Myasthenia Gravis Question

[Dan__0]

    
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Posted by ccf neuro M.D. on June 01, 1997 at 15:12:11:

In Reply to: Myasthenia Gravis Question posted by Dan on May 26, 1997 at 17:32:40:

: My mother-in-law has just been diagnosed with Myasthenia Gravis. She would like to know if others have had symptoms like hers and if the treatments seemed to have helped. Her symptoms are as follows: She cannot hold her head up, so her chin drops down to her chest. Also it feels as if she has 100lbs on her shoulders and back. Her most pronounced problem is her neck muscles dont seem to have any strength. Thanks in advance for any help anyone can offer.
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Weakness of the neck muscles that help hold the head up is a very common symptom of myesthenia gravis. Other commonly involved muscles are the muscles that control eye movements (resulting in double vision), and weakness of the muscles of the pharynx (throat) that result in trouble swallowing and a nasal voice. Generalized weakness and fatigue, especially as the day wears on, are symptoms of more generalized myesthenia gravis. Underlying conditions associated with myesthenia gravis include tumors of the thymus gland called thymomas, and hyperthyroidism (overactivity of the thyroid gland). Tests should be done to make sure that neither of these are present. The cause of myesthenia gravis is antibodies that cross-react with the small "junctions" between nerve fibers and muscles they go to, which block the chemical "messanger" from the nerve terminal to the muscle cell, preventing it from contracting as well as it should. Treatment measures are aimed at counteracting this problem. The first and simplest treat-
ment is with so-called acetylcholinesterase inhibitor drugs. These enzymes block an enzyme that normally digests away the chemical that transmits the message from the nerve to the muscle, allowing it to exist for a longer time and overcome the blocking effects of the antibodies. Mestinon (physostigmine) is the most commonly used example of such drugs. A second treatment option is cortisone drugs called corticosteroids. These work by suppressing the immune system cells that are producing the antibodies, but must be started very gradually, as the very same drugs have a temporary adverse effect on the "junctions", making the person weaker initially before s/he gets stronger. Thymectomy, removal of the thymus gland (which generates many of the immune cells that produce the antibodies) is another treatment option, but effects are not seen for years. Finally, in crisis situations, a treatment called plasmapheresis, where the plasma of the blood is removed (along with the antibodies responsible for producing the
disease can be used, and results in usually rapid reversal of symptoms; weakness returns, however, within a few weeks of completion of such treatments as more antibodies are produced. In rare instances, more potent and dangerous immune suppressant drugs like Imuran may be used, most often in conjunction with a corticosteroid medication, and/or in an effort to reduce the required doses of corticosteroids used to avoid complications of their long term use. The vast majority of patients with myesthenia gravis go on to lead essentially normal lives with well controlled symptoms. Long term management by a neurologist with experience in treating the disease is an essential component of successful control of the disease, and in handling the so-called "crises", or sudden worsenings of the disease, that sometimes occur. Also, the neurologist is essential to have whenever the patient is undergoing surgery or is in the hospital, as certain antibiotics, anesthetic agents, and other medications may greatly worsen weakness if accidentally given. The Myesthenia Gravis Foundation of America (MGF) is a good source of additional patient information on this disease. They may be contacted at: 222 South Riverside Plaza--Suite 1540 Chicago, IL 60606; phone # [phone]; FAX [phone]. If your mother would be intersted in seeing a Cleveland Clinic neurologist for management of her disease, I would suggest Dr. Kerry Levin, a world expert in the disease. Our appointment number is 1-800-223-2273 extension 45559. Information provided in the Neurology Forum is intended for general medical informational purposes only. Actual diagnosis, treatment, and management options for your specific medical condition should be strictly in conjunction with your treating physician(s). I hope you and your mother find this information helpful.