Abstract
3,4-diaminopyridine (3,4-DAP) is known as effective therapy in the acute management of Lambert-Eaton myasthenic syndrome (LEMS). There are also reports with positive results in long-term therapy. We report a patient with a 6-year history of slowly progressive LEMS who was treated for 20 months with 3,4-DAP. The therapy led to an objective increase in muscle power. Over the period of treatment, there was only a slight increase in muscle weakness, and attempts at withdrawal of the therapy confirmed this disease progression. The clinical features correlated with the results of the stimulation EMGs, which showed a distinct decrease in the compound muscle action-potential without therapy, but only a slight decrease with the 3,4-DAP therapy. 3,4-DAP seems to be more effective in cases where symptoms are more marked, and even the autonomic symptoms of our patient were relieved. The only notable side-effect was paraesthesiae. 3,4-DAP seems to be an effective and acceptable long-term symptomatic therapy in LEMS.
Published Version
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