#1010 What is Lou Gehrig’s disease?

What is Lou Gehrig’s disease?

What is Lou Gehrig’s disease? It is a disease that causes progressive degeneration of the nerve cells in the spine and brain.

The disease is called Lou Gehrig’s disease after a famous baseball player who died of it, but the disease’s actual name is amyotrophic lateral sclerosis (ALS). “Amyotrophic” comes from Greek. “A” means “no”. “Myo” means “muscle.” “Trophic” means “nourishment”. So, it means muscle malnourishment. “Lateral” comes from Latin and means “to the sides”. This refers to the parts of the spine that are damaged by the disease. “Sclerosis” comes from Greek and it means “harden”. It refers to the nerves that are hardened or scarred as the disease progresses. ALS was first discovered in 1869 by a French neurologist called Jean-Martin Charcot.

Lou Gehrig was a professional baseball player who was born in 1903 and played for the New York Yankees between 1923 and 1939. He played there at the same time as Babe Ruth. Gehrig was a hitter and he is classed as one of the greatest baseball players of all time. He’s ranked number 6 on a list of the 100 best baseball players of all time that I found on ESPN. However, unfortunately, it is for the ALS disease than for his baseball prowess that he is primarily remembered. Gehrig began experiencing symptoms during the 1938 season and in the 1939 season he had lost most of his coordination, speed, and endurance. Nobody knew what had caused his sudden loss of ability, but people suspected illness. He was diagnosed with ALS on June 19, 1939. His condition gradually worsened and he died on June 2, 1941, less than two years after he had been diagnosed, although he had probably had the disease for a while before he was diagnosed. His illness was very public and a lot more people came to know about the disease because of him. After his death, ALS became known as Lou Gehrig’s disease, at least in the United States. With the spread of American culture around the world, the name spread.

So, what is ALS? It is a disease where the motor neurons in the spine and the brain slowly degenerate until body function is no longer possible. At this point, the patient needs to be kept alive with a ventilator. Motor neurons are the nerves that our brain uses to send signals to make our muscles move. If you want to lift up a cup of coffee, it is your motor neurons sending the command, and if you want to walk, it is the motor neurons sending the commands to your leg muscles. Every voluntary movement you make happens because of the motor neurons. Motor neurons start in the brain and extend down the spinal cord before spreading out throughout the body. You can see how vital they are because if someone has a severe spinal cord injury, it can prevent them from using the muscles that are below that point of injury.

Scientists know what ALS does to the nerves and to the body, but they don’t know why it does it.  ALS usually first appears as a weakness in the muscles and then becomes a loss of control. People might drop things or fall over. Trouble speaking and walking follow. All of this happens because some of the neurons die and the signal can’t be sent as normal because they get interrupted where there are gaps. The signals are sent as an electrical signal that goes from neuron to neuron and is unable to jump across gaps. The consequence of this is that the muscles begin to atrophy, reaching the point where they can’t move at all. There are two patterns. In some cases, the lower neurons die first, which causes more atrophy. In other cases, the upper neurons die first, which causes more muscle stiffness and overactive reflexes.

There is no cure for ALS. Once the neurons have died they cannot be brought back. There are some drugs that can prolong the process or reduce the symptoms, but ALS is nearly always fatal. 50% of people who are diagnosed with it live for between three and five years after their diagnosis. 25% of people live between five and ten years. 10% live a little longer than ten years. And some people live a lot longer. Stephen Hawking was diagnosed with ALS when he was 21 and he died at the age of 76. Because scientists don’t know why the nerves start dying, they don’t know why some people live a long time and others don’t. That also means they don’t know how to cure it. In the end, ALS leads to complete paralysis and people cannot eat, swallow, or breathe. They have to be ventilated, and they usually die of a complication from that. Hopefully, a cure will be found. And this is what I learned today.

Image By Pacific & Atlantic Photos, Inc – Heritage Auctions, Public Domain, https://commons.wikimedia.org/w/index.php?curid=41470554

Sources

https://www.hopkinsmedicine.org/health/conditions-and-diseases/amyotrophic-lateral-sclerosis-als

https://en.wikipedia.org/wiki/ALS

https://en.wikipedia.org/wiki/Lou_Gehrig

https://www.als.org/understanding-als/what-is-als

https://www.espn.com/mlb/story/_/id/33158613/top-100-mlb-players-all-nos-25-1

https://www.nbcnews.com/health/health-care/stephen-hawking-had-als-55-years-how-did-he-do-n857006

https://www.hss.edu/condition-list_amyotrophic-lateral-sclerosis.asp

https://read.houstonmethodist.org/do-motor-neurons-first-die-in-the-brain

https://www.ninds.nih.gov/health-information/disorders/motor-neuron-diseases

https://my.clevelandclinic.org/health/diseases/16729-amyotrophic-lateral-sclerosis-als

https://www.hss.edu/condition-list_amyotrophic-lateral-sclerosis.asp