Stiripentol (Diacomit) treatment for child with myoclonic epilepsy in infancy resistant to conventional drugs

Severe myoclonoc epilepsy in infancy (SMEI) belongs to those epilepsies, which are difficult to treat. In most cases the seizures cannot be treated efficiently with the current antiepileptic drugs (AED). However, it is the reduction of seizures that influences the future development of the children to a high degree. Two children with SMEI where treated with stiripentol (STP), a cytochrom-450-inhibitor. At the early age of two months and 6 months a West-Syndrome has been diagnosed in both children. Although all different kinds of AED had been given, the frequency of seizures could not be reduced. A ketogene diet had not been tolerated by patient 1 whereas the diet had to be stopped with patient 2 because of considerable side effects. With increasing age of the children their epilepsies and EEGs changed to SMEI. At the age of 2 4/12 and 3 6/12 an add-on-therapy with STP and clobazam (CLB) was started. The dosage with STP finally was 50–60mg/kg, for CLB it was 0,2–0,25mg/kg. Within few weeks there was a significant reduction in frequency of seizures with both children. Apart from drowsiness no other clinical and chemical side effects could be stated. Unfortunately the frequency of seizures increased with patient 2 (first reduction of >50%) after a 6-week treatment, so STP had to be stopped. The treatment of patient 1 was continued. The attacks had nearly disappeared. After some months the frequency of seizures slightly increased. However the general state of health significantly improved.

Since 1978 STP has been known as an efficient AED. There are 2 major studies on epilepsy of infancy. One of these studies (Perez, J. et al. Epilepsia 1999) analyzes 212 children with partial epilepsy and SMEI, whereas 41 children were treated with SMEI in Chiron's study (Chiron, C. et al. Lancet 2000). In both studies a high rate of responders is described.

In one of our two cases there was a loss of efficacy. However, the seizure rate of the second patient could be improved on a long-term basis.