Nelson syndrome

General: Caused by elevated, incompletely suppressible, or nonsuppressible levels of circulating adrenocorticotropic hormone (ACTH) and possibly melanocyte-stimulating hormone; originally reported as hyperpigmentation with associated pituitary inactivity following bilateral adrenalectomy for Cushing syndrome; appears that age at the time of adrenalectomy is an important predictive factor for development of this disorder.

Ocular: Progressive visual deterioration; recurrent visual field defects with bilateral hemianopsia caused by a pituitary mass diagnosed as chromophobe adenoma.

Clinical: Hyperpigmentation; intermittent headaches.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Hoffmann JE, et al. Dissociation of plasma and spinal fluid ACTH in Nelson syndrome. JAMA 1974; 228:491.

Kemink L, et al. Patient's age is a simple predictive factor for the development of Nelson's syndrome after total adrenalectomy for Cushing's disease. J Clin Endocrinol Metab 1994; 79:887-889.

Nelson DH, et al. ACTH-producing tumor of the pituitary gland. N Engl J Med 1958; 259:161.