Amyotrophic lateral sclerosis (ALS), discovered by French neurologist Jean-Martin Charcot in 1869, is a progressive disease affecting nerve cells, or motor neurons, in the brain and spinal cord. “Amyotrophic” has its origins in Greek: “a” means “no” while “myo” refers to muscle. “Trophic" is a term that describes feeding or nourishment. So, “amyotrophic” simply means “no muscle nourishment.” “Lateral” identifies the areas in the spinal cord where the motor neurons that control the muscles are located. As the disease causes this area to deteriorate, it leads to scarring or hardening (“sclerosis”) eventually resulting in nerve cell death, and the brain loses the ability to control muscle movement.1
ALS became widely known around the world when it ended the career of New York Yankees’ star Lou Gehrig, one of Major League Baseball’s most famous players. Gehrig was diagnosed with ALS in 1939 at the Mayo Clinic during a visit prompted by bothersome symptoms he had noticed on the field. Shortly afterward, Gehrig retired from baseball, giving a heartfelt farewell speech to 62,000 fans at Yankee stadium on July 4, 1939. Since then, ALS is often called Lou Gehrig’s disease.2
What are the symptoms of ALS?
The symptoms of ALS vary from person to person and may be noticed in different parts of the body, depending on the type of onset, or beginning, of the disease. Limb onset ALS starts in the arms or legs affecting things like buttoning a shirt, writing or walking. With bulbar onset, the symptoms affect speech and swallowing. But no matter where they start, ALS symptoms will spread to other parts of the body.3
ALS generally begins with muscle weakness that spreads and gets worse over time. Symptoms may include:4
- Trouble walking or doing simple, daily activities
- Tripping and falling
- Weakness in legs, feet or ankles
- Hand weakness or clumsiness
- Slurred speech or trouble swallowing
- Muscle cramps and twitching in arms, shoulders and tongue
- Changes in thinking or behavior
There’s generally no pain in the early or late stages of ALS, and it doesn’t usually affect bladder control or the ability to touch, taste, smell or hear.5 But when the breathing muscles are affected, the person with ALS will eventually need to be on a ventilator permanently in order to breathe.6
What are the causes of ALS?
The Centers for Disease Control and Prevention (CDC) estimates that 12,000 to 15,000 people in the United States have ALS, and approximately 5,000 people are diagnosed each year.7 But what causes ALS? The short answer is that researchers don’t know for sure.8
Studies continue, however, and most theories involve a complex combination of genetic and environmental risk factors that include:9
- Genetics
In 10% of those with ALS, a risk gene was passed down from a family member.
- Age
ALS is most common in people between the ages of 60 and 85.
- Gender
Before age 65, more men than women develop ALS; that difference disappears after age 70.
- Smoking
Women who smoke have the highest risk, especially after menopause.
- Exposure to toxins
Lead in the home or workplace may be linked to the development of ALS.
- Military service
Exposure to certain chemicals, trauma, viruses or intense exertion may be to blame.
What complications are associated with ALS?
As ALS progresses, it may cause a variety of complications like these:10
- Breathing problems
ALS weakens the muscles needed to breathe, and the most common cause of death for people with the disease is breathing failure.
- Difficulty speaking
People with ALS may lose the ability to verbally communicate due to weakness of the muscles that produce speech.
- Eating problems
Weakness of the muscles involved with swallowing may make eating difficult. Foods, liquids or saliva may also get into the lungs, causing pneumonia.
- Dementia
Some people with ALS may have trouble with language or decision-making and eventually be diagnosed with fronto-temporal dementia.
How is ALS diagnosed?
The healthcare provider will usually perform a physical exam, consider a person’s medical history and order a variety of tests to confirm an ALS diagnosis. These include:11
- Blood and urine tests
- Neurological examination to test reflexes
- Electromyogram (EMG) to measure electrical activity of nerves and muscles
- Nerve conduction study to test signaling ability
- Magnetic Resonance Imaging (MRI) to scan for brain or spine damage
How is ALS treated?
Just as there is currently no cure for ALS, there’s no treatment that can reverse the damage to motor neurons. But these medications that are approved by the Food and Drug Administration (FDA), within an individualized treatment plan, may make ALS easier to live with:12
- Riluzole, given orally, is believed to reduce damage to motor neurons and aid nerve function
- Edevarone is administered intravenously and has helped maintain the daily functioning of people with ALS
Other medications can also be prescribed to help people with ALS manage symptoms that may include muscle cramps, stiffness, excess saliva or phlegm, crying/laughing episodes, depression or sleep disruption.13
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Sources:
1“What is ALS?” ALS Association, last accessed May 8, 2023,
2“Lou Gehrig and the History of ALS,” ALS Association, last accessed May 8, 2023,
3“Amyotrophic Lateral Sclerosis (ALS),” Cleveland Clinic, last accessed May 8, 2023,
4“Amyotrophic Lateral Sclerosis (ALS),” Mayo Clinic, last accessed May 8, 2023,
5“Amyotrophic Lateral Sclerosis (ALS),” Mayo Clinic.
6“ALS Symptoms and Diagnosis,” ALS Association, last accessed May 8, 2023,
7“National Amyotrophic Lateral Sclerosis Registry – FAQ,” Centers for Disease Control and Prevention, last accessed May 8, 2023,
8“Amyotrophic Lateral Sclerosis (ALS),” Cleveland Clinic.
9“Amyotrophic Lateral Sclerosis (ALS),” Mayo Clinic.
10“Amyotrophic Lateral Sclerosis (ALS),” Mayo Clinic.
11“Amyotrophic Lateral Sclerosis (ALS),” Cleveland Clinic
12“Amyotrophic Lateral Sclerosis (ALS),” National Institute of Neurological Disorders and Stroke, last accessed May 8, 2023,
13“Amyotrophic Lateral Sclerosis (ALS),” National Institute of Neurological Disorders and Stroke.
This information is provided for educational purposes only. It is not to be used for medical advice, diagnosis or treatment. Consult your healthcare provider if you have questions or concerns.
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