Bilgin Y M, van der Wiel H E, Fischer H R, De Herder W W
Department of Internal Medicine, IJsselland Hospital, 2900 AR Capelle aan den IJssel, The Netherlands.
J Endocrinol Invest. 2007 Oct;30(9):776-9. doi: 10.1007/BF03350817.
Severe psychosis in patients with Cushing's syndrome is rare and generally difficult to treat. We report a 46-yr-old woman suffering from Cushing's syndrome caused by an inoperable ACTH-producing lung carcinoma. She was initially treated with chemotherapy and radiotherapy. Six months later she presented with severe psychosis. Laboratory findings revealed a severe hypokalemia and metabolic alkalosis, which was caused by extremely high serum ACTH (788 ng/l) and cortisol (4.2 micromol/l). She was unresponsive to treatment with conventional antipsychotic drugs; she was therefore sedated and intubated. Treatment was started i.v. with etomidate, which blocks the cortisol synthesis, and orally by nasogastric tube with mifepristone, which competes with cortisol for binding to their receptors. To counteract adrenal insufficiency, she received corticosteroids. After 5 days there was a normalization of the ACTH, cortisol levels, and the metabolic disorders. After discontinuing etomidate she was extubated; there were no signs of psychosis observed. Computed tomography (CT) scan of the brain showed no metastasis, however CT scan of the abdomen showed liver metastasis and bilateral adrenal enlargement. Unfortunately, the clinical situation worsened and the patient died due to progression of the metastasis. This case report demonstrates the efficacy of a treatment of mifepristone with etomidate in a patient with an ectopic ACTH-producing Cushing's syndrome.
库欣综合征患者出现严重精神病较为罕见,且通常难以治疗。我们报告一例46岁女性,患有由无法手术切除的促肾上腺皮质激素(ACTH)分泌型肺癌引起的库欣综合征。她最初接受了化疗和放疗。6个月后,她出现了严重精神病。实验室检查发现严重低钾血症和代谢性碱中毒,这是由极高的血清ACTH(788 ng/l)和皮质醇(4.2 μmol/l)所致。她对传统抗精神病药物治疗无反应;因此对其进行了镇静和插管。开始静脉注射依托咪酯,其可阻断皮质醇合成,并通过鼻胃管口服米非司酮,米非司酮可与皮质醇竞争结合其受体。为对抗肾上腺功能不全,她接受了皮质类固醇治疗。5天后,ACTH、皮质醇水平及代谢紊乱恢复正常。停用依托咪酯后她被拔管;未观察到精神病迹象。脑部计算机断层扫描(CT)显示无转移,但腹部CT扫描显示有肝转移和双侧肾上腺增大。不幸的是,临床情况恶化,患者因转移进展而死亡。本病例报告证明了在一例异位ACTH分泌型库欣综合征患者中,米非司酮联合依托咪酯治疗的有效性。