How to Know if You Have Cushings Syndrome

Cushing's Syndrome/Disease | American Association of Neurological Surgeons

Cushing's syndrome is a disorder acquired by the body'due south exposure to an backlog of the hormone cortisol. Cortisol affects all tissues and organs in the body. These effects together are known every bit Cushing'due south syndrome.

Cushing'southward syndrome tin be caused by overuse of cortisol medication, as seen in the treatment of chronic asthma or rheumatoid arthritis (iatrogenic Cushing's syndrome), excess production of cortisol from a tumor in the adrenal gland or elsewhere in the body (ectopic Cushing's syndrome) or a tumor of the pituitary gland secreting adrenocorticotropic hormone (ACTH) which stimulates the over-production of cortisol from the adrenal gland (Cushing's illness).

Cortisol is a normal hormone produced in the outer portion of the adrenal glands. When functioning correctly, cortisol helps the body reply to stress and change. It mobilizes nutrients, modifies the body'south response to inflammation, stimulates the liver to enhance claret carbohydrate and helps command the amount of water in the body. Cortisol production is regulated by the ACTH produced in the pituitary gland.

• An estimated 10-15 per million people are affected every yr.
• Pituitary adenomas (Cushing's disease) account for more than lxx pct of cases in adults and about threescore-70 pct of cases in children and adolescents.
• Cushing'southward syndrome about commonly affects adults ages twenty-50 and is more prevalent in females, accounting for about seventy percent of all cases.

Pituitary adenomas are benign tumors of the pituitary gland that secrete increased amounts of ACTH causing excessive cortisol production. Most patients have a single adenoma. First described in 1912 by neurosurgeon Harvey Cushing, Physician, in his book The Pituitary Body and Its Disorders, Cushing'south disease is the most common cause of spontaneous Cushing's syndrome, accounting for 60-seventy percent of all cases.

Some benign or cancerous (cancerous) tumors that arise outside the pituitary gland can produce ACTH. This condition is known every bit ectopic ACTH syndrome. Lung tumors (malignant adenomas or carcinoids) crusade more than than 50 percentage of these cases. Other less common types of tumors that tin can produce ACTH are thymomas, pancreatic islet prison cell tumors and medullary carcinomas of the thyroid.

An abnormality of the adrenal glands, such as an adrenal tumor, may cause Cushing's syndrome. Nigh of these cases involve non-cancerous tumors called adrenal adenomas, which release excess cortisol into the blood.

Adrenocortical carcinomas, or adrenal cancers, are the to the lowest degree common crusade of Cushing's syndrome. Cancer cells secrete excess levels of several adrenal cortical hormones including cortisol and adrenal androgens. Adrenocortical carcinomas often cause very high hormone levels and a rapid onset of symptoms.

Most cases of Cushing's syndrome are non genetic. Even so, some individuals may develop Cushing'due south syndrome due to an inherited tendency to develop tumors of one or more endocrine glands. In main pigmented micronodular adrenal disease, children or young adults develop small cortisol-producing tumors of the adrenal glands. In multiple endocrine neoplasia type I (MEN I), hormone-secreting tumors of the parathyroid glands, pancreas and pituitary gland can occur. Cushing's syndrome in MEN I may be due to a pituitary, ectopic or adrenal tumor.

• Weight gain in confront (moon face)
• Weight proceeds above the neckband bone (supraclavicular fat pad)
• Weight gain on the back of cervix (buffalo hump)
• Skin changes with easy bruising in the extremities and evolution of purplish stretch marks (striae) particularly over the abdomen or axillary region
• Red, round face (plethora)
• Central obesity with weight gain centered over the chest and abdomen with thin arms and legs
• Excessive hair growth (hirsutism) on face, neck, chest, abdomen and thighs
• Female person balding
• Generalized weakness and fatigue
• Blurry vision
• Vertigo
• Muscle weakness
• Menstrual disorders in women (amenorrhea)
• Decreased fertility and/or sexual practice bulldoze (libido)
• Hypertension
• Poor wound healing
• Diabetes mellitus
• Severe depression
• Extreme mood swings

The diagnosis of Cushing's syndrome is based on clinical suspicion combined with a review of the patient's medical history, physical test and laboratory tests to help make up one's mind the existence of excessive levels of cortisol. A patient suspected of having Cushing's syndrome must initially have an appearance suggestive of excess cortisol product combined with an elevated 24-hour urinary excretion of cortisol. In assessing the physical features of Cushing's syndrome, it tin often be benign to compare old and recent photographs to reveal the archetype changes in facial and trunk appearance associated with this disorder.

The post-obit tests tin can help determine if there are excessive levels of cortisol being produced:

• The 24-60 minutes urinary cortisol test measures the amount of cortisol being produced within the urine over the course of an entire twenty-four hour period. Levels higher than fifty-100 micrograms per day in an adult advise the presence of Cushing'southward syndrome. Although the bulk of patients with Cushing's syndrome accept elevated levels of cortisol, it is becoming increasingly axiomatic that many patients with a mild case of Cushing'due south syndrome may also have normal levels of cortisol resulting in several 24-hr urine collections to ostend a diagnosis.
• The low-dose dexamethasone suppression test measures the response of the adrenal glands to ACTH and has been widely utilized for four decades. It involves taking a modest dose of a cortisol-like drug, dexamethasone (1 mg), at eleven p.one thousand., so having claret drawn to screen for cortisol the following forenoon. In patients without Cushing's syndrome, the morning level of cortisol is typically very low, indicating that ACTH secretion was suppressed by the evening dose of dexamethasone. In patients with Cushing's syndrome, the morning cortisol level volition be loftier. It is axiomatic that normal patients will suppress their cortisol to a very depression level (i.eight mg/dl), whereas those suffering from Cushing's syndrome volition not. Using this strict criterion, this test should provide an estimated 95-97 percent diagnostic accuracy charge per unit. However, some patients with a mild case of Cushing's syndrome can suppress their cortisol to low levels making it hard to fully diagnose utilizing this exam.
• The tardily-night salivary cortisol test is a relatively new exam that checks for elevated levels of cortisol in the saliva betwixt 11 p.m. and midnight. Cortisol secretion is usually very low late at night, but in patients with Cushing's syndrome, the level volition always exist elevated during this fourth dimension. Collection of saliva requires special sampling tubes; still, this is an easy test for patients to perform and tin exist done multiple times. Normal levels of late-dark salivary cortisol virtually exclude the diagnosis of Cushing's syndrome. When administered correctly, this test should provide an estimated 93-100 per centum diagnostic accuracy rate.

After a definitive diagnosis has been made, the source must then be determined. The first step in distinguishing the underlying cause is the measurement of ACTH. Patients with ACTH-secreting tumors will either have a normal or elevated level of ACTH. In dissimilarity, patients with an excess of adrenal cortisol will accept a subnormal level. Performing a high-dose dexamethasone suppression test may exist helpful in this situation. This test, like to the low-dose dexamethasone suppression test, involves taking a high dose of dexamethasone (8 mg) at eleven p.thousand. then having blood drawn to screen for the presence of cortisol the following morn. In normal patients, the morn level of cortisol will again be very depression. Patients with pituitary tumors will also suppress their serum cortisol level, but those with adrenal tumors volition maintain a loftier level of cortisol production. A loftier level of cortisol points to a non-pituitary source.

Despite the tests described above, distinguishing a pituitary from a non-pituitary ACTH-secreting tumor can be diagnostically challenging. The majority of patients with ACTH-secreting tumors have a pituitary lesion that is often very small.

The following tests are recommended:

• Magnetic Resonance Imaging (MRI) of the pituitary gland with gadolinium enhancement is a recommended approach. When an obvious pituitary tumor (>v mm) is identified with an MRI, further diagnostic evaluation may not be needed depending on the clinical presentation. However, well-nigh 50 percent of patients will accept a "normal" MRI of the pituitary, with 10 percent having incidental tumors unrelated to ACTH production. In such cases, the MRI will not provide a definitive diagnosis and additional tests will exist required.
• Petrosal sinus sampling is a test used to distinguish the source of ACTH secretion and should only be performed afterward the diagnosis of Cushing's syndrome has been confirmed. ACTH and other pituitary hormones produced in the pituitary gland enter the blood stream by drainage through veins chosen the inferior petrosal sinuses. To perform this process, a catheter is placed in both veins at the same time and blood is sampled for ACTH before and after the administration of the corticotropin-releasing hormone (CRH) and at two, five and 10 minute intervals. This invasive technique should be performed at a medical heart by a medical professional with extensive experience in this procedure. When administered correctly, this test should provide an estimated 95-98 percent diagnostic accuracy rate.

Treatment of Cushing's syndrome depends on the underlying crusade of excess cortisol but may peradventure include surgery, radiation, chemotherapy or the use of cortisol-inhibiting drugs. If the cause is iatrogenic, from long-term use of glucocorticoid hormones to treat another disorder, the physician will gradually reduce the dose of the externally administered steroid to the everyman dose acceptable for control of that disorder. Once control is established, the dose of glucocorticoid hormones may be given on alternate days to lessen side effects for the patient.

Microsurgical resection of an ACTH-secreting pituitary adenoma is the optimum treatment for Cushing'southward disease with cure rates of lxxx-90 percent if a tumor is found. Surgery is most often done through a transnasal transsphenoidal arroyo, which will non leave a visible scar. This blazon of surgery may be performed, per the surgeon's preference, using either a minimally invasive operating microscope or with the utilize of an endoscope. Partial removal of the pituitary gland (subtotal hypophysectomy) may be used in patients without clearly identifiable adenomas.

In patients to whom a remission is not obtained afterwards surgery, the use of various medications that can inhibit cortisol product may exist used, such as:

• Ketoconazole (Nizoral)
• Mitotane (Lysodren)
• Metyrapone (Metopirone)
• Mifepristone (Korlym)

These agents are non as effective as surgery and are considered a 2nd–line class of treatment. In some patients who are particularly resistant to all forms of therapy, removal of the adrenal glands bilaterally (bilateral adrenalectomy) may be considered.

Post-surgery, with successful tumor removal, the production of ACTH drops beneath normal. This subtract is natural and temporary, and patients are prescribed a synthetic class of cortisol such as hydrocortisone or prednisone to compensate this change. About patients can discontinue replacement therapy within six to 12 months, but others may crave the use of oral steroids for several years to, mayhap, their lifetime. Patients who need adrenal surgery may also require steroid replacement therapy.

One boosted treatment option in patients who have failed surgical procedures or are besides high run a risk medically to bring to surgery is radiations therapy. Radiation therapy, which is administered over a six-week period to the pituitary gland, has yielded improvement in xl-50 percent of adults and up to 85 percent of children.

Stereotactic radiosurgery has also been found to exist an effective treatment for patients with ACTH-secreting pituitary adenomas. A contempo study demonstrated endocrine remission in 54 pct of patients and tumor-growth command in 96 per centum. Even so, ongoing observation is required to monitor for tumor growth, disease recurrence, the development of new hormone deficiencies and damage to the optic pathways and brain.

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Source: https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Cushings-Disease

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