Amyotrophic lateral sclerosis (ALS) is a debilitating disease that disrupts motor neurons, causing rapid muscle weakness, atrophy, spasticity, and difficulties with speech, swallowing, and breathing. The cause is unknown but may involve mutations in the SOD1 gene. The only FDA-approved treatment, Rilutek, can slow progression but also endangers liver health. Physical and speech therapy can help delay muscle degeneration and promote independence. There is no cure or prevention for ALS, which affects 20,000-30,000 Americans each year, usually between ages 40-60, with men more likely than women.
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Amyotrophic Lateral Sclerosis is defined
as a debilitative disease that disrupts the
functions of the motor neurons causing
complications within the patient that
include: Rapidly progressing weakness,
muscle atrophy, muscle spasticity,
difficulty speaking (Dysathria), difficulty
swallowing ( dysphagia), and difficulty
breathing (dyspnea).
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The cause has not yet been fully concluded although
certain researchers believe the disease is produced
through a mutation within the gene that produces the
SOD1(superoxide dismutase) enzyme which aids
within mitochondrial purification. It is theorized that the
mutation causes the accumulation of free radicals
which catalyze motor neuron degeneration. Scientists
also theorize the involvement of several factors
contribute to the development of Amyotrophic lateral
sclerosis, these include: electric Shock, military
Service, chemical exposure, and cranial trauma.
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The only medication that has provided results seems to be the
medication Rilutek. Rilutek only seems to slow the progression of
the disease, but it presents certain dangers toward the patient’s
liver health. Other Pharmaceutical treatments may be prescribed
in order to relieve fatigue and muscle spasticity.
The patient will also need to receive Physical and speech therapy
in order to delay muscular degeneration and promote
independence. The treatments consist of physical rehabilitation
sessions that assist patients with tasks such as walking and other
physical endeavors and speech therapy which instruct the patient
upon the procedures that will allow them to communicate during
the progression of their illness. Individuals affected will also need
respiratory assistance due to their failing respiratory muscles.
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There is no way in which Amyotrophic
Lateral Sclerosis can be prevented.
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As many as 20,000-30,000 individuals within the
united states are affected by Amyotrophic
Lateral Sclerosis and 5,000 individuals are
diagnosed every year. Amyotrophic Lateral
Sclerosis is the most common neuromuscular
diseases worldwide. Amyotrophic Lateral
Sclerosis commonly affects individuals
between the ages of 40-60, men are affected at
a higher quantity than women.
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1824-Charles Bell writes a report about Amyotrophic Lateral Sclerosis.
1850- English scientist Augustus Waller describes the appearance of shriveled
nerve fibers.
1869-French Physician Jean-Martin Charcot first describes ALS in scientific
literature.
1881- “Amyotrophic Lateral Sclerosis” is translated into English and translated in a
three-volume edition of Lectures on the Diseases of the Nervous System.
1939-ALS is recognized within the united states due to Lou Gerhig’s case.
1950’s-ALS epedemic occurs among the Chamorro people within Guam.
1991-Researchers link chromosome 21 to Familial Amyotrophic Lateral Sclerosis.
1993-SOD1 gene on chromosome 21 is connected to the development of Familial
Amyotrophic Lateral Sclerosis.
1996_ Rilutek is approved by the FDA in order to treat the disease.
1998- A criteria is developed in order to diagnose the disease.
1999-The revised ALS functional rating scale is approved.
2011-Non coding repeat expansions within C9ORF72 are found to be a major cause
of ALS and fronto temporal dementia.
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