Myasthenia Gravis

The "acquired" form of myasthenia gravis (MG) is an autoimmune disease with an incidence approaching 1 in 10,000. It involves an antibody response against part of the a-subunit of the AChR in muscle. This leads to degradation of AChRs, reduction of synaptic infoldings, and weakness due to diminished postjunctional response. Because anti-AChR antibodies are found in nearly 90% of MG patients, serum testing can serve as a diagnostic tool. MG is often found associated with abnormalities of the thymus, which contains a protein that is immunologically related to muscle AChR.

Standard treatment consists of anti-AChE agents such as pyridostigmine, whose effect is long-lasting and predictable. A short acting anti-AchE, such as edrophonium, can be used to differentiate between a myasthenic crisis (insufficient treatment) and a cholinergic crisis (overtreat-ment with anti-AChEs). Thymectomy is a good option for patients under 50 years of age. Immunosuppressive agents, such as corticosteroids and possibly azathioprine or cyclosporine A, are also effective. Plasmapheresis is beneficial, but the effect is transient.

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