Amyotrophic Lateral Sclerosis (ALS-Lou Gehrig's Disease)

Smart Choice MRI home » Illness & Injury » Illnesses » A » Amyotrophic Lateral Sclerosis (ALS-Lou Gehrig's Disease)

What is Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost. Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person’s thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

Is there any treatment?

No cure has yet been found for ALS. However, the FDA has approved the first drug treatment for the disease—riluzole. Riluzole is believed to reduce damage to motor neurons and prolongs survival by several months, mainly in those with difficulty swallowing. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS. Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation. Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).

What is the prognosis?

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals have increasing problems with moving, swallowing, and speaking or forming words. Eventually people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with ALS survive for 10 or more years.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) conducts research in its laboratories at the National Institutes of Health (NIH) and also supports additional research through grants to major medical institutions across the country. The goals of this research are to find the cause or causes of ALS, understand the mechanisms involved in the progression of the disease, and develop effective treatments.

Results of an NINDS-sponsored phase III randomized, placebo-controlled trial of the drug minocycline to treat ALS were reported in 2007. This study showed that people with ALS who received minocycline had a 25 percent greater rate of decline than those who received the placebo, according to the ALS functional rating scale (ALSFRS-R).

NIH Patient Recruitment for Amyotrophic Lateral Sclerosis Clinical Trials

Organizations

ALS Association 27001 Agoura Road Suite 250 Calabasas Hills, CA 91301-5104 advocacy@alsa-national.org http://www.alsa.org Tel: 818-880-9007 800-782-4747 Fax: 818-880-9006	Les Turner ALS Foundation 5550 W. Touhy Avenue Suite 302 Skokie, IL 60077-3254 info@lesturnerals.org http://www.lesturnerals.org Tel: 888-ALS-1107 847-679-3311 Fax: 847-679-9109
Muscular Dystrophy Association 3300 East Sunrise Drive Tucson, AZ 85718-3208 mda@mdausa.org http://www.mda.org Tel: 520-529-2000 800-572-1717 Fax: 520-529-5300	Project ALS 3960 Broadway Suite 420 New York, NY 10032 info@projectals.org http://www.projectals.org Tel: 212-420-7382 800-603-0270 Fax: 212-420-7387
ALS Therapy Development Institute 215 First Street Cambridge, MA 02142 info@als.net http://www.als.net Tel: 617-441-7200 Fax: 617-441-7299

Related NINDS Publications and Information

Amyotrophic Lateral Sclerosis Fact Sheet

Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's disease) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Doubling Up: Researchers Combine a Common Dietary Supplement with an Antibiotic to Treat Lou Gehrig's Disease
January 2003 news summary on a drug combination that slows the progression of amyotrophic lateral sclerosis (ALS) in an animal model.
Minocycline Delays Onset and Slows Progression of ALS in Mice
May 2002 news summary on a possible new treatment for amyotrophic lateral sclerosis (ALS)
Testimony on Amyotrophic Lateral Sclerosis, May 2000
NINDS Director's testimony before the Senate Appropriations Subcommittee on Labor, Health and Human Services, Education and Related Agencies on Amyotrophic Lateral Sclerosis (ALS), May 18, 2000.