Sporadic Amyotrophic Lateral Sclerosis

Categories: Disease

Amyotrophic Lateral Sclerosis, also known as ALS, is a disease that thousands of people deal with day to day. Before we jump into an expert definition, I’m going to break down the word part by part. With the help of a class previously taken (Medical Terminology), I’ve learned that ‘a’ refers to no, ‘myo’ refers to muscle, and ‘trophic’ refers to nourishment. Putting that together, it comes out to mean “no muscle nourishment. The ALS Association defines Amyotrophic Lateral Sclerosis as a neurodegenerative disease that is universally fatal and affects nerves in the brain as well as the spinal cord that controls voluntary actions (ALS 2016).

Amyotrophic lateral sclerosis originally came into view in 1869 by Jean-Martin Charcot, a French neurologist. Unfortunately, this disease did not gain publicity until it was contracted by a famous New York Yankees baseball player, Lou Gehrig. This ultimately ended Gehrig’s favorable sports career (ALS 2016). Now this disease is more famously known as Lou Gehrig’s Disease.

In 2014, ALS took storm over social media by producing the “Ice Bucket Challenge.” This challenge is when a bucket full of ice water is poured all over another person, usually video recorded, and those in the video would then nominate others to do the same. If those nominated did not want to endure the ice water, they could donate to the ALS foundation instead. Until this challenge became about, I knew absolutely nothing about this disease. Unfortunately, the majority of those doing this challenge were younger children. In my opinion, I think that it is very uncommon that the majority of those did not know what ALS stood for (like myself), most probably had to look up what it was, but stopped as soon as they saw the definition.

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On a positive note, this was a nation-wide challenge and ultimately it brought more people aware of this foundation.

There are two categories to ALS; familial and sporadic (Bradley et al., 2013). Familial ALS is the inherited version of this diseases, where the offspring have a 50% chance of developing symptoms if they come from family that has this gene. Luckily, Familial ALS contribute to a small amount of diagnosis (Bradley et al., 2013). The majority of ALS victims fall under the sporadic category, where nobody knows the exact reason why or how they received this disease. A question to ask yourself is, if you knew that you or your partner had this gene, would you still choose to have children despite the risks?

As of now, there is only one FDA approved medication for ALS management. Riluzole, or the generic version Rilutek, aids in slowing down the profession of symptoms. These symptoms include, but are not limited to, paresthesia and paralyzation. Paresthesia is an abnormal sensation, such as burning, prickling, or formication (Medical Dictionary Online). Riluzole works by inhibiting the excitatory transmitter glutamate (Cheah et al., 2010). The only thing that can better the target treatment for this disease is increasing the knowledge we have of the cause of Sporadic ALS.

Recently there have been several studies of BMAA, beta-methylamino-L-alanine. There is a theory that this is a factor of Sporadic ALS. The theory is that an increased ingestion of exposure to BMAA can lead to Sporadic ALS development later in life. BMAA is a non-protein encoding neurotoxic amino acid that is produced mainly by cyanobacteria (Cheah et al., 2010). Cyanobacteria is a class of bacterial that employ photosynthesis in order to secure energy to sustain life (Bradley et al., 2013). Cyanobacteria live in several different habitats, but mainly are found in water and soil. It has blooms that contain BMAA, which then can be inhaled or ingested orally. Not only is MBAA found in humans, it has shown up in animals. This becomes worrisome when humans consume animals that are infected with this ingestion. (Banack et al., 2010; Berlin et al., 1997; Bradley et al., 2009).

Although Sporadic ALS does not have a specific location of where has it, it is obvious to understand that it would be common in a place with plenty of water. Cyanobacterial infected marine habitats have an accumulation of BMAA within the native shellfish population (Banack et al., 2014). Banack interviewed a Sporadic ALS patient from Florida who reported eating lobsters that were caught from a local bay for at least thirty years. This specific bay is known to contain an abundance of cyanobacterial blooms that could possibly contaminate wildlife that it has contact with.

Amyotrophic Lateral Sclerosis is a devastating neurodegenerative disease that thousands of people suffer and die from daily. Although it can be genetic passed down, only about 5-10% of documented cases meet the requirements to be considered Familial ALS (Bradley et al., 2013). Therefore, showing that the abundant of cases are solely documented as being Sporadic ALS. However, there will always be a need for more information about diseases, but especially this one.

References

  1. About ALS. (2016). Retrieved December 5, 2018, from http://www.alsa.org/about-als/
  2. Banack, S. A., Metcalf, J. S., Bradley, W. G., & Cox, P. A. (2014). Detection of cyanobacterial neurotoxin β-N-methylamino-l-alanine within shellfish in the diet of an ALS patient in Florida. Toxicon, 90, 167-173. Retrieved March 23, 2016, from http://www.sciencedirect.com.libproxy.uwyo.edu/science/article/pii/S0041010114002
  3. Bradley, W. G. (2009). Possible therapy for ALS based on the cyanobacteria/BMAA hypothesis. Amyotrophic Lateral Sclerosis, 10118-123. doi:10.3109/17482960903285951
  4. Bradley, W. G., Borenstein, A. R., Nelson, L. M., Codd, G. A., Rosen, B. H., Stommel, E. W., & Cox, P. A. (2013). Is exposure to cyanobacteria an environmental risk factor for amyotrophic lateral sclerosis and other neurodegenerative diseases?. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 14(5/6), 325-333.
  5. Cheah BC, Vucic S, Krishnan AV, Kiernan MC. (2010). Retrieved March 28, 2016. Riluzole, Neuroprotection and Amyotrophic Lateral Sclerosis. Current Medicinal Chemistry 17(18):1942-1959.

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Sporadic Amyotrophic Lateral Sclerosis. (2021, Apr 22). Retrieved from http://studymoose.com/sporadic-amyotrophic-lateral-sclerosis-essay

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