Abstracts

Rationale: To determine the efficacy of add-on pulse dose steroid treatment (PDST) for pediatric patients with intractable seizure disorders. The clinical effectiveness of PDST has previously been reported in the Landau-Kleffner and autistic spectrum disorder populations, but as yet has not been evaluated in patients with primary diagnoses of epileptic disorders in childhood. Methods: Seventeen consecutive patients (10 M, 7 F, mean age at treatment start = 5.91 yrs.) with an abnormal 24-hour EEG and history of medically intractable seizures were enrolled. Seizure types included complex partial, myoclonic, primary generalized, tonic clonic, and tonic clonic with secondary generalization. Ss had failed an average of 3.67 medications prior to start. Ten were on the Ketogenic diet prior to beginning steroids; 3 were maintained on the diet throughout the study. Five Ss were on daily steroids prior to starting the pulse dosage. All Ss were on a daily anticonvulsant at the time of add-on therapy and were given 5-10mg/kg of prednisone or prelone in a divided dose q 5-7days. Results: 11/17 children (65%) showed EEG improvements when re-evaluated at a minimum of 3 mos after initiation of the dosage; 3 had complete normalization of their EEGs after 6 months of treatment. 10/17 had improved seizure frequency (59%). Side effects were minimal (e.g., tired, hyperactive, mood swings); and children did not show Cushingoid features. Children remained on PDST for an average of 1.20 yrs (range .33-3.04 yrs). Reasons for discontinuing PDST included no decrease in seizure frequency (n=4), addition of new anticonvulsants (n=3), or aggressiveness (n=2). Conclusions: PDST as an add-on regimen may help improve epileptiform activity and decrease overt clinical seizure frequency in pediatric Ss with intractable seizure disorders who have been maintained on chronic anticonvulsant or ketogenic diet therapies without benefit. This offers another option for intractable patients without the risk of Cushingoid side effects seen with daily steroids or ACTH. In patients who had previously responded to daily steroids, PDST may offer a long-term option to maintain seizure control.