What is myasthenia gravis (MG)?
Myasthenia gravis is an autoimmune disease that affects the
transmission of signals from nerves to muscles. The name myasthenia
gravis comes from Greek and Latin words meaning "grave muscle
weakness." Today, however, most cases of MG are not as "grave" as
the name implies. In fact, most people with MG can expect to live
normal or nearly normal lives.
The hallmark of MG is muscle weakness that increases during activity
and improves after rest. MG often involves muscles that control eye
and eyelid movement, facial expression, chewing, talking, and
swallowing. The muscles that control breathing and neck and limb
movements may also be affected.
The thymus gland, part of the immune system, is abnormal in most MG
cases. Some people with MG have benign (noncancerous) tumors of the
thymus gland called thymomas.
Some drugs like antibiotics can trigger or worsen MG symptoms. The
list is as follows.
- Telithromycin: Exacerbation within 2 hours of administration
- Prednisone (High dose): Onset days after administration
- NMJ blockers
- Non-depolarizing (Vecuronium)
What causes MG?
MG is caused by a defect in the transmission of nerve signals to
muscles. Normally, nerve endings release a substance called
acetylcholine that binds or attaches to receptors on the muscle.
This leads to muscle contractions. In MG, the body's own immune
system produces antibodies that block this transmission.
Who gets myasthenia gravis?
Estimates of the number of people affected by MG vary, ranging from
five to 14 people per 100,000.
MG occurs in all ethnic groups and both genders. It most commonly
affects young adult women (under 40) and older men
(over 60), but it can occur at any age. Children sometimes develop
MG is not directly inherited nor is it contagious. Sometimes the
disease may occur in more than one member of the same family. If a
woman with MG becomes pregnant, sometimes the baby acquires
antibodies from the mother and has MG symptoms for a few weeks or
months after birth. This is called neonatal myasthenia, and the
symptoms can be treated.
In rare cases, myasthenia is caused by a defective gene and appears
in infants born to non-myasthenic mothers. This type is called
What is the role of the thymus gland in MG?
The thymus gland, found in the upper chest area beneath the
breastbone, is a part of the body's normal immune system. In most
adults with MG, the thymus gland is abnormal. Some people with MG
develop thymomas or tumors on the thymus gland. Generally thymomas
are benign, but they can become malignant (cancerous). The removal
of the Thymus Gland can bring a cure in the Myasthenic patient.
What are the signs and symptoms of MG?
The muscles that control eye and eyelid movement, facial expression,
and swallowing are most often affected. The onset of the disorder
may be sudden, by the first symptom being eyelid droop. Symptoms
often are not immediately recognized as MG.
In most cases, the first noticeable symptom is weakness of the eye
muscles. In others, difficulty in swallowing and slurred speech may
be the first signs. While rare, first signs of MG can also include
difficulty with breathing. The degree of muscle weakness involved in
MG varies greatly among persons with this disease. Symptoms, which
vary in type and severity, may include:
Drooping of one or both eyelids (ptosis)
Blurred or double vision (diplopia) due to weakness of the muscles
that control eye movements
Unstable or waddling gait
Weakness in arms, hands, fingers, legs, and neck
Change in facial expression
Difficulty in swallowing and shortness of breath
Impaired speech (dysarthria)
Shortness of breath
How is MG diagnosed?
Unfortunately, a delay in diagnosis of one or two years is not
unusual in cases of MG. Weakness is a common symptom of many other
disorders. The diagnosis is often missed in people who have mild
weakness or in those whose weakness is restricted to only a few
The first steps of diagnosing MG include a review of the person's
medical history and physical and neurological exams. If the doctor
suspects MG, several tests are available to confirm the diagnosis.
Antibody blood test. A special blood test can detect the antibodies
that prevent nerves from signaling to muscles. While most people
with MG have abnormally high levels of these antibodies, some
individuals (about 10 percent) can actually test negative for
antibodies. And, antibodies may not be detected if only eye muscles
Edrophonium test. When this drug is injected, the weak eye muscles
of people with MG will briefly get stronger.
Nerve conduction test/repetitive stimulation. This is a test of
specific muscle fatigue by repetitive nerve stimulation.
Single fiber electromyography (EMG). In this test, pairs of single
muscle fibers are stimulated by electrical impulses. It can detect
impaired nerve-to-muscle transmission.
Computed tomography (CT) or magnetic resonance imaging (MRI). These
tests can help identify an abnormal thymus gland or a thymus gland
What is the treatment for MG?
Today, MG can be controlled. There are several therapies available
to help reduce muscle weakness. Most persons with MG have good
results from treatment. In some people MG, like many other
autoimmune diseases, may go into remission (a period of time without
symptoms) and muscle weakness may disappear completely.
Remission or improvement can occur without treatment in some cases.
According to the Muscular Dystrophy Association, up to 20 percent of
person with MG may have complete remission of symptoms without any
treatment, and another 20 percent may improve without treatment.
These spontaneous improvements are more likely to occur during early
stages of MG.
Treatment of MG may include:
Medications. Drugs used include cholinesterase inhibitors such as
neostigmine and pyridostigmine. These drugs help improve nerve
signals to muscles and increase muscle strength. Immunosuppressive
drugs such as prednisone, cyclosporine, and azathioprine may also be
used to suppress the production of abnormal antibodies. They must be
used with careful medical followup because they can be associated
with major side effects.
Thymectomy, the surgical removal of the thymus gland (which
is abnormal in most persons with MG). This surgery is done for
persons with MG who have tumors, as well as for individuals without
tumors. It improves symptoms in more than half of individuals
without tumors. It may cure some people with MG, possibly by
re-balancing the immune system.
Other therapies sometimes used to treat MG during especially
difficult periods of weakness include:
Plasmapheresis or plasma exchange. This is a procedure that removes
abnormal antibodies from the blood.
High-dose intravenous immune globulin. This treatment temporarily
interferes with the ability of the immune system to damage the nerve
muscle junction. Treatment options for a person with MG depend on
the severity of the weakness, which muscles are affected, and the
person's age and other medical problems.
In a few cases, MG may cause severe weakness resulting in acute
respiratory failure. But most people can expect to lead normal or
nearly normal lives.
What is a myasthenic crisis?
A myasthenic crisis occurs when weakness affects the muscles that
control breathing. This can create a medical emergency requiring a
respirator to help the person breathe or measures to prevent a
person from taking in, or aspirating, too much air into their lungs.
In individuals whose respiratory muscles are weak, infection, fever,
a reaction to medication, or emotional stress can trigger a crisis.
How can I help take care of myself if I have MG?
You can follow a few simple steps to help cope with the condition in
your daily life. Plenty of rest and a well balanced, potassium-rich
diet can help ease fatigue. Good sources of potassium include
oranges, orange juice, and bananas. It is important to avoid
overexertion, and if necessary, to rest the eyes or to lie down
briefly a few times a day.
Is MG associated with other conditions?
Since it is an autoimmune disease, it may occur in combination with
other autoimmune conditions such as rheumatoid arthritis, Sjorgrens
syndrome, lupus, pernicious anemia, or autoimmune thyroiditis.