Amyotrophic Lateral Sclerosis

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Amyotrophic Lateral Sclerosis

Motor Neuron Disease Maladie de Charcot Lou Gehrig's Disease

What is the Disease?

ALS is an extremely deadly disease affecting the nerve cells that control the victim's voluntary muscles. These nerves shrink and eventually die, leaving the muscles without stimulation. As these muscles go without stimulation, they too eventually shrink and die. The victim progressively weakens to the point of complete paralysis of all voluntary muscles and some involuntary muscles, such as breathing and swallowing, and soon after this point, death is inevitable.

'A' means "Without" 'Myo' means "Muscle" 'Trophic' means "Nourishment" 'Lateral' refers to uneven development of symptoms between right and left sides
'Sclerosis' refers to "destruction" of tissue

The History of ALS

A French doctor named Charcot first identified ALS in 1874. It is one of the most devastating diagnoses a person can receive. ALS is said to start between the years of 40 and 70, with the exact average being 45.6 years old.
The most classic case of Amyotrophic Lateral Sclerosis is Lou Gehrig. Lou
Gehrig was a New York Yankees first baseman, who from 1923 to 1939, had never missed a game and had a life time batting average of .340. However, the symptoms of ALS emerged in 1938, and in 1939, he was diagnosed with the disease.
At that time doctors knew little to nothing about the disease and the only suggested treatment was the untested vitamin E. So Gehrig ate a daily plate full of garden grass, until June 2, 1941 when he died at the age of 37.
ALS affects approximately 1 out of every 100,000 people. In the United
States there are around 30,000 Americans affected by ALS, and 3,000 more are diagnosed with the disease each year, with men being affected slightly more than women, and in some cases, running in families. However while this is the same number of new cases as Multiple Sclerosis, Multiple Sclerosis affects around
350,000 Americans. The difference is that 50% of ALS patient's die within three years, and 80% die within five. The disease is in some ways quite similar to
Alzheimer's except with Alzheimer's you have a body walking around with a diseased brain, whereas with ALS you have a healthy brain trapped inside a diseased body.

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... members of the European Union. Gabapentin is also similar to Riluzole and is being tested for approval by the FDA. More importantly, a drug known as Myotrophin is being tested as well by the FDA and may be the first drug to slow the progress of paralysis. Because Myotrophin acts differently than Riluzole, they, hopefully, can be used in synch as well.

Rescources

Science News, Vol. 145, page 202 The Sacramento Bee, March 2, 1994, A8 The
Sacramento Bee, June 9, 1996 The Wall Street Journal, June 13, 1995, B7 Applied
Medical Informatics (AMI), 1994 Muscular Dystrophy Association (MDA), January 31,
1996 The New York Times, May 9, 1995 The New York Times, June 13, 1995 Gene
Therapy, March 1995 Mayo Clinic Health Letter, April 1996, page 5 Rhone-Poulenc
Rorer, August 1995 The ALS Association and the Neuromuscular Research Foundation

Internet Sites:

http://www.caregiver.org/fs/fs_als.html http://www.medicinenet.com/ http://www.phoenix.net/~jacobson/guide2.html
http://www.familyvillage.wisc.edu/lib_als.htm

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