Rasmussen Encephalitis

Rasmussen encephalitis (RE) is a rare epileptic disorder that is characterized by the presence of unihemispheric seizures coinciding with inflammation. The disease mostly presents in children. Clinically, patients often reach a residual stage with drug resistant seizures and severe neurological deficits. Pathologically, at this stage, the brain shows variable neuronal loss. The etiology is unknown but the infiltration of large numbers of CD8 positive T lymphocytes suggests that this is an autoimmune disease. Treatment consists of anti-inflammatory therapy (IVIG or tacrolimus), which, however, does not reduce the drug-resistant seizures. Therefore, hemisperectomy is the most effective treatment of RE.