Journal Article

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis

C. G. Bien, T. Granata, C. Antozzi, J. H. Cross, O. Dulac, M. Kurthen, H. Lassmann, R. Mantegazza, J.-G. Villemure, R. Spreafico and C. E. Elger

in Brain

Published on behalf of The Guarantors of Brain

Volume 128, issue 3, pages 454-471
Published in print March 2005 | ISSN: 0006-8950
Published online February 2005 | e-ISSN: 1460-2156 | DOI: http://dx.doi.org/10.1093/brain/awh415
Pathogenesis, diagnosis and treatment of Rasmussen encephalitis

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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; AED = anti-epilepsy drug; BBB = blood–brain barrier; CTL = cytotoxic T lymphocyte; EPC = epilepsia partialis continua; FDG = fluorodeoxyglucose; GluR3 = glutamate receptor subunit 3; GrB = Granzyme B; HE = hemispherectomy or any of its variants; IVIG = intravenous immunoglobulins; PEX/PAI = plasma exchange/protein A IgG immuno-adsorption; RE = Rasmussen encephalitis; SPECT = single photon emission computed tomography; TCR = T cell receptors

Journal Article.  15129 words.  Illustrated.

Subjects: Neurology ; Neuroscience

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