Rasmussen's encephalitis: advances in management and patient outcomes

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Caitlin E HoffmanJames T Rutka

Abstract

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. A retrospective chart review was performed in children diagnosed with RE from 1983 to 2012. 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 wer...Continue Reading

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Citations

Apr 20, 2018·Journal of Neurosurgical Sciences·Johannes Herta, Christian Dorfer
Aug 10, 2017·Journal of Neuropathology and Experimental Neurology·Guoming LuanTianfu Li
Dec 5, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Joceli Rodrigues SilvaAna Paula Hamad
Jul 22, 2020·World Neurosurgery·Claudia M Kuzan-FischerCaitlin E Hoffman
Mar 12, 2018·Neurología : publicación oficial de la Sociedad Española de Neurología·O Fernández-ConcepciónC Vásquez-Ham
Aug 10, 2020·Seizure : the Journal of the British Epilepsy Association·A OrsiniP Striano
Jul 18, 2020·Journal of Neurosurgery. Pediatrics·Nikhil BellamkondaAria Fallah

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