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Myasthenia Gravis

Definition
Myasthenia gravis (mi-uhs-THE-ne-uh GRA-vis) is characterized by weakness and
rapid fatigue of any of the muscles under your voluntary control. The cause of
myasthenia gravis is a breakdown in the normal communication between nerves and
muscles.

There is no cure for myasthenia gravis, but treatment can help relieve signs and
symptoms — such as weakness of arm or leg muscles, double vision, drooping
eyelids, and difficulties with speech, chewing, swallowing and breathing.

While myasthenia gravis can affect people of any age, it's more common in women
younger than 40 and in men older than 60. The disorder occurs in one or two people
per 10,000.

Symptoms
Muscle weakness caused by myasthenia gravis worsens as the affected muscle is used
repeatedly. Since symptoms typically improve with rest, your muscle weakness may
come and go. While myasthenia gravis can affect any of the muscles that you control
voluntarily, certain muscle groups are more commonly affected than others.

Eye muscles
In more than half the people who develop myasthenia gravis, their first signs and
symptoms involve eye problems, such as:

 Drooping of one or both eyelids (ptosis)


 Double vision (diplopia), which may be horizontal or vertical
 Blurred vision, which may come and go

Face and throat muscles


In about 15 percent of people with myasthenia gravis, the first symptoms involve face
and throat muscles, which can cause difficulties with:

 Speaking. Your speech may be very soft or sound nasal, depending upon
which muscles have been affected.
 Swallowing. You may choke very easily, which makes it difficult to eat, drink
or take pills. In some cases, liquids you're trying to swallow may come out
your nose.
 Chewing. The muscles used for chewing may wear out halfway through a
meal, particularly if you've been eating something hard to chew, such as steak.
 Facial expressions. Family members may note that you've "lost your smile" if
the muscles that control your facial expressions are affected.
Arm and leg muscles
Myasthenia gravis can cause weakness in your arms and legs, but this usually happens
in conjunction with muscle weakness in other parts of your body — such as your
eyes, face or throat. The disorder usually affects arms more often than legs. However,
if it affects your legs, you may waddle when you walk.

When to see a doctor


Talk to your doctor if you have trouble:

 Breathing
 Seeing
 Swallowing
 Chewing
 Walking

Causes

Receptors

Thymus gland
Your nerves communicate with your muscles by releasing chemicals, called
neurotransmitters, which fit precisely into receptor sites on the muscle cells. In
myasthenia gravis, your immune system produces antibodies that block or destroy
many of your muscles' receptor sites for a neurotransmitter called acetylcholine. With
fewer receptor sites available, your muscles receive fewer nerve signals, resulting in
weakness.

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. But, in some adults
with myasthenia gravis, the thymus is abnormally large. Some people also have
tumors of the thymus. Usually, thymus gland tumors are noncancerous.

Some factors can make myasthenia gravis worse, including:

 Fatigue
 Illness
 Stress
 Extreme heat
 Some medications — such as beta blockers, calcium channel blockers, quinine and some
antibiotics

Complications
Complications of myasthenia gravis are treatable, but some can be life-threatening.

Myasthenic crisis
Myasthenic crisis is a life-threatening condition, which occurs when the muscles that
control breathing become too weak to do their jobs. Emergency treatment is needed to
provide mechanical assistance with breathing. Medications and blood-filtering
therapies help people recover from myasthenic crisis, so they can again breathe on
their own.

Thymus tumors
About 15 percent of the people who have myasthenia gravis have a tumor in their
thymus, a gland under the breastbone that is involved with the immune system. Most
of these tumors are noncancerous.

Other disorders
People who have myasthenia gravis are also more likely to have the following
problems:

 Underactive or overactive thyroid. The thyroid gland, located in the neck,


secretes hormones that regulate your metabolism. If your thyroid is
underactive, your body uses energy more slowly. An overactive thyroid makes
your body use energy too quickly.
 Lupus. Lupus is a disease in which your immune system attacks certain parts
of your body. Common symptoms include painful or swollen joints, hair loss,
extreme fatigue and a red rash on the face.
 Rheumatoid arthritis. This type of arthritis is caused by problems with your
immune system. It is most conspicuous in the wrists and fingers, and can
result in joint deformities that make it difficult to use your hands.

Preparing for your appointment


While you might first discuss your symptoms with your family doctor, he or she will
probably refer you to a neurologist for further evaluation.

What you can do


Because appointments can be brief, plan ahead and write lists of important
information, including:

 Detailed descriptions of all your symptoms, including whether anything seems


to make them better or worse
 All your medications and dosages, including nonprescription drugs and
supplements
 Questions for the doctor, such as what tests or treatments he or she may
recommend

What to expect from your doctor


Your doctor will want a detailed description of your symptoms and your past medical
history. In addition to a physical exam, your doctor may also check your neurological
health by testing your:

 Reflexes
 Muscle strength
 Muscle tone
 Senses of touch and sight
 Coordination
 Balance

Tests and diagnosis


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

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 breaks down
acetylcholine, the chemical that transmits signals from your nerve endings to your
muscle receptor sites.

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.
Repetitive nerve stimulation
This is a type of nerve conduction study, in which electrodes are attached to your skin
over the muscles to be tested. Small pulses of electricity are sent through the
electrodes to measure the nerve's ability to send a signal to your muscle. To diagnose
myasthenia gravis, the nerve will be tested many times to see if its ability to send
signals worsens with fatigue.

Single-fiber electromyography (EMG)


Electromyography (EMG) measures the electrical activity traveling between your
brain and your muscle. It involves inserting a very fine wire electrode through your
skin and into a muscle. In single-fiber EMGs, a single muscle fiber is tested. Most
people find this test to be somewhat uncomfortable.

Imaging scans
Your doctor may order a CT scan or an MRI to see if there's a tumor or other
abnormality in your thymus.

Treatments and drugs


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

Medications

 Cholinesterase inhibitors. Drugs such as pyridostigmine (Mestinon) enhance


communication between nerves and muscles. These drugs don't cure the
underlying problem, but they do improve muscle contraction and muscle
strength.
 Corticosteroids. These types of drugs 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, increased
risk of some infections, and an increase and redistribution of body fat.
 Immunosuppressants. Your doctor may also prescribe other medications that
alter your immune system, such as azathioprine (Imuran), cyclosporine
(Sandimmune, Neoral) or mycophenolate (CellCept).

Therapy

 Plasmapheresis (plaz-muh-fuh-RE-sis). This procedure uses a filtering


process similar to dialysis. Your blood is routed through a machine that
removes the antibodies that are blocking transmission of signals from your
nerve endings to your muscles' receptor sites. However, the beneficial effects
usually last only a few weeks.
 Intravenous immune globulin. This therapy provides your body with normal
antibodies, which alters your immune system response. It has a lower risk of
side effects than do plasmapheresis and immune-suppressing therapy, but it
can take a week or two to start working and the benefits usually last less than a
month or two.
Surgery
About 15 percent of the people who have myasthenia gravis have a tumor in their
thymus, a gland under the breastbone that is involved with the immune system. If you
have such a tumor, you'll need to have your thymus removed.

For people with myasthenia gravis who don't have a tumor in the thymus, it's unclear
whether the potential benefit of removing the thymus outweighs the risks of surgery.
This is an individualized decision between you and your doctor, but most doctors
don't recommend surgery if:

 Your symptoms are mild


 Your symptoms involve only your eyes
 You're over 60 years old

Lifestyle and home remedies


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.
Take your time eating and rest between bites. More frequent, smaller meals
may be easier to handle. Also, try soft foods and avoid sticky foods that
require lots of chewing.
 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 problem. Wear the patch while you read or watch television. To
avoid eyestrain, periodically switch the patch from one eye to the other.
 Plan. 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.