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Nelson's Syndrome

General Information

  • Nelson’s syndrome is an iatrogenic condition (caused by a physician) that occurs as a result of treating Cushing's disease patients by removal of both adrenal glands.
  • In Cushing’s disease, blood cortisol is elevated due to excessive secretion of the hormone ACTH by a pituitary adenoma. ACTH signals the adrenal gland to produce cortisol. In the past, the identification of a pituitary adenoma was more difficult, and therefore patients underwent removal of the adrenal glands in order to lower the blood levels of cortisol. Because the pituitary tumor was not removed, the tumor enlarged and excessive production of ACTH continued. The hormone ACTH, in excessive amounts, can stimulate pigment production in the skin. Nelson’ syndrome develops within one to four years of surgery in about 15 percent to 25 percent of patients.

Symptoms

  • A key finding is darkening of skin color and increased pigmentation of nevi.
  • Because ACTH-secreting tumors are typically aggressive and invasive, patients may develop large tumors resulting in visual loss, pituitary failure and headache.

Diagnosis

  • The clinical history and skin pigmentation findings are usually obvious.
  • Hormonal tests generally show elevated ACTH levels in the blood but low or normal levels of cortisol.
  • Magnetic Resonance Imaging (MRI) or computed tomography (CT) scan is important for the identification of a pituitary adenoma.

Treatment

The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the NeuroICU attending physician and team members direct your family member's care while in the ICU.  The NeuroICU team consists of the bedside nurses, nurse practitioners, physicians in specialty training (Fellows) and attending physicians.

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