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IntroductionThe term 'leukodystrophy' actually refers nonspecifically to a group of inherited, disorders called the leukodystrophies. These can onset at any stage of life and progress with varying rapidity. However the most common course is rapid progression starting in babyhood or toddlerhood. Leukodystrophy causes the brain to gradually lose myelin; the fat that surrounds nerves. This means the nerves first become progressively impaired, and then stop working altogether - a little like electrical cables that are stripped of their rubber insulation. The result of this, without any cure or treatment, is similar to motor neurone disease; progressive loss of movement, speech and senses, resulting in ultimate death. The cause of death is often pneumonia, choking on fluid that is stuck in the lungs, or inability to breathe due to paralysis. Sometimes dementia occurs, but in many cases the person's sounds, body language or facial expressions in response to various stimuli make it plain that they are fully aware of everything happening to them and around them.
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