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Published in: Child's Nervous System 4/2016

01-04-2016 | Original Paper

Rasmussen’s encephalitis: advances in management and patient outcomes

Authors: Caitlin E. Hoffman, Ayako Ochi, Orlando Carter Snead III, Elysa Widjaja, Cynthia Hawkins, Martin Tisdal, James T. Rutka

Published in: Child's Nervous System | Issue 4/2016

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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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Metadata
Title
Rasmussen’s encephalitis: advances in management and patient outcomes
Authors
Caitlin E. Hoffman
Ayako Ochi
Orlando Carter Snead III
Elysa Widjaja
Cynthia Hawkins
Martin Tisdal
James T. Rutka
Publication date
01-04-2016
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 4/2016
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-015-2994-x

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