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Rasmussen’s encephalitis: advances in management and patient outcomes

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Abstract

Purpose

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.

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Author information

Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Caitlin E. Hoffman, Martin Tisdal & James T. Rutka

  2. Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Ayako Ochi & Orlando Carter Snead III

  3. Division of Diagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Elysa Widjaja

  4. Division of Neuropathology, Hospital for Sick Children, Toronto, ON, Canada

    Cynthia Hawkins

  5. Department of Neurological Surgery, Weill Cornell Medical College, New York, NY, USA

    Caitlin E. Hoffman

Authors
  1. Caitlin E. Hoffman
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  2. Ayako Ochi
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Correspondence to Caitlin E. Hoffman.

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The authors declare that they have no conflict of interest with regard to this study.

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No funding was received with regard to this study.

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Hoffman, C.E., Ochi, A., Snead, O.C. et al. Rasmussen’s encephalitis: advances in management and patient outcomes. Childs Nerv Syst 32, 629–640 (2016). https://doi.org/10.1007/s00381-015-2994-x

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  • DOI: https://doi.org/10.1007/s00381-015-2994-x

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