Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

Myasthenia gravis is one of the neuromuscular autoimmune abnormalities and disorders that weaken voluntary muscle movements in humans. According to statistics in the United States of America, it is estimated that 20 people out of every 100,000 people are affected by this rare disease. Of course, myasthenia gravis is not something that can be easily diagnosed in the first place. This complication can appear in different people regardless of age, gender and even race. On the other hand, myasthenia gravis will cause many problems in terms of endangering a person's physical health.

BingMag.com Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

Myasthenia gravis is one of the neuromuscular autoimmune abnormalities and disorders that weaken voluntary muscle movements in humans. According to statistics in the United States of America, it is estimated that 20 people out of every 100,000 people are affected by this rare disease. Of course, myasthenia gravis is not something that can be easily diagnosed in the first place. This complication can appear in different people regardless of age, gender and even race. On the other hand, myasthenia gravis will cause many problems in terms of endangering a person's physical health.

As mentioned, myasthenia gravis is an autoimmune condition that causes the wasting of striated and voluntary muscles. . With the passage of time, the amount of muscle weakness also increases. The muscles of the body from the respiratory area to the muscles of the upper and lower limbs can be involved in this rare and dangerous disease. In fact, the word myasthenia gravis has roots in two languages, German and Latin. Gravis is derived from the word Grave which means acute and serious. Myasthenia also means muscle weakness. Unfortunately, at the time of writing this article, a definitive treatment for this disease has not been discovered, and there are only certain treatment processes to reduce the progress of the signs and symptoms of the disease routinely used in different societies. In general, there are currently several treatment processes at the level of different societies in order to control the symptoms and maintain the quality of life of the patients to some extent. If the disease of myasthenia gravis is under the control of the attending physician, usually patients will experience a normal quality of life and do not suffer constantly like other rare diseases.

Signs and symptoms of myasthenia gravis disease

  • Weakening of the eye muscles (also known as ocular myasthenia)
  • Falling. eyelid on one or both sides (ptosis)
  • double vision or blurred vision (diplopia)
  • changes in facial muscles
  • difficulty swallowing
  • Short and difficult breathing
  • Inability to speak properly (dysarthria)
  • Weakness of the muscles of the hands, feet and neck muscles

Sometimes Sometimes it is possible that the muscles of the respiratory area have problems and the person faces a serious danger in breathing. In this case, emergency measures should be taken immediately.

Myasthenic crisis; an emergency disorder that must be taken seriously Do not breathe. In this case, the members of the emergency medical team should reach the place quickly and do something by connecting the patient to a ventilator so that he can resume his interrupted breathing. This terrifying challenge, known as myasthenic crisis, can be triggered by stress, various infections, surgery, and any adverse reaction to multiple medications. It is reported that approximately fifteen to twenty percent of patients with myasthenia gravis have experienced such an incident at least once. However, some percentages of this statistical community have stated that they are unaware of the cause of the myasthenia gravis crisis. During various researches, certain drugs can enhance the destructive effects of myasthenia gravis.

The cause of myasthenia gravis disease

As it was said at the beginning, myasthenia gravis is an autoimmune condition. ; This means that the cells of the immune system attack the internal cells instead of attacking foreign pathogens. This disease occurs due to an error in the transmission of nerve messages to the muscles. The cause of myasthenia gravis disease can be related to the role of antibodies as well as the thymus gland.

The role of antibodies on the occurrence of the disease

Usually, the normal communication between nerve and muscle is interrupted by neuromuscular gaps. It is short. Neuromuscular clefts are actually the places where nerve cells meet muscles; A muscle that should be controlled by the mentioned nerves.

Neurotransmitters are usually special chemicals that use nerve cells to transmit information. Naturally, when the electrical messages from these cells travel to a motor neuron, the nerve produces a substance called acetylcholine at its terminal. This substance is known as a neurotransmitter that binds to sites called acetylcholine receptors in the muscle. In fact, the binding of acetylcholine to its receptor in the muscle activates the muscle tissue and then muscle contraction occurs.

In myasthenia gravis disease, antibodies (immune system proteins produced by the body's immune system) block, change, or destroy acetylcholine receptors in the muscle-nerve gap. For this reason, in patients with myasthenia gravis, the muscle does not have the strength to contract or this procedure is performed with difficulty. In this case, the antibody itself usually binds to the acetylcholine receptor.

The role of the thymus gland

The thymus gland can regulate the use of the immune system. This control can be related to myasthenia gravis disease. The thymus gland grows over time to finally reach its final development at the age of puberty. Then, over time, the tissue of this gland becomes smaller and fat tissue replaces different parts of the gland. The thymus gland plays a very important role in the development of the body's immune system; because it is responsible for the production of T lymphocyte cells; A type of white blood cell that protects the body against infections and viruses.

In many adults with myasthenia gravis, the thymus gland remains large. Patients with this condition usually have an accumulation of immune cells in their thymus gland and may turn into thymoma (called tumor cells of the thymus gland). Thymomas are usually harmless, but there is always a risk that the tumors will one day become cancerous and spread to other parts of the body. Scientists believe that the thymus gland can issue incorrect instructions for the development of immune cells. Perhaps the reason for immune cells attacking self cells and producing antibodies against the acetylcholine receptor lies in the fact that the thymus does not do its job properly.

People prone to myasthenia gravis

BingMag.com Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

Can anyone get myasthenia gravis? In response, it should be said that every man and woman anywhere in the world can experience this disease. Having said that, adult women under forty years old and adult men over sixty years old are more susceptible to this disease. However, this disease has been seen even in children. Myasthenia gravis is neither hereditary nor transmissible to another healthy person. However, it has been seen that this disease has appeared in more than one member in the same family.

Even babies are not safe from myasthenia gravis; Because if the mother suffers from this disease, the mother's antibodies will be transferred to the fetus through the placenta and cause neonatal myasthenia gravis. However, usually this type of disease is temporary and two to three months after birth, the symptoms of the disease disappear forever. This point is also possible because of the presence of a defective gene in the DNA of the fetus, abnormal proteins have been created and this issue causes symptoms similar to the symptoms of myasthenia gravis disease.

Diagnosis of myasthenia gravis disease

The attending physician can choose six approaches to accurately diagnose myasthenia gravis, which are briefly as follows:

Neurological and physical examination

The physician, with a method similar to other neurological diseases, First, he performs a physical examination of the patient and also records the history of the patient. During the physical tests, the doctor should check muscle strength, balance between muscles, sense of touch, as well as eye muscle movement problems.

Edrophonium test

In this test, a substance called The name of edrophonium chloride is injected to the patient suspected of this disease to reveal the muscle weakness caused by myasthenia gravis. This drug overtly blocks the breakdown of acetylcholine and temporarily increases the level of acetylcholine in the neuromuscular junction. This test is usually used to test eye muscle weakness.

Blood test

BingMag.com Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

In most patients with myasthenia gravis, the level of acetylcholine receptor antibodies is unimaginably high. Also, the second antibody called anti-MuSK was seen in half of the patients with myasthenia gravis, in whom the acetylcholine receptor antibody was not seen. A proper blood test can detect this antibody. However, in some rare cases, neither of these two antibodies may be detected. In this case, it is usually said that the person has seronegative myasthenia.

Electrical diagnosis

Some tests are based on continuous nerve stimulation. . This is done by electrical devices. The treating doctor tries to stimulate the person's nerves with short electrical pulses several times so that the muscle shows the necessary reaction to these impulses. Muscle fibers in patients with myasthenia gravis do not respond to electrical stimulation as well as healthy people. The single strand electromyography (EMG) test is currently the most sensitive test for diagnosing myasthenia gravis, which detects disorders of the nerve-to-muscle transmission process. Other tests may fail to detect this disease, but the EMG technique can correctly diagnose myasthenia gravis with the severity of typical symptoms.

Images

Medical imaging such as CT scan or MRI can be used to diagnose thymus gland tumors. The aforementioned imaging can correctly detect other signs and physical changes.

Respiratory system function test

By measuring the strength of breathing, it is possible to predict whether the patient's respiratory condition is facing a problem and whether Sooner or later, the patient will face myasthenia gravis or not.

With all these interpretations, muscle weakness is a disorder that cannot be deduced from it alone whether a person has myasthenia gravis or not; Because the identification of myasthenia gravis can take up to a year because, for example, only a few specific muscles may have problems in contraction.

How to treat myasthenia gravis disease

BingMag.com Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

Currently, there is no definitive treatment reported for myasthenia gravis and only various procedures can be used to improve muscle condition as treatment in commented In order to reduce the symptoms of the disease, five models of treatment approaches are used globally. With this method, we can hope that the amount of antibodies against the muscular system will be less secreted and, consequently, the severity of the disease will decrease significantly. In a specified research, thymectomy has been able to improve muscle weakness and also reduce the need for immunosuppressive drugs. Antibody to acetylcholine receptor is disturbed. Eculizumab, which was approved by the US Food and Drug Administration in 2017, is used as a suitable drug for this purpose.

Anticholinesterase drugs

BingMag.com Familiarity with myasthenia gravis; Amitabh Bachchan’s 40-year illness

Some drugs are designed and prescribed to slow the breakdown of acetylcholine at the neuromuscular junction. If this happens, muscle strength can be increased. However, this process is temporary and after the drugs are removed, the rate of acetylcholine breakdown will return to the previous state. is the administration of immunosuppressive drugs to produce fewer abnormal antibodies. Prednisone, azathioprine, mycophenolate, mofetil and tacrolimus can be mentioned in this category of chemical drugs. Such drugs usually have several side effects. The most important complication of these types of drugs is the reduction of the immune system's ability to detect pathogens. For this reason, the body will be more susceptible to various diseases, especially infectious diseases. Such an approach can be more effective than other therapies. In this case, patients can have antibodies in their plasma that attack the muscle-nerve gap. This treatment process can destroy harmful antibodies. However, it should be noted that this treatment process can last for several weeks. More briefly, plasmapheresis and intravenous antibodies can be defined as follows:

  • Plasmapheresis: a process performed by a machine. Harmful antibodies in the plasma are removed and replaced with good plasma or another substitute.
  • Intravenous Antibody: Injection of highly concentrated antibodies from healthy donors that can temporarily stop the system from working. Change the body's immunity. These healthy antibodies bind to the problematic antibodies to remove them from the circulation.

Researchers are trying to use current methods, better tools and biomarkers for more accurate and faster diagnosis. find Undoubtedly, the sooner this disease is detected, the easier it is to fight its consequences. Currently, some of the antibodies that cause this disease cannot be identified by current treatment solutions. Finding biomarkers to discover such antibodies is on the agenda of many researchers interested in this field. Many doctors around the world are trying to use more optimized surgical methods to remove the thymus gland; Because the removal of this organ can cause serious and fatal injuries to a person. However, what is certain is that drug therapy is not supposed to be the main treatment for myasthenia gravis; Because only for a temporary period of time, it can resolve complications such as muscle wasting. The secret of success for the treatment of myasthenia gravis is in plasma and surgical procedures. Perhaps in the near future, stem cells can be used to treat such a neurological condition; Moreover, this method is currently used to treat other autoimmune diseases.

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