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Monday, April 26, 2021

Myasthenia gravis

 diagnosis of myasthenia gravis might include:

Edrophonium test

Injection of the chemical edrophonium chloride that results in a sudden, temporary improvement in muscle strength might indicate that you have myasthenia gravis.

Edrophonium chloride blocks an enzyme that breaks down acetylcholine, the chemical that transmits signals from your nerve endings to your muscle receptor sites.

Ice pack test

If you have a droopy eyelid, your doctor might place a bag filled with ice on your eyelid. After two minutes, your doctor removes the bag and analyzes your droopy eyelid for signs of improvement.

Blood analysis

A blood test might reveal the presence of abnormal antibodies that disrupt the receptor sites where nerve impulses signal your muscles to move.

Repetitive nerve stimulation

In this nerve conduction study, doctors attach electrodes to your skin over the muscles to be tested. Doctors send small pulses of electricity through the electrodes to measure the nerve's ability to send a signal to your muscle.

To diagnose myasthenia gravis, doctors will test the nerve repeatedly to see if its ability to send signals worsens with fatigue.

Single-fiber electromyography (EMG)

This test measures the electrical activity traveling between your brain and your muscle. It involves inserting a fine wire electrode through your skin and into a muscle to test a single muscle fiber.

Imaging

Your doctor might order a CT scan or an MRI to check if there's a tumor or other abnormality in your thymus.

Treatment

Various treatments, alone or in combination, can relieve symptoms of myasthenia gravis. Your treatment will depend on your age, how severe your disease is and how fast it's progressing.

Medications

  • Cholinesterase inhibitors. Medications such as pyridostigmine (Mestinon, Regonal) and neostigmine (Bloxiverz) enhance communication between nerves and muscles. These medications aren't a cure, but they can improve muscle contraction and muscle strength in some people.

    Possible side effects include gastrointestinal upset, diarrhea, nausea, and excessive salivation and sweating.

  • Corticosteroids. Corticosteroids such as prednisone inhibit the immune system, limiting antibody production. Prolonged use of corticosteroids, however, can lead to serious side effects, such as bone thinning, weight gain, diabetes and increased risk of some infections.
  • Immunosuppressants. Your doctor might also prescribe other medications that alter your immune system, such as azathioprine (Azasan,, Imuran) mycophenolate mofetil (Cellcept), cyclosporine (Sandimmune) methotrexate (Trexall) or tacrolimus (Astrograf XL, Prograf). These drugs, which can take months to work, might be used with corticosteroids.

    Side effects of immunosuppressants, such as increased risk of infection and liver or kidney damage, can be serious.

Intravenous therapy

The following therapies are usually used in the short term to treat a sudden worsening of symptoms or before surgery or other therapies.

  • Plasmapheresis (plaz-muh-fuh-REE-sis). This procedure uses a filtering process similar to dialysis. Your blood is routed through a machine that removes the antibodies that block transmission of signals from your nerve endings to your muscles' receptor sites. However, the good effects usually last only a few weeks, and repeated procedures can lead to difficulty accessing veins for the treatment.

    Risks associated with plasmapheresis include a drop in blood pressure, bleeding, heart rhythm problems or muscle cramps. Some people develop an allergic reaction to the solutions used to replace the plasma.

  • Intravenous immunoglobulin (IVIg). This therapy provides your body with normal antibodies, which alters your immune system response. Benefits are usually seen in less than a week and can last three to six weeks.

    Side effects, which usually are mild, can include chills, dizziness, headaches and fluid retention.

  • Monoclonal antibody. Rituximab (Rituxan) and the more recently approved eculizumab (Soliris) are intravenous medications used in some cases of myasthenia gravis. These drugs are usually used for people who don't respond to other treatments. They can have serious side effects.

Surgery

Some people with myasthenia gravis have a tumor in their thymus gland. If you have a tumor, called a thymoma, doctors will surgically remove your thymus gland (thymectomy).

Even if you don't have a tumor in the thymus gland, removing the gland might improve your myasthenia gravis symptoms. However, the benefits of thymectomy can take years to develop.

A thymectomy can be performed as an open surgery or as a minimally invasive surgery. In open surgery, your surgeon splits the central breastbone (sternum) to open your chest and remove your thymus gland.

Minimally invasive surgery to remove the thymus gland uses smaller incisions. It might also involve:

  • Video-assisted thymectomy. In one form of this surgery, surgeons make a small incision in your neck or a few small incisions in the side of your chest. They then use a long, thin camera (video endoscope) and small instruments to see and remove the thymus gland.
  • Robot-assisted thymectomy. In this form of thymectomy, surgeons make several small incisions in the side of your chest and remove the thymus gland using a robotic system, which includes a camera arm and mechanical arms.

These procedures might cause less blood loss, less pain, lower mortality rates and shorter hospital stays compared with open surgery.

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