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Rasmussen’s encephalitis ( RE ) , which was first described by Rasmussen in 1958 , is a rare, dispersed, and progressive neurological syndrome that is characterized by focal epilepsy, unilateral inflam-mation of the cerebral cortex, progressive hemiplegia and cognitive deterioration. The etiology of this syn-drome remains under investigation, and it is hypothe-sized and widely accepted that RE is a T-cell-mediated autoimmune response. However, the antigenic epitopes and mechanisms are still unknown. The patho-logical characteristics of RE are cortical inflammation, neuronal loss, and gliosis that are confined to one cere-bral hemisphere. Hemispherectomy remains the only cure for the seizures and cognitive deterioration associ-ated with the disease, but this procedure results in inev-itable functional loss in the brain. Compared with sur-gery, immunomodulatory treatments are expected to cause less neurological deficits, but with limited clinical effect.