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About Lou Gehrigs Disease

Lou Gehrigs Disease is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles.

In Lou Gehrigs Disease, 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 Lou Gehrigs Disease 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.

In most cases the disease does not impair a person's mind, personality, intelligence, or memory, nor does it affect a person's ability to see, smell, taste, hear, or recognize touch.

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 Lou Gehrigs Disease is not known, and scientists do not yet know why Lou Gehrigs Disease strikes some people and not others.

Treatment

No cure has yet been found for Lou Gehrigs Disease. 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 Lou Gehrigs Disease.

Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation. Individuals with Lou Gehrigs Disease 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 Lou Gehrigs Disease 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 Lou Gehrigs Disease.

Most people with Lou Gehrigs Disease die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with Lou Gehrigs Disease survive for 10 or more years.

Research

Public Health Authorities conduct research in laboratories and support additional research through grants to major medical institutions across the country.

The goals of this research are to find the cause or causes of Lou Gehrigs Disease, understand the mechanisms involved in the progression of the disease, and develop effective treatments.

Reference for Lou Gehrigs Disease Article

National Institutes of Health

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