Rasmussen’s encephalitis: advances in management and patient outcomes

Rasmussen’s encephalitis: advances in management and patient outcomes

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 e...

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Bibliographic Details
Published in:Child's nervous system Vol. 32; no. 4; pp. 629 - 640
Main Authors: Hoffman, Caitlin E., Ochi, Ayako, Snead, Orlando Carter, Widjaja, Elysa, Hawkins, Cynthia, Tisdal, Martin, Rutka, James T.
Format: Journal Article
Language:English
Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-04-2016
Subjects:
Adolescent
Child
Child, Preschool
Cognition Disorders - etiology
Cognition Disorders - therapy
Disease Management
Encephalitis - complications
Encephalitis - diagnosis
Encephalitis - therapy
Female
Follow-Up Studies
Hemispherectomy
Humans
Immunotherapy
Magnetoencephalography
Male
Medicine
Medicine & Public Health
Neuroimaging
Neurosciences
Neurosurgery
Original Paper
Retrospective Studies
Seizures - etiology
Seizures - therapy
Treatment Outcome
Hemispherotomy
Intractable epilepsy
Immunotherapy
MEG
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Summary: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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ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-015-2994-x