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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.

Kavita Srivastava
Bharati Vidyapeeth University Medical College, Pune, India
India

Brahmini Korrai
Bharati Vidyapeeth University Medical College, Pune, India
India

Sneha Mahajan
Bharati Vidyapeeth University Medical College, Pune, India
India

 


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