Lou Gehrig's Disease: Amyotrophic Lateral Sclerosis

Lou Gehrig's Disease: Amyotrophic Lateral Sclerosis
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  • 0:08 Proper Signal and Function
  • 0:35 What Is Amyotrophic…
  • 1:21 Why Does Lou Gehrig's…
  • 3:00 Clinical Signs,…
  • 4:00 Treatment of ALS
  • 4:32 Lesson Summary
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Lesson Transcript
Instructor: Artem Cheprasov
In this lesson, we will cover a deadly condition known as amyotrophic lateral sclerosis. We'll learn its alternative name, what occurs in your body as a result of this disease, and if there is any way to treat it.

Proper Signal and Function

If you were to unplug a television from the wall socket, it would shut down and stop working. If you were to stop pumping a hot air balloon with hot air, it would deflate. These two scenarios both showcase the removal of an important signal for something else to work or function properly. If your body were to lose an important signal, then devastating consequences may result as this lesson will showcase with a very famous disease.

What Is Amyotrophic Lateral Sclerosis?

This disease is called amyotrophic lateral sclerosis (ALS), or more colloquially, Lou Gehrig's disease. This is an incurable and deadly condition that destroys nerves that control muscles of voluntary movement. Besides Lou Gehrig, many well-known individuals have been afflicted with this terrible disease, notably physicist Stephen Hawking and perhaps even Mao Zedong. Individuals who get this disease typically do so between the ages of forty and sixty, more men than women are affected, and most will pass away from respiratory failure within five to ten years after they experience the first symptoms of this disease.

Why Does Lou Gehrig's Disease Occur?

No one knows for certain why people suffer from this condition. It seems more random than anything else. Genetic components, increased amounts of a neurotransmitter called glutamate, and autoimmune components have all been implicated in causing ALS. However, we know that the end result amounts to amyotrophy, or the atrophy of muscles as a result of nerve disease. Atrophy refers to the wasting away of muscles associated with this malady.

It occurs because the nerves involved in stimulating your muscles to move, called upper and lower motor neurons, die. If the signal from these nerves stops as a result of their death, your muscles essentially shut down. This goes back to our introductory example where I mentioned the fact that if you pull the plug that supplies the electrical signal to your TV, then the TV will shut down. Here, the plug is pulled by way of destruction of the nerves that send electrical and chemical signals to your muscles.

These signals tell your muscles to move. If your muscles get these signals, the muscles want to stay strong and big in order to move you from point A to point B, run, catch a ball, and hit a home run (all the things Lou Gehrig had to do). However, if the signal from these nerves disappears, your body begins to think your muscles don't need to move around anymore and diverts critical resources important for growth to other parts of your body, resulting in the deflation of your muscles, just like the hot air balloon was deflated when the hot air, its signal to inflate, was removed.

Clinical Signs, Symptoms, and Diagnostics

Symptoms and signs associated with these pathological changes include:

  • Muscle fasciculation, more commonly known as muscle twitches
  • Difficulty walking, moving your arms, speaking, and swallowing
  • And, eventually, respiratory failure, as the muscles involved in helping you breathe begin to fail just like the muscles that help you move fail as well

If you can't breathe, then you obviously are unable to live. This respiratory failure is the eventual cause of death for most people with ALS.

There is unfortunately no definitive test that can tell someone if they have ALS, but a combination of tests can be used to rule out other causes of those signs in order to reach such a diagnosis. Many of these procedures center on looking at your muscles and nerves and testing their capability to conduct and receive electrical signals. Some of these tests are called an electromyogram, nerve conduction velocity test, and a muscle biopsy.

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