Vigabatrin and High-Dose Prednisolone Add-on Therapy for Patients with West Syndrome
Abstract number :
2.133
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2018
Submission ID :
501315
Source :
www.aesnet.org
Presentation date :
12/2/2018 4:04:48 PM
Published date :
Nov 5, 2018, 18:00 PM
Authors :
Ha Neul Lee, Yongin Severance Hospital, Yonsei University College of Medicine; Ara Ko, Pusan National University Children’s Hospital, Yangsan, Republic of Korea; Song Ee Youn, Severance Children's Hospital, Yonsei University College of Medicine; Hee
Rationale: Hormonal therapy and vigabatrin are now accepted as first-line or standard therapy for West syndrome (WS). However, the superiority among these drugs or between monotherapy or combination therapy is still in question. In this study, we designed a treatment protocol for WS and prospectively assessed its efficacy for controlling spasms, stabilizing electroencephalography (EEG), and developmental catch-up. Methods: In patients diagnosed WS, vigabatrin was first tried for 2 weeks, and prednisolone was added-on to vigabatrin after 2 weeks if patients do not respond to vigabatrin. More detailed protocol was as follows: vigabatrin 50 mg/kg/day for 1 day, followed by vigabatrin 100 mg/kg/day for 3 days, vigabatrin dose up to 150 mg/kg/day if spasms were still present or burden of amplitudes and epileptiform discharges (BASED) score of EEG was = 3 at day 5, 40 mg/day of prednisolone add-on if spasms were still present or BASED score of EEG was = 3 at day 14, and then prednisolone dose up to 60 mg/day if spasms were still present or BASED score of EEG was = 3 at day 21. Results: Sixty-six patients newly diagnosed with WS (median seizure onset age 5.7 [IQR, 4.1-7.1] months, median age at diagnosis 6.6 [IQR, 5.4-8.1] months, 40 [60.6%] boys) were subject to the vigabatrin and prednisolone add-on therapy protocol. Of 66 patients, 22 (33.3%) patients showed resolution of spasms and BASED score of = 2 after vigabatrin, and 26 (39.4%) patients showed resolution of spasms and BASED score of = 2 after prednisolone add-on, making total of 48 (72.7%) patients responsive to the protocol without relapse until 7 months after the diagnosis. The mental and psychomotor age quotients were higher at time of diagnosis and remained significantly higher 6 months after the diagnosis in responsive patients (all, p < 0.001). Serious adverse reactions leading to discontinuation or stepping down of the protocol were not observed. Conclusions: The treatment protocol with vigabatrin and prednisolone add-on therapy for West syndrome is effective and safe. Funding: None