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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.1 Immunotherapies may slow down tissue and function loss, whereas convincing effects on seizure activity have rarely been reported.2 Functional hemispherectomy (HE) is highly effective for elimination of seizures but can only be offered if no indispensable function resides in the affected hemisphere.1 We present a patient with such an HE contraindication who was treated with natalizumab, a blocker of T-cell entry into the CNS.
Acknowledgments
Acknowledgment: The authors thank O. Grauer and C. Gross for supervision of the flow cytometric experiments.
Footnotes
↵* These authors contributed equally to this work.
Author contributions: S.B. wrote the initial draft of the manuscript, which was corrected by S.G.M., H.W., and C.G.B. S.B., O.J.S., and K.G. treated the patient in Münster under supervision of S.G.M. and H.W. C.G.B. treated the patient in Bielefeld-Bethel.
Study funding: No targeted funding reported.
Disclosure: S. Bittner, O. Simon, and K. Göbel report no disclosures. C. Bien served on a scientific advisory board of UCB and Eisai, Germany; undertook industry-funded travel with support of Eisai, UCB, Desitin, and Grifols, Germany; and obtained honoraria for educational speaking engagements from Eisai, UCB, and Desitin, Germany. S. Meuth has received honoraria for lecturing and travel expenses for attending meetings from Bayer Health Care, Biogen Idec/Elan Corporation, Merck Serono, Novartis, Sanofi Aventis, and Teva Neuroscience; has served/serves as a consultant for Biogen Idec, Merck Serono, Novartis Pharma, and Sanofi-Aventis; and receives research support from Bayer Schering Pharma, Biogen Idec/Elan Corporation, Merck Serono, Novartis, Novo Nordisk, and Sanofi-Aventis. H. Wiendl has received honoraria for lecturing and travel expenses for attending meetings from Bayer Health Care, Biogen Idec/Elan Corporation, Lilly, Lundbeck Merck Serono, Novartis, Sanofi Aventis, and Teva Neuroscience; has served/serves as a consultant for Biogen Idec, Merck Serono, Novartis Pharma, and Sanofi-Aventis; and receives research support from Bayer Schering Pharma, Biogen Idec/Elan Corporation, Merck Serono, Novartis, Novo Nordisk, and Sanofi-Aventis. Go to Neurology.org for full disclosures.
- Received December 3, 2012.
- Accepted in final form April 4, 2013.
- © 2013 American Academy of Neurology
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