1. What is the mechanism behind LES?
2. What is the presentation of LES?
3. What does muscle biopsy show?
4. What are the EMG findings in LES?
5. What is the treatment of LES?
Answers:
1. Autoimmune attack on the presynaptic membrane, decreasing Ca entry into the cell and decreased release of ACh. Associated with small cell lung cancer.
2. Proximal fatigue and weakness, LE>UE, abnormal MSR, exacerbated with rest and improved with exercise, rarely involves neck or face or bulbar.
3. Overdevelopment of neuromuscular junction.
4. Normal SNAP, low amp CMAP, unstable MUAP.
5. Treat underlying malignancy, corticosteroids, immunosuppression, plasmapheresis, guanidine (incr ACh quanta).
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