What Is Lou Gehrig Disease?
by Aimee Amodio | More from this Blogger

The disease commonly known as Lou Gehrig disease is officially known as amyotrophic lateral sclerosis or ALS. It is also called Charcot disease or motor neuron disease.

Basically, amyotrophic lateral sclerosis is a progressive disease that damages motor neurons -- the specialized cells in the spinal cord and brain stem that control muscle movement. As the disease progresses, muscle control degenerates.


The initial signs of Lou Gehrig disease are subtle -- minor enough that they can easily be overlooked. Early symptoms may include muscle twitching, muscle cramping, stiff muscles, or weakness. As the disease progresses, a person's speech may become slurred. Eventually, a person with ALS has trouble chewing and swallowing. The muscles become weaker as the disease progresses, taking strength and eventually the ability to walk or use arms and legs. The arms and legs will look thinner as the muscle tissue atrophies. In the late stages of the disease, the muscles of the respiratory system weaken, making it difficult to breathe. Most people with ALS die from respiratory failure.

Most cases of Lou Gehrig disease -- more than ninety percent of cases -- occur in people with no family history of the disease. The cause of onset in these cases is unknown. Only a small percentage of cases develop from a known genetic mutation -- these cases are known as inherited ALS or familial ALS.

There are eight different types of ALS, distinguished by genetic cause, pattern of inheritance, age at onset, and progression of the disease. For example, ALS types 1 and 8 are adult-onset ALS. Type 1 ALS progresses very quickly; most people with type 1 ALS die of respiratory failure within five years of onset. ALS types 2 and 4 are juvenile or early onset ALS. Types 2, 4, and 8 are relatively rare. Approximately twenty percent of familial cases of ALS and three percent of sporadic cases of ALS are type 1.

Around the world, Lou Gehrig disease appears in between four and eight people out of every 100,000. In the United States, approximately five thousand people are diagnosed with ALS each year.

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