Medical treatment of Rasmussen's Encephalitis: A systematic review

Area of Science:

• Neurology
• Immunology
• Pediatric Neurology

Background:

• Rasmussen's encephalitis (RE) is a severe, rare, chronic inflammatory brain disease.
• It leads to drug-resistant epilepsy, progressive unilateral brain destruction, and neurological deficits.
• RE involves immune-mediated processes, including CD8+ T cells, microglial activation, and inflammasome pathways.

Purpose of the Study:

• To review the pathophysiological mechanisms of Rasmussen's encephalitis.
• To summarize existing literature data on immunotherapies for RE.
• To discuss limitations and future research directions for RE treatment.

Main Methods:

• Literature review of pathophysiological mechanisms and immunotherapies for RE.
• Analysis of clinical, electrophysiological (EEG), and MRI findings in RE diagnosis.
• Summary of reported immunotherapy trials (corticosteroids, IVIg, tacrolimus, azathioprine, adalimumab, mycophenolate mofetil, natalizumab).

Main Results:

• Diagnosis relies on intractable epilepsy, unilateral EEG slowing, and hemiatrophy on MRI.
• Standard antiseizure medications are often ineffective.
• Hemispherotomy is effective but causes permanent deficits; immunotherapy is recommended for early or slow-progressing cases.

Conclusions:

• Immunotherapies are crucial for managing Rasmussen's encephalitis, particularly in early stages.
• Current immunotherapy data is limited to small cohorts without comparative studies.
• Further research is needed to establish optimal immunotherapy protocols and address treatment limitations.