Pseudotumor cerebri in patients with Cushing's disease.

OBJECTIVE

To discuss the manifestation of pseudotumor cerebri during the course of correcting the hypercortisolism of Cushing's disease.

METHODS

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.

RESULTS

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.

CONCLUSION

Symptoms of intracranial hypertension such as headache occurring in a patient recently withdrawn from exogenous or endogenous corticosteroids should prompt consideration of the presence of pseudotumor cerebri.