Article

Nelson's syndrome; a new look

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Abstract

Nelson's syndrome is defined as the development of an ACTH-secreting pituitary tumor following bilateral adrenalectomy for pituitary-dependent Cushing's syndrome.1, 2 The potential seriousness of these tumors has been repeatedly emphasized.3–6 Polycycloidal polytomography of the sella allows the detection of minor defects compatible with small adenomas. It is obvious that the patients were not studied this way initially.

We reviewed 38 cases of Cushing's disease, that is, ACTH-dependent adrenocortical hyperplasia treated from 1951 to 1973 at the Cleveland Clinic (Table 1). Five men and 12 women who had undergone either total (eight cases) or subtotal (nine cases) adrenalectomy for Cushing's syndrome between 1951 and 1973 were examined from 1976 to 1979. Two additional women treated with yttrium-90 implantation were also studied. The original diagnosis of Cushing's disease was verified in all cases by combining clinical findings with biochemical studies including the standard dexamethasone suppression test (2 mg/day for 2 days followed by 8 mg/day for 2 days, given orally in divided doses). Pathologic findings confirmed the diagnosis in all surgical cases of bilateral adrenal hyperplasia.

Roentgenographic evaluation consisted of polytomography of the sella in all 19 patients. Full visual field examinations were performed in all 19 patients in the Department of Ophthalmology. Laboratory studies were done at 8:00 a.m., at least 12 hours after the last dose of glucocorticoid therapy.

Assays

ACTH was measured by radioimmunoassay with dextran-coated charcoal to separate bound and free ligand.7 Reagents for assay were obtained from Amersham Corporation (Arlington Heights, Illinois 60005). Twenty . . .


 

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