Adult onset Rasmussen’s encephalitis associated with reflex language induced seizures responsive to Rituximab therapy

Rasmussen’s encephalitis (RE) is a rare, focal and progressive inflammatory disorder of unknown aetiology, resulting in unilateral hemispheric atrophy, contralateral hemiparesis and hemianopia, dysphasia with language-dominant hemispheric involvement and intractable epilepsy. RE is associated with polymorphic seizure types including epilepsia partialis continua (EPC) [1]. Current treatment strategies principally involve the combined use of corticosteroids, intravenous immunoglobulin (IVIG) and in adults, plasma exchange (PEX) although the response is variable and may be short-lived. Other treatments with greater efficacy and the potential to alter the natural history of RE are needed. Rituximab, a chimeric monoclonal antibody against CD20, has shown promise in RE although reports of its use in this disorder are few. Here, we present an unusual case of language-induced reflex seizures occurring in the context of adult-onset Rasmussen’s encephalitis and report on the clinical response to immunomodulation and the efficacy of Rituximab.