What is ALS disease?

ALS

ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. 

Amyotrophic translates from Greek to “no muscle nourishment”. When muscles lack nourishment, in an ALS patient this is happens because of the progressive degeneration of motor neurons from their brain and spinal cord, the muscles atrophy or waste away. The term lateral is in reference to portions of the spinal cord where nerve cells that signal and control muscles are located. Sclerosis is scarring or hardening that is the result of this area’s degeneration.

ALS is also known as Lou Gehrig’s Disease and motor neurone disease.

Familial Compared to Sporadic ALS

There are two different types of ALS, one of which is genetic and one that can seemingly affect anyone. The genetic form of ALS, Familial ALS (FALS) is much less common than Sporadic ALS. FALS accounts for about 5-10% of all ALS cases in the US with Sporadic making up the rest.  FALS is inherited and in families that carry this gene, there is a 50% chance that their offspring will inherit the gene mutation and become afflicted with the disease. 

ALS Facts

People are usually diagnosed with ALS between the ages of 40 and 70 but people can develop it in their twenties or thirties. ALS is 20 percent more common in men than it is in women but in older people, the incidence of the disease becomes more equal between men and women. In the United States, approximately 5,000 people are diagnosed with ALS each year and as many as 16,000 people are living with the disease at any given time.

There have been many research studies and many more are being done to investigate possible risk factors associated with ALS. It is not yet known whether or not genetic and/or environmental factors contribute to the development of ALS. One documented fact is that military veterans are approximately twice as likely to develop ALS as civilians are.

About half of the people who are diagnosed with ALS live for three years or more after their diagnosis; twenty percent live five years or more; and up to 10 percent will live more than 10 years. While this is not typical, the well-known physicist, cosmologist and author, Stephen Hawking, lived with ALS for 55 years from the time he was diagnosed at 21. 

People with ALS often experience muscle stiffness, muscle twitching, and gradually worsening weakness due the muscles throughout their body are decreasing in size. Most people with ALS eventually lose the ability to walk, use their hands, speak, swallow and breathe. 

There is no known cure for ALS and up until 1995 there were no medications to combat any of the side effects or to prolong an ALS patient’s life. Until a cure is found, the goal of treatment is to improve symptoms and to slow the degenerative process down.

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