Rasmussen's Encephalitis: A Literary Review

Abstract

Usually affecting one hemisphere of the brain, Rasmussen's encephalitis (RE) is a persistent inflammatory disease of unclear origin. Rasmussen and colleagues presumed a viral etiology of the sickness in their first description. Later, the condition was linked to autoantibodies that were in the blood. Recently, it was shown that the cause of RE was a cytotoxic T-cell reaction to neurons. RE may be identified histopathologically by cortical inflammation, neuronal degeneration, and cerebral hemispheric-specific gliosis. The hemisphere is affected by increasing multilocular inflammation. To diagnose patients sooner and to evaluate whether the aforementioned phenomena are primary or secondary, it is essential to continue the search for a primary immunological or viral component. This information is crucial for determining the effectiveness of immunotherapy. RE-related seizures can only now be managed surgically. The only procedure that works is complete hemispheric disconnection (hemidisconnection), which may be done as either a (functional) hemispherectomy or hemispherectomy. Although thalidomide has been anecdotally reported, its safety profile prevents it from being used as a first-line treatment despite having a noticeable effect on the frequency and severity of seizures. Finding the disease's root causes more quickly by combining descriptive clinical studies, genetic testing, and early histological evaluation of RE tissue specimens to check for viral and autoimmune pathogenesis. Creating appropriate in vitro or animal models will enable the study of causality, perhaps directing clinical trials.

Keywords: epilepsia partialis; glur3 antibodies; hemidisconnection; meningitis; nmda receptor-mediated neuronal excitability; rasmussen's encephalitis.

Figure 1. The selection process of articles used in this study.

Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) flowchart for the keywords used in the literature review.

Figure 2. Pathogenetic model of Rasmussen encephalitis.

This image is taken from an open-access journal licensed under a Creative Commons Attribution 4.0 International License (Orsini et al. [40]). MMF: Mycophenolate mofetil

Figure 3. Therapeutic approach to Rasmussen encephalitis.