What is Adrenomyeloneuropathy?

Stephany Seipel
Stephany Seipel
Anatomical model of the human body
Anatomical model of the human body

Adrenomyeloneuropathy (AMN) is a form of a rare metabolic disorder called adrenoleukodystrophy. This condition deteriorates the adrenal gland and degenerates the myelin sheath, a fatty covering that coats the brain's nerve fibers. It can lead to brain damage, physical disability and eventual death. This disorder usually appears around the age of 28, but it can occur anywhere between the ages of 20 and 50. It affects both the brain and spinal cord in some patients, but other patients suffer damage only to the spinal cord.

AMN occurs when the enzymes in the brain do not break down fatty acids. As a result, long fatty acid chains form in the adrenal cortex and brain. Patients who develop adrenomyeloneuropathy might experience changes in their gait or have problems keeping their balance while walking. They have difficulty moving their legs or coordinating their leg muscles. The muscles in the lower body gradually become stiffer and more difficult to control as the disease progresses.

Other symptoms include impotence, bladder control issues, upset stomach and weight loss. Patients might develop seizure disorders or experience changes in behavior and attitude. As the adrenal gland degenerates, the patient also suffers from a loss of adrenal hormones, which are hormones that control bodily functions such as heart rate and blood pressure.

Medical professionals diagnose the disease by taking blood tests to look for long chain fatty acids in the brain. They conduct magnetic resonance imaging tests on the brain to look for abnormalities. Blood tests can also be performed to examine the patient's DNA.

There was no cure for adrenomyeloneuropathy as of early 2011. Some patients benefit from a mixture of glyceryl trioleate and glyceryl trierucate; these oils might help normalize the levels of fatty acids. This method has been considered experimental, and insurance might not cover it. In addition, the oil does not slow the disease's progression if both the brain and spinal cord are affected. Other patients are treated with steroids that stabilize the adrenal hormone levels.

The prognosis varies depending upon the patient. Young people who suffer from adrenomyeloneuropathy of the brain and spinal cord do not usually do as well in the long term as patients whose brains are not affected. Patients who have cerebral involvement might become completely disabled and can eventually die from the disease. Those who do not have cerebral involvement can often manage their symptoms with physical therapy and counseling.

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      Anatomical model of the human body