Monitoring and Optimizing Treatment Response in LEMS Management

This segment outlines practical strategies for monitoring therapeutic response, managing side effects, and titrating amifampridine in patients with Lambert-Eaton myasthenic syndrome (LEMS). Expert faculty explain that amifampridine is generally well tolerated, with early symptoms such as distal tingling, perioral numbness, or mild gastrointestinal upset often resolving over time. They emphasize the importance of vigilance for rare but serious risks—particularly seizures at doses above 100 mg/day, especially in individuals with brain metastases or prior seizure history, and potential cardiac effects at very high doses in those with underlying abnormalities. Standard practice begins with 10–20 mg divided three times daily, titrated toward a maximum of 80–100 mg as tolerated. They monitors patients monthly, adjusting more quickly when benefits are unclear. Additional therapies such as prednisone, IVIG, or long-term immunosuppressants require close surveillance for infection, metabolic complications, or hypercoagulability. The panel notes that while amifampridine remains the cornerstone of treatment, emerging therapies are limited, underscoring the need for multidisciplinary collaboration.