Amyotrophic Lateral Sclerosis or ALS is an Insidious Disease

Muscular Dystrophy or ALS (Amyotrophic Lateral Sclerosis), a hidden threat

Droopy eyelids, weak arms and legs are symptoms of Muscular Dystrophy, a hidden danger caused by the body's immune system working abnormally. Muscular Dystrophy is more common in women than men, especially in young adults, with an average of 3 people per 100,000 population, making it a hidden danger. Although the outside may still look healthy, the internal muscular condition may be gradually weakening.

At present, it is not clear why nerve cells carrying commands are deteriorating, but it is assumed that Muscular Dystrophy, ALS, is caused by multiple factors that lead to the disease.

What is ALS?

The abbreviation ALS stands for Amyotrophic Lateral Sclerosis, a disease that is not directly related to muscles, but is a result of abnormalities in the nerve cells that carry commands, causing muscle weakness due to the lack of nerve cells to control them. These nerve cells are found in the spinal cord and brain, and they gradually deteriorate and die. Because ALS is a disease caused by abnormalities in motor neurons, it is also known as motor neuron disease (MND) or amyotrophic lateral sclerosis. In the United States, this disease is known as Lou Gehrig's disease, named after the famous baseball player who was diagnosed with the disease in 1930.

Cause of Disease:

Currently, it is still uncertain why nerve cells degenerate due to loss of command. It is assumed that the disease, Amyotrophic Lateral Sclerosis (ALS), is caused by several factors that lead to the disease. These include certain genetic factors that are not yet fully understood, making nerve cells more vulnerable to deterioration than others. Environmental factors, such as exposure to toxins like insecticides, heavy metals, radiation, or certain types of viruses can stimulate nerve cells to malfunction. In addition, aging contributes to the deterioration of cells over time, which is due to mitochondria, the battery that powers the cells, being abnormal. However, these assumptions have not been clearly proven.

Who is at risk:

In the UK, ALS is found in every 100,000 people, with approximately 2 people per year. The average age of onset of the disease is between 60-65 years old. Therefore, the likelihood of developing ALS in older individuals is greater than in younger ones. Generally, the disease is more commonly found in women than men, with approximately 1.5 times more prevalence. Additionally, about 90% of ALS patients do not have a clear genetic cause, so individuals with no clear family history have a very low risk of developing the disease in future generations. Moreover, there is no clear evidence that athletes are more susceptible to the disease than other professions.

Symptoms and Progression of the Disease:

At the onset, the patient will experience weakness in the muscles of one side of the body, such as the hand, arm, leg, or foot. For example, they may not be able to lift their arm above their head, hold objects, or may experience dropping of the wrist or ankle. They may also have difficulty walking, climbing stairs, or getting up from a seated position. The muscle weakness will gradually worsen until it affects both sides. Some patients may experience weakness in both arms or legs from the beginning. In addition to muscle weakness, there may also be muscle atrophy and twitching. Some patients may first see a doctor because of breathing difficulties due to weak respiratory muscles, blurred speech, or choking.

Some patients may be diagnosed with weak respiratory muscles at their first doctor's visit, which causes easy fatigue, especially when lying down or waking up in the middle of the night. Because the symptoms of ALS are similar to those of other diseases, patients with ALS in the early stages may be diagnosed with other diseases. As the symptoms worsen, the patient will have muscle weakness and atrophy in the arms, legs, and speech muscles, causing difficulty in swallowing and eventually requiring a feeding tube. Breathing muscles may also become weak to the point of requiring a ventilator. There is currently no cure for ALS, and approximately 50% of patients die within about 2 years after experiencing symptoms. The cause of death is often due to respiratory failure and pneumonia, resulting from aspiration.

How to diagnose ALS, a muscle weakness disease?

As ALS (Amyotrophic Lateral Sclerosis) is an incurable disease, its diagnosis is important and must be conducted by a neurologist specializing in geriatrics, who has experience in taking medical history, performing physical examinations, and conducting further laboratory investigations. Additional laboratory tests, such as nerve conduction studies and electromyography, can help support the diagnosis of ALS.

Once diagnosed with the disease, how should you manage it?

Once diagnosed with the disease, it is important to receive quality care and encouragement from caregivers and family members, as this will help the patient to not lose hope and maintain a good quality of life. In the future, there may be advancements in the treatment of ALS, similar to other types of neurological diseases.

Currently, the only medication accepted in the medical field to slow down the progression of ALS is Riluzole (Rilutek), which has the ability to inhibit glutamate, a neurotransmitter that, if too much is present, can cause cell death. There is currently no evidence to support the use of other medications or stem cell therapy for treating ALS.

In addition to medication, managing the disease through a holistic approach is also important for overall health benefits, such as regular exercise, engaging in activities and physical therapy to strengthen weakened muscles, preventing contractures that may result from lack of muscle use, proper nutrition, and sufficient rest. If the patient has difficulty lying down or becomes fatigued due to weakened respiratory muscles, the use of non-invasive ventilation devices at home can help the patient sleep comfortably and improve their quality of life.

Soft and weak muscles (dangerous) if left untreated can lead to complications!

The danger of muscular dystrophy is that patients can still perform daily activities normally at the beginning of the day, but gradually muscle weakness can occur. If the patient takes a short break or stops using the affected muscles, they can recover and use them normally again. However, it is better if the patient does not allow the symptoms of muscle weakness to recur repeatedly, as the severity of the disease can lead to other complications such as:

• Respiratory failure due to abnormal respiratory muscle function
• Toxic thyroid syndrome due to abnormal thyroid gland function
• Risk of aspiration due to abnormal throat muscle function
• Risk of autoimmune disease or lupus (SLE)

If you or someone close to you experience any symptoms of muscular dystrophy, it is recommended to take a break and seek medical diagnosis and treatment to prevent the possibility of complications.

Best wishes from Vibhavadi Hospital.

Information source: Daily News.