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Lou Gehrig's Disease

What is Lou Gehrig's Disease?

Lou Gehrig's Disease is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.

Lou Gehrig's Disease is also called Amyotrophic lateral sclerosis or ALS.

What are Motor Neurons?

Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain are transmitted to motor neurons in the spinal cord and from them to particular muscles. In Lou Gehrig's 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.

Consequences of Lou Gehrig's Disease

Lou Gehrig's Disease causes weakness with a wide range of disabilities Eventually, all muscles under voluntary control are affected, and patients lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without ventilatory support. Most people with Lou Gehrig's Disease die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of Lou Gehrig's Disease patients survive for 10 or more years.

Because Lou Gehrig's Disease affects only motor neurons, 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. Patients usually maintain control of eye muscles and bladder and bowel functions.

Who gets Lou Gehrig's Disease?

As many as 20,000 Americans have Lou Gehrig's Disease, and an estimated 5,000 people in the United States are diagnosed with the disease each year. Lou Gehrig's Disease is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. Lou Gehrig's Disease most commonly strikes people between 40 and 60 years of age, but younger and older people also can develop the disease. Men are affected more often than women.

What are the symptoms of Lou Gehrig's Disease?

The onset of Lou Gehrig's Disease may be so subtle that the symptoms are frequently overlooked.

The most common symptoms of Lou Gehrig's Disease are:

  • twitching
  • cramping, or stiffness of muscles
  • muscle weakness affecting an arm or a leg
  • slurred and nasal speech
  • or difficulty chewing or swallowing

What causes Lou Gehrig's Disease?

The exact cause of Lou Gehrig's Disease is not known.

How is Lou Gehrig's Disease treated?

No cure has yet been found for Lou Gehrig's Disease. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease—riluzole (Rilutek). Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months, mainly in those with difficulty swallowing. The drug also extends the time before a patient needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs.

Other treatments for Lou Gehrig's Disease are designed to relieve symptoms and improve the quality of life for patients.

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