We read with interest Joseph Sullivan's commentary on the systematic meta-analysis by Kassai et al.,1 who reviewed the data available on the treatment of severe myoclonic epilepsy in infancy (SMEI) or Dravet syndrome. We agree with Sullivan's view that the accurate diagnosis and classification of children into specific epilepsy syndromes can be extremely helpful for all clinicians who care for children with epilepsy, not only for counseling purposes but also for guiding treatment decisions.
SMEI is one of the most deleterious epilepsy syndromes during childhood and its treatment remains challenging. The only randomized controlled trials in SMEI published to date2 showed the efficacy of stiripentol when added to clobazam and valproate treatment, even though the duration of follow-up was short and the number of enrolled children was small.
We recently conducted an open-label, add-on trial to evaluate the efficacy and safety of levetiracetam in a large series of patients with SMEI, and showed that treatment with relatively high doses of levetiracetam (50–60 mg/kg daily) markedly reduced tonic–clonic, myoclonic and partial seizures in approximately two thirds of the patients.3 These data suggest that levetiracetam is another treatment option for SMEI.
The evaluation of drug efficacy in a homogeneous subgroup of patients is an interesting strategy in the search for treatments for rare and severe diseases such as SMEI. Withdrawal-design trials with replacement of conventional treatments by new antiepileptic drugs seem to be appropriate and ethical tools in this context. However, although seizure response is a clinically relevant end point, long-term follow-up should also address the important issues of cognitive, developmental and behavioral outcomes in these patients.
Kassaï B et al. (2007) Severe myoclonic epilepsy in infancy: a systematic review and a meta-analysis of individual patient data. Epilepsia 49: 343–348
Chiron C (2008) et al. (2000) Stiripentol in severe myoclonic epilepsy in infancy: a randomised placebo-controlled syndrome-dedicated trial. STICLO study group. Lancet 356: 1638–1642
Striano P et al. (2007) An open-label trial of levetiracetam in severe myoclonic epilepsy of infancy. Neurology 69: 250–254
The authors declare no competing financial interests.
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Striano, P., Striano, S. & Zara, F. Reply to “Epilepsies in children—the power of making a syndrome diagnosis”. Nat Rev Neurol 4, E3 (2008). https://doi.org/10.1038/ncpneuro0831