The Janus-faced nature of Rasmussen’s Encephalitis

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,...

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Bibliographic Details
Published in:Seminars in pediatric neurology 2014-06, Vol.21 (2), p.129-136
Main Authors: Press, Craig, MD, PhD, Wallace, Adam, MD, Chapman, Kevin E., MD
Format: Article
Language:English
Subjects:
Brain - diagnostic imaging
Brain - pathology
Brain - surgery
Child, Preschool
Encephalitis - diagnostic imaging
Encephalitis - pathology
Encephalitis - physiopathology
Encephalitis - therapy
Female
Humans
Magnetic Resonance Imaging
Male
Neurology
Pediatrics
Positron-Emission Tomography
Seizures - diagnostic imaging
Seizures - pathology
Seizures - physiopathology
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Description
Summary: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.
ISSN:1071-9091
1558-0776
DOI:10.1016/j.spen.2014.04.018