Abstract

Investigators from the National Epilepsy Center, Shizuoka, Gifu University, Tokyo Women’s Medical University, National Center of Neurology and Psychiatry, and Okayama University School of Medicine, Japan, examined seizure, cognitive, and motor outcomes in 49 patients referred with Rasmussen encephalitis (RS) and recently had received initial immunomodulatory therapy: regular IV immunoglobulin (IVIg) at a dose of 100 mg/kg/day, methylprednisolone steroid pulse therapy at a dose of 30 mg/kg/day (children) for 3 days, and tacrolimus 0.1 mg/kg/day.

Highlights

  • Investigators from the National Epilepsy Center, Shizuoka, Gifu University, Tokyo Women’s Medical University, National Center of Neurology and Psychiatry, and Okayama University School of Medicine, Japan, examined seizure, cognitive, and motor outcomes in 49 patients referred with Rasmussen encephalitis (RS) and recently had received initial immunomodulatory therapy: regular IV immunoglobulin (IVIg) at a dose of 100 mg/kg/day, methylprednisolone steroid pulse therapy at a dose of 30 mg/kg/day for 3 days, and tacrolimus 0.1 mg/kg/day

  • IQ higher than 80 was attained by 50% of patients treated with steroid pulse therapy, in 43% following IVIg therapy, in 29% after tacrolimus therapy, and 0% in patients treated surgically by functional hemispherectomy (FH)

  • Motor function was aggravated in 100% patients treated by FH, 62% by regular IVIg, and 10% by regular steroid pulse therapy

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Summary

Introduction

Investigators from the National Epilepsy Center, Shizuoka, Gifu University, Tokyo Women’s Medical University, National Center of Neurology and Psychiatry, and Okayama University School of Medicine, Japan, examined seizure, cognitive, and motor outcomes in 49 patients referred with Rasmussen encephalitis (RS) and recently had received initial immunomodulatory therapy: regular IV immunoglobulin (IVIg) at a dose of 100 mg/kg/day, methylprednisolone steroid pulse therapy at a dose of 30 mg/kg/day (children) for 3 days, and tacrolimus 0.1 mg/kg/day. Seizure-free rate was 71% after functional hemispherectomy (FH), seizure response rate was 81% for regular steroid pulse therapy, 42% for tacrolimus therapy, and 23% for regular IVIg therapy. IQ higher than 80 was attained by 50% of patients treated with steroid pulse therapy, in 43% following IVIg therapy, in 29% after tacrolimus therapy, and 0% in patients treated surgically by FH.

Results
Conclusion
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