Abstracts

Severe myoclonic epilepsy of infancy (SMEI) or Dravet syndrome is a catastrophic encephalopathy with onset in the first year of life, more often with febrile hemiclonic or generalized status epilepticus, and secondary developmental delay. Although valproate, topiramate and stiripentol may give some benefit, drug treatment of SMEI is usually disappointing. Levetiracetam (LEV) is a new antiepileptic drug (AED) with a mechanism of action distinct from that of other AEDs and broad spectrum of activity including antimyoclonic activity. We evaluated the efficacy and tolerability of LEV in patients with diagnosis of SMEI., 10 children (mean age 8.4 years; range 4-16 years) with clinical diagnosis of SMEI were enrolled in a multicentric, prospective, open-label study. 5 out of 10 patients showed [italic]SCN1A [/italic]mutation. A mean of 2 (range: 1-5) AEDs were tried before LEV trial. LEV was given in add-on up to the dose of about 50-60 mg/kg/day. Responders were defined as having more than 50% reduction in the frequency of seizures compared with baseline., After a mean period of 8.5 months of treatment (range 6-12 months), 7 patients had more than 50% reduction in seizure frequency. None of the patient was seizure-free. LEV was generally well-tolerated. Mild adverse events (i.e., sedation, irritability) occurred in three patients., This pilot study suggests that LEV may be a well-tolerated drug that may give some benefit in SMEI. Randomized placebo-controlled studies should be considered to evaluate further this promising therapy.,