Long term spasm control and developmental outcomes after combined therapy (prolonged oral steroids and Vigabatrin) in children with West syndrome secondary to perinatal brain injury.

Background: We wanted to study whether prolonged oral steroids (six months) combined with Vigabatrin results in better long term outcome in terms of spasm control, EEG improvement and developmental gains. Methodology: Between April 2018 to December 2021, 56 children with symptomatic West syndrome (Perinatal insult) were enrolled and advised 2 weeks of daily followed by 4 weeks of alternate day (i.e. 6 weeks) of ACTH with Vigabatrin for 6 months. After ACTH, all patients were advised prolonged low dose oral Prednisolone, which was continued for 6 months. Results: Follow up was available in 22 (3 and 6 months), 21 (9 months), 20 (1 year) and 19 patients at 2 years. Seven patients who received six months or more of oral steroids and Vigabatrin showed spasm control in 100% at 3, 6 and 9 months and 85% at 2 years. The developmental gains were seen in 71% (3 months), 85% at 6 months, 57% at 9 months and 2 years. In contrast, 15 patients (who received either one or none of both) showed spasm control in 60% (3 months), 78% (9 months) and 83% . Their developmental gains were considerably lower 60% (3 months), 64% (6 months), 43% (9 months) and 41% at 2 years, though not statistically significant. Oral steroids were well tolerated Conclusion- Combined ACTH plus Vigabatrin followed by prolonged low dose oral steroids was associated with sustained spasm control and better developmental outcomes at 2 years. More RCT to confirm these findings are needed.
Keywords: west syndrome, spasms, outcome, development, steroids, vigabatrin