Rickels Michael R, Nichols Charles W
Department of Medicine, Division of Endocrinology, Diabetes &, Metabolism, University of Pennsylvania School of Medicine, Philadelphia, 19104, USA.
Endocr Pract. 2004 Nov-Dec;10(6):492-6. doi: 10.4158/EP.10.6.492.
To discuss the manifestation of pseudotumor cerebri during the course of correcting the hypercortisolism of Cushing's disease.
We describe the clinical, biochemical, and neuro-ophthalmologic findings in a patient in whom pseudotumor cerebri developed during correction of the hypercortisolism of Cushing's disease. In addition, we review the relationship between pseudotumor cerebri and Cushing's disease in the literature.
A 44-year-old woman with Cushing's disease underwent total adrenalectomy after two unsuccessful transsphenoidal operations. She developed daily headaches 2 weeks postoperatively while taking 60 mg of hydrocortisone daily. She noticed a visual floater 8 weeks postoperatively, and a prompt ophthalmologic evaluation revealed papilledema. We diagnosed pseudotumor cerebri on the basis of symptoms and signs of intracranial hypertension, unremarkable findings on neuroimaging and cerebrospinal fluid analysis, and a substantially increased cerebrospinal fluid pressure. After 8 weeks of treatment with furosemide, the headaches and papilledema resolved, and the patient was able to reduce her dose of hydrocortisone to 30 mg daily. Pseudotumor cerebri has been associated with adrenocortical insufficiency and gluco- corticoid withdrawal and can lead to loss of vision. A literature review revealed 6 previous patients in whom pseudotumor cerebri developed in association with Cushing's disease. In all but one case, the pseudotumor cerebri manifested 2 to 4 weeks after treatment of hypercortisolism or cessation of hydrocortisone replacement.
Symptoms of intracranial hypertension such as headache occurring in a patient recently withdrawn from exogenous or endogenous corticosteroids should prompt consideration of the presence of pseudotumor cerebri.
探讨库欣病高皮质醇血症纠正过程中假脑瘤的表现。
我们描述了一名在库欣病高皮质醇血症纠正过程中发生假脑瘤患者的临床、生化及神经眼科检查结果。此外，我们还回顾了文献中假脑瘤与库欣病的关系。
一名44岁库欣病女性患者在两次经蝶手术失败后接受了全肾上腺切除术。术后2周，她每日服用60mg氢化可的松时出现每日头痛。术后8周，她注意到有视物漂浮物，眼科检查迅速发现视乳头水肿。我们根据颅内高压的症状和体征、神经影像学及脑脊液分析无明显异常发现以及脑脊液压力大幅升高诊断为假脑瘤。使用呋塞米治疗8周后，头痛和视乳头水肿消失，患者能够将氢化可的松剂量减至每日30mg。假脑瘤与肾上腺皮质功能不全及糖皮质激素撤药有关，可导致视力丧失。文献回顾显示，此前有6例患者在库欣病相关情况下发生假脑瘤。除1例病例外，所有病例的假脑瘤均在高皮质醇血症治疗或氢化可的松替代治疗停止后2至4周出现。
近期停用外源性或内源性皮质类固醇的患者出现头痛等颅内高压症状时，应考虑假脑瘤的存在。
