Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease or classical motor neuron 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. Muscle weakness and atrophy occur on both sides of the body. Individuals with ALS lose their strength and the ability to move their arms and legs, and to hold the body upright. 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 involving 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.
No cure has yet been found for ALS. However, the drug riluzole--the only prescribed drug approved by the Food and Drug Administration (FDA) to treat ALS--prolongs life by 2-3 months but does not relieve symptoms. The FDA has also approved the NeuRx Diaphragm Pacing System, which uses implanted electrodes and a battery pack to cause the diaphragm (breathing muscle) to contract, to help certain individuals who have ALS and breathing problems an average benefit of 16 months before onset of severe respiratory failure. 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).
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.
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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.
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.
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.
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.
ALS Therapy Development Institute
Founded in 1999, the ALS Therapy Development Foundation is dedicated to discovering and developing treatments for ALS. The organization, which focuses on a concentrated drug discovery program for ALS, has collaborated with 28 for-profit companies and academic researchers to perform advanced investigations.
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NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke (NINDS). This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 250 identified neurological disorders.