Amyotrophic Lateral Sclerosis (ALS) is a group of rare neurological diseases involving nerve cells (neurons) responsible for regulating voluntary muscle movement. Voluntary muscles produce activities such as chewing, walking and talking.
The disease is progressive, meaning the symptoms worsen over time. Currently, there is no cure for ALS, and there is no effective treatment to prevent or reverse the progression of the disease.
ALS belongs to a wide range of disorders known as motor neuron diseases, which gradually lead to degeneration (atrophy) and death of motor neurons. Motor neurons are nerve cells that extend from the brain to the spinal cord and muscles throughout the body. These motor neurons initiate and provide essential communication connections between the brain and the spontaneous muscles.
The message travels from the motor neurons in the brain (called upper motor neurons) to the motor neurons in the spine and from the motor nuclei of the brain (called the lower motor neurons) and from the spinal cord and motor nucleus of the brain to a specific muscle or muscle.
In ALS, the upper motor neurons and lower motor neurons degenerate or die and stop sending messages to the muscles. When unable to function, the muscles gradually weaken, begin to flex (called attraction), and become dysfunctional (atrophy). Eventually, the brain loses the ability to initiate and control voluntary movements.
Early symptoms of ALS are usually muscle weakness or stiffness. Gradually voluntary control affects all muscles and individuals lose the ability to speak, eat, walk and breathe.
Most people with ALS die of respiratory failure, usually within 3 to 5 years of the first appearance of symptoms. However, 10 per cent of people with the disease live for ten or more years.
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Who Gets ALS?
The Centers for Disease Control and Prevention estimates that ALS is among 14,000 – 15,000 Americans in 2016. ALS is a common neuromuscular disease worldwide. It affects people of all castes and ethnic backgrounds.
Several potential risk factors for ALS are:
Ages. Although the disease can occur at any age, symptoms usually develop between the ages of 55 and 75 years.
Gender. Women are slightly more likely than men to develop ALS. However, in our age, the difference between men and women disappears.
Race and ethnicity. Caucasians and non-Hispanics are more likely to develop the disease.
Some studies suggest that military veterans are approximately 1.5 to 2 times more likely to develop ALS. Although the reason for this is unclear, potential risk factors for veterans include exposure to lead, pesticides and other environmental toxins. The US Department of Veterinary Affairs has identified ALS as a service-related disease.
Most cases of ALS (90 per cent or more) are occasionally considered. This means that the disease occurs randomly without risk factors and a family history of the disease. Although family members of those with scattered ALS are at higher risk for the disease, the overall risk is much lower, and most do not develop ALS.
Family (Genetic) ALS
About 5 to 10 percent of all ALS cases are familial, meaning that one person inherited the disease from their parents. The domestic form of ALS usually requires only one parent to carry the gene that caused the infection.
Family ALS has been found to cause mutations in more than a dozen genes. About 25 to 40 percent of family cases (and a small percentage occasionally) are caused by a genetic defect called “chromosome 9 open reading frame 72,” or C9ORF72.
Interestingly, the same mutation is associated with the degeneration of the frontal-temporal lobe of the brain, leading to frontal-temporal lobe dementia. Some people carrying this mutation may show symptoms of both motor neuron and dementia symptoms (ALS-FTD). Another 12 to 20 percent of family cases are caused by mutations in the gene that provide clues to the production of the enzyme copper-zinc superoxide dismutase 1 (SOD1).
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What Are the Symptoms?
The onset of ALS symptoms may be subtle enough to be ignored. Still, gradually these symptoms develop into a more pronounced weakness or atrophy, which may cause the physician to suspect ALS. Some of the early features are:
- The attraction of arms, legs, shoulders or tongue (muscle cramps)
- Muscle cramps
- Tight and tight muscles
- Muscle weakness that affects one arm, one leg, neck or diaphragm.
- Thin and resonant speech
- Challenging to chew or swallow.
For most people, the first sign of ALS appears on the hand or arm when they are having trouble with simple tasks such as pressing a button, typing or turning a key on a key. In other cases, the symptoms initially affect one of the legs, and people may experience strange gait or run or notice that they are mostly tripping or making mistakes.
When symptoms begin in the arms or legs, it is referred to as “organ onset” ALS. Other people first notice speech or swallowing problems called “bulb onset” ALS.
Despite the progression of the disease, the symptoms first appear here, with muscle weakness and atrophy spreading to other parts of the body. Individuals may develop problems such as walking, swallowing (dysphagia), speaking or speech (dysarthria) and breathing (dystopia).
Although the sequence of emerging symptoms and the rate of progression of the disease varies from person to person, eventually individuals will not be able to stand or walk, get out of bed on their own, or use their hands and arms.
People with ALS usually have trouble swallowing and chewing food, which generally makes it harder to eat and increases the risk of suffocation. They burn calories faster than most people who do not have ALS. Because of these factors, people with ALS can lose weight quicker and switch to malnutrition.
As people with ALS generally maintain the ability to develop higher mental processes such as reasoning, remembering, understanding and problem solving, they become aware of the progressive loss of their performance and become prone to anxiety and depression.
There is growing evidence that a small percentage of individuals may experience language or decision-making problems and that some form of dementia may also develop over time.
People with ALS have difficulty breathing due to the weakening of the respiratory muscles. They eventually lose their ability to live on their own and have to rely on a ventilator. Affected individuals are at increased risk of pneumonia in the later stages of the disease. In addition to uncomfortable muscle aches, some people with ALS may develop traumatic neuropathy (neurological disease or damage).
How is ALS Diagnosed?
No single test can provide an accurate diagnosis of ALS. ALS is primarily tested based on a detailed history of symptoms and signs observed by the physician during the physical examination, along with a series of tests to detect other mimetic diseases. However, the presence of upper and lower motor neuron symptoms strongly suggests the presence of illness.
Physicians review a person’s complete medical history and regularly perform neurological tests to determine if symptoms such as muscle weakness, muscle wasting, and elasticity are progressively worse.
ALS symptoms in the early stages of the disease may be similar to other, more treatable diseases or a variety of disorders. Appropriate testing may rule out the possibility of other conditions.
Muscle and Imaging tests
Electromyography (EMG), a unique recording technique that detects the electrical activity of muscle fibres, can help diagnose ALS. Another standard test is a neural conduction study (NCS), which measures the electrical activity of nerves and muscles by assessing the ability of a nerve to send signals to a nerve or muscle.
For example, specific abnormalities in the NCS and EMG may indicate that the person has peripheral neuropathy (damage to peripheral nerves outside the brain and spinal cord) or myopathy (muscle disease) rather than ALS.
A physician may also order a magnetic resonance imaging (MRI) test. This non-surgical procedure uses a magnetic field and radio waves to produce detailed images of the brain and spinal cord. Standard MRI scans are usually common in people with ALS.
However, they can reveal other problems that can cause symptoms such as a spinal tumour, herniated disc in the neck that compresses the spine, syringomyelia (a cyst in the spinal cord) or cervical spondylosis (affecting abnormal wear). Neck).
Depending on the person’s symptoms, test results and test results, the doctor may order tests on blood and urine samples to rule out the possibility of other diseases.
Testing for other Diseases and Disorders
Neurological disorders such as multiple sclerosis, post-polio syndrome, multicellular motor neuropathy, and spinal and bulbar muscle degeneration (Kennedy’s disease) also mimic specific symptoms of the disease. Physicians should consider them as an attempt to diagnose. Comfort and muscle aches also occur in benign conditions.
This diagnosis and due to various diseases or disorders is similar to the early stage of ALS disease, due to which people can get a second nervous impression.
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Treatment or treatment to stop or reverse the progression of ALS is currently unknown. However, FDA-approved drugs Rilozol (brand names Rilutech, Teglutic) and Edaravone (Radicawa) have been shown to slow the progression of ALS. Also, many fair clinical trials are going on around the world, giving important information about how to deal with this disease.
As the search for effective treatment and prevention continues, multi-disciplinary teams around the world are helping patients and their families with the many challenges of living with ALS. These teams of specialists use tools and therapies that help patients manage their ALS symptoms and maintain their independence and quality of life for those with the disease. This multi-disciplinary approach has also been shown to prolong public survival with ALS.
Treatment and intervention may include:
- Proper body position
- An exercise regimen, physical and occupational therapy
- Supports devices and people who help walk
- Braces and splints for legs and arms
- Custom wheelchair
- Home evaluation makes it easy to turn around the home
- Technology tools that help people communicate
- Tips for easily swallowed foods and fluids
- Nutritionist help
- Feeding tube
- Diaphragm pacers
- Aids for breathing
Many people with ALS and other neurological disorders decide to take part in research studies to help test new drugs and therapies aimed at curing the disease. To learn more about these studies, visit the US National Institute of Health Clinical Trial Registry.
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