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. …show more content…
Riluzole or rilutek, 2-amino-6-trifluromethoxy benzothiazole, is the only medication available to treat ALS. However, it cannot cure the disease, it can only slow down the progression. Riluzole is only moderately affective in slowing the progression because it only prolongs a patient’s life by three months. Early research suggested that Riluzole acted as a inhibitor of glutamate release, however more recent studies suggest that Riluzole has several functions including a potent neuroprotective agent that modulates GABAergic systems as well as an antagonist of protein kinase c and neuronal nitric oxide. It has also been found that Riluzole acts as a Ca2+, Na2+ channel blocker. It also inhibits pertussis toxin/cholera toxin sensitive G-proteins. The main metabolic pathway of Riluzole in humans is the hydroxylation of the primary amine group producing N-hydroxyriluzole. In humans there are two different types of hepatic enzymes engaged in the biotransformation of Riluzole in the liver. These two types of hepatic enzymes are monooxygenases and UDP glucuronosyltransferases. Riluzole is predominately metabolized by CYP1A2 …show more content…
At the Washington School of Medicine, scientists performed an experiment using glial cell line derived neurotrophic factor protein, GDNF, for a single growth-factor therapy. GDNF is a protein that is important in ensuring neuron survival and development in the human body. They performed multiple experiments that were designed to answer whether or not target-derived GDNF, skeletal muscle (myo-GDNF/G93A-SOD1) and centrally derived GDNF, astrocytes in the CNS, could be effective treatments for amyotrophic lateral
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.
Neurodegeneration is used mainly for diseases that are characterised by progressive loss of structure and function of neurons. There are many neurodegenerative diseases including amyotrophic lateral sclerosis that...
Amyotrophic Lateral Sclerosis is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Amyotrophic Lateral Sclerosis is better known as ALS or Lou Gehrig’s disease. Amyotrophic Lateral Sclerosis was not brought to International or national attention until Famous New York Yankees baseball player, Lou Gehrig, was diagnosed with it in 1939. Jon Stone, the writer and creator of Sesame Street, was also diagnosed with Amyotrophic Lateral Sclerosis. Amyotrophic Lateral Sclerosis is very deadly and it physically handicaps a person as it progresses. There are two types of Amyotrophic Lateral Sclerosis, Sporadic and Familial. Sporadic is the most common cause in some cases and Familial is inherited, which is rare. Amyotrophic Lateral Sclerosis is one of the most aggressive muscular atrophy disorders, it has many signs and symptoms, and it can be treated but cannot be cured.
Amyotrophic Lateral Sclerosis, commonly referred to as ALS, is a disease that can alter the daily life of a human in monumental and unending ways. In one of her articles about ALS, Caroline Ingre (2015) states that the disease is a “fatal neurodegenerative disorder” and further supports this by noting how the disease is marked by the degeneration in motor neurons in the brain, brainstem, and spinal cord (p. 181). This basically means that
As stated by the A.D.A.M Medical Encyclopedia, “ALS affects approximately 5 out of every 100,000 people worldwide” (Amyotrophic lateral sclerosis- National Library of Medicine, 2012). ALS is also known as amyotrophic lateral sclerosis. It is often called Lou Gehrig’s disease. The name derived from the famous New York Yankees player, Lou Gehrig, who lost his battle to ALS in 1941. Out of this tragedy came some light; ALS had the attention of the nation.
Lewy body dementia (LBD) is a progressive dementia and the second most common type of dementia after Alzheimer's disease dementia. Protein deposits, called Lewy bodies, develop in nerve cells in the brain regions involved in motor control such as, thinking, memory and movement (Mayo Clinic). LBD has similar symptoms with both Alzheimer's disease and Parkinson's disease and is often misdiagnosed. Of particular note, Robin Williams suffered from this debilitating disease which lead to his suicide in 2014. It was not discovered until his autopsy three months after his death.
performance that involves, but is not limited to, a loss in at least 2 of the
Alzheimer’s disease, named after Dr. Alois Alzheimer, is a disease that is on the rise in America and the rest of the world. People should learn as much as they want about this disease, because as you age, your chances of becoming an Alzheimer’s Disease, or AD, patient increases. It is estimated that approximately 3 percent of Americans between the ages of 65 and 74 have the illness, and more than half of all people over age 85 have the ailment.
Alzheimer’s disease is a complex illness that affects the brain tissue directly and undergoes gradual memory and behavioral changes which makes it difficult to diagnose. It is known to be the most common form of dementia and is irreversible. Over four million older Americans have Alzheimer’s, and that number is expected to triple in the next twenty years as more people live into their eighties and nineties. (Johnson, 1989). There is still no cure for Alzheimer’s but throughout the past few years a lot of progress has been made.
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.
Alzheimer’s disease or AD is an incurable disorder of the brain that results in loss of normal brain structure and function. In an AD brain, normal brain tissue is slowly replaced by structures called plaques and neurofibrillary tangles. The plaques represent a naturally occurring sticky protein called beta amyloid and in an Alzheimer’s brain, sufferer’s tend to accumulate too much of this protein. Neurofibrillary tangles represent collapsed tau proteins which, in a normal brain along with microtubules, form a skeleton that maintains the shape of the nerve cells. In Alzheimer’s disease, the tau proteins break loose from their normal location and form tangles. Without the support of these molecules, nerve cells collapse and die. As normal brain structure is lost with progression of the disease, brain function also degenerates. Patients afflicted with Alzheimer’s disease display a gradual mental decline. Initially, and most apparently, there is a loss of short-term memory. Eventually, as a patient progresses to later stages of the disease, the brain becomes so damaged that patients can no longer communicate or recognize immediate family or even themselves. They have difficulty walking and standing and frequently fall. In the final stages, they lose bladder and bowel control and have difficulty with swallowing, frequently leaving them malnourished and dehydrated. Eventually, they are forced to remain bedridden and, without the help of life-prolonging measures provided in a hospital, die. However, this level of deterioration is severe and may take as long as twenty years. Because of the disease’s slow progress and its usual later start in a person’s life, a victim of AD will usually die first of natural causes. Under the objectives ...
In this day and age, it seems as though almost everyone has experience a loved one taken away form a very serious disease known as Alzheimer’s disease. Alzheimer’s disease is unbelievably devastating for everyone affected by it. This disease is causing major economical problems such as less occupancy in the nursing homes, and hospitals due to the rising population of elderly men and women being diagnosed with it everyday. Because there is not yet a cure for this disease and the percent of the population being diagnosed keeps rapidly rising, more time and money needs to go towards Alzheimer’s research.
Alzheimer's Disease Introduction to Alzheimer's Alzheimer's disease is a progressive, degenerative disease of the brain. It was first described by the German neuropathologist Alois Alzheimer (1864-1915). in 1905. This disease worsens with advancing age, although there is no evidence. that it is caused by the aging process.
Amyotrophic lateral sclerosis (ASL), Lou Gehrig’s disease, a brutal, unforgiving illness of the neurological system with no known cure.
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