Takahashi M P, Miyai I, Matsumura T, Nozaki S, Kang J
Department of Neurology, Toneyama National Hospital.
Rinsho Shinkeigaku. 1997 Aug;37(8):704-7.
We report a 28-year-old man of Kallmann syndrome with arachnoid cyst and empty sella. At age 22, he was admitted with acute slipped capital epiphysis and diagnosed as primary hypogonadotropinemia, because of no response to LH-RH before and after 7-day LH-RH injection. He was treated with androgen for only one year. On his second admission due to femoral head necrosis at age 28, the endocrinological evaluation suggested hypothalamic hypogonadotropinemia. Although he had mild hyposomia, we diagnosed him as Kallmann syndrome, because abnormalities of rhinencephalon was present on MRI. Arachnoid cyst in the middle cranial fossa and empty sella were also observed on MRI and the ballooning of the sella had been advanced on plain X-ray for these 6 years. As Kallmann syndrome is known to be accompanied with midline craniofacial anomalies, the dysplasia of sellar diaphragm might be originated by the same pathogenesis. In this case, empty sella might be caused by impaired CSF dynamics due to arachnoid cyst as well as possible constitutional anomaly of the diaphragm.
我们报告一例患有蛛网膜囊肿和空蝶鞍的28岁卡尔曼综合征男性患者。22岁时，他因急性股骨头骨骺滑脱入院，由于在7天促黄体生成素释放激素（LH-RH）注射前后对LH-RH无反应，被诊断为原发性性腺功能减退症。他仅接受了一年的雄激素治疗。28岁时因股骨头坏死再次入院，内分泌评估提示下丘脑性性腺功能减退症。尽管他有轻度身材矮小，但我们诊断他为卡尔曼综合征，因为磁共振成像（MRI）显示嗅脑存在异常。MRI还观察到中颅窝蛛网膜囊肿和空蝶鞍，并且在这六年中，蝶鞍的膨隆在X线平片上逐渐加重。由于已知卡尔曼综合征伴有中线颅面畸形，蝶鞍膈发育异常可能由相同的发病机制引起。在这种情况下，空蝶鞍可能是由于蛛网膜囊肿导致脑脊液动力学受损以及蝶鞍膈可能存在的先天性异常所致。
