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A and P

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Submitted By chriso78
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Amyotrophic lateral sclerosis is a devastating disease that is caused by the degeneration of motor neurons that affect voluntary movement. This will cause paralysis as the disease progresses and eventually will lead to death. ALS is difficult to diagnose since it resembles many other neurological disorders. It is not known what causes ALS and as of now there is no cure and very limited medications and treatments for those diagnosed with the disease. ALS is a disease that will ultimately make you a prisoner in your own body, mentally alert but unable to move.

Amyotrophic Lateral Sclerosis is a progressive neurodegenerative disease that affects the nerve cells in the brain and spinal cord. The body uses motor neurons to send information to and receive information from the Central Nervous System. They travel from the brain to the spinal cord and from the spinal cord to the muscles resulting in voluntary movement. ALS will cause these neurons to progressively degenerate. The neurons will no longer be able to send impulses to muscle fibers that normally cause muscles to function. Eventually the brain will lose its ability to communicate with the body and to control muscle movement. There are many types of nerves in the body. ALS only affects the nerves that are responsible for voluntary movements. These movements are mainly controlled by the muscles in the arms and legs. The heart and digestive system are also made of muscle but they under involuntary control. Everything that these organs do, they do automatically. ALS does not affect these types of muscles. That leaves a body function that is technically voluntary and involuntary, breathing. While breathing tends to happen automatically, a person can still hold his or her breath when they choose to. So it has been seen in some cases that ALS can cause issues with a person’s ability to breathe normally. (ALS association)
ALS is characterized by dysarthria, dysphagia, brisk reflexes, pyramidal signs, fasciculations, progressive atrophy and muscle weakness (Maruyama, 2013, Jul). Early symptoms of ALS are subtle. They can be described by muscle weakness or stiffness. Weakness will become more debilitating and over time will cause paralysis to muscles pertaining to the limbs and trunk will occur. Muscles that play roles in functions such as speech, swallowing and eventually breathing will also be affected as the disease progresses. ALS is not contagious and can affect anyone. Racial, ethnic or socioeconomic boundaries do not play a part in the development of the disease. ALS is thought to be caused by both environmental and genetic factors (ALS association). Genetically, there is a 10-fold greater risk of ALS among the first-degree relatives of ALS probands compared with the general population (Maruyama, 2013, Jul). ALS causes approximately 2 deaths for per hundred thousand annually. About 5,600 people are diagnosed with ALS every year which breaks down to about 2 people per 100,000 (ALS association).
ALS was discovered by Jean-Marie Charcot who was a noted French neurologist that is often referred to as “the Father of Neurology”. He made this discovery in 1874 through careful clinical observation and meticulous work in his laboratory while detailing the pathophysiology of some disorders that he was attempting to prove were separate from each other. He conducted studies between 1865 and 1869 which allowed him to declare the ALS was a specific neurological disease. He found that lesions within the lateral column in the spinal cord resulted in chronic progressive paralysis and contractures (no atrophy of muscles). Also that lesions of the anterior horn of the spinal cord resulted in paralysis without contractures (with atrophy of muscles) (US National Library of Medicine).
Despite remarkable advances in medicine and the understanding of ALS, no cure is currently available (van Zundert, 2012). There are also limited treatments and medications for ALS. Rilutek is a medication which is used to slow the disease’s progression by decreasing glutamate levels. There are also clinical trials under way that are testing the use of other agents that can hopefully target possible causes of the disease. When ALS starts to cause respiratory complications then noninvasive ventilation, respiratory management, and nutritional interventions are used. ALS can also be treated by utilizing occupational and physical therapists. This can help treat specific symptoms, improve the quality of life and maximize a person’s ability to function with ALS (ALS hope foundation).
The impact of this disease can be stressful for the person and family involved. A person with ALS will basically become more and more dependent on others with every aspect of their everyday life. A person with ALS will no longer be able to work and support his or her family. And there can be monumental costs for medical care, equipment and either home health caregiving or skilled nursing facility assistance (ALS association).

Bibliography
(n.d.). Retrieved from ALS association: http://www.alsa.org
(n.d.). Retrieved from US National Library of Medicine: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3064755/
(n.d.). Retrieved from ALS hope foundation: https://www.alshopefoundation.org/understanding-als/treatment-als.php
Maruyama, H. (2013, Jul). Optineruin and Amyotrophic Lateral Sclerosis. Geriatrics and Gerontology International, Vol 13 pp 528-32. van Zundert, B. (2012). Early pathogenesis in the adult-onset neurodegenerative disease amyotrophic lateral sclerosis. Journal of Cellular Biochemistry, Vol 113, pp3301-12.

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