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In the Spotlight

MYASTHENIA GRAVIS: When Ones Muscles Are Attacked From Within!

By Adam Brochert, M.D.
Personal MD.com
Medical Contributor

 
Muscles Feel Weak?

Myasthenia gravis is an unusual disorder affecting muscle function. Normally, when a person decides to move, an impulse is sent down a nerve to the muscle. When the impulse reaches the end of the nerve, the nerve releases a substance called acetylcholine. Acetylcholine then normally binds to the muscle and causes the muscle to contract, which creates movement.

In myasthenia gravis, the body produces antibodies which attack the acetylcholine receptors found on muscles. The purpose of these muscle receptors is to bind the acetylcholine that is released from the nerves, which lets the muscle know when to contract. When the antibodies bind to these muscle receptors, the receptors are then blocked or destroyed, making them unable to bind acetylcholine (See Figure).

This causes muscle weakness, because the signals sent by the nerve cannot get to the muscle. So, while the nerves and muscles both technically are okay in myasthenia gravis, they are blocked from interacting because of the antibodies that are produced.

Because the antibodies produced in myasthenia gravis attack the persons own body, myasthenia gravis is known as an autoimmune disease. There are many other autoimmune diseases, such as lupus, rheumatoid arthritis and scleroderma. It is not known why a persons body would produce antibodies that attack their own body, but unfortunately, taken as a group, autoimmune diseases are fairly common.

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Myasthenia gravis tends to affect younger women, however older persons and males can also be affected. The primary symptom is muscle weakness and fatigue. Classically, the weakness gets worse as the day progresses.

The eye muscles are almost always affected, which can lead to a drooping of the eyelids and double vision because the eyes fail to move in a coordinated fashion. The more a muscle is used, the weaker it becomes. If the muscle becomes very weak, a period of rest will often improve its strength.

When suspected, a simple test can be done to make the diagnosis of myasthenia gravis. A short acting medication called an anticholinesterase can be given. This medicine works by increasing the amount of acetylcholine around muscles.

lupus
any of several diseases (as lupus vulgaris or systemic lupus erythematosus) characterized by skin lesions
rheumatoid arthritis
a usually chronic disease that is considered an autoimmune disease and is characterized especially by pain, stiffness, inflammation, swelling, and sometimes destruction of joints
scleroderma
a usually slowly progressive disease marked by the deposition of fibrous connective tissue in the skin and often in internal organs

If a person has myasthenia, this medication will make their weak muscles stronger. If they dont have myasthenia gravis, their muscles will not get stronger with the medication. A blood test that detects the muscle receptor antibodies is often used to confirm the diagnosis.

Once the diagnosis is confirmed, treatment may be started. The course of myasthenia gravis is unpredictable and its severity varies widely. Some people have a very mild disease, which resolves on its own spontaneously. Others have such severe disease that their respiratory muscles become paralyzed and they can die without the assistance of a breathing machine. Because of the unpredictable course of the disease, treatment may be held initially if symptoms are mild. When symptoms are interfering with daily life, however, treatment is started.

Long acting forms of anticholinesterase medicine are the treatment of choice. The most commonly prescribed drug in this class is Mestinon (pyridostigmine bromide). This medication must often be given four times a day, and if the severity of the disease changes, the dose will need to be adjusted. Close follow up with a doctor is usually required.

Another aspect of treatment is surgery. Removal of the thymus gland often can produce a permanent relief of symptoms. The thymus gland is in the chest just above the heart and behind the upper portion of the breastbone (known as the sternum).
The thymus has an important role in the development of immune cells in the body, so it is not surprising that removal of this gland can improve symptoms in a disease caused by an overactive immune system. Unfortunately, this treatment is generally not very effective in other autoimmune diseases.

In patients who have particularly severe disease or who fail to respond to having their thymus gland removed, steroid medications, such as Prednisone (corticosteroids), may be needed, usually in high doses. In an acute crisis, hospitalization may be required to stabilize the patient. Sometimes special procedures, such as plasmapheresis, that either add or remove antibodies from the blood may be needed.

Most patients with myasthenia gravis can be effectively treated with medications and surgery. This unique disease which affects muscles is fortunately uncommon. Usually, when our muscles are weak, it is simply because we need a break!

 

Copyright © 1999 PersonalMD.com. All rights reserved.

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Copyright © 1999 PersonalMD.com. All rights reserved.


 
     
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