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What is ALS?

Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's disease," is a progressive neuro-degenerative disease that attacks nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually lead to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With all voluntary muscle action affected, patients in the later stages of the disease become totally paralyzed. Yet, through it all, for the vast majority of people, their minds remain unaffected.

A-myo-trophic 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.

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing and breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (waste away). Limbs begin to look "thinner" as muscle tissue atrophies.

In 1939, New York Yankees superstar Lou Gehrig was diagnosed with Amyotrophic Lateral Sclerosis. Two years later, he died at age 38. Gehrig's unusually high profile gave the relatively unknown disease a nickname that has persisted since then, "Lou Gehrig's Disease."

Over 300,000 Americans alive and well today will die from ALS (8,000 in Massachusetts). The disease usually strikes those aged 40 - 70, although younger people develop the disease too. 30,000 Americans have the disease currently. One half of those with the disease die in three years. The life expectancy of an ALS patient averages two to five years after diagnosis. Every year 5,000 people are newly diagnosed with the disease. More people die each year from ALS than Multiple Sclerosis and Huntington's disease. ALS is not rare. Ninety percent of the cases strike people with no family history of the disease.

ALS can strike anyone. United States Senator Jacob Javits, actor David Niven, jazz great Charlie Mingus, Vice President Henry Wallace, baseball hall-of-famer Catfish Hunter, and actor Michael Zaslow all succumbed to ALS.

The ALS Association Massachusetts Chapter is the only not-for-profit voluntary health organization in the Commonwealth dedicated solely to the fight against ALS through patient services, awareness, education dissemination and research.

For more information, visit The ALS Association Massachusetts Chapter or the National ALS Association.


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