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Myasthenia gravis weakness may be stopped
Myasthenia gravis is an autoimmune disease that involves not only the severe muscle weakness but also resultant complications such as difficulty in breathing, difficulty in chewing and swallowing, slurred speech, droopy eyelids, and blurred or double vision.While myasthenia gravis cannot be cured, it is sometimes treated with surgery to remove the thymus, which plays a role in the immune system, or with various drugs, some having serious side effects. By preventing or reversing the muscle weakness, the other symptoms can be prevented or reversed as well.Now researchers at the Saint Louis University School of Medicine have shown that severe muscle weakness in myasthenia gravis can be prevented or reversed by blocking a key step in the immune response that brings on the disease.
Te immune response at the heart of the process is called a complement cascade.This is a complex chain of chemical reactions in which proteins bind together to attack a cell by punching a hole in it.When acetylcholine receptors are damaged in this way, muscle movement is severely impaired.
Using an animal model, the scientists found that they could prevent muscle weakness caused by myasthenia gravis or restore muscle strength by stopping the complement cascade at a step called C5 before the series of chemical reactions had finished.They did this by administering an anti-C-5 agent which targets one of the proteins in the cascade, thus stopping the process.
According to Henry J. Kaminski, M.D., professor and chairman of the Department of Neurology and Psychiatry at the Saint Louis University School of Medicine and one of the study's authors, the findings are encouraging enough that human clinical trials involving the C5 agent, eculizumab, are likely within the year.Dr. Kaminski states, "We believe this therapeutic approach has strong potential for improving the lives of patients with myasthenia gravis.And if it proves successful there, it could also one day help us find new therapies for other autoimmune disorders, such as rheumatoid arthritis and lupus."
Source: Saint LouisUniversityMedicalCenter, December 2007
