Myasthenia Gravis

Myasthenia gravis, an autoimmune disorder affecting the myoneural junction, is characterized

by varying degrees of weakness of the voluntary muscles. Approximately 60,000 people have
myasthenia gravis in the United States. Women are affected more frequently than men, and they tend
to develop the disease at an earlier age (20 to 40 years of age, versus 60 to 70 years for men).
Causes
Often, the cause of MG contraction is not known for the particular patient. It is possible to
contract MG from Penicillamine, which is used to treat Wilsons disease, rheumatoid arthritis, other
chronic autoimmune diseases, and cystinuria. It is also possible to contract MG by being treated with
interferon alpha, or through a bone marrow transplant.
Signs and Symptoms
The initial manifestation of myasthenia gravis usually involves the ocular muscles. Diplopia
(double vision) and ptosis (drooping of the eyelids) are common. However, the majority of patients also
experience weakness of the muscles of the face and throat (bulbar symptoms) and generalized
weakness. Weakness of the facial muscles results in a bland facial expression. Laryngeal involvement
produces dysphonia (Voice impairment) and increases the patients risk for choking and aspiration.
Generalized weakness affects all the extremities and the intercostal muscles, resulting in decreasing vital
capacity and respiratory failure. Myasthenia gravis is purely a motor disorder with no effect on sensation
or coordination.
Pathophysiology
Normally, a chemical impulse precipitates the release of acetylcholine from vesicles on the
nerve terminal at the myoneural junction. The acetylcholine attaches to receptor sites on the motor
endplate and stimulates muscle contraction. Continuous binding of acetylcholine to the receptor site is
required for muscular contraction to be sustained.
In myasthenia gravis, antibodies directed at the acetylcholine receptor sites impair transmission
of impulses across the myoneural junction. Therefore, fewer receptors are available for stimulation,
resulting in voluntary muscle weakness that escalates with continued activity). These antibodies are
found in 80% to 90% of the people with myasthenia gravis. Eighty percent of people with myasthenia
gravis have either thymic hyperplasia or a thymic tumor, and the thymus gland is believed to be the site
of antibody production. In patients who are antibody negative, researchers believe that the offending
antibody is directed at a portion of the receptor site rather than the whole complex.
Diagnosis
1. Acetylcholinesterase inhibitor test
- Used to diagnose myasthenia gravis
- Stops the breakdown of acetylcholine, thereby increasing availability at the
neuromuscular junction.
2. Edrophonium chloride (Tensilon)
- A fast-acting acetylcholinesterase inhibitor
- Administered through IV to diagnose myasthenia gravis
- Thirty seconds after injection, facial muscle weakness and ptosis should resolve for
about 5 minutes, immediate improvement in muscle strength after administration
of this agent represents a positive test and usually confirms the diagnosis.
- Atropine should be available to control the side effects of edrophonium, which
include bradycardia, sweating, and cramping.
3. Ig Serum
- The presence of acetylcholine receptor antibodies is identified in serum.
4. Repetitive nerve stimulation
- Demonstrates a decrease in successive action potentials.
5. MRI
- The thymus gland, a site of acetylcholine receptor antibody production, may be
enlarged in myasthenia gravis.
- Identify an enlarged thymus gland.

Prevention
Because the cause of myasthenia gravis is unknown, there is no way to prevent it. However,
once the disease has developed, there may be ways to prevent episodes of worsening symptoms or
flare-ups:
- Give yourself plenty of rest.
- Avoid strenuous, exhausting activities.
- Avoid excessive heat and cold.
- Avoid emotional stress.
Whenever possible, avoid exposure to any kind of infection, including colds and influenza (flu).
You should be vaccinated against common infections, such as influenza.
Work with your doctor to monitor your reactions to prescription medications. Some drugs
commonly prescribed for other problems, such as infections, heart disease or hypertension, may make
myasthenia gravis worse. You may need to choose alternative therapies or avoid some medications
entirely.

Treatment
Management of myasthenia gravis is directed at improving function and reducing and removing
circulation antibodies. Therapeutic modalities include:
1. Administration of anticholinesterase medications
- help improve neuromuscular transmission and increase muscle strength
- E.g. neostigmine and pyridostigmine
2. Immunosuppressive therapy
- Improve muscle strength by suppressing the production of abnormal antibodies.
- Their use must be carefully monitored by a physician because they may cause major
side effects.
- E.g. prednisone, azathioprine, cyclosporine, mycophenolate mofetil, and tacrolimus
3. Plasmapheresis
- A technique used to treat exacerbations. The patients plasma and plasma
components are removed through a centrally placed large-bore double-lumen
catheter. The blood cells and antibody-containing plasma are separated after which
the cells and a plasma substitute are reinfused. Plasma exchange produces a
temporary reduction in the level of circulating antibodies.
4. Thymectomy
- Surgical removal of the thymus gland. It can produce antigen-specific
immunosuppression and result in clinical improvement. The procedure results in
either partial or complete remission
Prognosis
With treatment, most individuals with myasthenia can significantly improve their muscle
weakness and lead normal or nearly normal lives. Some cases of myasthenia gravis may go into
remissioneither temporarily or permanentlyand muscle weakness may disappear completely so that
medications can be discontinued. Stable, long-lasting complete remissions are the goal of thymectomy
and may occur in about 50 percent of individuals who undergo this procedure. In a few cases, the severe
weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency
medical care.
Epidemiology
The prevalence of myasthenia gravis in the United States is estimated at 14 to 20 per 100,000
population, approximately 36,000 to 60,000 cases in the United States. The most common age at onset
is the second and third decades in women and the seventh and eighth decades in men.
History
The first described case of MG is likely that of the Native American Chief Opechancanough, who
died in 1664. In 1672, the English physician Thomas Willis described a patient with the fatigable
weakness of limbs and bulbar muscles characteristic of MG. In the late 1800s, the first modern
descriptions of patients with myasthenic symptoms were published, and the name myasthenia
gravis was coined by fusing the Greek terms for muscle and weakness to yield the noun myasthenia and
adding the Latin adjective gravis, which means severe.

References
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Frequently Asked Questions. Myasthenia Gravis: Frequently Asked Questions.
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