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ASK THE EXPERTS: Q What is acromegaly and how does it progress? Does catching it early make a difference?


doi: 10.1097/01.NNN.0000390000.33588.cb
DEPARTMENTS: Your Questions Answered: ACROMEGALY

Answers to your questions about cluster headaches, acromegaly, pesticides and ADHD, and treatment for pediatric brain tumors.

Myrna Rosenfeld, M.D., Ph.D., is professor of neurology and division chief of neuro-oncology at the University of Pennsylvania.

A Acromegaly is a hormonal disorder in which the pituitary gland produces excess growth hormone. The pituitary gland is responsible for secreting and regulating hormone functions in the body and can develop benign masses called adenomas that overproduce a variety of hormones. Acromegaly is not hereditary.

The overproduction of growth hormone has many effects. Affected children can grow excessively tall, known as gigantism. In adults, bones grow wider rather than longer; and the feet, hands, nose, and tongue often enlarge.

Acromegaly symptoms occur over several years and often go unnoticed, even when rings become tight or hats and shoes no longer fit. The excessive growth hormone also alters one's metabolism, leading to weight gain, diabetes, high blood pressure, arthritis, and heart disease. Due to the insidious nature of these changes, most patients have symptoms for an average of 10 years before a diagnosis is made. The diagnosis is confirmed by measuring growth hormone levels in a blood sample. In addition, a CT scan of the brain is important, because some adenomas can grow very large, causing headaches or vision loss by pressing on the brain. While many problems can improve after successful therapy, some changes may be permanent and lead to persistent problems—so early diagnosis and intervention is important.

Acromegaly is traditionally treated through transsphenoidal surgery, in which the adenoma is removed through a small incision made in the back of the nose. The procedure requires a short hospital stay and also recovery time. Eighty percent to 85 percent of patients with tumors less than 1cm in size (microadenomas) typically experience long-term cure, compared to 50 percent to 60 percent of patients with larger tumors (macroadenomas). Those patients whose symptoms persist after surgery are treated with drugs that block the actions of the growth hormone. These may include somatostatin analogues, effective in 50 percent to 70 percent of patients; indopamine agonists, effective in up to 90 percent of patients; and growth hormone antagonists, which aren't as effective as the previous two drugs and can be prescribed in addition to somatostatin analogues. All patients with acromegaly should be seen by an endocrinologist, who can make sure that the treatment is effective and that all other hormone functions are working well.


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©2010 American Academy of Neurology