How many people have Adrenoleukodystrophy?

Answer 1

Adrenoleukodystrophy is an X-linked peroxisomal disease that is characterized by progressive demyelination beginning around 6 years of age in the classical form. A more aggressive, infantile form also exists. A key to the diagnosis is the presence of high blood levels of very long chain fatty acids, which are NOT oxidized normally because of abnormal peroxisomal function. The most salient manifestations of this entity is: hyperactivity, impaired auditory discrimination, impaired spatial orientation, visual disturbances, strabismus, and seizures. There is no treatment for this condition and the prognosis is poor. Death usually occurs early.

Answer 2

Progressive neurological degeneration makes the prognosis generally poor. Death occurs within one to ten years following presentation of symptoms. Treatment is palliative. The use of Lorenzo's Oil or a bone marrow transplant are currently under investigation. However, there are many boys who have been treated with a bone marrow transplant and have survived over twenty years with no further progression of the disease and are still alive today without any further complications of the disease and no further need to supplement diet with Lorenzo's oil.

Answer 3

It is estimated that 1 per 20,000 people suffer from Adrenoleukodystrophy. This disorder is considered rare and shows similar symptoms of Addison disease.