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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Rasmussen's encephalitis: advances in management and patient outcomes.

Caitlin E Hoffman1,2, Ayako Ochi3, Orlando Carter Snead3

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Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
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PubMed
Summary

Early or late hemispherotomy surgery for Rasmussen's encephalitis (RE) offers good seizure control and functional outcomes. This epilepsy surgery improves cognition and motor function, even in cases with dominant hemisphere involvement.

Keywords:
HemispherotomyImmunotherapyIntractable epilepsyMEG

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Area of Science:

  • Pediatric Neurology
  • Neurosurgery
  • Inflammatory Disorders

Background:

  • Rasmussen's encephalitis (RE) is a severe inflammatory brain disorder causing progressive epilepsy, hemiparesis, and cognitive decline.
  • Optimal surgical timing for RE remains debated, particularly concerning language dominance and functional status.

Purpose of the Study:

  • To evaluate the outcomes of hemispherotomy in children with Rasmussen's encephalitis.
  • To inform treatment decisions regarding surgical timing and its impact on seizure control and functional recovery.

Main Methods:

  • Retrospective chart review of 13 children diagnosed with RE between 1983 and 2012.
  • Analysis of outcomes including seizure freedom, cognitive function, ambulatory status, and language improvement post-surgery.

Main Results:

  • Hemispherotomy led to seizure freedom in 63% and improved seizure control in 100% of surgically treated patients.
  • Significant improvements were observed in cognitive function (90%) and language (83% in dominant disease).
  • All surgically treated patients remained ambulatory, with no association between outcome and age at treatment or pre-operative seizure duration.

Conclusions:

  • Hemispherotomy is an effective treatment for Rasmussen's encephalitis, providing substantial seizure control and cognitive improvement.
  • Surgical timing, whether early or late, and dominant hemisphere involvement did not negatively impact outcomes.
  • Hemispherotomy can be considered at various stages of the disease with positive expectations for seizure control and functional recovery.