What Is ALS?

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. It is also called Lou Gehrig’s disease or motor neuron disease. ALS means that over time neurons stop working and die back. In ALS, the neurons in the brain, spinal cord, and those outside of the spinal cord that go to muscles, waste away.

The disease causes muscle weakness that gets worse over time. It can affect the arms, hands, legs, or feet as well as the muscles used to speak, swallow, and breathe. People with ALS have difficulty with things like walking, standing, reaching for the phone, or using a fork to eat because the motor nerves that allow movement are affected.

ALS is a terminal disease because there are no medicines or treatments that can completely stop or reverse the dying back of the nerves going to the muscles. A person with ALS will slowly get weaker and eventually die.

The progression and symptoms of ALS are different for everyone. Some people have long gaps of no progression. Some progress very slowly while others have faster progression. No matter how your disease progresses, it is very important to get your care from an ALS interdisciplinary team. This will not only help prolong your life but improve your quality of life as well.

Symptoms

In the beginning, you may not notice the slight changes that occur. As time goes on the symptoms become more noticeable. 

The first symptoms of ALS can vary between people. Symptoms that begin in the arms or legs are referred to as limb onset ALS. They include:

• Muscle twitching (fasciculations) or muscle cramping

• Weakness in the arms, legs, hands, or feet

• Clumsiness or falling

Symptoms that begin with speech or swallowing difficulty are known as bulbar onset ALS. They include:

• Choking when eating

• Progressive shortness of breath

• Slurred speech

ALS affects the motor nerves but does not cause any change to sensation, hearing, smell, vision, or taste.

Risk factors

The precise cause of ALS is not clear and very few risk factors for developing the disease have been identified. 

• It is estimated that about 30,000 people in the U.S. are living with ALS.

• About 5,000-6,000 people are diagnosed each year.

• Most patients with ALS are white, and more than half are men.

• The average age at diagnosis is age 55.

• Most patients are between ages 40 and 70 when diagnosed. However, ALS can occur at any age.

For unknown reasons, military service is a risk factor for future diagnosis. Several studies have documented an increased risk for veterans to be diagnosed with ALS. It is now accepted that a U.S. veteran who served between 1910 and 1982 is about 1.5 times more likely to be diagnosed with ALS compared with someone who was never in the service. This risk does not depend on how, where, or when you served. 

ALS is not contagious.

Forms of ALS

Most people with ALS have sporadic disease. This means they did not inherit the disease from their parents and are unlikely to pass it on to their children. A small number of patients with ALS have familial ALS. This is an inherited form of ALS that is passed from parent to child. Some of these mutations have been identified and researchers are working on developing treatments with this information. There is now 1 medicine available for people with a specific kind of genetic mutation (SOD-1) causing ALS. For this reason, if you have a family history of ALS and develop the disease, ask your ALS specialist about genetic testing.

Diagnosis

ALS can be difficult to diagnose because there is not one specific test to confirm that a person has the disease. Doctors use a physical exam to look for features that are characteristic of the disease. The work-up may also include these tests to rule out other health problems:

• Nerve conduction tests (NCV) and Electromyography (EMG)

• MRI

• Blood tests

Because ALS can be so difficult to diagnose, it can take an average of 1-2 years to reach a diagnosis of ALS. Therefore, it is important to see a neuromuscular specialist. This type of doctor specializes in diseases of the peripheral nervous system and muscles.

Treatment of ALS

The treatment of ALS is divided into two categories:

• Symptomatic treatments. These try to help any problems you may develop from the disease. This would include drooling, pain, cramping, difficulty sleeping, depression, increased crying or laughing (pseudobulbar affect), difficulty walking, swallowing, and breathing. You will be given durable medical equipment to help you keep as much independence as possible.

• Disease modifying therapy. A number of medicines are now available to slow progression of ALS. Some of these medicines are approved for people with inherited forms of ALS with a specific genetic mutation. Others are most effective when given to people with early stages of the disease. It’s important to discuss the risks and benefits of medicine therapy with an ALS specialist to decide what treatments would be best to meet your goals and priorities.

Veterans will also be provided grants to help with the following:

• Adaptive wheelchair vans

• Daily home health assistance

• Skilled nursing care when appropriate

• Modifying their homes to improve accessibility

• Monthly financial compensation to help provide additional care

Living with ALS

ALS is a life-changing diagnosis. Getting the right care requires medical experts from different fields working together. This is often called a multidisciplinary or interdisciplinary team. People with ALS who get care from ALS centers that have a specialized team have a higher quality of life.

The life expectancy after diagnosis is 3 to 5 years. Half of people will live longer than 3 years after being diagnosed. However, some people with ALS will live longer. About 10% of patients will live for 10 or more years after their diagnosis.