Myasthenia gravis (autoimmune disease of the nerves, muscles)


Description

An autoimmune disorder characterized by progressive fatigue and muscle weakness. Muscle strength may be normal at the beginning of a task such as talking; then it becomes progressively weaker as the activity continues. Antibodies block, alter, or destroy the receptors for acetylcholine at the neuromuscular junction, preventing normal muscle contraction. It is more common in young females. It is sometimes associated with a tumor of the thymus (thymoma).

Symptoms

Muscle weakness, muscle weakness that becomes worse as the activity continues and returns after rest, double vision, slurred speech, swallowing problems.

Tests

Common tests used for diagnosis and treatment

Workup:
A history and physical exam will be performed. The following tests are done to confirm the diagnosis: EMG, acetylcholine receptor antibodies, and/or Tensilon (edrophonium) test. A chest CT or MRI scan may be done to look for a thymus tumor (thymoma).

Tests:
CT Scan, Magnetic resonance imaging (MRI)

Other Specific Tests: EMG, acetylcholine receptor antibodies, Tensilon (edrophonium) test

Specialists:
Neurology, Pediatric Neurology

Treatment

No known cure is available and treatment is aimed at reducing the weakness. Medications to increase the amount of acetylcholine at the muscle receptor are prescribed (neostigmine, pyridostigmine). Medications to suppress the immune system may be recommended (prednisone, azathioprine, cyclosporine, mycophenolate mofetil). Symptoms can vary. Severe symptoms may be treated with plasmapheresis or intravenous immunoglobulin. 
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