Amyotrophic Lateral Sclerosis (ALS), knows as Lou Gehrig's Disease in the U.S.A., is a degenerative neurological disease which causes the neurons responsible for movement and motor control to die, leading to profound paralysis and death from respiratory failure. The disease begins with muscle weakness and twitches, but progresses, over many years, to the loss of all voluntary muscle control and, eventually, to the loss of involuntary muscle control.
Perhaps the most devestating feature of ALS is that while the patient loses complete control over their body, their cognitive function remains intact. The victim of ALS is completely aware of their imprisonment in a body that no longer responds to their commands.
ALS is one of the most common neuromuscular diseases in the world. 1 to 2 people per 100,000 will develop ALS each year. The disease usually manfiests between the ages of 40-60, but younger and older people can also fall victim.
No one has discovered a definitive cause of ALS, but the disease has been linked to neurotoxins, spinal surgery, pesticides used on sports fields, heavy metals and DNA defects. There is no cure for ALS and direct treatment options are limited. Riluzole is the only drug on the market designed to treat ALS. Studies have shown that it can delay death by several months. Lithium has also shown some promise in delaying death. Most of the treatments for ALS involve making life easier for the patient as they lose more and more of their muscle control.
Most ALS patients die within 3-5 years from the onset of symptoms. However some can live for 10 years or longer with the disease.
Due to the fact that this sick is uncomfortably common, has no cure and limited treatments; that it causes devestation to the body while leaving the mind conscious of that devestation; and because the disease is 100% fatal over a moderate period of time, I rate it a:
5 on my "lethality scale" (1-10)
and a 7 on my "disturbing scale" (1-10)
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