Nelson's syndrome is a rare group of symptoms that occurs after the adrenal glands are removed or destroyed. They are caused by a rapidly developing pituitary adenoma. What are the symptoms of Nelson's syndrome and how is it treated?

Nelson's syndromeis a rare disease that occurs in people who have undergone bilateral adrenalectomy, that is, after removal of the adrenal glands.

Removal of the adrenal glands leads to the growth of the pituitary adenoma, which, by secreting hormones, affects the human endocrine system. A growing tumor can damage surrounding tissue and cause neurological symptoms.

Nelson's syndrome is characterized by excessive skin pigmentation, neurological symptoms and elevated serum ACTH levels.

The main cause of the syndrome is the abolition of the inhibitory effect of cortisol on the pituitary cells, which leads to adenomatous growth.

Nelson's syndrome and the hypothalamic-pituitary-adrenal axis

Nelson syndrome develops disorders on the hypothalamic-pituitary-adrenal axis. Properly functioning individual cells operate on the principle of a negative feedback mechanism. When there are problems at one of the stages, the others also change their work.

Physiologically, the hypothalamus produces hormones that regulate the pituitary gland, stimulate and inhibit it. The pituitary gland responds adequately to signals from the hypothalamus and secretes hormones that affect the adrenal glands. The ACTH released from the pituitary gland reaches the adrenal cortex, where it stimulates it to produce, inter alia, cortisol.

Nelson's syndrome - symptoms

The symptoms of Nelson's syndrome are caused by the pressure of the growing tumor on the surrounding tissues:

  • headaches - resulting from stretching the dura mater
  • visual disturbance - due to pressure on nerves III, IV, VI
  • excessive pigmentation of the skin and mucous membranes - hormone secretion disorders

Increased ACTH concentration affects the skin color, because this hormone is similar to melanotropin, which in a he althy person affects the amount of melanin - a pigment in the skin.

After removal of the adrenal glands in Nelson's syndrome, the deficiency of the hormones produced by them must be compensated by supplementation. The sick person should be under the care of an endocrinologist.

Nelson's syndrome - diagnosis

The clinical suspicion of Nelson's syndrome is confirmed by the measurement of plasma ACTH concentration. Significantly increased values ​​may exceed 1000pg / ml.

ACTH levels decrease during a dexamethasone inhibition test, but higher doses are needed than for Cushing's disease.

Imaging and ophthalmological examinations should be performed, as in the case of diagnostics of other pituitary tumors.

Nelson's syndrome - treatment

Surgical removal of the adenoma is the treatment of choice for Nelson's syndrome. When this is impossible, conservative treatment is applied, consisting in the introduction of drugs that inhibit the secretion of ACTH. These include:

  • cyproheptadine
  • pizotyphen
  • derivatives of valeric acid
  • bromocriptine

Long-acting somatostatin analogues can be used in some cases of Nelson's syndrome.

In severe cases of Nelson's syndrome, radiotherapy is required. Radiotherapy is a beneficial solution for patients who cannot be treated surgically, but it carries a risk of complications. There may be problems with concentration, memory or visual disturbances.

About the authorNatalia MłyńskaStudent of medicine at the Medical University of Lodz. Medicine is her greatest passion. He also loves sports, mainly running and dancing. She would like to treat her future patients in such a way as to see them as a human being, not only a disease.

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