Amyotrophic Lateral Sclerosis ( ALS ) - How It Effect Brain Cell

Amyotrophic lateral sclerosis belongs to the category of motor neuron disease. In simpler terms, it is a neurological disease which is progressive in nature and leads to improper functioning of the nerves that are responsible for the movement of the muscles. This is a fatal disease as in the later stages of the disease the breathing capability of a person gets hampered. Amyotrophic lateral sclerosis can be referred to as Lou Gehrig's disease. The ALS disease affects the motor neurons present in the brain and spinal cord. As this disease progresses the motor neurons tend to degenerate and die. This hampers the voluntary movement of the muscles. A person suffering from the condition of ALS is no longer in control of his/her arms, legs, and face. Only 5 percent of the people who have been diagnosed with ALS tend to live for nearly 20 years. Nearly half of the people suffering from this condition tend to survive for only 3 years after the diagnosis has been made. In some rare cases like that of famous physicists Stephen Hawking, a person can live for more than 20 years after the diagnosis.

The signs and symptoms exhibited by a person suffering from the condition of ALS are given below

• Twitching or cramping in the tongue, shoulders, and arms
• Weakness in ankles, hands, feet, and legs
• Difficulty in carrying out the routine activities
• Pain
• Tiredness
• The problem in holding the head in an upright position
• Difficulty in swallowing the food
• Enhanced clumsiness
• Problems with mucus and saliva
• Difficulty in speaking
• During the later stages breathing difficulty may also develop
• The problem in decision making
• Hampered memory leading to dementia

The researchers and doctors have still not been able to identify the cause of ALS. To understand the possible causes of ALS the researchers are carrying out several studies. Some of the studies which are being carried out are given below Disorganized immune response: in some cases, the immune system of an individual may start attacking the healthy cells of one's own body. This can lead to the destruction of the nerve cells. Gene mutation: the presence of several genetic mutations can cause inherited amyotrophic lateral sclerosis. However, the symptoms of both the inherited and non-inherited ALS are the same.

Protein mishandling: when the nerve cells are not able to handle the proteins present within them this leads to a slow accumulation of the protein in an abnormal form within the cells. This mishandling of protein can lead to the death of the nerve cells Chemical imbalance: glutamate is a chemical substance present in the brain. It is also present in the spinal cord around the nerve cells. People who suffer from the problem of ALS usually have an increased level of glutamate. It is a well-known fact that enhanced levels of glutamate can be toxic for the nerve cells.

There are some environmental factors as well that can lead to the development of ALS condition in an individual like

• Military service
• Exposure of enhanced levels of agricultural chemicals
• Electrical or mechanical trauma
• Exposure to increased levels of heavy metals

There is no proof which suggests that if certain changes are made to the lifestyle of an individual then the risk of ALS would reduce.

ALS can have severe detrimental effects on the body of an individual who is suffering from this condition. Some of the effects of the disease which may be observed are

• Difficulty in speaking: the majority of the people who have been diagnosed with the problem of ALS develop difficulty in speaking. This problem with speech initially begins with little slurring of words but it eventually develops into a severe problem. It becomes increasingly difficult to understand the speech of the people suffering from this condition. In order to communicate the people with ALS make use of communication technologies.
• The problem in eating food: the damage to the muscles that control the process of swallowing can lead to dehydration and malnutrition in people with ALS. There is also a chance that they might develop pneumonia if food, saliva or liquid passes into their lungs. To ensure proper hydration and nutrition people with ALS make use of the feeding tube.
• The problem in standing and sitting: the damaging of the nerve cells of the brain and spinal cord causes severe weakness in the limbs of the people with ALS. People suffering from ALS often make use of wheelchairs to go from one place to another.
• Breathing difficulty: as the ALS diseases progress in an individual it tends to paralyze the muscles which help in breathing. People with ALS make use of either BiPAP or CPAP machine to provide assistance in breathing especially at night.

It is very difficult to diagnose ALS in its early stages as it tends to mimic the symptoms of several other neurological disorders. For the purpose of diagnosing and ruling out other neurological conditions a doctor may order the following diagnostic tests

• Blood and urine test is ordered by the doctor rule out other plausible causes of the symptoms.
• Electromyogram (EMG): it is a test in which needle electrodes are inserted through the skin into the muscles of the patient. This test helps in evaluating a muscle’s electrical activity in different states like while relaxing and contracting. If any irregularities are observed then it can provide assistance to the doctor in diagnosing ALS.
• Nerve conduction study: this study helps the doctor in taking into account the ability of the muscles to send the impulses to various parts of the body. This test can also help in understanding whether a person has nerve damage or any other disease of the muscles.
• MRI: an MRI scan is ordered by the doctor to rule out other conditions which may be causing similar symptoms.

The damage done by the amyotrophic lateral sclerosis cannot be reversed through any treatment. however, treatment may be helpful in slowing down the progression of the symptoms associated with the disease and prevent further complications. Currently, there are two medications that have been approved by the FDA which help in slowing down the progression of the disease.

Radicava (Edaravone): this drug got approved in the year 2017 by the FDA. The clinical trial of the drug indicated a decline in the progression of the disease. The doctor gives these drugs to the patient in the form of an intravenous infusion for 10 to 14 days in a row within a month. Some of the side effects associated with the use of this drug are shortness of breath, bruising, swelling and hives.

Rilutek (Riluzole): this drug was approved in the year 1995 by the FDA. This drug helps in slowing down the progression of the disease by reducing the glutamate level of the body. This medicine is available in the form of oral tablets. The side effects associated with oral consumption of Rilutek include dizziness, flatulence, and abnormal liver function. Some of the therapies which may be needed by a person suffering from the problem of ALS include

Physical therapy: This therapy helps in managing the pain and the issues related to mobility. A physical therapist may also help the individual in adjusting to walker, brace, and wheelchair.

Occupational therapy: despite the disability of the arms and the legs occupation therapy can help a person with ALS to find ways of remaining independent and performing their daily activities.

Speech therapy: speech therapy helps people with ALS by teaching them adaptive techniques to speak clearly.

Nutritional support: nutritionist help in preparing meals that are not only rich in nutritional values but they are easy to swallow as well.

ALS is a progressive neurological disease which has damaging effects on the body of an individual suffering from it. There is no treatment available that can reverse the effect of this disease. People who have been diagnosed with this condition must practical about their future. A proper plan is required to be made so that possible restrictions do not hamper the daily activities of a person.