What is Adrenomyeloneuropathy?
Adrenomyeloneuropathy (AMN) is the adult onset of Adrenoleukodystrophy (ALD): a rare and terminal disease affecting approximately 13,600 people in the United States, according to Health Research Funding. An X-linked chromosome disorder, the disease knows no ethnic discriminators, and while it occasionally presents in females, it appears to be much more prevalent in males. The mortality rate with AMN is high; however, ALD patients usually face certain death.
AMN occurs when the X chromosome mutates or in some way becomes defective. Because of this, the mutant gene prevents the body from being able to create the necessary proteins that would normally bind to the very long chains of fatty acids (VLCFA.) Instead AMN patients’ bodies store these acids, so they’re not broken down into smaller chains. This creates a deterioration of the myelin sheaths around the body’s nerve cells which results in neurological degeneration.
In most adult patients, AMN affects the neurological functions and message delivery systems in the spinal cord, which results in a loss of neuromuscular control and inhibits motor skills. As the disease progresses, patients usually develop moderate to severe walking handicaps and often require mobility assistance, such as canes and wheelchair aids.
Because of the rarity of AMN diagnoses, very little is known about the disease and adult prognoses are often quite ambiguous. There is currently no cure; however, there are a variety of clinical and dietary treatment methods available to patients to help manage pain and disease symptoms.
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