Adrenoleukodystrophy

Adrenoleukodystrophy (ALD) is a degenerative disorder of the sheath covering nerve fibers, known as myelin. A type of leukodystrophy, the victims of ALD are typically male, 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 other reasons.

Symptoms

The clinical presentations is largely dependant on the age of onset of the disease. The most frequent type is the childhood-onset one, 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.

Related Topics:
Seizure - Ataxia - Adrenal insufficiency

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In the adolescent-onset form, the spinal cord dysfunction is more prominent and therefore is called adrenomyeloneuropathy or "AMD". 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 female carriers rarely exhibit symptoms similar to AMD.

Related Topics:
Spinal cord - Urination - Defecation

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Adult and neonatal (which tend to affect both males and females and be inherited in an autosomal recessive manner) forms of the disease also exist, they are extremely rare. Some patients may present with sole findings of adrenal insufficiency (Addison's disease).

Related Topics:
Autosomal recessive - Adrenal insufficiency

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