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.
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).
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).
ALS Association 27001 Agoura Road Suite 150 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 800-344-4863 Fax: 520-529-5300 |
Project ALS 900 Broadway Suite 901 New York, NY 10003 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 |
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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Last updated September 09, 2008