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What is Lou Gehrig's disease?

Genetic research and drug therapy offer some hope in the search for a cure for Lou Gehrig's disease. But for people with the condition, answers can't come soon enough.

Amyotrophic lateral sclerosis (ALS), known as Lou Gehrig's disease because of the famous baseball player who died from it, is a neuromuscular disease that attacks nerve cells and pathways in the brain and spinal cord, according to the American Academy of Neurology.

As ALS progresses, motor neurons—nerve cells in the body's spinal cord and muscles—die and the brain is no longer able to initiate or control muscle movement. Without messages from the brain, muscles begin to atrophy, or waste away.

The Centers for Disease Control and Prevention estimates that about 30,000 people in the U.S. have ALS. Most people are diagnosed between the ages of 55 and 75.

Symptoms of ALS

ALS symptoms are different for each person. Possible signs include:

  • Twitching and cramping of muscles.
  • Problems with speaking.
  • Impaired use of arms and legs.
  • Difficulty walking.
  • Shortness of breath.
  • Difficulty swallowing.
  • Difficulty breathing.

ALS usually does not affect cognitive functioning. People with the disease are still able to taste, see, smell, hear, recognize touch and think clearly. However, research suggests that a small percentage of people with ALS may experience problems with decision-making and memory skills.

The loss of the ability to speak is a frustrating disability that accompanies ALS. Electronic devices and computers have been developed to help people with ALS communicate, regardless of their physical capabilities.

Because muscular weakness can be caused by other diseases with symptoms similar to ALS, people with symptoms are usually referred to a neurologist for diagnosis.

Causes

There are no known causes for ALS. In 5% to 10% of cases, a genetic defect may play a role. 

The majority of ALS cases are called sporadic because they are unpredictable and can affect anyone.

The life expectancy of people with ALS averages about two to five years from the time of diagnosis, according to the ALS Association. About 20% of those with ALS live five years, and 10% live 10 years.

Managing symptoms

The primary treatment for ALS is managing symptoms. Physical, occupational, speech, respiratory and nutrition therapy are all used. Moderate exercise can help maintain muscle strength and functioning.

As the disease progresses, various devices may help people with ALS avoid injury and maintain their independence, including:

  • Leg or ankle braces to give support and help prevent falls.
  • Reclining chairs to relieve fatigue of neck muscles.
  • Cervical collars to support weakened neck and trunk muscles when sitting becomes difficult.

Other devices can help with feeding, dressing and maintaining personal hygiene.

Hope for a cure

Researchers are hopeful that an understanding of the genetic form of ALS may lead to treatment for both forms of the disease, since their symptoms and progression are similar. Some drugs have been shown to affect nerve cell activity, which may increase the survival time for those with ALS.

Reviewed 9/27/2023

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