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Anti-AChR antibodies reduce the number of available AChRs at the NMJ. Postsynaptic folds are flattened or “simplified,” with resulting inefficient neuromuscular transmission. During repeated or sustained muscle contraction, decrease in amount of ACh released per nerve impulse (“presynaptic rundown,” a normal occurrence), combined with disease-specific decrease in postsynaptic AChRs, results in pathologic fatigue. Thymus is abnormal in 75% of pts (65% hyperplasia, 10% thymoma). Other autoimmune diseases may coexist: Hashimoto's thyroiditis, Graves' disease, rheumatoid arthritis, systemic lupus erythematosus.

Outline

Section 14. Neurology