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- Autoantibodies are formed which bind to acetylcholine receptors on skeletal muscle.
- Anti-acetylcholine antibodies impair transmission at the neuromuscular junction
- Symptoms and sings: ptosis, diplopia, dysarthria (speech disorder), dysphagia (difficulty swallowing), facial paresis, proximal limb weakness, and shortness of breath
- Management
- Acetylcholinesterase inhibitors (e.g. pyridostigmine)
- Plasmapheresis may directly remove auto-antibodies.
- Immunosuppressive therapies are aimed at reduction in the synthesis of auto-antibodies
- Indication for mechanical ventilation includes forced vital capacity (FVC) 15 mL/kg or less (normal ≥60 mL/kg) and negative inspiratory force (NIF) 20 cm H2O or less (normal ≥70 cm H2O).
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