3,4-Diaminopyridine for the treatment of myasthenia gravis with electrophysiological patterns of Lambert-Eaton myasthenic syndrome

作者：Lee Moon Kyu; Sunwoo Il Nam; Kim Seung Min^*

来源：Journal of Clinical Neuroscience, 2018, 50: 194-198.

DOI：10.1016/j.jocn.2018.01.024

• 3,4-Diaminopyridine
• Treatment
• Anti-acetylcholine receptor antibody
• Myasthenia gravis
• Lambert-Eaton myasthenic syndrome

3,4-Diaminopyridine (34DAP) is a presynaptic transmission enhancer. Its efficacy for Lambert-Eaton myasthenic syndrome (LEMS) and myasthenia gravis (MG) was demonstrated. However, there are cases sharing the characteristics of both disease and the effect of 34DAP in "gray zone" patients is sparse. Recently, we prescribed 34DAP to five anti-acetylcholine receptor antibody-positive MG patients with electrophysiological LEMS patterns and three LEMS patients, and carefully monitored the responses. Sero-positive MG patients exhibited more favorable responses than LEMS patients. The combination of 34DAP and pyridostigmine resulted in the best outcomes. No significant side effects were recorded during the follow-up period. In conclusion, this study results provide evidence that 34DAP could be effective in sero-positive MG patients with pre-synaptic dysfunction.

• 出版日期2018-4