Advancements in pathogenesis studies of Rasmussen's encephalitis

Abstract

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 inflammation of the cerebral cortex, progressive hemiplegia and cognitive deterioration. The etiology of this syndrome remains under investigation, and it is hypothesized and widely accepted that RE is a T-cell-mediated autoimmune response. However, the antigenic epitopes and mechanisms are still unknown. The pathological characteristics of RE are cortical inflammation, neuronal loss, and gliosis that are confined to one cerebral hemisphere. Hemispherectomy remains the only cure for the seizures and cognitive deterioration associated with the disease, but this procedure results in inevitable functional loss in the brain. Compared with surgery, immunomodulatory treatments are expected to cause less neurological deficits, but with limited clinical effect.

Keywords

Rasmussen’s encephalitis(RE) / seizures / neuron degeneration / autoantibodies / T-cell cytotoxicity