The short-term efficacy of combined hormone therapy in West syndrome

Abstract

SUMMARY Background Effective treatment protocols development for West syndrome (WS) is scientifically and economically significant. Aim To evaluate the comparative short term efficacy and tolerability of tetracosactide at a dose of 0.03– 0.05 mg/kg and dexamethasone at a dose of 0.3–0.5 mg/kg both combined with valproate at a dose of 30–40 mg/kg/day for WS therapy. The regimen was: 1 injection daily for 10 days, following with 5 injections every other day, then 5 injections every two days, plus a valproate. Material and Methods 79 infants (Group 1) received tetracosactide, 18 infants (Group 2) – dexamethasone. The demographic data and the main characteristics of WS were similar in both groups. Results The efficacy of tetracosactide exceeded that of dexamethasone: there were more responders to therapy in Group 1: 92.4% vs 72% (p = 0.0017). Tetracosactide produced faster results: 50.5% of patients in Group 1 experienced cessation of infantile spasms within the first 5 days of therapy versus 38.7% of patients in Group 2; infantile spasms ceased in 34% of patients in Group 1 on day 6–10, versus 22.2% of patients in Group 2. 74.6% of patients in Group 1 experienced normalization of EEG on day 10, versus 33.3% of patients in Group 2 (p = 0.04). A higher percentage of patients treated with dexamethasone exhibited multiregional activity on EEG by day 10. Tolerability was similar in both groups. All adverse effects were of mild to moderate severity Conclusion Tetracosactide therapy in combination with average therapeutic doses of valproate proved to be more effective in treating WS than the combination of dexamethasone and valproate.