Lou Gehrig's Disease
Amyotrophic Lateral Sclerosis (ALS) Causes Motor Neurons to Die
The development of Lou Gehrig's disease, a disease of the motor neurons, leads to progressive disability as neural control of the skeletal muscles of the body is lost.
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive, debilitating neuromuscular disorder where motor neurons degenerate and die. Unfortunately most patients die of this disease within 5 years of initial diagnosis. As motor neurons die, skeletal muscle control is lost leading to paralysis, an inability to speak and swallow, and quite often fatal respiratory consequences.
When Motor Neurons Die Skeletal Muscle Control is Lost
When New York Yankees great Lou Gehrig died at age 37 in 1941, the symptoms of the illness that would ultimately bear his name had caused him to retire from baseball only two years earlier. In the year prior to his retirement he and his teammates had noticed a sharp decrease in his abilities. This occurs because the motor neurons of the spinal cord and the brain, which control muscular contraction, progressively die off.
What is the Pattern of Disability in ALS
No two patients with ALS likely present to their physician with exactly the same symptoms. Commonly, things as simple as unexplained muscular weakness, an increased frequency of tripping, routine falls, cramping in the hands and feet and many other events are all part of the complex of symptoms that may be the initial indicator of ALS. For some patients weakness in a leg may be the first sign while for another it could be difficulty speaking. As more and more motor neurons die the disabilities spread and the condition worsens.
What Causes Motor Neurons to Die in ALS
For the vast majority of ALS patients there is no known reason for the progressive degeneration and subsequent death of their motor neurons. For a small percentage of ALS cases there appears to be a genetic, or inherited, component. The mutations most commonly seen would seem to indicate that oxidative damage to motor neurons is part of the cause of their death, at least for these familial cases. Irrespective of the cause, motor neurons continue to degenerate and die leading to increasing disability and, for most patients, death results from respiratory problems.
What Treatments are Available for ALS
Numerous clinical trials have been undertaken with the hope of at least slowing the progression of the disease. One of the difficulties faced in undertaking these clinical trials is the relative rarity of ALS in the population. Without a large enough sample of patients it is difficult to evaluate potential treatments effectively. Nonetheless, there has been approval of a drug (riluzole) by the US Food and Drug Administration for slowing the progression of ALS.
What does the future hold? Continuing research identifies new avenues for study every day. New genetic mutations associated with the disease are being investigated. And animal models of the disease are being utilized to test new factors that may help the remaining motor neurons to survive.
