PLASMAPHERESIS IN RASMUSSENS ENCEPHALITIS

Rasmussen's encephalitis (RE) is a progressive childhood disorder char acterized by intractable focal seizures, hemiplegia, dementia, and inf lammatory histopathology. The process is typically limited to one cere bral hemisphere. We report four patients with pathologically confirmed RE who were treated with repeated plasmapheresis. Three patients exhi bited repeated, dramatic, transient responses to plasmapheresis, manif ested by reduced seizure frequency and improved neurologic function. O ne patient exhibited marginal improvement after treatment with plasmap heresis. These observations indicate that circulating factors, likely autoantibodies, are pathogenic in at least some patients with RE and s uggest that RE is an autoimmune disease. Plasmapheresis may be a usefu l adjunctive therapy in RE, specifically for treatment of patients wit h acute deteriorations such as status epilepticus, and can also aid in assessment of residual function in the diseased hemisphere before sur gical resection.