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Proposal Requirement 3 Example: Amyotrophic Lateral Sclerosis (ALS)

Writer: Natasha Matta

Instagram: @natashakmatta

Email: matta.natashak@gmail.com


Summary of the Significance

Amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s disease, dubbed after the famous baseball player with the condition, is a progressive neurodegenerative disease that harms motor neurons in the brain and spinal cord, causing gradual muscle weakness, loss of movement control, and eventual paralysis. An estimated 30,000 people in the United States alone are affected by the disease annually, and 5,000 new cases are diagnosed each year. Researchers estimate that ALS causes around 5 of every 100,000 deaths in people aged 20 or older. After diagnosis, 50% of ALS patients live three years or longer, 20% live five years or longer, and 10% live ten years or longer.

Motor neurons coordinate signals between the brain, spinal cord, and muscles that control voluntary and involuntary movement, and these neuronal cells are attacked and killed in ALS. Degeneration of motor neurons results in muscle weakness, gradual loss of controlled movement, and paralysis. In the earlier stages of the disease, ALS patients often experience muscle weakness, atrophy (muscle deterioration), and cramps, and they may more easily lose their balance when walking, slur their speech, have a weak grip, or struggle with other basic motor skills. Middle stages of ALS are characterized by more widespread and severe muscle weakness and paralysis, and patients might experience difficulties talking, swallowing, or breathing as muscles in the mouth, throat, and chest grow weaker. In the final stages of ALS, the majority of voluntary muscles become paralyzed, and speech, eating, drinking, and breathing become difficult and sometimes impossible, and thus, respiratory failure is the most common cause of death for individuals with ALS.

Additionally, ALS is incredibly difficult to diagnose: there is no single test or set of procedures to definitively diagnose the condition. An ALS diagnosis is given through a series of tests, the majority of which are aimed at ruling out diseases that present similar symptoms to ALS. Electromyography (EMG), nerve conduction velocity (NCV), analysis of thyroid and parathyroid hormone levels and amount of heavy metals in blood and urine samples, spinal tap, x-rays and MRIs of the brain and spinal cord, myelogram of the cervical spine, muscle and nerve biopsies, and genetic testing if ALS runs in the family can all be used in diagnosis. Currently, there is no cure for ALS, but various treatments and therapies can slow the loss of muscle strength and function and help patients with symptoms like fatigue, making patients more comfortable and self-sufficient. ALS is detrimental to quality of life, and even though current treatment options can slow down progression of the disease, they do not halt it. More research is needed into new avenues of treatment for ALS to improve the hundreds of thousands of lives of ALS patients across the globe.


Analysis

The author begins by briefly explaining what amyotrophic lateral sclerosis is (a progressive neurodegenerative disease in which motor neurons are killed) and immediately establishes the importance of the issue through the high prevalence rate of the condition (it affects tens of thousands of people in the United States alone) and the short life expectancy for patients diagnosed with ALS (typically only 3-10 years after diagnosis). She then explains mechanisms underlying ALS like degeneration of motor neurons and which symptoms these cause like muscle weakness, loss of voluntary muscle control, and eventual paralysis. Scientific terminology like motor neurons and muscle atrophy were defined, so that the section is easy for someone who is not well-versed in neuroscience to read and understand. By the end of the piece, the author successfully convinced the reader that there is a need for further research into ALS treatment because of how difficult it is for patients and their families to cope with. The author used reputable sources including research papers from peer-reviewed science journals, hospitals, and universities and wrote professionally, using third person and employing correct spelling, grammar, and formatting.


References

“What is ALS?”. ALS Therapy Development Institute. https://www.als.net/what-is-als/

“ALS - Amyotrophic Lateral Sclerosis.” Johns Hopkins Medicine. https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/als/conditions/als_amyotrophic_lateral_sclerosis.html#:~:text=ALS%20Statistics&text=It%20affects%20as%20many%20as,among%20persons%20over%20age%2060

“What is ALS?” The ALS Association. https://www.als.org/understanding-als/what-is-als

“ALS Facts and Statistics.” ALS News Today. https://alsnewstoday.com/als-facts-statistics/

Ciervo, Yuri, et al. (2017). “Advances, challenges, and future directions for stem cell therapy in amyotrophic lateral sclerosis.” Molecular Neurodegeneration. https://www.researchgate.net/publication/321042958_Advances_challenges_and_future_directions_for_stem_cell_therapy_in_amyotrophic_lateral_sclerosis

“Types of Neurons.” The University of Queensland. https://qbi.uq.edu.au/brain/brain-anatomy/types-neurons#:~:text=Motor%20neurons%20of%20the%20spinal,all%20of%20our%20muscle%20movements

“Stages of ALS.” ALS Association Texas Chapter. https://www.alstexas.org/understanding-als/stages/#:~:text=As%20the%20disease%20progresses%20to,%2C%20eat%2C%20or%20drink%20normally

“Stages of ALS.” Muscular Dystrophy Association. https://www.mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als

“Amyotrophic Lateral Sclerosis (ALS) Fact Sheet.” National Institute of Neurological Disorders and Track. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet