Amyotrophic Lateral Sclerosis - Muscular Atrophy: an Annotated Bibliography

Have you ever heard of ALS, better known as Lou Gehrig’s disease? For many people, ALS is a disorder that they may not know much about. I never heard of it either until my father was diagnosed with this disease in 2006. Because there is no known cure, it is important to detect this disease early, so that proper treatments and preparation can be done before it’s too late.

People first need to know what ALS is. ALS is very difficult to diagnose. So far there is no

The two different forms are Sporadic and Familial. The Sporadic ALS makes up approximately 90 to 95 percent of the people who have this disease in the US. The other 5 to 10 percent cases of ALS in the US are made up by Familial ALS. When someone is diagnosed with Familial ALS it means that they inherited it.

There are two different types of ALS, sporadic and familial. Sporadic ALS is the most common, 90 to 95 percent of people with this disease have sporadic ALS. However, the other 5 to 10 percent of people have familial ALS, meaning it is inherited. People with familial ALS have a 50 percent chance that their offspring will inherit the gene mutation and could possibly inherit the disease. Regardless if it is sporadic or familial, other than the gene being passed along the disease is the same. Amyotrophic lateral sclerosis (ALS), is a neurological disease that mainly involves the nerve cells. Nerve cells are responsible for controlling voluntary muscle movement; such as walking, talking, and eating. However, this disease causes the upper and lower motor neurons to degenerate or die. When

Amyotrophic Lateral Sclerosis, otherwise known as ALS or Lou Gherig’s disease, is a neurological disease which attacks nerve cells causing them to die and leave the muscles paralyzed. People with ALS lose strength and control of muscles, weakening them and causing them to start to wear away. Eventually the muscles stop working all together and cause paralysis in places such as arms, legs and other parts of the body. When the nerve cells in the diaphragm and chest wall are attacked and the muscles are paralyzed, the person can lose the ability to breathe without assistance, or sometimes at all. Respiratory failure is the cause of death for most people with ALS. Currently there are approximately 2500-3000 Canadians that are living with ALS and according to www.als.ca “Every day two or three Canadians die of the

As many as 30 thousand Americans are currently living with or have been diagnosed with ALS in the United States. ALS is a life threatening disease that scientists have yet to discover treatments for, but as of right now, ALS can not be treated. ALS can be hard to understand, many wonder how does one get ALS? What are the symptoms ? How is ALS diagnosed? Treatment options, and what is the prognosis of the disease?

There are three forms of ALS. “Familial”, which is hereditary and passed through the genes. Another form of ALS Nonhereditary, which is “sporadic’ and ALS that targets the brain called “ALS/Dementia”. ALS is typically not inherited.

Amyotrophic derives from Greek having ‘a’ mean ‘no’, ‘myo’ mean muscle and ‘trophic’ mean ‘nutrition’ translating to ‘no muscle nutrition.’ Lateral is the area in the spinal cord where signal cells are located and Sclerosis meaning scarring or hardening which is the result of no muscle nutrition. ALS is a progressive, degenerative disease meaning that as time passes, the conditions worsen and that it causes deterioration. The way ALS can be developed is through repetitive head trauma that causes toxic protein from the brain called “tau” to seep into the spinal cord and lead to the symptoms of the disease. The most heart wrenching aspect of the disease is that it attacks and destroys every part of the body, such as voluntary muscles, except the brain and the bladder. This essentially leads to the person with it able to know what is going on but has no control over it. Since ALS also impairs speech and mobility, one feels basically lifeless except for the fact that the brain can still process and think. The disease affects the voluntary muscles of one, throughout later stages, all mobility is lost and leads to paralyzation and eventual death. The ages where people are typically diagnosed with it are forty to seventy and the life expectancy is three to five years. However, athletes have been getting diagnosed with it at a younger age being 25 to 35 years of

After many years of studies there is a way to manage the symptoms and to extend the life of the patients. In 1994, Rilutek was approved and is the only drug used for ALS (Bensimon G, Lacomblez L. Meninger V 1994). This drugs benefits still really low when the progression of ALS is really fast, Rilutek only slow the progression of the disease by ten percent. Unfortunately, the benefit is not the same for every patient and many can not tolerated or see any benefits from taking the medication. Also, the high prices for Rilutek make it even more difficult for patient without any insurance or monetary availability to pay for the drug. The care for an ALS patient required a lot of education, counseling and symptoms

Intervention arms: The trial here wishes to evaluate a new drug for ALS compared to placebo. As there is a drug available to treat ALS (Riluzole)(1), the decision to use placebo over available treatment would need to be clearly justified and evaluated in detail.

Etiology - Amyotrophic lateral sclerosis is a disease that affects the nervous system. It affects the brain and the spinal cord and it controls the voluntary muscle movements and disables the body from being able to control movements such as speech, swallowing, and movement of the limbs. Unfortunately, there is no cure for Amyotrophic lateral sclerosis. There are two different kinds of amyotrophic lateral sclerosis, sporadic and familial. The sporadic type means that there is no specific cause for the disease. The familial type only affects about 5 to 10 percent of cases. This disease isn’t easily understood due to the severity of the disease. It is hard for many doctors to understand what causes this disease in most patients. Some things that could contribute to amyotrophic lateral sclerosis are: free radical damage, an immune response that targets motor neurons, imbalances in the chemical messenger glutamate, or a buildup of abnormal proteins inside nerve

Amyotrophic lateral Sclerosis (ALS) is a neurodegenerative disease. It is a disease which involves the degeneration of motor neurons. Not only does it degrade the motor neurons, It also degenerates the corticospinal neurons of the motor cortex. ALS is also known as Lou Gehrigs disease. This name came about after a New York Yankees baseball player developed this disorder and died from it in 1936. ALS is a heterogenous disorder. Multiple genetic factors contribute to the onset of ALS. More than 20 genes are known to have a role to play in ALS pathogenesis.In most cases of ALS, the disease is not hereditary. The cause of ALS is still unknown. A small amount of cases are made up of a few hereditary forms of ALS.

Neuromuscular conditions include issues involving nerves and muscle. Amyotrophic Lateral Sclerosis is a disease in which degeneration of the motor cells happens. The muscles begin to deteriorate which causes muscle weakness. This disease doesn’t weaken a person’s intellect, eyesight, hearing, taste, the ability to smell, and touch. Myasthenia gravis is a chronic disease in which antibodies destroy the nerves and muscle connections. This is the starting point to communication problems between the nerves and the muscles which results in weakening of the skeletal

The manifestations of ALS are caused by the location of motor neuron death. When upper motor neurons die, the symptoms include problems controlling fine movements, spasms, dysphagia, dysphonia and dysarthria (Porth & Matfin, 2009). “Manifestations of lower motor neuron destruction include fasciculations, weakness, muscle atrophy, and hyporeflexia” (Porth & Matfin, 2009, p. 1284). Patients with early signs of ALS usually complain of feeling weak on one side, which is due to the slowing of electrical impulses to that group of muscles (Ignatavicius & Workman, 2010). Since the impulses are slowed, they are not receiving adequate electrical stimulation to move and the person feels weak. As the disease progresses, all the motor neurons die and are not regenerated so, the patient is left paralyzed, losing the ability to speak, swallow and breathe (Ignatavicius & Workman, 2010).

Regardless of which occur first, affected individuals will generally experience most of the symptoms as the disease progresses. Specifically, symptoms that include difficulty swallowing, moving, forming words, tight muscles, spasticity, and/or exaggerated reflexes occur when the upper motor neurons have been damaged. When lower motor neurons are damaged, symptoms such as muscle weakness, atrophy, cramping, and twitching will occur. In some cases, a few of the muscle neurons that