ALS (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.  Symptoms are usually first noticed in the arms and hands, legs, or swallowing muscles.  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 to function properly, 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.  Although the disease does not usually impair a person's mind or personality, several recent studies suggest that some people with ALS may develop cognitive problems, such as with word fluency, decision-making, and memory.  The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

Treatment

No cure has yet been found for ALS. However, the drug riluzole -- the only prescribed drug approved by the Food and Drug Administration to treat ALS -- prolongs life by 2-3 months but does not relieve symptoms. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS. Drugs are available to help individuals with spasticity, pain, panic attacks, and depression. Physical therapy, occupational therapy, and rehabilitation may help to prevent joint immobility and slow muscle weakness and atrophy. Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).

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.

Research

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).

NINDS is awaiting the results of a phase III trial of intravenous ceftriaxone in ALS that recently concluded recruitment. Preliminary studies showed promise, including safety and tolerability.

Both the NINDS and the Centers of Disease Control and Prevention (CDC) are committed to studies of disease patterns or risk factors among persons with ALS in order to better understand the causes of ALS, the mechanisms involved in the progression of the disease, and to develop effective treatments. In keeping with this goal, the CDC has launched the National ALS Registry, a program to collect, manage and analyze data about persons with ALS. The Registry includes data from national databases as well as de-identified information provided by persons with ALS. Persons living with ALS who choose to participate can add their information to the Registry.

Organizations

ALS Association
Nonprofit voluntary health organization dedicated to the fight against amyotrophic lateral sclerosis. Funds global research and sponsors advocacy programs, a network of chapters, and certified centers and clinics located nationwide.

1275 K Street, N.W.
Suite 1050
Washington, DC 20005
advocacy@alsa-national.org
http://www.alsa.org
Tel: Washington
Fax: 202-289-6801

Les Turner ALS Foundation
Supports medical research, patient services, and promotes awareness and education to find the cause(s), effective treatments, and ultimately, a cure for amyotrophic lateral sclerosis.

5550 W. Touhy Avenue
Suite 302
Skokie, IL 60077-3254
info@lesturnerals.org
http://www.lesturnerals.org
Tel: Skokie
Fax: 847-679-9109

Muscular Dystrophy Association
Voluntary health agency that fosters neuromuscular disease research and provides patient care funded almost entirely by individual private contributors. MDA addresses the muscular dystrophies, spinal muscular atrophy, ALS, Charcot-Marie-Tooth disease, myasthenia gravis, Friedreich's ataxia, metabolic diseases of muscle, and inflammatory diseases of muscle, for a total of more than 40 neuromuscular diseases.

3300 East Sunrise Drive
Tucson, AZ 85718-3208
mda@mdausa.org
http://www.mda.org
Tel: Tucson
Fax: 520-529-5300

Project ALS
Not-for-profit organization that raises awareness and significant funds toward effective treatments and a cure for ALS, also known as Lou Gehrig’s disease.

3960 Broadway
Suite 420
New York, NY 10032
info@projectals.org
http://www.projectals.org
Tel: New York
Fax: 212-420-7387

Content Provided By

NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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