Rasmussen syndrome and long-term response to thalidomide

We report a 13-year-old female who experienced symptoms and signs of Rasmussen encephalitis for the first time at the age of 5 years. Various therapeutic procedures, including conventional and new antiepileptic drugs, steroids, immunoglobulin, plasma exchanges, and partial hemispherectomy, were applied, but their results were unsatisfactory. During one of the exacerbations, when the patient's life was endangered, thalidomide was administered. Frequency and intensity of epileptic seizures were reduced significantly, and the quality of her life improved. Except for moderate neutropenia, the other adverse effects were not recognized. In our opinion, thalidomide is not a first-choice drug for Rasmussen encephalitis but is a good alternative only for cases refractory to other well-known and accepted therapeutic procedures. (C) 2003 by Elsevier Inc. All rights reserved.