Much mystery surrounds amyotrophic lateral sclerosis (ALS), a form of motor neuron disease that attacks nerve cells in the brain and spinal cord — the nerve cells that allow you to control your muscles. Eventually a person loses strength and cannot move.
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In 2014, the ALS Ice Bucket Challenge brought awareness of this deadly disease to countless people who had never heard of it before. The challenge involved people getting doused with buckets of ice water on video, posting that video to social media, then nominating others to do the same (or make a donation or both), all in an effort to raise ALS awareness and funds toward its research.
ALS also is known as Lou Gehrig’s disease, after the famous New York Yankees baseball player who was forced to retire after developing the disease in 1939. Well-known physicist and best-selling author Stephen Hawking, who died March 14, 2018, also had ALS.
ALS affects about 30,000 people living in the United States, with about 5,600 new patients diagnosed each year.
ALS affects men slightly more frequently than women, except for those under the age of 50. In their 30s and 40s, men are affected about three or four times as often as women.
About 10 percent of cases are genetic or hereditary and the other 90 percent are sporadic. For the 90 percent, we don’t know the cause or trigger but in the hereditary cases, we can now identify the responsible gene about 70 percent of the time. Much research is ongoing to explain 100 percent of the genes causing hereditary ALS.
Because there is no known cause for most forms of ALS, we have no cure. The hope is that soon a cure will be found or at least a treatment that slows down the disease and makes it more of a chronic disease, like most cases of multiple sclerosis.
Riluzole (Rilutek®) is the only medication so far approved by the Food and Drug Administration (FDA) for ALS treatment. Although it slows disease progression and extends survival by several months, it is not a cure and we must find something better.
The first symptoms of ALS can vary in type and location, but the most significant one is muscle weakness, which can begin anywhere in the body, including the limbs, face, tongue, throat, or even the diaphragm. Because ALS is a progressively degenerative disease, it gradually spreads to affect other muscles in the body.
ALS affects everyone differently and progresses differently. Approximately 20 percent of people with ALS will live for five years or more, and up to 10 percent will live more than 10 years. Unfortunately, the majority will not survive longer than two to five years after onset of symptoms.
The average delay from onset of symptoms to diagnosis of ALS is about 12 months. By increasing awareness of ALS and seeing a specialist as quickly as possible once symptoms begin, a correct diagnosis can be made and supportive care and therapy started sooner.
Although thinking and memory are usually preserved in ALS, they can also be affected. Research shows that up to half of ALS patients can have some degree of cognitive or behavioral impairment, and approximately 10 percent have dementia in addition to the motor weakness. Awareness of this possibility is important when it comes to making important decisions during the course of the disease.
Genetic studies tell us that ALS is probably not just a single disease, but a conglomerate or combination of several related diseases with a similar end result. This may be a reason why we have been unsuccessful in finding one treatment that will be effective for every patient.
What does the future hold?
Dr. Pioro says continuing research offers hope for progress in the fight against ALS.
“There’s been a huge amount of research in the last five to 10 years that is giving us new insights into what may be causing ALS,” Dr. Pioro concludes. “So there is great hope that we are getting closer to discovering a way to slow it down significantly and hopefully to stop it.”