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Brain Advance Access originally published online on February 2, 2005
Brain 2005 128(3):454-471; doi:10.1093/brain/awh415
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© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please e-mail: journal.permissions{at}oupjournals.org

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis

A European consensus statement

C. G. Bien1, T. Granata2, C. Antozzi2, J. H. Cross3, O. Dulac4, M. Kurthen1, H. Lassmann5, R. Mantegazza2, J.-G. Villemure6, R. Spreafico2 and C. E. Elger1

1 University of Bonn, Department of Epileptology, Bonn, Germany, 2 Instituto Nazionale Neurologico ‘C. Besta’, Milan, Italy, 3 Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK, 4 Service de Maladies Métaboliques et Neurologie Hôpital Necker Enfant Malades, Paris, France, 5 Medical University of Vienna, Brain Research Institute, Vienna, Austria, and 6 Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

Correspondence to: Dr Christian G. Bien, University of Bonn, Department of Epileptology, Sigmund-Freud-Str. 25, 53105 Bonn, Germany E-mail: christian.bien{at}ukb.uni-bonn.de

Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.


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