Abstracts

Rationale: Levetiracetam showed to be effective in the treatment of myoclonic seizures. The aim of this study is to evaluate the efficacy of levetiracetam (LEV) in the treatment of myoclonus in Creutzfeldt-Jacob disease (CJD). Methods: Since 2003 we treated with LEV 6 patients with CJD who presented, initially or during the course of the disease, mild, moderate or intense myoclonus involving subcontinuously face and arms. Patients were three men (mean age at onset 56 yrs ) and three women (mean age at onset 61 yrs). Three patients had a sporadic and three a genetic form of CJD. Five patients had a polymorphism at codon 129 Met/Met and only one had no polymorphism but showed E200K gene mutation of PRP. EEG showed in two cases periodic slow waves complex , in two frontal intermittent rythmic delta activity and in two diffuse, asymmetrical theta. MRI showed in 4 cases caudate and lenticular hyperintensity and 2 diffuse cerebral atrophy. LEV was administered at doses of 2000-3000 mg/daily with low titration. Treatment lasted 3-6 months. At present, only one patients is still alive; the survival of dead ones was 2-12 months. Results: All patients showed during treatment, a marked reduction of myoclonus from moderate intense to mild form; some patients were myoclonus-free during part of the day.Conclusions: Initially or during the course of CJD, approximately 90% of patients present movement disorders, especially generalised myoclonus. Many drugs have been used in the treatment, including prednisone, levodopa, haloperidol, benzodiazepines and anticonvulsants. On the basis of our results, we can recommend LEV as a safe and effective treatment of myoclonus in CJD.