Introduction
Amyotrphic Lateral Sclerosis or ALS is a progressive neurodegenrative disease that affects nevrous cells in the braina and the spinal cord. When broken down, A-myo-trophic means, "no muscle nurishment." The word lateral refers to the part of the body that is affected, the spinal cord. And sclerosis refers to the scaring or hardening of skin in the region affected by the disease.
Two people you may have heard of having this disease include Lou Gahrig a famous baseball player, and Stephen Hawking, and english theoretical physicist. The disease was discovered by French neurologist Jean-Martin Charot in 1869.
The following link gives a good overview of what ALS is , how it affects the body, and how many people are affected on average.
https://www.youtube.com/watch?v=tKDET9t8VTI
Task
How ALS works away at the body?
Our body has motor neurons that reach from the brain to the spinal cord and from the spinal cord to different muscles all over the body. Once these neurons begin to die, the brain looses its ablility to control muscles throughout the body. This leads to individual diagnosed with ALS to loose functions such as speach, eating, moving around, and even breathing. Specifically it is the volentary movements provided by motor neurons that are targeted by the disease and end up dying.
There are two different types of ALS, sporadic and familial. Sporadic, being the most common form making up 90-95% of all cases, can affect anyone at random. Familial is passed down by genetics.
The followin link will take you to a video that gives some good graphics on how this disease works against its victims muscles over time.
Process
Who does ALS affect?
According to U.S. polulation studies, around 5,600 people in the U.S. are diagnosed with ALS each year. According to the ALS CARE database, about 60% of those diagnosed are men, and 93% of those in the database are caucasian, so if you are a white male, watch out!
Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. This does not mean that you cannot get the disease earlier.
Half of all people diagnosed with ALS live at least three or more years after diagnosis. Around 20% will live more than five years and around 10% will live more than ten years.
Symtoms:
ALS is typically a disease that invloves a gradule onset, meaing it doesnt just happen over night. Symtoms can begin in the muscles that control speech, swallowing, as well as the persons hands, arms, or legs. However everyone is different when it comes to this disease so symtoms are not always the same. One thing that is universal with ALS is the progressive muscle weakness and paralysis.
The most common symtoms include gradual onset, and painless, progressive muscle weakness. Other early symtoms include tripping, dropping things, abnormal fatigue of the arms/hands and legs, slurred speech, muscle cramps and twitches, and uncontrollable laughter or crying.
Luckly our sense of sight, touch, hearing, taste and smell cannot be affected by ALS because the disease only targets motor neurons.
Diagnosis:
This can be a very hard disease to diagnose because of other diseases that mimic its symtoms. A comprehensive diagnostic workup incluedes most, if not all, of the following procedures:
- Electrodiagnostic tests such as electomyography (EMG) and nerve conduction velocity (NCV)
- Blood and urine studies
- Spinal tap
- X-rays
- Myelogram of cervical spine
- Muscle and/or nerve biopsy
- Thorough neurological examination
Evaluation
Treatment:
There is currently one FDA approved drug, riluzole, which mostly helps slow down the progression for most people with the disease. There is no current cure or treatment that can stop or reverse the disease from taking over.
The best thing for people with this disease can be to attend support groups, ALS Association Certified treatment center, or a Recognized Treatment Center. It really comes down to managing the symtoms and learning to live with the disease.
The following video is about stem cell treatment for a woman with ALS.
Conclusion
Becuse this is a disease that can affect anyone, it is important to spread awareness and support for those affected. Please take a moment to remember how lucky we all are to have our volentary motor functions and not take them for granted. As they say, you never know what you got until it't gone.