Myasthenia gravis

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Superficial anterior muscles

Ptosis, drooping of the eyelid

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Immune response
Antibody
Autoimmune disorders
Hyperthyroidism
Rheumatoid arthritis
Systemic lupus erythematosus

Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of

voluntary muscles, which often improves with rest and worsens with activity. The
condition is caused by an abnormal immune response.

Causes

In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain
movement does not adequately reach the muscle cells. This is caused when immune cells
target and attack the body's own cells (an autoimmune response). This immune response
produces antibodies that attach to affected areas, preventing muscle cells from receiving
chemical messages (neurotransmitters) from the nerve cell.

The cause of autoimmune disorders such as myasthenia gravis is unknown. In some

cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the
immune system). Patients with myasthenia gravis have a higher risk of having other
autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus
erythematosus.

Myasthenia gravis affects about 3 of every 10,000 people and can affect people at any
age. It is most common in young women and older men.

Symptoms

• Muscle weakness, including:

 o Swallowing difficulty, frequent gagging, or choking
o Paralysis
o Muscles that function best after rest
o Drooping head
o Difficulty climbing stairs
o Difficulty lifting objects
o Need to use hands to rise from sitting positions
o Difficulty talking
o Difficulty chewing
• Vision problems:
o Double vision
o Difficulty maintaining steady gaze
o Eyelid drooping

Additional symptoms that may be associated with this disease:

• Hoarseness or changing voice

• Fatigue
• Facial paralysis
• Drooling
• Breathing difficulty

Exams and Tests

Examination may be normal or may show muscle weakness that progressively worsens as
the muscle is used. In many patients the eye muscles are affected first. Reflexes and
sensation are normal. Weakness may affect the arms, legs, breathing or swallowing
muscles, and any other muscle group.

• Repetitive stimulation (type of nerve conduction study) is more sensitive.

• Single-fiber EMG can be very sensitive.
• Acetylcholine receptor antibodies may be present in the blood.
• A Tensilon test is positive in some cases but must be interpreted carefully by an
experienced doctor. Baseline muscle strength is evaluated. After Tensilon
(edrophonium, a medication that blocks the action of the enzyme that breaks
down the transmitter acetylcholine) is given, muscle function may improve.
• The patient may need a CT or MRI scan of the chest to look for a possible tumor
in the thyroid.

Treatment

There is no known cure for myasthenia gravis. However, treatment may result in
prolonged periods of remission.

Lifestyle adjustments may enable continuation of many activities. Activity should be

planned to allow scheduled rest periods. An eye patch may be recommended if double
vision is bothersome. Stress and excessive heat exposure should be avoided because they
can worsen symptoms.

Some medications, such as neostigmine or pyridostigmine, improve the communication

between the nerve and the muscle. Prednisone and other medications that suppress the
immune response (such as azathioprine, cyclosporine, or mycophenolate mofetil) may be
used if symptoms are severe and there is inadequate response to other medications.

Plasmapheresis, a technique in which blood plasma containing antibodies against the

body is removed from the body and replaced with fluids (donated antibody-free plasma
or other intravenous fluids), may reduce symptoms for up to 4 - 6 weeks and is often used
to optimize conditions before surgery.

When other treatments do not improve systems, patients may receive intravenous
immunoglobulin.

Surgical removal of the thymus (thymectomy) may result in permanent remission or less
need for medicines.

Patients with eye problems may try lens prisms to improve vision. Surgery may also be
performed on the eye muscles.

Several medications may make symptoms worse and should be avoided. Therefore, it is
always important to check with your doctor about the safety of a medication before
taking it.

Crisis situations, where muscle weakness involves the breathing muscles, may occur.
These attacks seldom last longer than a few weeks. Hospitalization and assistance with
breathing may be required during these attacks. Often plasmapheresis is used to help end
the crisis.

Support Groups

The stress of illness can often be helped by joining support groups where members share
common experiences and problems. See myasthenia gravis - support group.

Outlook (Prognosis)

There is no cure, but long-term remission is possible. There may be minimal restriction
on activity in many cases. Patients that only have eye symptoms (ocular myasthenia
gravis), may progress to have generalized myasthenia over time.

Pregnancy is possible for a woman with myasthenia gravis but should be closely
supervised. The baby may be temporarily weak and require medications for a few weeks
after birth but usually does not develop the disorder.
Possible Complications

• Restrictions on lifestyle (possible)

• Side effects of medications (see the specific medication)
• Complications of surgery
• Myasthenic crisis (breathing difficulty), may be life threatening

When to Contact a Medical Professional

Call your health care provider if symptoms suggesting myasthenia gravis occur.

Go to the emergency room or call the local emergency number (such as 911) if breathing
difficulty or swallowing problems occur.