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Adrenoleukodystrophy (ALD) is a degenerative disorder of the fatty white sheath covering nerve fibers, known as myelin. A type of leukodystrophy, the victims of ALD are typically male, usually ages 5 to 10, as the disease is usually inherited in a sex-linked manner on the X chromosome. Leukodystrophies are disorders that affect the growth and/or development of myelin, a complex fatty neural tissue that insulates many nerves of the central and peripheral nervous systems. Without myelin, nerves are unable to conduct an impulse, leading to increasing disability as myelin destruction increases and intensifies. Leukodystrophies are different from demyelinating disorders such as multiple sclerosis, in which myelin is formed normally, but is lost by immunologic dysfunction or for other reasons.

Symptoms


The clinical presentation is largely dependent on the age of onset of the disease. The most severe type is the childhood cerebral form, which normally occurs in males between the ages of 5 and 10 and is characterized by failure to develop, seizures, ataxia, adrenal insufficiency and degeneration of visual and auditory function. This form can also occur in adolescents and, very rarely, adults.

In another form of ALD, which primarily strikes young men, the spinal cord dysfunction is more prominent and therefore is called adrenomyeloneuropathy or "AMN." The patients usually present with weakness and numbness of the limbs and urination or defecation problems. Most victims of this form are also males, although some female carriers exhibit symptoms similar to AMN.

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Hematopoietic stem cell gene therapy with a lentiviral vector in X-linked adrenoleukodystrophy. http://www.ncbi.nlm.nih.gov/pubmed/19892975
neuropot (neuropot (beta)) Sat, 19 Dec 2009 20:58:36 -0000
Hematopoietic stem cell gene therapy with a lentiviral vector in X-linked adrenoleukodystrophy. http://www.ncbi.nlm.nih.gov/pubmed/19892975
Hematopoietic stem cell gene therapy with a lentiviral vector in X-linked adrenoleukodystrophy. http://www.ncbi.nlm.nih.gov/pubmed/19892975
neuropot (neuropot (beta)) Sat, 19 Dec 2009 03:58:40 -0000
Hematopoietic stem cell gene therapy with a lentiviral vector in X-linked adrenoleukodystrophy. http://www.ncbi.nlm.nih.gov/pubmed/19892975
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Adrenoleukodystrophy (ALD) Foundation - A non-profit organization created to educate about ALD by providing educational materials and links to information associated with the disease.
Meta Description: [ The Adrenoleukodystrophy Foundation, a non-profit organization created to educate about ALD, AMN and Addison's Disease. Our motto: Care Today for a Cure Tomorrow. ]

Human and Scientific Story of Adrenoleukodystrophy - Description, discussion of treatments, and links.

MCW HealthLink - Adrenoleukodystrophy - Information sheet from the Medical College of Wisconsion about Adrenoleukodystrophy.
Meta Description: [ Adrenoleukodystrophy (ALD) is a rare, genetic disorder. It causes breakdown or loss of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal glands. ]

The Amn-Ald Community Pages - Support site for families dealing with the diseases AMN/ALD (one form of the many Leukodystrophies).

404 Virtual Hospital: Adrenal Leukodystrophy (ALD) - Radiology resident case with references and images.
Meta Description: [ Virtual Hospital was a digital library of health information in pediatrics, paediatrics, and radiology for pediatric education and radiology education ]

X-linked Adrenoleukodystrophy Mutations - This database has been initiated to collect data on mutations found in the gene (ABCD1) responsible for X-linked adrenoleukodystrophy.
Meta Description: [ This database has been initiated to collect data on mutations found in the gene (ABCD1) responsible for X-linked adrenoleukodystrophy and provide information on X-ALD. ]

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