Amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s Disease, has gotten some serious attention after Pete Frates and his family started the ALS Ice Bucket Challenge to raise money for and awareness of the incurable neurodegenerative disease.
The ALS Association explains that ALS degenerates motor neurons used to control muscle movements, meaning the brain can’t send messages to muscle fibers in order to move them. The degeneration stems from an inability to “nourish” the muscles connected to the spinal cord, which causes them to atrophy, or waste away. These atrophied muscles may then harden or scar the area in which they are located.
According to the ALS Association, the degeneration of motor neurons prevents impulses from being sent to muscle fibers that cause muscle movement. As a result, early symptoms of ALS include varying kinds of muscle weakness, typically involving the arms and legs, as well as difficulties swallowing, speaking and breathing. ALS impacts the body’s ability to perform voluntary movements, which includes breathing, since people are able to hold their breath. The ALS Association adds the limbs of ALS patients tend to look thinner as their muscles atrophy.
There’s currently no cure for ALS, although the FDA has approved one drug that helps slow the progression of the disease, according to the ALS Assocation. That drug, riluzole, has helped some patients delay the need to go on a ventilator or undergo a tracheotomy, in which a tube is inserted into a person’s throat in order to help them breathe. Riluzole may increase survival by approximately two-to-three months in some patients. The drug is expensive, however, as each individual tablet costs roughly $10. The ALS Assocation said there are several drugs currently in clinical trials that hold promise in terms of fighting the disease.
According to The National Center for Biotechnology Information, Jean-Martin Charcot described and diagnosed the first cases of ALS in studies conducted from 1865 to 1869. Charcot first used the term amyotrophic lateral sclerosis in a compilation of his lectures published in 1874. ALS is still referred to as Charcot’s disease in many parts of the world, The National Center for Biotechnology Information reports. Charcot is also credited as the founder of multiple sclerosis and the study of modern neurology.
Although the disease was discovered in the mid-1800s, it didn’t gain national attention until 1939 when Lou Gehrig was diagnosed with ALS, forcing him to retire from baseball, and leading to the disease’s more common name. Theoretical physicist Steven Hawking was diagnosed with ALS when he was 21. Upwards of 30,000 Americans have ALS at one time and the disease typically affects people ages 40 to 70. Other notable individuals with ALS include former U.S. Vice President Henry A. Wallace, Senator Jacob Javits and Sesame Street creator Jon Stone, according to the ALS Association.
The Ice Bucket Challenge, started by Massachusetts resident and ALS patient Pete Frates, has brought Lou Gehrig's Disease into the national consciousness as the campaign has gone viral. Read more on the disease here.