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Myasthenia gravis

Myasthenia gravis is a chronic disorder characterized by weakness and rapid fatigue of any muscles under your voluntary control. It results from a breakdown in the normal communication between nerves and muscles.

The disorder affects only the function of your nerves and muscles - the muscle weakness you experience improves with rest. In moderate to severe cases, myasthenia gravis may cause difficulties with speech, chewing, swallowing and breathing, as well as weakness of your limbs.

Myasthenia gravis can affect people of any age, but it's more common in women younger than 40 and in men older than 60. Treatments are available to control myasthenia gravis and relieve symptoms

Signs and symptoms

Myasthenia gravis may affect any of the muscles that you control voluntarily. It most commonly affects certain muscles, including those of the face, eyes, arms and legs, and those involved in chewing, swallowing and breathing. Signs and symptoms may include:

• Facial muscle weakness, including drooping eyelids

• Double vision

• Difficulty in breathing, talking, chewing or swallowing

• Muscle weakness in your arms or legs

• Fatigue brought on by repetitive motions

The more often a muscle action is repeated, the worse the weakness becomes. In myasthenia gravis, good days alternate with bad. Remissions may occur, however, and can last for months. In rare cases, breathing or swallowing problems worsen markedly, requiring emergency medical care.

Causes

When your nervous system functions normally, the chemical acetylcholine transmits nerve impulses to your muscles. At specialized areas of your muscles, called neuromuscular junctions, receptor sites receive impulses and signal your muscles to contract at certain times, such as when you raise a spoon to your mouth.

In myasthenia gravis, there's a breakdown in communication between your nerves and your muscles. The culprit is your immune system. For unknown reasons, myasthenia gravis causes your immune system to produce antibodies that block or destroy many of your receptor sites for acetylcholine. With fewer receptor sites, your muscles receive fewer nerve signals, resulting in the weakness. The degree of muscle weakness varies greatly from person to person.

It's believed that the thymus gland, a part of your immune system located in the upper chest beneath the breastbone, may trigger or maintain the production of these antibodies. Large in infancy, the thymus is small in healthy adults. Some people with myasthenia gravis, however, have an abnormally large thymus gland. Some of these develop tumors of the thymus. Usually, thymus gland tumors are noncancerous (benign).

Some factors can make myasthenia gravis worse, including illnesses such as a cold, stress and overexertion.

When to seek medical advice

If you experience muscle weakness or have difficulty controlling the muscles of your eyes, face and mouth, have trouble breathing or experience fluctuating weakness in your arms and legs, see your doctor. These signs could be indications of myasthenia gravis. Although there's no cure for myasthenia gravis, the outlook for managing its symptoms is good. The earlier you see your doctor, the sooner treatments can be initiated to help you improve your muscle strength, and the sooner you can learn about strategies to help you use your energy in the most efficient ways.

Screening and diagnosis

The key symptom that points to the possibility of myasthenia gravis is muscle weakness that improves with rest. Tests to confirm the diagnosis may include:

• Neurologic examination. This may include testing of your reflexes, muscle strength, muscle tone, senses of touch and sight, gait, posture, coordination, balance and mental skills.

• Electromyography. During the first part of this test, a small electrical impulse is applied to your skin, stimulating your nerves in order to test the strength of your muscle contraction. In the second part, a thin-needle electrode inserted into one of your muscles helps measure patterns of electrical activity in your muscle at rest and with slight muscle contraction.

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

• Edrophonium test. Injection of the chemical edrophonium (Tensilon) may result in a sudden, although temporary, improvement in your muscle strength, an indication that you may have myasthenia gravis. Edrophonium acts to block an enzyme that inhibits the transmission of signals from your nerve endings to your muscle receptor sites.

Treatment

Doctors use a variety of treatments, alone or in combination, to relieve symptoms of myasthenia gravis:

• Medications. Several drugs, called cholinesterase inhibitors, enhance communication between nerves and muscles. These drugs don't treat the underlying problem, but they do improve muscle contraction and muscle strength. Cholinesterase inhibitors include neostigmine (Prostigmin) and pyridostigmine (Regonol, Mestinon). Corticosteroids inhibit the immune system, limiting antibody production. Prolonged use of corticosteroids can lead to serious side effects, such as bone thinning, weight gain, diabetes, increased risk of some infections and a redistribution of body fat. Your doctor may also prescribe other medications that alter your immune system, such as cyclophosphamide (Cytoxan, Neosar) and azathioprine (Imuran).

• Surgery. Removal of the thymus gland brings marked relief to more than half the people with severe myasthenia gravis, but the effect is often delayed for many years, and the response is more dramatic in younger people.

• Plasmapheresis. This procedure can remedy life-threatening stages of myasthenia gravis. Plasmapheresis involves removal of antibodies from your blood that block transmission of signals from your nerve endings to your muscles' receptor sites. Blood is taken from your body, passed through a filter that removes the specific antibodies and then returned to your body. This approach is expensive and time-consuming. Also, other forms of therapy are necessary for long-term restoration of muscle strength. Otherwise, the immune system soon makes new antibodies to replace those that have been removed.

As part of your treatment, your doctor may suggest physical therapy and occupational therapy to help you adjust to tasks you need to do around the house and in your job.

Therapeutic plasmapheresis is a process in which the fluid part of the blood, called plasma, is removed from blood cells by a device known as a cell separator.

Coping skills

Supplementing your medical care with these approaches may help you make the most of your energy and cope with the symptoms of myasthenia gravis:

• Adjust your eating routine. Try to eat when you have good muscle strength, possibly an hour after taking your medication. Also, take your time eating and rest between bites. Try soft foods and avoid sticky foods.

• Use safety precautions at home. Install grab bars or railings in places where you may need support, such as next to the bathtub. Keep the floors and halls in your house clear of clutter, cords and loose rugs. Outside your home, keep the steps, sidewalk and path to your car clear.

• Use electric appliances and power tools. Save your energy in the bathroom, in the kitchen or at the workbench by using electric appliances, such as toothbrushes, can openers and screwdrivers.

• Wear an eye patch. If you have double vision, using an eye patch can help relieve this effect. Wear the patch while you read or watch television. To avoid eyestrain, occasionally switch the patch from one eye to the other.

• Plan ahead. If you have a chore to do around the house, shopping to do or an errand to run, plan the activity to coincide with the time at which your medication provides your peak energy level. If you're working on a project at home, gather everything you need for the job at one time, to eliminate extra trips that may drain your energy.

• Ask for help. Depending on your energy level, you may not be able to do everything you have planned around the house or run every errand that you need to. Ask family members and friends to lend a hand.

• Manage stress. Because emotional stress can make myasthenia gravis worse, look for ways to reduce stress. These may include relaxation techniques such as biofeedback and meditation.

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