Rasmussen encephalitis treated with natalizumab

Rasmussen encephalitis (RE) is characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable seizures. Inflammatory lesions consist of oligoclonally expanded cytotoxic CD8+ T cells attacking neurons and astrocytes. Immunotherapies may slow down tissue and function lo...

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Veröffentlicht in:Neurology 2013-07, Vol.81 (4), p.395-397
Hauptverfasser: Bittner, Stefan, Simon, Ole J, Göbel, Kerstin, Bien, Christian G, Meuth, Sven G, Wiendl, Heinz
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Sprache:eng
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Zusammenfassung:Rasmussen encephalitis (RE) is characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable seizures. Inflammatory lesions consist of oligoclonally expanded cytotoxic CD8+ T cells attacking neurons and astrocytes. Immunotherapies may slow down tissue and function loss, whereas convincing effects on seizure activity have rarely been reported. Functional hemispherectomy (HE) is highly effective for elimination of seizures but can only be offered if no indispensable function resides in the affected hemisphere. We present a patient with such an HE contraindication who was treated with natalizumab, a blocker of T-cell entry into the CNS.
ISSN:0028-3878
1526-632X
DOI:10.1212/WNL.0b013e31829c5ceb