Nelson syndrome is a disorder characterized by abnormal hormone secretion, enlargement of the pituitary gland (hypophysis), and the development of large and invasive growths known as adenomas. It occurs in an estimated 15 to 25 percent of people who undergo surgical removal of the adrenal glands for Cushing disease. Symptoms associated with Nelson syndrome include intense skin discoloration (hyperpigmentation), headaches, vision impairment, and the cessation of menstrual periods in women.
Symptoms of Nelson syndrome include intense skin pigmentation, headaches, visual field disturbances and the cessation of menstrual periods in females. Blood levels of the pituitary hormones adrenocorticotrope hormone (ACTH) and beta-melanocyte stimulating hormone (beta-MSH) are abnormally high. The pituitary gland gets abnormally large in Nelson syndrome, causing headaches and visceral symptoms.
Nelson syndrome can be caused by surgical removal of the adrenal glands on both sides of the body (bilateral adrenalectomy). Removal of the adrenal glands is a treatment for Cushing disease. Cushing disease is the name given to a condition in which Cushing syndrome, an endocrine disorder, occurs because of the presence of benign (non-cancerous) tumors on the pituitary gland. Following removal of these adrenal glands, some people will develop Nelson syndrome. Growth of a pre-existing or a concealed (occult) tumor of the pituitary gland may also cause this disorder.
Nelson syndrome affects approximately 15 to 25% of people who have undergone surgical removal of their adrenal glands. It affects males and females in equal numbers. Cases caused by tumors are very rare.
When Nelson syndrome is suspected, blood samples are analyzed for the presence or absence of cortisol and/or aldosterone. Imaging studies such as CAT scans or magnetic resonance imaging may also be used.
Treatment for Nelson syndrome consists of radiation to limit abnormal growth of the pituitary gland. If the pituitary gland increases so much in size that it encroaches on surrounding brain structures, it may be surgically removed.
Microsurgical removal of Nelson syndrome adenomas through the bone at the base of the skull (transsphenoidal) may be recommended.
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Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:109.
Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:959.
Wilson JD, Foster DW., eds. Textbook of Endocrinology. 8th ed. W.B. Saunders Company. Philadelphia, PA; 1992:283-84; 286.
Hentschel SJ, McCutcheon IE. Stereotactic radiosurgery for Cushing disease. Neurosurg Focus. 2004;16:E5.
Jane JA Jr, Vance ML, Woodburn CJ, et al. Stereotactic radiosurgery for hypersecreting pituitary tumors: part of a multimodality approach. Neurosurg Focus. 2003;14:e12.
Kobayashi T, Kida Y, Mori Y. Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg. 2002;97(5 Suppl):422-28.
Kelly PA, Samandouras G Grossman AB, et al. Neurosurgical treatment of nelson’s syndrome. J Clin Endocrinol Metab. 2002;87:5465-69.
Pollock BE, Young WF Jr. Stereotactic radiosurgery for patients with ACTH-producing pituitary adenomas after prior adrenalectomy. Int J Radiat Oncol Biol Phys. 2002;54:640-41.
Xing B, Ren Z, Su C, et al. Microsurgical treatment of Nelson’s syndrome. Chin Med J (English). 2002;115:1150-52.
Kasperlik-Zaluska AA, Jeske W. Management of Nelson’s syndrome: observations in fifteen patients. Clin Endocrinol (Oxf). 2001;55:819.
Arafah BM, Nasrallah MP. Pituitary tumors, pathophysiology, clinical manifestations and management. Endocr Relat Cancer. 2001;8:287-305
Andreassen M, Kristensen LO: Rosiglitazone for prevention or adjuvant treatment of Nelson’s syndrome after bilateral adrenalectomy.;Eur J Endocrinol. 2005 Oct;153(4):503-5
Munir A, Song F, Ince P, Ross R, Newell-Price J: A Pilot Study of Prolonged High Dose Rosiglitazone Therapy (12mg/day) in Nelson’s Syndrome. Endocrine Abstracts (2004) 8 OC24
Cushing HW. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 1932;50:137-195.
FROM THE INTERNET
Findling J. What is Nelson’s syndrome? Cushing’s Support & Research Foundation. nd. 1p.www.csrf.net
Nelson Syndrome. Health On the Net Foundation. Last modified: Wed Mar 9 2005. 3pp.
What is Nelson’s syndrome? Endocrine Surgeon. nd. 1p.
Sidhaye AR. MedlinePlus. Medical Encyclopedia. Cushing’s disease – exogenous. Update Date: 8/6/2004. 3pp.
Crousus GP, Nelson syndrome; eMedicine; August 2004 www.emedicine.com/ped/topic1558.htm
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