Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement

CG Bien, T Granata, C Antozzi, JH Cross, O Dulac… - Brain, 2005 - academic.oup.com
CG Bien, T Granata, C Antozzi, JH Cross, O Dulac, M Kurthen, H Lassmann, R Mantegazza…
Brain, 2005academic.oup.com
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.
Abstract
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.
Oxford University Press