What Is Adrenomyeloneuropathy?

Adrenomyeloneuropathy (AMN) is an inherited condition that affects the spinal cord. It is a form of X-linked adrenoleukodystrophy. On average, people with AMN begin to develop features in the late...

What Are the Signs and Symptoms of Adrenomyeloneuropathy?

Adrenomyeloneuropathy (AMN) is a form of X-linked adrenoleukodystrophy. On average, people with AMN begin to develop symptoms at age 28; however, the age of onset can range from the second to the...

What Causes Adrenomyeloneuropathy?

Adrenomyeloneuropathy (AMN) is caused by changes (mutations) in the ABCD1 gene. This gene gives the body instructions to make the adrenoleukodystrophy protein (ALDP) which helps transport...

How Is Adrenomyeloneuropathy Inherited?

Adrenomyeloneuropathy (AMN) is inherited in an X-linked manner. A condition is considered X-linked if the responsible gene is located on the X chromosome, one of the two sex chromosomes. The Y...

How Is Adrenomyeloneuropathy Diagnosed?

A diagnosis of adrenomyeloneuropathy (AMN) is typically suspected based on the presence of characteristic signs and symptoms. A blood test that measures the levels of a specific type of fat (very...

How Might Adrenomyeloneuropathy Be Treated?

Treatment of adrenomyeloneuropathy (AMN) varies based on the signs and symptoms in each person. For example, steroid replacement therapy may be prescribed in people with adrenal insufficiency.

What Is the Long-Term Outlook for People with Adrenomyeloneuropathy?

The long-term outlook (prognosis) for people with adrenomyeloneuropathy (AMN) varies depending on the subtype. In general, AMN with cerebral involvement (both brain and spinal cord affected) has a...

Adrenomyeloneuropathy

Because men have one copy of the X chromosome, a mutated copy of the responsible gene in each cell is enough to cause signs or symptoms of the condition. A man with an X-linked condition will pass the mutation to all of his daughters and none of his sons.

Because women have two X chromosomes, the effect of the mutation may be masked by the other, normal copy of the gene, on the other X chromosome. Women with one mutation are often referred to as "carriers" of an X-linked condition. In most cases, carrier women have no symptoms or are mildly affected. However, some may be just as severely affected as males with the condition. Women who carry an X-linked condition have a 50% chance of passing the mutation on with each pregnancy.

In AMN approximately 20% of women who carry one ABCD1 mutation will develop progressive stiffness and weakness in their legs, often in middle age or later. These women typically have normal adrenal function.

Adrenomyeloneuropathy

Summary

Symptoms

Cause

Inheritance

Diagnosis

Treatment

Prognosis

Summary

What Is Adrenomyeloneuropathy?

Symptoms

What Are the Signs and Symptoms of Adrenomyeloneuropathy?

Cause

What Causes Adrenomyeloneuropathy?

Inheritance

How Is Adrenomyeloneuropathy Inherited?

Diagnosis

How Is Adrenomyeloneuropathy Diagnosed?

Treatment

How Might Adrenomyeloneuropathy Be Treated?

Prognosis

What Is the Long-Term Outlook for People with Adrenomyeloneuropathy?

Related HealthJournals

X-Linked Adrenoleukodystrophy

Leukodystrophy

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Adrenomyeloneuropathy