Case report: Rasmussen’s Encephalitis treated successfully with rituximab

Juliana Lockman,Kenneth D Laxer,Scheherazade Le,Robert S Fisher

doi:10.7759/cureus.136

Abstract

Rasmussen's encephalitis (RE), or chronic focal encephalitis, is a progressive neurologic condition consisting of cognitive regression, hemiparesis, and intractable epilepsy. While the etiology is unknown, evidence supports an autoimmune basis. Although steroids, plasmapheresis, and intravenous immunoglobulin (IVIg) may afford limited benefit, standard of care for pediatric cases typically involves hemispherectomy. In this report, we present a case of adult-onset Rasmussen's encephalitis with intractable epilepsy, ultimately responsive to a rituximab (Rituxan®) anti-B lymphocyte treatment protocol.

Highlights

Rasmussen's encephalitis (RE) or chronic focal encephalitis is a progressive neurologic disease characterized by cognitive regression, hemiparesis, and focal onset epilepsy [1]
Epilepsy can become intractable and is frequently heralded by epilepsia partialis continua progressing to other seizure types
A key question is whether the improvement was attributable to the RTX. This cannot be ascertained in an uncontrolled case report, but it is notable that she had no such improvement for the six prior years and improvement occurred again after a second RTX course

Summary

Introduction

Rasmussen's encephalitis (RE) or chronic focal encephalitis is a progressive neurologic disease characterized by cognitive regression, hemiparesis, and focal onset epilepsy [1]. Her exam showed normal language skills but an irregular, moderate-to-high amplitude twitching movements of her right hand at times with extension of her hand or individual fingers. The motor function in her right arm worsened and seizures spread more often to the right leg and face She experienced a decline in her ability to do activities, such as dancing, painting, and writing. One year follow-up revealed substantial improvement in seizure frequency and right hand dexterity She was able to regain her ability to do activities, such as painting, dancing, and writing. Interval neuroimaging in 2012 continued to show slight progressive left hemispheric atrophy

Discussion

Disclosures

Findings

Granata T