- Last Update On : 2013-08-04
Myasthenia gravis is an acquired autoimmune disease characterized by muscle weakness and progressive fatigability. The fundamental defect is failure of neuromuscular transmission due to formation of specific autoantibodies directed against the acetylcholine receptor (AChR) in the postsynaptic membrane of skeletal muscle. These antibodies block acetylcholine-binding sites, damage the postsynaptic membrane, and accelerate receptor degradation. Synaptic transmission fails when the autoantibodies cause a critical loss of the cation channel protein, which is required to activate the muscle action potential.
Acetylcholine receptor (AChR) binding antibody is positive in approximately 90% of nonimmunosuppressed patients with generalized MG. Values >0.02 nmol/L are consistent with a diagnosis of acquired myasthenia gravis in patients with supportive clinical and electrophysiological findings. Antibody levels are usually high in generalized myasthenia gravis and in myasthenia gravis associated with thymoma. Results may be negative in the first 12 months after symptoms of MG appear or during immunosuppressant therapy. The frequency of AChR binding antibody detection is lower in MG patients with weakness clinically restricted to ocular muscles (70%), and antibody titers are generally lower (0.03-1.0 nmol/L). Positive AChR results may also be found in patients with Lambert Eaton Syndrome and autoimmune liver disease. Generally, there is not a good correlation between antibody titer and the severity of weakness.
AChR binding antibody is recommended as the initial case finding test because it is faster and more economical to perform. If negative, the AChR modulating test is recommended. AChR modulating antibodies are indicated when AchR binding antibody is negative and when myasthenia gravis is mild, ocular restricted, or of less than one year duration. Reference range for AChR modulating antibody 0-20%. Results are reported as the percent loss of AChR.
Specimen requirement is a red top of tube of blood.