Definition of Adrenoleukodystrophy

Reviewed on 3/29/2021

Adrenoleukodystrophy: A rare genetic (inherited) disorder characterized by the breakdown or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland. Adrenoleukodystrophy (ALD) is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath of the nerve fibers in the brain. The myelin sheath is a fatty covering which acts as an electrical insulator.

There are several forms of ALD:

The treatment for all forms of ALD is symptomatic and supportive. Physical therapy, psychological support, and special education may be useful for some individuals.

The 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.

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