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ALS Etiology

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Pathology and Etiology Amyotrophic Lateral Sclerosis (ALS) is a relatively elusive disease. ALS is a disease that affects the large upper and lower motor neurons of the motor cortex, brain stem, and spinal cord . The destruction of the upper and lower motor neurons causes progressive paralysis. Even with research of ALS beginning in 1869, and becoming more widely known in 1939 (About ALS), the cause is still being heavily researched. Luckily, scientists have come across quite a few theories as to the cause of ALS. The validity of many of the theories are still being tested, but a few of them have found their way to being a staple in ALS etiology. Sporadic ALS (sALS) accounts for 90% of diagnosed cases. The exact cause of sALS is unknown, …show more content…

EEA2 transporters are primarily found in the central nervous system (CNS) and are responsible for moving glutamate away from synapses shortly after it is released; the swift removal of glutamate is meant to prevent prolonged excitation of nerve cells (Foran & Trotti, 2009). With the dysfunction of EEA2 transporters, glutamate over excites the postynaptic membranes of nearby nerve cells—the over abundance of the excitatory neurotransmitter, as well as the prolonged contact, causes an increase in calcium (Ca2+) permeability of surrounding nerve cells. The increase of Ca2+ permeability also causes the release of intracellular Ca2+ stores. Along with the influx of intracellular Ca2+, sodium (Na+) follows the Ca2+ into the cell, causing further stimulation. As a result of the increased intracellular levels of Ca2+ and Na+, potassium (K+) leaves the cell—the resulting effect is cell swelling and death of the neuron. Glutamate isn't the only factor that can play into the fatal disease though, oxidative stress can also be a contributing …show more content…

ROS are produced by metabolic processes in the body (Oxidative Stress, 2014). In aerobic metabolism the mitochondria uses about 90% of oxygen—this means that the mitochondria are most responsible for the production of ROS (Barber, S. C., Meade, R. J., & Shaw, P. J.,2006). The over abundance of intracellular Ca2+ levels in the neuron is closely linked with oxidative stress. Excessive intracellular Ca2+ are not only responsible for the swelling and death of neurons, but it also is one of the causes of the production of ROS. When ROS are over produced they cause the inhibition of glutamate uptake through EEA2 transporters, hence the vicious cycle of excitotoxicity. When the intracellular levels of Ca2+ increase, it is the job of mitochondria to take in Ca2+; the intake of Ca2+ by the mitochondria causes increased ROS production. ROS cause damage to DNA and protein, which causes the destruction of neurons (Barber, S. C., Meade, R. J., & Shaw, P. J. 2006.) Although studies have shown the link between sALS and oxidative stress, further research is being done to prove or disprove this

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To encourage a positive attitude, emotional support from friends and family help the patient mentally (Larson). ALS is a very mystifying disease that in 90 percent of cases, scientists do not know the cause. Scientists say that nothing in the ALS patient’s diet, where they live, or how they have lived their life can be a credible case for ALS. However, in a small percent of cases, a genetic history can exist. In these cases around half of the entire family is diagnosed with ALS (Causes of

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The overall prevalence of ALS in the United States has been estimated to be between one and two cases per 100,000 people. The overall prevalence worldwide is estimated to be between four and ten per 100,000 people. Even though ALS can occur at any particular age, the most common age at onset is the mid-to-late 50s. Men are affected slightly more than women, according to most studies, by a ratio of five to three. The vast majority of patients with ALS have no prior family history of the disease.

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there are still some people who are not sure what this disease actually is. Well it is a neurological condition that a person is born with. The main causes of this disease are still not known to the scientist

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