Amyotrophic Lateral Sclerosis (ALS)

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Many people have heard of, or participated in, the ALS Ice Bucket Challenge but many people fail to realize what ALS is. Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig’s disease is a motor neuron disease that causes degeneration of neurons that control voluntary muscles. ALS is not a single disease, but a clinical diagnosis for many different pathophysiologic diseases that share a common factor of progressive loss of motor neurons and break down of the motor neuron system. ALS is known by upper and lower motor neuron degeneration. As the upper motor neurons deteriorate, the cells “suffer from a retrograde axonal loss with secondary myelin pallor and gliosis”. While these changes are more severe at the brainstem and upper spinal cord, they …show more content…
Patients will not only develop weakness and stiffness of limb muscles, but also weaker respiratory muscles. With weaker respiratory muscles, the ventilation process of breathing becomes harder for those with ALS. Many studies have linked metabolism with respiratory function. Those with ALS are found to manifest a state of hyper metabolism. Sleep disorders are also common with those with ALS and can be an early sign of the disease but many times go undetected until later. With decreased respiratory function, changes of breathing can be fatal during sleep. The most well known treatment for sleep disorders and respiratory function is non-invasive positive pressure ventilation (1). Although the cause of ALS is unknown, it can sometimes be linked to family history. Through research, scientists have found over a dozen genetic mutations, one that includes mutations in the gene that produces SOD1 (Protein Coding gene) …show more content…
The study was intended to link the psychophysiological subjective quality of life and how one copes with a harsh and terminal disease. The subjective quality of life is defined as “the difference between the hopes and expectations of the individual and that individual’s present experiences”. The study consisted of 21 patients from the ALS outpatient clinic of the Department of Neurology, University Hospital of Wurzburg. The only disqualifying factors were if patients had a history of any neurological or psychiatric disorder. Before all the data could be collected, 2 male patients and 1 female patient aborted the study for different reasons, thus making the final sample size 18

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