- Discussion:
- rare disease usually found in association with occult neoplasms of the lung or other organs;
- defect lies in the release of acetylcholine from prejunctional axon terminals;
- main danger to patients is the manifestation of the underlying neoplasm;
- Clinical Findings:
- fluctuating weakness involving proximal limb girdle;
- unlike Myasthenia Gravis, weakness is worse on initiating exercise but then subsequently improves w/ exercise;
- various therapeutic regimens have not helped this disease;
- muscle weakness tends to be relatively slight