Progressive Unilateral Encephalopathy of Childhood (Rasmussen's Syndrome): A Reappraisal

Abstract

Twelve children with Rasmussen's encephalitis underwent hemispherectomy and have been followed for an average of 9.15 years. Our decision-making process leads to early, rather than late operation. Because hemiplegia is inevitable with or without operation, earlier operation may allow such children to resume a more normal life. We postulate that earlier operation may prevent some of the intellectual decline that accompanies the continued seizures and their treatment. In view of the variable pathologic findings and lack of evidence of a viral etiology, we suggest either use of the term "progressive unilateral encephalopathy of childhood" or "Rasmussen's syndrome" in recognition of its heritage.