Adrenoleukodystrophy

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Adrenoleukodystrophy (ALD, also called Siemerling-Creutzfeldt Disease or Addison-Schilder's disease:545) is a rare, inherited disorder that leads to progressive brain damage, failure of the adrenal glands and eventually death.

ALD is a disease in a group of genetic disorders called leukodystrophies, whose chief feature is damage to myelin.

In adrenoleukodystrophy, over-accumulation of VLCFAs leads to damage to the brain, adrenal gland, and peripheral nervous system on the age of onset of the disease.

The classical, severe type is the childhood cerebral form which, as an X-linked disease, affects primarily males.

Symptoms normally start between the ages of 4 and 10 and include loss of previously acquired neurologic abilities, seizures, ataxia, Addison's disease, and degeneration of visual and auditory function.