Objective: Hormonal therapy and vigabatrin are now accepted as the first-line or standard therapies for West syndrome (WS). However, the superiority of these drugs in terms of monotherapy or combination therapy is still in question. In this study, we designed a treatment protocol for WS and prospectively assessed the efficacy of these therapies in controlling spasms, stabilizing electroencephalography (EEG), and allowing for developmental catch-up. Methods: In patients diagnosed with WS, vigabatrin was first administered alone for 2 weeks, and then prednisolone was administered in combination with vigabatrin if patients did not respond to vigabatrin. The detailed drug administration protocol was as follows: vigabatrin 50 mg/kg/day for 1 day, followed by vigabatrin 100 mg/kg/day for 3 days, vigabatrin 150 mg/kg/day if spasms were still present or the burden of amplitudes and epileptiform discharges (BASED) score on EEG was ≥3 on day 5; 40 mg/day of prednisolone was added if spasms were still present or the BASED score was ≥3 on day 14. The prednisolone dose was increased to 60 mg/day if spasms were still present or the BASED score was ≥3 on 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, n = 40 [60.6%] boys) were subjected to the vigabatrin and prednisolone therapy protocol. Of the 66 patients, 22 (33.3%) patients showed resolution of spasms and a BASED score of ≤2 after vigabatrin alone, and 26 (39.4%) patients showed resolution of spasms and a BASED score of ≤2 after a combination of vigabatrin and prednisolone, for a total of 48 (72.7%) patients who were responsive to the protocol without relapse for at least 7 months after WS diagnosis. The mental and psychomotor age quotients were higher at the time of diagnosis and remained significantly higher 6 months after the diagnosis in responsive patients (p < 0.001). No serious adverse reactions leading to discontinuation or reduction of drug doses were observed. Conclusion: Using a treatment protocol involving vigabatrin and prednisolone for WS, 72.7% of patients showed resolution of spasms and a BASED score of ≤2. This study also found that this drug administration protocol was safe. However, further studies are warranted as this study describes results from observational study with limited sample size.
Bibliographical noteFunding Information:
This research was supported by a grant from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health & Welfare, Republic of Korea (grant number: HI15C1601).
© 2018 Elsevier B.V.
All Science Journal Classification (ASJC) codes
- Clinical Neurology