Imagine being not able to move your muscles freely everyday, and that process has to coordinate between the brain and the spinal cord to tell the muscles to move. However, when this coordination is challenged and can not convey signals, the results are no muscle movement. At first, muscles in the hands, arms, and legs feel weak. The hands and feet twitch. Speaking and eventually breathing become difficult, and paralysis occurs in the final stages, although thinking and other cognitive functions remain intact. Death usually occurs within five years of the onset of symptoms.
This rare, progressive disorder is also known as Lou Gehrig’s disease, after a famous baseball player whose life was cut short by it. What is Amyotrophic Lateral Sclerosis? Nowadays, more and more persons are being detected with a condition that is signified as “Amyotrophic Lateral Sclerosis”. In the history, this neurological situation was signified by several as “Lou Gehrig’s disorder” (Hohnsbeen, 2008). Those that suffer from this medical condition endure great complexities. This is essentially because of the fact that the disorder quickly wages war against some nerve cells in the body.
These cells are recognized as neurons by medical experts. The neurons that are alluring to the disorder are the ones that are absolutely liable for the control of muscles that are deemed to be voluntary. The muscles in the upper body as well as those in the lower body are influenced. The influenced neurons are situated in the brain as well as the spinal cord and finally die (Hohnsbeen, 2008). In this direction, one will learn the facts adjacent the neurological state of amyotrophic lateral sclerosis. When a person is detected with amyotrophic lateral sclerosis, the advancement of the disorder may be gradual or it may be rapid.
It all depends on the general health of the individual, how long they have had the condition previous to diagnosis, plus any other medical circumstances that they may endure from (Phillies, 1995). Seeing that the reason of this severe neurological condition is unidentified, medical experts often have a hard time deciding the speed in which the condition improvements. Nevertheless, it is a fact that it will improve and signs observed will become more demanding to the functionality of the body. Difficulties that engage the muscles of the body that are utilized for different purposes will be experienced (Phillies, 1995).
It is not common for the amyotrophic lateral sclerosis sufferer to experience difficulties such as problems in walking, situations in which it is challenging to eat and swallow as suitable, and several may even face severe medical emergencies such as the failure to breathe properly. The cause of amyotrophic lateral sclerosis is not identified, though a significant step toward deciding the cause came in 1993 when scientists found that changes in the gene that generates the Cu/Zn superoxide dismutase (SOD1) enzyme were related with some cases (about twenty-percent) of familial Amyotrophic lateral sclerosis.
This enzyme is an influential antioxidant that defends the body from harm caused by superoxide, a toxic free radical produced in the mitochondria (William, 2000). Free radicals are very reactive molecules made by cells during normal metabolism again mostly by the mitochondria. Free radicals can build up and cause harm to both mitochondrial and nuclear DNA and proteins within cells. Proof proposes that inability of defenses against oxidative pressure up-regulates programmed cell death (apoptosis), among several other potential results.
Although it is not yet obvious how the SOD1 gene mutation leads to motor neuron degeneration, examiners have theorized that an accumulation of free radicals may consequence from the faulty functioning of this gene. New study, nevertheless, points out that motor neuron death is not probably a consequence of lost or compromised dismutase activity, proposing mutant SOD1 induces toxicity in some other way (William, 2000). Researches engaging transgenic mice have yielded many hypotheses about the role of SOD1 in mutant SOD1 familial amyotrophic lateral sclerosis.
Mice lacking the SOD1 gene totally do not usually develop amyotrophic lateral sclerosis, although they do demonstrate an acceleration of age-associated muscle atrophy (sarcopenia) and a shortened lifespan (Wade, 2001). This points out that the toxic properties of the mutant SOD1 are a consequence of a gain in function rather than a loss of normal function. Additionally, aggregation of proteins has been found to be a common pathological aspect of both familial and sporadic amyotrophic lateral sclerosis.
Fascinatingly, in mutant SOD1 mice, totals of mutant SOD1 were discovered only in disordered tissues, and large amounts were detected during motor neuron degeneration. It is observed that whole deposit of mutant SOD1 carries out a role in disturbing cellular functions by harmful mitochondria, proteasomes, protein folding chaperones, or other proteins (Phillies, 1995). Any such trouble, if shown, would lend large credibility to the hypothesis that aggregates are engaged in mutant SOD1 toxicity.
Opponents have noted that in humans, SOD1 mutations cause only two-percent or so of general cases and the etiological mechanisms may be different from those liable for the sporadic type of the disorder. To date, the amyotrophic lateral sclerosis SOD1 mice remain the best model of the disorder for preclinical researches but it is expected that more helpful models will be developed (Wade, 2001). There are several signs that are indicated to the situation of amyotrophic lateral sclerosis. In most sufferers, the first sign related with the situation was a weakness in the body. This weakness is generally related with a particular muscle.
It may happen in the face. Several have endured from a common weakness in the tongue. It could also happen in other parts of the body, such as the hands or the legs. This is a symptom that the neurons that are liable for motor functions are facing death (Mitsumoto, 2009). In several cases, the weakness will expand to other parts of the body. As the neurons experience death as a consequence of amyotrophic lateral sclerosis, they are not capable to relay the suitable signals to the brain. As a consequence, the muscles simply do not realize that they are supposed to move in one way or another.
Over time, they are employed less and less. Weakness sets in to the muscles, and then they lose their mass. As the neurological situation improvements, other signs will start to happen. Muscle twitching is an ordinary sign. The twitching may be in the type of sore cramps for several, but this is not always the situation. An individual that has amyotrophic lateral sclerosis may start to notice that simple tasks engaging the hands may become more challenging. Several may discover that their talking and the capability to use their mouth for eating and swallowing are more complicated. Finally, walking will become problem.
Those that are detected with amyotrophic lateral sclerosis will often find that they feel weak and comparatively tired, but they are capable to carry on doing tasks that engage cognitive capability such as concentrating on projects such as reading, memorization and often capable to focus with little to no difficulties at all (Mitsumoto, 2009). Conclusion Amyotrophic lateral sclerosis is a situation that is deemed to be deadly. Unluckily, there is no treatment for this situation. Once the disorder has started to impact the body, the harm that it produces will extend (Hohnsbeen, 2008).
The collapse to the condition is that most of the sufferers that endure it will be influenced with a failure of the respiratory system. The average time span in which this happens is within 3 to 5 years from the onset of the disease. This generally results in death. Nevertheless, there have been several people that have surpassed a lifespan of ten years once diagnosed. If you have currently been examined, keep in mind to work carefully with your physician when it comes to cures. Finding a support group can also show to be extremely helpful when it comes to coping with the progress of the neurological situation.