Cushing's syndrome, also called hypercortisolism (overproduction of the hormone cortisol), is a rare endocrine disorder characterized by a variety of symptoms and physical abnormalities. It may be caused by either prolonged exposure of the body's tissues to high levels of the hormone cortisol or due to the overproduction of cortisol in the body. Cortisol is a natural substance produced by the adrenal gland. It can also be produced synthetically.

Common features of Cushing's syndrome include:

• upper body obesity
• severe fatigue and muscle weakness
• uneven sleep patterns
• high blood pressure
• backache
• elevated blood sugar
• easy bruising
• bluish-red stretch marks on the skin.
• In women:
  • increased growth of facial and body hair
  • menstrual periods may become irregular or stop completely

Exposure to too much cortisol can occur for different reasons:

• long-term use of glucocorticoid hormones to treat inflammatory illnesses
• pituitary adenomas (benign tumors of the pituitary glands) which secrete increased amounts of adrenocorticotropic hormone (ACTH)
• ectopic ACTH syndrome (a condition in which ACTH is produced by various types of potentially malignant tumors that occur in different parts of the body)
• and adrenal tumors (tumors of the adrenal glands).

Testing for

The diagnosis of Cushing's can be done with several blood tests.

1. Cortisol Uptake Test

A baseline blood sample is drawn in the morning, an injection of dexamethasone given and a follow-up blood drawing and test done 8 hours later.

• In a normally functioning adrenal gland, dexamethasone should suppress cortisol levels in the blood stream.
  
• After 8 hours, if the cortisol levels have not been reduced, then Cushings Syndrome may be indicated.

Once Cushings Syndrome is indicated, further testing is required to find out if the adrenal glands are causing the excess cortisol production directly (Primary Cushings Syndrome) or the pitiutary glands are are producing too much ACTH for the adrenal glands causing the adrenals to produce the excess cortisol (Secondary Cushings Syndrome).

    2.  ACTH Response Test (This test is also used to diagnose Addisons Disease, or hypocortisolism, the opposite of Cushings Syndrome)

In this test, blood is drawn in the morning, prior to fasting the night before and after eating no breakfast. It is a test for free ACTH over time.

As soon as the blood is drawn, an injection of cortisol is administered.  The patient is asked to rest or return in exactly one hour, and another vial of blood is drawn and tested for the body's reaction to the cortisol injection.

• if the test shows that the cortisol level has dropped to within normal ranges and the ACTH levels are normal, then the pituitary glands and the adrenal glands are working fine together. Whatever is wrong with the patient is something else.
  
• if the test shows that the cortisol levels are high, and the ACTH levels are high, then the cause is probably in the pituitary gland producing too much ACTH. Within a few minutes, the inj ected cortisol should have been detected by the hypothalamus and subsequently shut off ACTH production instructions to the pituitary gland. However, with ACTH production still high after 1 hour, then the diagnosis is probably secondary hypercortisolism, and there is probably a pituitary tumor causing the problem.
  
• if the test shows that the cortisol levels are still high, but the ACTH levels are below normal, then the cause is in the adrenal gland(s) themselves. The injected over-supply of cortisol did shut off ACTH production, but the adrenal glands keep on producing too much cortisol.
  
•  if the test shows that the cortisol levels are low, and the ACTH levels normal, then for some reason, the adrenal glands are not functioning. This situation indicated Addison's Disease, the opposite of Cushings Syndrome.

Treatments

Treatment of Cushing's syndrome depends on the cause of the overproduction of cortisol. If the cause is long-term use of a medication being used to treat another disorder, the physician may reduce the dosage until symptoms are under control. Surgery or radiotherapy may be used to treat pituitary adenomas (growths). Surgery, radiotherapy, chemotherapy, immunotherapy, or a combination of these may be used to treat ectopic ACTH syndrome. The aim of treatment is to cure the hypercortisolism and to eliminate any tumor that threatens the individual's health, while minimizing the chance of endocrine deficiency or long-term dependence on medications.

Very high blood pressure, obesity and elevated blood sugar. Can lead to diabetes and heart attack or stroke.

None, except if the cause is other drugs used, then adjustments are necessary. Treatment is usually surgical if the cause is pituitary growths to remove them.

Lifestyle changes (before a treatment, during, and after)

The prognosis for individuals with Cushing's syndrome varies depending on the cause of overproduction of cortisol. With treatment, most individuals show significant improvement, while improvement for others may be complicated by various aspects of the causative illness. Some kinds of tumors may recur. Most cases of Cushing's can be cured.

Addison and Cushing International Foundation:  http://www.nvacp.nl

Asbury, A, et al (eds)
Diseases of the Nervous System: Clinical Neurobiology , vols. I & II, 2nd edition, W.B. Saunders Co., Philadelphia, pp. 191, 574-575 (vol. I), pp. 1526-1527 (vol. II) (1992).

Bradley, W, et al (eds)
Neurology in Clinical Practice: Principles of Diagnosis and Management vols. I & II, 2nd edition, Butterworth-Heinemann, Boston, pp. 753-754, 758 (vol. I), p. 926 (vol. II) (1996).

Orth, D.
Cushing's Syndrome New England Journal of Medicine, 332:12; 791-803 (March 23, 1995).

Rowland, L (ed)
Merritt's Textbook of Neurology 9th edition, Williams & Wilkins, Baltimore, pp. 888-889, 898 (1995).

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