Adrenoleukodystrophy (ALD) Medical Journal - Symptoms, Causes and Treatment of Adrenoleukodystrophy not in Woman

Adrenoleukodystrophy or ALD a rare hereditary disease, only affecting males, which usually appears between the age of 5 to 10 years. The recessive gene responsible for ALD is transmitted on the X sex chromosome and the disorder is characterized by atropy (shrinking) of adrenal glands and degenerative changes in the nerves and brain.

Causes of Adrenoleukodystrophy

These are caused by a defect in fat metabolism so that certain ling chain fatty acids are not broken down but accumulate in the central nervous system. This result in progressive mental deterioration, loss of speech and blindness.

What are the symptoms of X-linked adrenoleukodystrophy?

There are a wide range of clinical severities of X-linked adrenoleukodystrophy (X-ALD), and these have been classified into six broad categories: childhood cerebral ALD, adolescent cerebral ALD, adult cerebral, adrenomyeloneuropathy, adrenal insufficiency-only, and symptomatic heterozygotes.

Treatment of Adrenoleukodystrophy

Treatment with a combination of certain oils and a restricted diet has been found to reduce the accumulation of harmful fatty acids and lessen brain damage. The first treatment deals with the replacement of the faulty adrenal function often present in X-ALD. The adrenal glands are next to the kidney, and produce certain important hormones.

Prognosis for patients with ALD is generally poor due to progressive neurological deterioration. Death usually occurs within 1 to 10 years after the onset of symptoms.