What Is Meant by Amyotrophic Lateral Sclerosis?
Amyotrophic lateral sclerosis, also called Lou Gehrig's condition, affects the motor neurons responsible for the muscle's voluntary movements. The voluntary movements of the muscle include activities like chewing, talking, walking, etc. In this condition, the nerves which send signals from the brain to the spinal cord and then to the muscles get affected and damaged. ALS worsens over time; the muscles get weaker and get wasted. This makes the person challenging to talk, walk, chew, and even breathe.
Most people affected by ALS die due to respiratory issues. There is no permanent cure for the condition. Still, the proper combination of treatment modalities can reduce the disease's pace at which it progresses and enhance the quality of the affected person's life.
How Does Amyotrophic Lateral Sclerosis Affect a Person?
As the disease progresses, the nerve cells get more affected, which impairs the signals sent from the brain to the muscles. There are two types of motor neurons; they are:
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Upper Motor Neurons: Consist of nerve cells in the brain and spinal cord. The upper motor neurons relay signals from the brain and spinal cord to the lower motor neurons. When the upper motor neurons are affected, it causes muscle stiffening.
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Lower Motor Neurons: This consists of nerve cells in the brainstem and the spinal cord, which receives signals from the upper motor neurons. These signals are then passed on to the muscles instructing them to move. If these nerve cells are affected, it results in a loss of muscle tone.
How Does ALS Affect Respiratory Functioning?
ALS usually affects breathing when the condition worsens as the muscles responsible for the movement of the chest and lungs get affected. The diaphragm muscles get damaged, separating the chest and the belly. The muscles present between the ribs also get affected. When ALS affects the respiratory system, which includes symptoms such as
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Breathing difficulties even at rest.
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Weak and continuous cough.
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Difficulty in clearing the throat and lungs.
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Excess saliva.
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Difficult to lie flat.
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Pneumonia, infection of lung air sacs.
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Respiratory system failure.
What Is the Causative Factor of Amyotrophic Lateral Sclerosis?
The exact cause of the condition is not evident. But some factors are believed to cause this condition, such as:
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Genetics: Mutation in some genes can result in ALS.
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Environmental Factor: Exposure to a toxic substance, virus, or physical trauma.
What Are the Signs and Symptoms Associated With ALS?
Some common symptoms are:
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Muscle cramp.
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Difficulty while walking.
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Weakness of the muscles.
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Weight loss.
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Breathing difficulties.
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Difficulty while chewing and swallowing.
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Depression and anxiety.
How Is Amyotrophic Lateral Sclerosis Diagnosed?
Diagnosing this condition is difficult as they show symptoms related to other medical conditions. Some tests are performed to rule out ALS; these are,
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Electromyogram: A small needle is inserted into various muscles through the skin during this procedure. During electromyography (EMG), the electrical activity of a muscle is recorded both during contraction and periods of rest. This helps to rule out the issue of whether it is present in the muscle or the nerve.
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Nerve Conduction Study: This procedure monitors the nerves' ability to signal to the muscles in various body areas. This helps to identify the presence of any nerve damage. Most of the time, EMG and nerve conduction tests are performed together.
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MRI (Magnetic Resonance Imaging): MRI shows detailed and precise brain and spinal cord images using magnetic fields and radio waves. MRI is used to rule out any presence of a spinal cord tumor, herniated disc, or any other condition that causes the signs and symptoms.
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Laboratory Test: Blood and urine tests are performed to rule out the presence of any other medical condition. Blood samples are used to measure the level of serum neurofilament light, which is usually very high in those people affected by ALS.
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Lumbar Puncture: In this procedure, a small amount of the spinal fluid is collected for laboratory testing with the help of a small needle. In people with ALS, the spinal fluid is typical.
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Muscle Biopsies: A muscle biopsy is performed when the doctors doubt muscle condition rather than ALS. In this procedure, a small muscle section is removed and sent for laboratory testing.
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Nerve Biopsy: A small section of never is removed and tested when the doctor doubts any nerve condition rather than ALS.
How Is ALS Managed?
There is no permanent cure for this condition, as the damage caused by ALS cannot be reversed. Treatment can effectively slow down the progression of the condition and mitigate the occurrence of complications. A group of doctors from various specialties plan treatment for the affected person to increase survival. The treatment modalities include:
1.Medication:
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Riluzole: This medication is taken orally, which can enhance life expectancy by 25 percent. Intake of this medicine can result in some side effects like dizziness, liver damage, and gastrointestinal conditions. A regular liver checkup is performed when the person is under this medication.
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Edaravone: This medication helps to reduce the progression of the condition, which is taken intravenously or orally as a liquid. Side effects caused are bruising, headache, and difficulty in walking. This is prescribed daily for about two weeks every month.
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Sodium-Phenylbutyrate-Taur Ursodiol: This medication is known to reduce the progression of the disease by 25 percent. This is present in powdered form, and the side effects caused are diarrhea, belly pain, nausea, etc.
2.Therapies:
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Breathing Improvement: In the long term, ALS tends to affect the person's breathing as the muscles of the chest and lungs weaken. The doctors monitor the breathing regularly, and devices like mechanical ventilators are provided to assist breathing at night.
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Physical Therapy: A physical therapist can help with pain, walking, mobility, etc., and trains the person to manage things independently. Practicing exercise regularly can help to maintain cardiovascular health and muscle strength.
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Occupational Therapy: Occupational therapy helps the affected person to be independent even if the arms and hands are weak. Some special equipment is used to do daily activities such as dressing, eating, bathing, etc.
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Speech Therapy: Speech therapists help to improve speech through various techniques.
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Nutritional Support: Nutritional specialist discusses with the family members and drafts a diet plan that is easy for the person to swallow and meets every nutritional need.
Conclusion
ALS is a motor neuro condition that causes muscle weakness and worsens over time. This causes difficulty in walking, swallowing, chewing, etc. This condition is not entirely curable, but the combination of various treatments can help to reduce the pace at which this condition progresses and help to prevent complications. The doctors can help to diagnose the condition at the earliest and provide the best treatment method to deal with this condition.
