Open trial with levodopa-carbidopa combination to patients with long-standing progressive myoclonus epilepsy

Six adult patients with long-standing progressive myoclonus epilepsy (PME) received levodopa combined with carbidopa in addition to their usual anticonvulsant regimen. During 5 weeks of treatment an improvement in daily activities and a decrease of action myoclonus were observed. The spontaneous EEG did not differ from the recordings prior to the trial, but the provoked EEG showed fewer reactions during provocations in 5 out of 6 patients. The treatment had no effect on psycho-motor reaction time or epileptic seizures. Pre-trial HVA values in the CSF of the PME patients were significantly lower than those of their epileptic controls, but during the trial CSF HVA values in PME patients increased markedly. No untoward side-effects were noted. The results suggest that there could be an alteration of dopaminergic tone in PME.