What causes the disease adrenoleukodystrophy?”
Adrenoleukodystrophy (ALD) (also known as “Addison-Schilder Disease,” “Siemerling-Creutzfeldt Disease,” and “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 one disease in a group of inherited disorders called leukodystrophies. Adrenoleukodystrophy progressively damages the myelin, a complex fatty neural tissue that insulates many nerves of the central and peripheral nervous systems, eventually destroying it. Without myelin, nerves are unable to conduct an impulse, leading to increasing disability as myelin destruction increases and intensifies. An essential protein, called a transporter protein, is missing in sufferers. This protein is needed to carry an enzyme which is used to break down very long-chain fatty acids found in the normal diet. Lack of this protein can give rise to a build-up of very long-chain fatty acids, (VLCFA) in the body which can damage the brain and the adren
Question Demyelinating disease: What causes it? What is the cause of demyelinating disease? Answer from Jerry W. Swanson, M.D. A demyelinating disease is any condition that results in damage to the protective covering (myelin sheath) that surrounds nerves in your brain and spinal cord. When the myelin is damaged, nerve impulses slow or even stop, causing neurological problems. Multiple sclerosis (MS) is the most common demyelinating disease. In this disorder, your immune system mistakenly attacks the myelin sheath or the cells that produce and maintain the myelin sheath. This causes inflammation and injury to the sheath and ultimately to the nerves that it surrounds. The result may be multiple areas of scarring (sclerosis), which can eventually slow or block nerve signals that control muscle coordination, strength, sensation and vision. Other types of demyelinating disease include: * Optic neuritis, a condition due to inflammation of the optic nerve in one or both eyes, which can lead
