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myasthenia gravis Definition
Myasthenia gravis is an autoimmune
disorder that causes muscle weakness. An autoimmune disorder is one
in which the body produces antibodies against its own tissues.
What is going on in the body?
For unknown reasons, myasthenia gravis causes a person's immune system to
produce antibodies that attack the muscles in his or her body. The antibodies
destroy the part of the muscle that receives signals from the nerves. When a
muscle is used a great deal, it may not work properly. After a period of rest,
the muscle generally improves.What are the signs and symptoms of the disease?Myasthenia gravis causes muscle weakness that is worsened by use
and improved with rest. For this reason, a person may have few or no symptoms
right after awakening or resting. As the person goes about his or her daily
activities, the symptoms may become obvious.
Eye muscle involvement may cause drooping eyelids and double vision. The person
may have a masklike expression if the facial nerves are affected. Head control,
chewing, swallowing, and speech may also be affected by muscle
weakness.
Approximately 15% to 20% of people who have myasthenia gravis have muscle
weakness in the arms and hands. Weakness in the leg muscles is less common.
Increasing weakness of the muscles in the chest may lead to difficulty
breathing. Respiratory distress, which is an emergency, can occur.
What are the causes and risks of the disease?
No one knows why a person with myasthenia gravis produces antibodies that
attack the muscles. This disorder occurs more often in women than in men. It
usually starts in women during their childbearing years. In men, the disease
most often starts in middle age. Rarely, a child may be born with myasthenia
gravis from a genetic defect.
New research findings suggest that autoimmune disorders
may be set off by a transfer of cells between the fetus and the mother during
pregnancy. The study
involved women with
scleroderma, an autoimmune disorder that affects the skin. These
women have more fetal cells in their blood even decades after a pregnancy than
women who don't have scleroderma. While further research is needed to confirm
these findings, the study does offer an explanation for the much higher
incidence of autoimmune disorders in women than in men.
Symptoms can be worsened by a number of factors, such as:
What can be done to prevent the disease?
There are no known ways to prevent myasthenia gravis.
How is the disease diagnosed?Diagnosis of myasthenia gravis begins with a medical history and
physical exam. The healthcare provider may ask the person to perform repetitive
movements of a muscle. The provider looks for signs of muscle weakness with
use. A blood test known as antibody titer can often detect the
antibodies that attack the musclesA Tensilon
test can be done to diagnose myasthenia gravis. A medicine called
Tensilon is given to the person intravenously, or into a vein. If the person
has myasthenia gravis, the Tensilon will temporarily strengthen the person's
muscles.
Electromyography, or EMG, can be done to
measure the way the muscles respond to electrical stimulation. When myasthenia
is present, the muscles have an abnormal response to the
stimulation.What are the long-term effects of the disease?The symptoms of myasthenia gravis often worsen over the first 5 to
7 years of the disease. After this time, the disease is usually not
progressive. Symptoms may worsen and improve as muscles are used and then
rested throughout the day.In some cases, myasthenia
gravis may get better or worse over time. About 20% of the people with
myasthenia gravis have a natural remission, or improvement, of the disease that
lasts a year or more. With good treatment, people with myasthenia gravis have
a normal life expectancy.What are the risks to others?
Myasthenia gravis is not contagious and poses no risk to others.
What are the treatments for the disease?There is no cure for myasthenia gravis. Lifestyle changes may
improve the symptoms. The following measures may be helpful:
- Avoid contact with people who have contagious infections.
- Dress appropriately in very cold or very hot environments.
- Eat a balanced diet high in
potassium.
- Follow the healthcare provider's recommendations for flu shots and pneumonia vaccines.
- Get plenty of sleep.
- Learn to manage
stress.
- Pace activities throughout the day to avoid fatigue.
- Rest the affected muscles often throughout the day.
- Seek effective treatment of underlying conditions, such as
hypothyroidism.
- Use good oral hygiene
and get regular dental care.
Treatment of myasthenia gravis often involves medicines, such as the following:
- anticholinesterases, such as neostigmine and pyridostigmine, which help
nerves send impulses to the muscles
- ephedrine sulfate, which can be given with other medicines to increase
muscle strength
- immunosuppressive medicines, such as prednisone, azathioprine,
cyclosporine, and cyclophosphamide, which suppress the body's immune attack on
the muscles
- intravenous human immune globulin, or IVIG, which works for a short period
of time by flooding the body with antibodies from many donors
Surgery to remove the thymus gland gets rid of the risk of tumors and often
improves symptoms. Fifteen percent of the people with myasthenia gravis have a
tumor of the thymus gland. This surgery may be recommended in a person who is
otherwise healthy.
Plasmapheresis is sometimes used. This technique allows
removal of some of the person's plasma and plasma components. The remaining
cells are reinfused. This produces a temporary reduction in circulating
antibodies. This process can cause remarkable improvement in some people, but
does not cure the disease. What are the side effects of the treatments?
Many of the medicines used to treat myasthenia gravis can cause allergic
reactions, stomach upset,
diarrhea, and nervousness. Medicines used to suppress the immune
system can increase the person's risk of infections. Surgery has a risk of
bleeding, infection, and allergic
reaction to
anesthesia.
What happens after treatment for the disease?
If the person improves, he or she can resume normal daily activities. If the
muscles involved in breathing are severely weakened, the individual may need a
ventilator to help with
breathing. Protection against choking or falls might be needed. Some people
require intense treatment for long periods of time.
A number of medicines aggravate myasthenia gravis.
People are advised to consult with their healthcare provider before taking any
new medicines. The person's dentist should be told that the person has
myasthenia gravis, and that local
anesthetic medicine such as novacaine might cause
problems.How is the disease monitored?
Any new or worsening symptoms should be reported to the healthcare
provider. | Author: Adam Brochert, MD Date Written: 04/13/00 Reviewer: Eileen McLaughlin, RN, BSN Date Reviewed: 09/11/01
Potential conflict of interest information for reviewers available on request
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