Vigabatrin and high-dose prednisolone therapy for patients with West syndrome.

Abstract

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.

• Authors: Ara Ko, Song Ee Youn, Hee Jung Chung, Se Hee Kim, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
• Journal: Epilepsy research (Epilepsy Res) Vol. 145 Pg. 127-133 (09 2018) ISSN: 1872-6844 [Electronic] Netherlands
• PMID: 29966811 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2018 Elsevier B.V. All rights reserved.

Chemical References

• Anti-Inflammatory Agents
• Anticonvulsants
• Prednisolone
• Vigabatrin

Topics

• Anti-Inflammatory Agents (therapeutic use)
• Anticonvulsants (therapeutic use)
• Child
• Child, Preschool
• Dose-Response Relationship, Drug
• Female
• Follow-Up Studies
• Humans
• Infant
• Male
• Prednisolone (therapeutic use)
• Spasms, Infantile (drug therapy, physiopathology)
• Treatment Outcome
• Vigabatrin (therapeutic use)