title: Rasmussen's encephalitis: advances in management and patient outcomes.
abstract: OBJECTIVE
  Rasmussen's encephalitis (RE) is a hemispheric inflammatory disorder resulting in progressive epilepsy, hemiparesis, and cognitive decline. Controversy surrounds the most effective timing of surgery with respect to language dominance, functional status, and seizure outcome. We describe our experience with RE to inform treatment decisions.
  
  
  METHODS
  A retrospective chart review was performed in children diagnosed with RE from 1983 to 2012.
  
  
  RESULTS
  Thirteen consecutive cases were identified: six males and seven females with a mean age of 10.6 years (range 5-18). Nine patients received immunotherapy, with transient benefit in three, treatment-associated complications in two, and no difference in their mean time to treatment (5.38 vs 6.37 years p = 0.74) or long-term outcome. Mean follow-up was 5.6 years (range 0.58-12.25). There was no difference in outcome based on pre-operative duration of seizures. At last follow-up, 63 % of surgically treated patients achieved seizure freedom, 100 % had improved seizure control, 90 % had improved cognitive function, 36 % stopped medication, and 63 % tapered medication. Language improved in 83 % of patients with dominant disease. These findings were not associated with age at treatment. All surgical patients were ambulatory at last follow-up.
  
  
  CONCLUSIONS
  Hemispherotomy achieves good seizure control with cognitive improvement and ambulatory status post-operatively. Time to surgery and dominant disease were not associated with outcome, suggesting that hemispherotomy can be offered early or late, with expectations of good seizure control and functional outcome, even with dominant disease.
authors.affiliation: Division of Pediatric Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. ceh2003@med.cornell.edu.|Department of Neurological Surgery, Weill Cornell Medical College, New York, NY, USA. ceh2003@med.cornell.edu.
authors.forename: Caitlin E
authors.initials: CE
authors.lastname: Hoffman
authors.affiliation: Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Ayako
authors.initials: A
authors.lastname: Ochi
authors.affiliation: Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Orlando Carter
authors.initials: OC
authors.lastname: Snead
authors.affiliation: Division of Diagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Elysa
authors.initials: E
authors.lastname: Widjaja
authors.affiliation: Division of Neuropathology, Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Hawkins
authors.affiliation: Division of Pediatric Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Martin
authors.initials: M
authors.lastname: Tisdal
authors.affiliation: Division of Pediatric Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: James T
authors.initials: JT
authors.lastname: Rutka
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology / Q000628:therapy; D019468:Disease Management; D004660:Encephalitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D038421:Hemispherectomy; D006801:Humans; D007167:Immunotherapy; D015225:Magnetoencephalography; D008297:Male; D059906:Neuroimaging; D012189:Retrospective Studies; D012640:Seizures / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome
keywords: Hemispherotomy; Immunotherapy; Intractable epilepsy; MEG