Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS), sometimes referred to as "Lou Gehrig's Disease," is a progressive fatal neuromuscular disease that attacks nerve cells and pathways in the brain and spinal cord. Motor neurons, among the largest of all nerve cells, reach from the brain to the spinal cord and from the spinal cord to muscles throughout the body with connections to the brain. When they die, the ability of the brain to start and control muscle movement dies with them. With all voluntary muscle action affected, ALS patients in the later stages are totally paralyzed; through it all, however, their minds remain unaffected.
Amyotrophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment--"No muscle nourishment." When a muscle has no nourishment, it "Atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates it leads to scarring or hardening ("Sclerosis") in the region.
Over 5,000 Americans are diagnosed with ALS each year. There is great variation in the course of the disease. Symptoms usually appear in individuals between the ages of 40-70, though the disease has been reported in both younger and older persons. Survival after the confirming diagnosis is, on average, two to five years. Progression of ALS varies with each individual; therefore, some will live longer--up to 10 years, and about five percent will exceed 12 years. In some cases, the disease seems to plateau. Many patients are able to live productive and satisfying lives especially with the use of assistive devices for daily living, and later in the disease, augmentative communication equipment.
Men and women are affected in almost equal numbers. Also, up to 10 percent of ALS cases are familial occurring more than once in a family lineage; but 90 percent of ALS cases show no hereditary pattern.
Approximately one-third of patients become aware of the onset of the disease when their hands become clumsy, causing difficulty in the performance of fine tasks. Another one-third experience
weakness in the legs and may trip because of mild foot drop. The
remaining one-third notice slowing of speech or difficulty in
swallowing. ALS may be present for some time before any...

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