Nelson syndromeE24.1

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

ACTH-secreting pituitary adenoma; Nelson's syndromes

Requires free registration (medical professionals only)

Please login to access all articles, images, and functions.

Our content is available exclusively to medical professionals. If you have already registered, please login. If you haven't, you can register for free (medical professionals only).


Requires free registration (medical professionals only)

Please complete your registration to access all articles and images.

To gain access, you must complete your registration. You either haven't confirmed your e-mail address or we still need proof that you are a member of the medical profession.

Finish your registration now

HistoryThis section has been translated automatically.

Nelson, 1958

DefinitionThis section has been translated automatically.

Syndrome characterized by disorders of hypophysial hormone secretion, enlargement of the pituitary gland, intense hyperpigmentary dyschromia of the skin, headache, visual disturbances, menstrual disorders, among others.

Occurrence/EpidemiologyThis section has been translated automatically.

Very rare. In 40% of patients with bilateral adrenalectomies. Incidence decreasing.

EtiopathogenesisThis section has been translated automatically.

Idiopathic. Consequence of therapy of pituitary tumors by bilateral adrenalectomy.

ManifestationThis section has been translated automatically.

Long term development (up to 20 years) after bilateral adrenalectomy. Predominant in younger women.

Clinical featuresThis section has been translated automatically.

Pronounced ubiquitous melanotic hyperpigmentation of the skin and mucous membranes (including scars, gingiva, nipples). Sclerae are usually not affected. Often there are visual disturbances, weight loss, hypotonus, anorexia, vomiting. Excessive ACTH secretion, increased α-MSH secretion.

LaboratoryThis section has been translated automatically.

Electrolytes, ACTH, α-MSH, POMC, pituitary hormones, hypothalamus hormones, thyroid hormones.

DiagnosisThis section has been translated automatically.

Medical history (almost always Cushing's), blood pressure, laboratory, MRI, CT, thyroid parameters.

Differential diagnosisThis section has been translated automatically.

Addison's disease; hemochromatosis; neurofibromatosis; dyschromasia.

TherapyThis section has been translated automatically.

Therapy of the underlying disease (hypophysectomy, radiotherapy of the pituitary gland). If necessary, hormone replacement therapy with glucocorticoids, mineralocorticoids, somatostatin analogues.

Progression/forecastThis section has been translated automatically.

Well, with timely treatment of the underlying disease.

LiteratureThis section has been translated automatically.

  1. Chapuis Y et al (1996) Role and operative risk of bilateral adrenalectomy in hypercortisolism. World J Surg 20: 775-779
  2. Clayton R et al (1977) Secondary pituitary hyperplasia in Addison's disease. Lancet 2: 954-956
  3. Imai T et al (1996) Adrenalectomy for treatment of Cushing's syndrome: results in 122 patients and long-term follow-up studies. World J Surg 20: 781-786
  4. Kelly PA et al (2002) Neurosurgical treatment of Nelson's syndrome. J Clin Endocrinol Metab 87: 5465-5469
  5. Kobayashi T et al (2002) Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg 97: (5Suppl): 422-428
  6. Nelson DH et al (1958) ACTH-producing tumor of the pituitary gland. N Engl J Med 259: 161

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer