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Brain Advance Access first published online on February 2, 2005
This version published online on February 3, 2005
Brain, doi:10.1093/brain/awh415
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© The Author (2005). Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received September 21, 2004
Revised January 6, 2005
Accepted January 7, 2005

Article

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis

C. G. Bien 1*, T. Granata 2, C. Antozzi 2, J. H. Cross 3, O. Dulac 4, M. Kurthen 1, H. Lassmann 5, R. Mantegazza 2, J.-G. Villemure 6, R. Spreafico 2, and C. E. Elger 1

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
6 Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

* To whom correspondence should be addressed.
C. G. Bien, E-mail: christian.bien{at}ukb.uni-bonn.de


  Abstract

Summary 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.

Keywords: encephalitis; epilepsy; pathophysiology; diagnostic criteria; therapy.
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