Amyotrophic Lateral Sclerosis

What is amyotrophic lateral sclerosis (ALS)?

Amyotrophic lateral sclerosis (ALS), often called Lou Gehrig's disease, is a disease of the nervous system. It attacks nerve cells called motor neurons in the brain and spinal cord. These neurons send messages from your brain and spinal cord to your voluntary muscles. These are the muscles you can control, such as those in your arms and legs.

ALS is a progressive disease, meaning it gets worse over time. As the motor neurons die, they stop sending messages to the muscles. As a result, the muscles become weak, twitch, and waste away.

Who is more likely to develop amyotrophic lateral sclerosis (ALS)?

ALS can happen at any age, but its symptoms most often begin between ages 55 and 75. There are certain factors that may raise your risk of developing ALS:

• Being male
• Being White and not Hispanic
• Having a family member with ALS

What causes amyotrophic lateral sclerosis (ALS)?

ALS can be sporadic or familial:

• Most of the time, ALS is sporadic, meaning that there is no family history of the disease. Researchers don't know what causes this type, but it may be a combination of genetics and environmental factors.
• Familial ALS is much less common. It is inherited (passed down through families).

What are the symptoms of amyotrophic lateral sclerosis (ALS)?

The early symptoms of ALS are often mild. They may begin in one arm or leg or in the muscles used for speaking and swallowing. You may notice:

• Muscle twitches or cramps
• Stiff or weak muscles in the neck, arm, or leg
• Trouble walking, running, or writing
• Slurred speech
• Trouble chewing or swallowing

Over time, weakness spreads to other parts of the body. You gradually lose the ability to move, speak, eat, and breathe on your own. Most people with ALS can still reason, remember, and understand, but some have changes in language, thinking, or behavior. Some may develop a form of dementia known as frontotemporal dementia (FTD-ALS).

How is amyotrophic lateral sclerosis (ALS) diagnosed?

There is no single test for ALS. Since the symptoms are like those of other diseases, ALS can be hard to diagnose, especially in its early stages.

Your health care provider will do a physical exam and a neurological exam and ask about your health history. You may also have tests to rule out other conditions, such as:

• An electromyography (EMG) and nerve conduction studies (NCS) to check how your nerves and muscles are working
• An MRI scan of your brain and spinal cord
• A spinal tap (lumbar puncture)
• Blood and urine tests

How is amyotrophic lateral sclerosis (ALS) treated?

There's no treatment to reverse the damage from ALS. But medicines may slow the loss of function, help relieve symptoms, and sometimes help you live longer.

A team of providers can help you stay active and independent for as long as possible, with treatments such as:

• Physical, occupational, and speech therapy
• Devices to help you communicate
• A feeding tube, if you can't eat safely
• Breathing support, such as a mask used during sleep, and later, a breathing machine

Most people with ALS die from respiratory failure, often within three to five years after symptoms begin. But some people live 10 years or longer.

Support groups and mental health counseling may help you and your family cope with the illness and its impact on your life.

Can amyotrophic lateral sclerosis (ALS) be prevented?

There is no known way to prevent ALS. If ALS runs in your family, talk with your provider about whether genetic counseling and testing may be right for you.

NIH: National Institute of Neurological Disorders and Stroke