Cushing’s Syndrome

Treatment Provided by Board-Certified Neurologists and Neurosurgeons

The Department of Neurosurgery at Rutgers Health and RWJBarnabas Health treats Cushing’s syndrome, a rare and serious endocrine disease characterized by excessively high cortisol levels in the blood. Cushing’s syndrome, also called hypercortisolism, causing symptoms of upper body obesity, a red “moon” face, and other symptoms resulting from too much of the hormone, cortisol, in the blood. Cushing’s syndrome is curable in most patients.

Our neurology and neurosurgery department and our team of distinguished, board-certified physicians offers comprehensive treatment of Cushing’s syndrome. If you suspect you or a loved one suffers from Cushing’s syndrome, or if you want a second opinion on a previous diagnosis, our neurologists and neurosurgeons are available to do a complete physical evaluation to determine what is causing your symptoms.

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What Is Cushing’s Syndrome?

To understand Cushing’s syndrome, it’s useful to know what the cortisol hormone does and what happens when the body makes too much of it. Often called the stress hormone, cortisol is a corticosteroid made by the adrenal glands and released during the body’s response to stress. At healthy levels, cortisol controls a wide range of bodily functions, acting as an anti-inflammatory, controlling metabolism, regulating blood sugar levels, influencing memory formation, and more.

Cushing’s syndrome is caused by long-term exposure to high levels of cortisol, either because the body produces it on its own, or from long-term use of synthetic glucocorticoid hormones taken either orally or via injectable corticosteroids. It is a rare condition, and the National Organization for Rare Disorders reports that approximately 13 million people experience Cushing’s syndrome annually. The Cushing’s Support & Research Foundation estimates fewer than 15 of every million people are diagnosed each year. Cushing’s syndrome is most common in adults under the age of 50, but it sometimes occurs in children. For reasons still not well understood, it affects women at least 5 times as much as men. About 40 to 70 people out of every million have endogenous Cushing’s syndrome, according to The National Institute of Diabetes and Digestive and Kidney Diseases.

Cushing’s Syndrome Symptoms

Depending on the level of excess cortisol in the body, symptoms of Cushing’s syndrome vary. Diagnosing Cushing’s syndrome can sometimes take time, because symptoms may not obviously point to this disorder. Also, other disorders are related to Cushing’s, such as polycystic ovary syndrome (PCOS), which can make it difficult to diagnose.

Some of the common signs of Cushing’s syndrome include:

  • New or worsened high blood pressure
  • Upper body obesity
  • A fatty hump between the shoulders
  • Red, round “moon face”
  • Severe fatigue and muscle weakness
  • Stretch marks appearing on the abdomen, thighs, breasts, and arms
  • Bruising easily
  • Poor wound healing
  • Acne
  • Decreased libido
  • Decreased fertility (and in women, absent or irregular menstruation)
  • Memory loss
  • Cognitive problems

Causes of Cushing’s Syndrome

Endogenous and Exogenous Cushing’s Syndrome

Cushing’s syndrome can be created either by the body itself producing too much cortisol (endogenous), or something outside the body can cause the disorder (exogenous), such as taking medication containing cortisol. If your body is overproducing cortisol on its own, discovering the underlying cause is important so it can be properly treated.

Endogenous Cushing’s syndrome may be associated with the following:

  • Pituitary gland tumor (pituitary adenoma): This is the most common form of Cushing’s syndrome. It involves a benign (non-cancerous) tumor growing on the base of the brain, in the pituitary gland. It is caused by overproduction of the adrenocorticotropic hormone (ACTH) stimulating the adrenal glands to produce more cortisol than normal.
  • Ectopic ACTH-secreting tumor: These tumors are in another part of the body than the brain and may be either benign or malignant (cancerous). These tumors are usually found in the lungs, pancreas, thyroid, or thymus gland.
  • Primary adrenal gland disease: This is not influenced by response to ACTH and is a result of the glands making too much cortisol.

Exogenous Cushing’s syndrome may be caused by long-term use of steroid medications such as prednisone. This is the most common cause of Cushing’s syndrome. Usually, steroid medications are taken to treat inflammatory diseases such as lupus, asthma, malignant tumors, chronic obstructive lung disease, leukemia, or rheumatoid arthritis. Although these medications can prove effective in the short term, they may cause Cushing’s syndrome as a side effect of treatment. If you take a steroid medication to treat another disorder, the dosage may be reduced until symptoms are under control. Medication must not be abruptly stopped, as it can cause a dangerous drop in cortisol levels.

Cushing’s Syndrome Diagnosis and Treatment

Fortunately, most cases of Cushing’s syndrome can be treated and cured. First, your physician must determine why you are producing too much cortisol to make a definitive diagnosis of Cushing’s syndrome. The cause of this excess cortisol must be determined in order to properly treat it.

If your doctor suspects you have Cushing’s syndrome, but you do not take any steroidal medications, blood and urine tests will be necessary to to measure the amount of cortisol in your body and confirm a diagnosis. Further tests may be required, such as a computerized tomography (CT) scan, or a magnetic resonance imaging (MRI) scan. These imaging tests will show your doctor whether you have tumors on the pituitary gland causing Cushing’s syndrome.

If your Cushing’s syndrome is caused by a tumor, surgery may be required to remove it from your pituitary gland. This type of surgery has a high success rate, but sometimes radiation therapy is required after surgery to lower the risk of a recurrence of any tumors. After your tumor is removed, a cortisol replacement medicine must be taken for at least a few months, because it will take time before your body will be able to produce normal amounts of cortisol again.

Complications of Cushing’s Syndrome and Prognosis

Untreated Cushing’s syndrome can have serious health consequences, including high mortality rates. Without treatment, Cushing’s can be fatal. Complications may include:

  • Bone loss (osteoporosis)
  • High blood pressure (hypertension)
  • Type 2 diabetes
  • Frequent infections
  • Loss of muscle strength

Prognosis of Cushing’s syndrome largely depends on what is causing it, but most patients can be cured, or at least show significant improvement from treatment. Some patients with tumors may experience recurrence of Cushing’s syndrome if their tumor returns.

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