Rasmussen's encephalitis: From immune pathogenesis towards targeted-therapy

A. Orsini, T. Foiadelli, N. Carli, G. Costagliola, B. Masini, A. Bonuccelli, S. Savasta, D. Peroni, R. Consolini, P. Striano

Research output: Contribution to journalReview article

Abstract

Rasmussen encephalitis (RE) is a unilateral hemispheric encephalitis whose main clinical features include refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline. Despite the autoimmune pathogenesis of RE, the only definitive therapeutic option is currently represented by surgery. We review the clinical features, the immune pathogenesis, and the available therapeutic options for RE, with special focus on immunosuppressive agents. The research includes systematic reviews, meta-analyses, observational studies, clinical trials, cases series and reports, until 2020. The use of immunosuppressive agents in RE is supported by the evidence of an autoimmune involvement of the central nervous system in this condition. Although often insufficient to modify the disease course and to achieve symptomatic control, immune therapy can be effective in patients with slow disease progression or in patients in which surgery is not applicable. Moreover, the documentation of T-cell involvement in the pathogenesis of RE, with a specific cytokine pattern, opens a window of opportunity for the use of T-targeted therapies and biologic drugs (i.e. anti-TNFα agents) in the treatment of this disease.

Original languageEnglish
Pages (from-to)76-83
Number of pages8
JournalSeizure
Volume81
DOIs
Publication statusPublished - Oct 2020

Keywords

  • Autoimmunity
  • Biologic drugs
  • Epilepsia partialis continua
  • Epilepsy
  • Immunosuppressive agents
  • Immunotherapy
  • Rasmussen encephalitis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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  • Cite this

    Orsini, A., Foiadelli, T., Carli, N., Costagliola, G., Masini, B., Bonuccelli, A., Savasta, S., Peroni, D., Consolini, R., & Striano, P. (2020). Rasmussen's encephalitis: From immune pathogenesis towards targeted-therapy. Seizure, 81, 76-83. https://doi.org/10.1016/j.seizure.2020.07.023