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Pathology of amyotrophic lateral sclerosis
Pathology of amyotrophic lateral sclerosis
Pathology of amyotrophic lateral sclerosis
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Amyotrophic Lateral Sclerosis
A Look at ALS Amyotrophic Lateral Sclerosis, or ALS, is a neurological disease that disrupts the functioning of motor neurons in the afflicted person. Commonly known as Lou Gehrig’s disease in the US, developing the disease usually guarantees that a premature death is unavoidable. ALS is a degenerative disease, which means nerve cells deteriorate, but all neurological disorders involve the exacerbation of neuron functioning, so what sets ALS apart from other neurological diseases? According to the National Institute of Neurological Disorders and Stroke (NINDS 2013), typical ALS patients are characterized by the steady failure and decline of functioning motor neurons. A common and fit person unaffiliated by ALS
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Primarily the basic functioning of the cell influences the development of the disease. Cells must be able to transport materials and messages from all over the body, and along a motor neuron’s axon in order to preserve the life of a nerve cell, but research suggests that the motor neurons can be vulnerable to any defect that might hinder axon transport. Apoptosis, or the systematic process of cell death, in which cells receive insufficient amounts of materials, has been a part of designing effective treatments for ALS, as it is at the core of the disease. Apoptosis is a normal physiologic occurrence; actually, being regarded as a central part of the nervous system’s growth (ALS Association, 2016). If there is a case of extreme apoptosis, problems may arise. Alternatively, while apoptosis might be part of the development of the nervous system, necrosis results from a direct injury or infection to the nervous system. The process of necrosis involves an explosion of the cellular content, creating a mess of …show more content…
Neurotransmitters, like glutamate, are messengers that pass signals from one neuron to another, thereby communicating to receiving neurons whether to fire off its own neurotransmitters, or not. If a neuron has a prolonged excitatory period from excessive glutamate, the results can be lethal (ALS Association, 2016). Glutamate, an excitatory neurotransmitter, becomes harmful when neuron’s messages become unusual and overwhelming e.g. in the case of a stroke. Responsibility of concentrating glutamate equally around the neurons falls to molecules called transporters. When it is not properly concentrated and causing an overflow of excitatory responses, glutamate is an issue. Nevertheless, excessive glutamate can be problematic in ALS patients and treatment involves being able to deliver glutamate transporters to ALS affected
On December 1, 2012, a patient by the name of John Dough walks into the medical assistant’s office. The patient is five foot 11 inches tall, currently he is 70 years old and weighs approximately 211 pounds. The patient has no known allergies does not smoke and has a relatively clean health record. After filling out the patient medical history forms, the patient is seen by the doctor. The patient explains to the doctor that lately he has had trouble lifting object he would not normally have trouble with, as well as walking short distances, and being very fatigued. After further examination the patient explains how he recently found a tick on his back and removed it, but now there is a red bullseye on his back. The physician suggests a blood sample be taken and sent to the laboratory. To help with weakness and fatigue he recommends the patient to get a good nights sleep and drink plenty of fluids to avoid dehydration. He also wants the patient to limit medication intake that could contribute to fatigue such as cold and allergy medicines and make sure to finish all daily exercising three to four hours before bed. The patient schedules a check up two weeks later.
There is no cure for ALS at this time and treatment is focused on management of the symptoms, involving a combination of physical therapy, occupational therapy, and speech, respiratory, and nutritional therapies. Moderate exercise may help maintain muscle strength and function. Drugs can also treat excessive saliva and drooling, and speech therapy can help compensate for loss of muscular control of the mouth. As the disease progresses and muscular degeneration spreads throughout the body, various devices may provide support, such as ankle braces, neck collars, reclining chairs, wheelchairs, and hospital beds. Respiratory support and feeding tubes are required when the person loses cont...
According to ALS Association (2016, para. 1), “Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in Amyotrophic Lateral Sclerosis eventually leads to their demise.” When our motor neurons die, our brain can no longer control our muscle movement. The survival time for a person living with Amyotrophic Lateral Sclerosis is up to ten years because eventually a person’s body will shut down completely. According to the Mayo Clinic (2016, para. 2), “As the disease advances and nerve cells are destroyed, your muscles progressively weaken. This eventually affects chewing, swallowing, speaking and breathing.” This disease is very scary to live with because you eventually die from
Lou Gehrig's disease is often referred to as Amyotrophic lateral sclerosis (ALS), this is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons come from the brain to the spinal cord and from the spinal cord to the muscles throughout the entire body. The progressive degeneration of the motor neurons in ALS would eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is also lost. With voluntary muscle action progressively affected, for this reason patients in the later stages of the disease may become totally paralyzed (Choi, 1988).
Different cellular signaling pathways respond to calcium levels. The inflow of calcium resulting from glutamate receptor stimulation leads to their activation.
ALS is a degeneration of motor neurons that move from the brain and down the
A piece of well-oiled machinery consists of an intricate and complex system: there are well-organized processes, mechanisms within the device work efficiently, and multiple processes function simultaneously to subsequently perform various functions. What happens when there is a glitch in the machine? When there is something wrong, such as connections between intricate processes, which do not follow through, the machine fails to function properly. In some cases, there are not any adjustment or fallback mechanisms. At that point, the damage can be irreversible and the machinery is no longer salvageable. [However, this can illustrate the interactions and processes within the complex machinery.]
Amyotrophic lateral sclerosis, or ALS, is a degenerative disease affecting the human nervous system. It is a deadly disease that cripples and kills its victims due to a breakdown in the body’s motor neurons. Motor neurons are nerve cells in the brainstem and spinal cord that control muscle contractions. In ALS, these neurons deteriorate to a point that all movement, including breathing, halts. Muscle weakness first develops in the muscles of body parts distant from the brain, such as the hands, and subsequently spreads through other muscle groups closer to the brain. Such early symptoms as this, however, can hardly be noticed.
Neurotransmitters can also produce their effects by modulating the production of other signal-transducing molecules ("second messengers"messengers") in the post-synaptic cells (Cooper, Bloom and Roth 1996). Nine compounds -- belonging to three chemical families -- are generally believed to function as neurotransmitters somewhere in the central nervous system (CNS) or periphery. In addition, certain other body chemicals, for example adenosine, histamine, enkephalins, endorphins, and epinephrine, have neurotransmitter-like properties, and many additional true neurotransmitters may await discovery.
Amyotrophic Lateral Sclerosis (ALS), more commonly known as Lou Gehrig’s disease, is an adult onset neurodegenerative disease. It is characterized by the death of motor neurons in the motor cortex, brainstem, and spinal cord. 90% of all cases of ALS are sporadic with no familial history, while 10% of cases of ALS have familial history. 20% of cases of familial ALS cases are linked to mutations in the SOD1 gene. Currently, the pathogenesis of the disease is unknown. However, multiple studies show that there are several mechanisms contribute to the progression of the disease. These include mitochondrial dysfunction, glutamate excitotoxicity, oxidative stress, axonal dysfunction, reactive astrocytosis, protein aggregation, and mutant SOD1 expression.
Imagine if you loss control of your body but your mind stayed unaffected. You would be a prisoner in your own body, all leading up to your death sentence. That is the sad fate for the people diagnosed with Amyotrophic lateral sclerosis (ALS). “Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder was first described by Ran in 1850. This description was then expanded in 1873 by Charcot, who emphasized the involvement of the corticospinal tracts. In the United States, ALS is often referred to as Lou Gehrig's disease, after the famous ball player who was stricken by the disease in the midst of his career. (Yale School of Medicine, 2014)” In this paper will go through the definition, the process, the signs, the risk factors, etiology, and discus the known people that have suffered with this terminal disease.
Neurodegenerative diseases are characterized by the presence of protein aggregations with a varying protein content depending on the type of disease formed. One of the prime diseases resulting from protein inclusion bodies (aggregations) is Amyotrophic Lateral Sclerosis (ALS), which was the broad scheme of focus throughout this study (NIH 2017). ALS is a fatal neurodegenerative disease that causes death of motor neurons in the motor cortex, brainstem and spinal cord, as well as peripheral skeletal muscles (first in the limbs, then progressively beyond those distal extremities). This leads to motor problems, muscle weakness, and paralysis. These motor impairments are gradually progressive, and therefore ALS is usually fatal within 3–5 years
With motor neurone disease it attacks the nerves, in the brain and spinal cord. This means messages gradually stop reaching muscles, which leads to weakness and wasting. In the case study the
This is a neurodegenerative disease, meaning it results in progressive loss or death of neurons. It often starts off with effecting simple motor skills like writing and holding things, after a few months usually patients start losing the ability to walk, talk, or move any of their limbs. Although the brain trauma is what causes it, ALS has little-no-effect on the brain. This fatal disease is typically diagnosed around age 60 and most patients are given about 3-5 years to live after being diagnosed. It has been found that 10% of cases are shown as genetic. It was brought to attention that athletes were beginning to get diagnosed with ALS at a younger age than most. After extensive research in the early 2000’s, Brain Analyst, Dr. Mckee ran tests and finally came to the conclusion that the toxic proteins in the brains of ALS patients were coming from repeated blows to the head. It was then made evident why so many athletes in contact sports such as football, soccer, boxing, etc… were being diagnosed at such a young age and more frequently than
A neurotransmitter is a chemical that is stored in the axon terminal buttons, and when the neuron fires it is released into the synapse where it interacts with the receptor. There are numerous neurotransmitters in the human nervous system. They control many different behaviors that we experience. The first neurotransmitter that scientists discovered was acetylcholine. Acetylcholine and dopamine are both involved in motor movements, memory, and learning. Acetylcholine is found in many different parts of the nervous system such as the autonomic, central, and peripheral nervous systems while dopamine is mostly found in the brain. When acetylcholine is released from motor neurons, it goes to the muscle fibers which make the muscles to contract. Science has linked acetylcholine to Alzheimer’s disease. Dopamine is also involved in motor movement, memory, and learning. Even though acetylcholine and dopamine are involved in the same processes they trigger different parts of the behavior. Dopamine initiates motor movement while acetylcholine causes the contraction. Lack of dopamine causes Parkinson’s disease which is a disease that causes tremors and uncontrolled movement.