What’s myasthenia gravis?

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Myasthenia gravis is an autoimmune condition that causes muscle weakness, particularly in the face, and is more common in women under 40 and men over 60. Diagnosis can be difficult, but treatments include drugs and thymectomy surgery. Ongoing research aims to find better treatments and a cure.

Myasthenia gravis, which results in severe muscle weakness, is an autoimmune condition that causes the body’s voluntary muscles to gradually weaken. Most often the muscles in the face, such as those that control eyelid movement, speech and chewing, are the first to be affected. These muscles can show involuntary movements or droop, or they can make speaking or swallowing very difficult.

Myasthenia gravis is not hereditary, although there have been some cases where there is more than one affected member of a family. Its occurrence is more common in women under the age of 40 and in men over the age of 60. The condition is mainly related to the thymus gland, which secretes abnormally immune cells that can attack the body’s nerve transmission signals and cut or distort them.

Symptoms progress from facial muscle weakness to weakness in the extremities, which can affect range of motion. Weakness is often shown more during activity. The most serious aspect of myasthenia gravis is its ability to cause a myasthenic crisis. During a seizure, the muscles that support breathing become too weak, and those experiencing a seizure generally require emergency services and/or hospitalization to provide respiratory assistance, such as with ventilators.

Methods for diagnosing myasthenia gravis are not perfect, and some with the condition can wait several years before receiving a positive diagnosis. Because these symptoms don’t go unnoticed, a doctor usually starts with a thorough medical history. The physical examination may include the patient demonstrating the ability to grasp objects or move certain muscles. Sometimes blood tests can detect the presence of abnormal immune cells.

Two other tests that may be done are single-fiber electromyography (EMG) and an edrophonium test. EMG tests individual muscle strands with electrical impulses to identify any damage of the neural transmission to the muscles. An edrophonium test uses intravenous administration of edrophonium to temporarily block the actions of immune cells. When tested, when the symptoms of myasthenia gravis improve, this can be considered a positive test.

Once diagnosed, there are several treatments for myasthenia gravis. In some cases, drugs are given that block immune responses, particularly those autoimmune cells made in the thymus. These include daily doses of drugs such as neostigmine. Occasionally, steroids such as prednisone are used to improve muscle strength, although these steroids can have extreme side effects.

When possible, a surgical procedure called a thymectomy removes the thymus gland. This is by far the most effective treatment for myasthenia gravis, with 70% of patients having a complete remission of symptoms. Because this option doesn’t completely eliminate the disease in some, ongoing research from the National Institute of Neurological Disorders and Stroke hopes to find both better treatments and, ultimately, a cure. For now, with current treatment, many with myasthenia gravis can be expected to lead normal, healthy lives.




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