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The Janus-faced nature of Rasmussen's encephalitis.

作者信息

Press Craig, Wallace Adam, Chapman Kevin E

机构信息

Department of Neurology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, CO.

Department of Neurology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, Aurora, CO.

出版信息

Semin Pediatr Neurol. 2014 Jun;21(2):129-36. doi: 10.1016/j.spen.2014.04.018. Epub 2014 Apr 16.

Abstract

Rasmussen encephalitis (RE) is an inflammatory unilateral progressive medically refractory epilepsy associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Here, we present 2 cases from our institution that demonstrate the dual nature of RE in 2 similarly aged children. Overall, 2 types of RE have been described: type 1 has a short prodromal phase and more explosive onset and type 2 has a longer prodromal of partial seizures followed by hemiparesis and atrophy. Younger patients are more likely to fit into the type 1 presentation and have been described as more likely to have dual pathology. Perhaps the patients with a more acute onset are more likely to have a dual pathology as is found in our 2 cases. We review the typical findings in RE and discuss current treatment options, highlighting new experimental treatments.

摘要

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