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Progressive unilateral encephalopathy of childhood (Rasmussen's syndrome): a reappraisal
E P Vining1, J M Freeman, J Brandt
1Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287-3141.
Insights
Early hemispherectomy for Rasmussen's encephalitis in children may improve quality of life and prevent intellectual decline. This approach aims to mitigate the effects of hemiplegia and ongoing seizures, offering a better long-term outlook.
Area of Science:
- Pediatric Neurology
- Neurosurgery
Background:
- Rasmussen's encephalitis is a rare, progressive neurological disorder affecting one hemisphere of the brain.
- The condition typically presents in childhood with intractable seizures and neurological deficits.
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.