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What Is ALS?
Amyotrophic Lateral Sclerosis (ALS), sometimes called Lou Gehrig's disease, is a degenerative neuromuscular disease affecting motor nerve cells in the brain and spinal cord. Typically, the first visible signs of ALS are weakness in the hands or legs, and slurring of speech. Eventually, the disease leads to paralysis of the "voluntary" muscles with loss of speech, swallowing and breathing capabilities. ALS can progress rapidly or plateau for unpredictable periods of time depending upon the location and extent of damage to the nervous system. Throughout the course of the disease, the mind remains alert. Generally not affected are vision, sensation, hearing, taste, smell, and the muscles of the eyes, bowel and bladder.
What Causes ALS?
Although ALS was first described in detail in 1869, the disease's cause, cure and means of prevention are still unknown. However, a number of theories have been suggested. Some consider ALS to be an autoimmune disease, the result of biochemical or metabolic abnormality, or a slow acting virus. Others are studying environmental toxins or genetic components. Ongoing research is directed toward finding a cure.
Who Is Affected?
Once considered a rare disease, ALS is estimated to affect more than 30,000 Americans at any given time. At least one in 20,000 upstate New York residents has ALS. Men are affected more often than women, usually between ages 35 and 70. The average survival from diagnosis is from two to five years. Twenty-five percent of people with ALS live five years or more, and up to 10 percent will survive more than ten years.
ALS affects people of all racial, ethnic and socioeconomic backgrounds. Some of the more well-known people whom ALS has affected are: baseball's Lou Gehrig, NY Yankee's Jim "Catfish" Hunter, famous USPGA caddy Bruce Edwards, actors David Niven and Michael Zaslow, renowned physicist Stephen Hawking and former US Senator Jacob Javits.
Is It Hereditary?
The most common form of ALS in the United States is known as "sporadic." It may affect anyone, anywhere. "Familial" ALS suggests that the disease is inherited. Approximately five percent of persons with ALS have a familial or hereditary form. In those families, 50 percent of the children are expected to have the disease. In 1993, researchers found a defective gene which appears to be the cause of half the cases of familial ALS.