Abstracts

West syndrome(WS) is a severe age-dependent intractable epilepsy in infants that frequently results in mental retaldation. ACTH or glucocorticoids are among several effective treatments in WS, but the advantages and disadvantages of these two therapies are still unknown. In the past study, liposteroid(dexamethasone palmitate)has been used for the treatment of WS and compared with ACTH therapy in relation to therapeutic effect and adverse reactions. Then, the initial effects observed are the same as for ACTH therapy. In this study, liposteroid(dexamethasone palmitate)therapy was tried in new protocol for WS and its related syndrome to shorten the therapeutic period and to reduce the relapse rate. A single intravenous injection of liposteroid(0.25mg/kg) was administered twelve times in one month(total dosage=3.0mg/kg) to one patient with WS aged 5 months and two patients with WS s epileptic sequelae after ACTH therapy aged 12 and 25 months. All three patients had uncontrollable daily seizures by the conventional antiepileptic drugs, such as VPA, CZP, or ZNS. Nodding spasm and hypsarrhythmia on EEG disappeared in one patient with WS within six doses, a 50% decrease of seizures and EEG improvement were found in another patient. No notable effects were seen in the other patient. There were no notable adverse reactions throughout the therapy. Efficacy can be determined in this new experimental liposteroid therapy earlier than with conventional liposteroid therapy. The liposteroid therapy under the new protocol can be performed safety and is useful for those susceptible for the adverse reactions by the conventional treatment or those without effective treatment.