Pulsatile steroid treatment per os: A therapeutic option for children with pharmacoresistant epilepsies

Objectives: For many years steroids have been used in the treatment of pharmacoresistant childhood epilepsies. However, severe side effects are not rare and, therefore, limit its use. Therapeutic trials in Multiple Sclerosis using intravenous pulsatile steroids demonstrated good efficacy with fewer side effects compared to conventional steroid treatment. Therefore, the following questions were asked in the present study:

1. Are these results transferable to the treatment of pharmacoresistant epilepsies in childhood?

2. Is a simplified scheme using pulsatile steroids per os in outpatients feasible?

Material and Methods: 13 patients from our department with drug resistant epilepsies (West syndrome N=6, electrical status epilepticus during slow sleep N=4, focal epilepsy with secondary bisynchrony in the EEG N=2, childhood absence epilepsy N=1) aged 6 months to 16 years were treated using a pulsatile steroid scheme per os (25mg/kg/d for three days once a week for four weeks followed by a variable tapering phase). Epilepsy and EEG data as well as adverse side effects were documented before treatment, four weeks later and at the end of the therapy.

Results: West syndrome (N=6): Three children became seizure free, and EEG improved markedly. In one child seizure frequency was reduced, and EEG improved. Two children showed no effect. Electrical status epilepticus during slow sleep (Landau Kleffner syndrome N=2, CSWS N=1, benign focal epilepsy N=1): EEG impropved markedly in one and slightly in two children. In one child no effect was found. The two children with focal epilepsy and secondary bisynchrony in the EEG showed no effect. The child with pharmacoresistant absence epilepsy became seizure free.

There have been no serious side effects in all of the children.

Conclusions: In this extremely pharmacoresistant cohort pulsatile steroid therapy per os was partly effective without showing severe side effects. In our opinion, a broader use of steroids in childhood epilepsies should be considered. Controlled prospective studies are needed to confirm these preliminary findings.