颅咽管瘤致低钠血症性代谢性脑病：一例报告

Nakayama Y, Tanaka A, Naritomi K, Yoshinaga S

Department of Neurosurgery, Fukuoka University, Chikushi Hospital, Japan.

Clin Neurol Neurosurg. 1999 Jun;101(2):114-7. doi: 10.1016/s0303-8467(99)00016-5.

Rathke's cleft cysts are sometimes associated with aseptic meningitis or metabolic encephalopathy due to hyponatremia. We treated such a case manifest by lethargy, fever and electroencephalographic abnormalities. A 68-year-old man was admitted to our ward after experiencing general malaise, nausea and vomiting and then high fever and lethargy. On admission, he was drowsy and had nuchal rigidity and Kernig's sign. Physically, he was pale with dry, thickened skin. He had lost 5.0 kg of body weight in the last month. His serum sodium was 115 mEq/l. He had a low serum osmotic pressure (235 mOsmol/l) and a high urine osmotic pressure (520 mOsmol/l). His urine volume was 1200-1900 ml/24 h with a specific gravity of 1008-1015. The urine sodium was 210 mEq/l. He did not have an elevated level of antidiuretic hormone. Electroencephalograms showed periodic delta waves over a background of theta waves. With sodium replacement, the patient become alert and symptom free, and his electroencephalographic findings normalized. However, the serum sodium level did not stabilize, sometimes falling with a recurrence of symptoms. Magnetic resonance imaging clearly delineated a dumbbell-shaped intrasellar and suprasellar cyst. The suprasellar component subsequently shrunk spontaneously and finally disappeared. An endocrinologic evaluation showed panhypopituitarism. The patient was given glucocorticoid and thyroxine replacement therapy, which stabilized his serum sodium level and permanently relieved his symptoms. A transsphenoidal approach was performed. A greenish cyst was punctured, and a yellow fluid was aspirated. The cyst proved to be simple or cubic stratified epithelium, and a diagnosis of Rathke's cleft cyst was made. The patient was discharged in good condition with a continuation of hormonal therapy. Rathke's cleft cyst can cause aseptic meningitis if the cyst ruptures and its contents spill into the subarachnoid space. Metabolic encephalopathy induced by hyponatremia due to salt wasting also can occur if the lesion injures the hypothalamus and pituitary gland.

拉克氏囊肿有时与无菌性脑膜炎或低钠血症所致的代谢性脑病相关。我们治疗了一例表现为嗜睡、发热及脑电图异常的此类病例。一名68岁男性在出现全身不适、恶心、呕吐，继而高热和嗜睡后入住我们病房。入院时，他嗜睡，有颈项强直及克氏征。体格检查发现他面色苍白，皮肤干燥、增厚。他在过去一个月体重减轻了5.0千克。他的血清钠为115 mEq/l。血清渗透压低（235 mOsmol/l），尿渗透压高（520 mOsmol/l）。他的尿量为1200 - 1900 ml/24小时，比重为1008 - 1015。尿钠为210 mEq/l。他的抗利尿激素水平未升高。脑电图显示在θ波背景上有周期性δ波。随着钠的补充，患者变得警觉且症状消失，脑电图结果恢复正常。然而，血清钠水平不稳定，有时会下降并伴有症状复发。磁共振成像清晰显示鞍内及鞍上有哑铃形囊肿。鞍上部分随后自发缩小并最终消失。内分泌评估显示全垂体功能减退。给予患者糖皮质激素和甲状腺素替代治疗，这使他的血清钠水平稳定并永久缓解了症状。采用经蝶窦入路。穿刺一个绿色囊肿，抽出黄色液体。囊肿证实为单层或立方分层上皮，诊断为拉克氏囊肿。患者在继续激素治疗的情况下状况良好出院。如果拉克氏囊肿破裂且内容物漏入蛛网膜下腔，可导致无菌性脑膜炎。如果病变损伤下丘脑和垂体，也可发生因盐耗所致低钠血症引起的代谢性脑病。