Amyotrophic Lateral Sclerosis (ALS)

What is ALS?

ALS is a short name for a disease called amyotrophic (ay-my-oh-TRO-fik) lateral (LATT-er-ol) sclerosis (skler-OH-sis). ALS also is known as Lou Gehrig’s disease.

How does ALS affect your body?

ALS affects certain nerve cells and nerve pathways in the brain and spinal cord. It is a neurologic
(NOOR-oh-LAW-jik) disease. The word “lateral” refers to the area of the spinal cord where nerves become diseased. As the nerves slowly die, hardened material replaces them. This hardening is called “sclerosis.” 

The affected nerves control muscles you normally can move on your own. Examples are muscles in your arms, legs, and chest. This type of muscle is called a voluntary muscle. With ALS, these muscles begin to waste away. “Amyotrophic” means loss of muscle. Some types of muscles are not involved. ALS does not affect muscles in internal organs such as your heart, bladder, and digestive tract.

What causes ALS?

Doctors do not know what causes ALS. Poisonous substances (toxins) in the environment or viruses may play a role. Researchers also are studying genetic and hormonal factors.

Who gets ALS?

Each year, about 5,000 people in the United States learn they have ALS. The disease most often strikes people ages 40 to 70. More men than women get it. ALS becomes more and more severe over time. The disease gets worse at a different rate with each person. About 50 percent of people with ALS live 2 to 5 years after learning they have it. About 10 percent live for 10 years.

How do you learn if you have ALS?

There are no medical tests for ALS. A nerve doctor, called a neurologist (noor-ALL-ohjist), asks you detailed questions about your health. The doctor gives you a physical exam and nerve tests. Another test given is an EMG, or electromyogram (ee-LEK-tro-MYoh-gram). An EMG tests how well the muscles work. Other tests may include blood and urine tests, x-rays, CT scans, and MRI (magnetic resonance imaging). These tests help to rule out other possible diseases.

What are the symptoms of ALS?

Often the first symptom of ALS is weakness in the arms or legs. Usually one side of the body is affected more than the other side. Frequently, weakness makes it hard to hold onto objects. Weakness may cause tripping when walking or running. Less often, the first muscles affected are in the mouth and throat. These muscles control speaking and swallowing. Speech may become slurred or hoarse when these muscles are affected. It may become hard to swallow.

Besides weakness, other symptoms of ALS are:

  • Wasting away of muscle, called atrophy (AT-trow-fee)
  • Feeling of fatigue all over
  • Cramping, spasms, and stiffness in muscles
  • Twitching in muscles, called fasciculation (fuh-SICK-you-LAY-shun)

How fast does ALS progress?

Very often, ALS spreads rapidly to most of the voluntary muscles in the body. It becomes harder to move these muscles. In time, the muscles will not move at all. The muscles become paralyzed.

When you have ALS, you lose the ability to stand and walk and to use your arms and hands. It becomes difficult or impossible to speak and swallow. Breathing and coughing get difficult. Lung infections, such as pneumonia, or failure of the breathing muscles lead to death. Muscles not affected are the eyes, bladder, and bowel. Your ability to think and your five senses usually are not affected.

How is ALS treated?

No cure for ALS has yet been found. Your health care team can offer services and equipment that help people with ALS. The goal is to improve your quality of life.

Medicine

The one drug approved for ALS is riluzole (RIL-you-zole). The brand name is Rilutek (RIL-you-tek). This medicine is believed to slow the rate of nerve damage. When taken regularly early in ALS, this drug may extend the life of an ALS patient by about 3 months. Other medicines are available to help manage symptoms, such as muscle spasms. Talk with your doctor for more information.

Supportive care

A physical therapist can provide an exercise program to help keep your muscles working. The therapist may also provide special equipment to help you function on your own as much as possible. Examples are walkers and wheelchairs.

An occupational therapist can offer advice on how to modify your home to help you perform daily tasks. A speech therapist teaches ways to cope with speech and swallowing problems. He or she can also provide devices to help you communicate.

Breathing assistance

As it gets more difficult to breathe, an ALS patient may want to use a mechanical ventilator (VEN-til-LAY-ter). This breathing machine can be used with a piece that goes into the mouth or nose. Another approach is a tube that goes directly into the windpipe. This method is called tracheostomy (tray-kee-OStuh-mee). Mechanical ventilators allow freedom to move around. These devices may extend life for a short time.

Other support

Nutrition specialists can help with meal planning and ways to prepare and take meals. Nurses, such as home care and hospice nurses, can give valuable assistance to ALS patients. Support groups can provide emotional help for patients and their caregivers. The ALS Association sponsors support groups. To find a support group, see the list of resources on this page.

If you have questions

If you have any questions or concerns, call a member of your ALS health care team.

Useful resources

The ALS Association Western Pennsylvania Chapter
One Station Square Drive, Suite 550
Pittsburgh, PA 15219
1-800-967-9296
ALSAWPA@aol.com
webwpawv.alsa.org

UPMC Neuromuscular Disease Division Department of Neurology
810 Kaufmann Building
3471 Fifth Avenue
Pittsburgh, PA 15213
412-692-4917

For an appointment or information:
1-800-533-UPMC (8762)

ALS Association of America National Office
27001 Agoura Road, Suite 150
Calabasas Hills, CA 91301

Information and referral service:
1-800-782-4747

All others: 818-880-9007
www.alsa.org

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