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 - Northwestern Memorial Hospital - Chicago

Overview

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive disease of the nervous system that attacks nerve cells (neurons) in the brain and spinal cord. 

The condition is more common in men, and the age of onset is usually between 40 and 60 years. Doctors and scientists are unsure what causes ALS, and while it may run in families, it may also appear randomly. ALS affects about 1 out of every 100,000 people.

Signs & Symptoms

ALS attacks the neurons that transmit messages from the brain and spinal cord to the voluntary muscles (the muscles you can control). Motor neurons degenerate or die, and as this happens, they stop transmitting messages to the muscles, which, over time, weaken, atrophy (waste away), and twitch. The brain gradually loses the ability to control voluntary movement.
 
As the disease progresses, muscle weakness and atrophy spread throughout the body. Symptoms include:
  • Muscle cramps
  • Difficulty running or walking
  • Difficulty with writing
  • Problems with speech
  • Swallowing difficulty
  • Muscle weakness
  • Paralysis
Symptoms may be mild at first, but worsen with time, as the neurons degenerate and die. From the initial muscle difficulties, muscle weakness and atrophy progress until even breathing is affected. There currently is no cure for ALS and most patients die from respiratory failure, typically within 3 to 5 years of onset of symptoms. Approximately 10 percent of people with ALS survive for 10 or more years.

Are there Treatments Offered?

Currently, there are some drugs that can help with symptoms and improve quality of life—drugs to treat pain, panic attacks, depression, and muscle twitches.

While there is no cure as yet for ALS, researchers at Northwestern University have found the malfunctioning protein recycling system at the heart of ALS. Efficient recycling of protein in the neurons is vital for their optimal function, and this failure to recycle the proteins is central to all three types of ALS.

The discovery of this root cause provides researchers a prime target for development of drug therapy, and, ultimately, effective treatment of ALS. This will allow researchers to test for drugs that regulate or optimize this protein pathway and restore normal function, in time. This breakthrough research may also help in the study of other neurodegenerative diseases such as Alzheimer's.

Physical and occupational therapies may help slow muscle weakness and atrophy, as well as prevent immobility in joints. Use of a ventilator for breathing becomes necessary as chest muscles eventually fail.

Resources & Support

For additional information and to learn about resources available for patients with ALS and their families, visit the following websites:

Last UpdateSeptember 1, 2011
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