Topic Overview

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis, or ALS, is a disease in which certain nerve cells in the brain and spinal cord slowly die. These nerve cells are called motor neurons, and they control the muscles that allow you to move the parts of your body. ALS is also called Lou Gehrig’s disease.

People who have ALS gradually become more disabled. How quickly the disease gets worse is different for everyone. Some people live with ALS for several years. But over time, ALS makes it hard to walk, speak, eat, swallow, and breathe. These problems can lead to injury, illness, and eventually death. In most cases, death will occur within 3 to 5 years after symptoms begin, although some people do live for many years, even decades.

It can be very scary to learn that you have ALS. Talking with your doctor, getting counseling, or joining a support group may help you deal with your feelings. Your family members may also need support or counseling as your disease gets worse.

ALS is rare. Each year in the United States and most of the world, only 1 to 2 people out of 100,000 get ALS. Men get ALS slightly more often than women do. ALS can occur at any age, but it most often starts in middle-aged and older adults.^{footnote 1}

What causes ALS?

Doctors don’t know what causes ALS. In about 1 case out of 10, it runs in families.^{footnote 1} This means that 9 times out of 10, a person with ALS doesn’t have a family member with the disease.

What are the symptoms?

The first sign of ALS is often weakness in one leg, one hand, the face, or the tongue. The weakness slowly spreads to both arms and both legs. This happens because as the motor neurons slowly die, they stop sending signals to the muscles. So the muscles don’t have anything telling them to move. Over time, with no signals from the motor neurons telling the muscles to move, the muscles get weaker and smaller.

Over time, ALS also causes:

• Muscle twitching.
• Trouble using your hands and fingers to do tasks.
• Problems with speaking, swallowing, eating, walking, and breathing.
• Problems with memory, thinking, and changes in personality. But these are not common.

ALS doesn’t cause numbness, tingling, or loss of feeling.

Respiratory problems and problems with swallowing and getting enough food are the most common serious complication of ALS. As the muscles in the throat and chest weaken, swallowing, coughing, and breathing problems tend to get worse. Pneumonia, pulmonary embolism, lung failure, and heart failure are the most common causes of death.

How is ALS diagnosed?

It can be hard for your doctor to tell if you have ALS. It may not be clear that you have the disease until symptoms get worse or until your doctor has done more testing. To find out if you have ALS, your doctor will do a physical exam and will ask you about your symptoms and past health. You will also have tests that show how your muscles and nerves are working.

Just because you have muscle weakness, fatigue, stiffness, and twitching doesn’t mean that you have ALS. Those symptoms can also be caused by other conditions. So talk to your doctor if you have those symptoms.

Tests to confirm ALS or look for other causes of your symptoms include:

• Electromyogram (EMG), which helps measure how well nerves and muscles work.
• Magnetic resonance imaging (MRI), which can show problems or injury in the brain.
• Nerve conduction studies to test nerve function.

If your doctor thinks that you have ALS, he or she will refer you to a neurologist to make sure.

How is it treated?

There is no cure for ALS, but treatment can help you stay strong and independent for as long as possible. For example:

• Physical and occupational therapy can help you stay strong and make the most of the abilities you still have.
• Speech therapy can help you with coughing, swallowing, and talking after weakness in the face, throat, and chest begins.
• Supportive devices and equipment can help you stay mobile, communicate, and do daily tasks like bathing, eating, and dressing. Some examples are canes, walkers, wheelchairs, ramps, handrails, raised toilet seats, and shower seats. You can also get braces to support your feet, ankles, or neck.
• Medicines can help relieve your symptoms and keep you comfortable. There are medicines that can help with many of the symptoms you might have, such as muscle problems (stiffness, cramps, twitching), drooling and extra saliva, depression and mood swings, and pain.
• A feeding tube can help you get enough nutrition to stay strong as long as you can.
• Breathing devices can help you breathe more easily as your chest muscles weaken.

A medicine called riluzole (Rilutek) may prolong survival by about 2 months.^{footnote 2} But it doesn’t improve symptoms or quality of life in ways that people with ALS, their caregivers, or their doctors have been able to notice. Most people tolerate riluzole very well, but it can cause side effects, including nausea, vomiting, weakness, dizziness, and coughing.

Another medicine, edaravone (Radicava), might also help slow down the progression of ALS in some people. Edaravone may cause side effects like bruising, headaches, and problems walking.

Treatment with riluzole or edaravone is expensive. But there may be programs that can help you pay for these medicines.

Palliative care

Palliative care is a kind of care for people who have a serious illness. It’s different from care to cure your illness. Its goal is to improve your quality of life—not just in your body but also in your mind and spirit.

You can have this care along with treatment to cure your illness. You can also have it if treatment to cure your illness no longer seems like a good choice.

Palliative care providers will work to help control pain or side effects. They may help you decide what treatment you want or don’t want. And they can help your loved ones understand how to support you.

If you’re interested in palliative care, talk to your doctor.

What decisions will you face as ALS progresses?

If you or a family member has ALS, learn as much as you can about the disease and how to take care of it. How much treatment you want for the problems caused by ALS is a personal choice that only you and your loved ones can make. Your values, wants, and needs are important things to think about as you make choices about your care.

As ALS symptoms get worse, you may have to choose which treatments you want for issues like breathing and eating problems. For example, would you consider using a machine to help you breathe if your breathing problems become severe? Do you want a feeding tube placed in your stomach if you are losing weight, unable to get enough calories by eating, or if you lose your ability to swallow?

As you make these choices, keep in mind that what’s right for one person with ALS may not feel right for another. It’s also okay to revisit your choices throughout the course of the disease. You may change your mind over time. Be sure to talk about your treatment options and share your concerns with your doctor.

You may want to put your health care choices in writing. This is called an advance directive or a living will. It gives you control over your own medical care when you can’t make decisions or speak for yourself. You may also want to choose a friend or family member to speak for you. This is called a durable power of attorney. Making these plans ahead of time will help make sure that your health care choices are followed.