Détail de la notice
Titre du Document
The natural history of Rasmussen's encephalitis
Auteur(s)
BIEN Christian G. ; WIDMAN Guide ; URBACH Horst ; ...
Résumé
Rasmussen's encephalitis (RE) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. In the present study, a comprehensive assessment of the natural history of the disorder is presented. Seizure frequency, degree of hemiparesis and degree of cerebral hemiatrophy in 13 patients with histopathologically proven RE are analysed over the time course prior to resective epilepsy surgery or introduction of long-term immunosuppressive pharmacotherapy. For the assessment of the degree of cerebral hemiatrophy, on defined slices comprising the Sylvian fissure of hard copies of serial MRI investigations, the hemispheric ratio (HR) was determined. The data show an initial prodromal phase with an intermediate frequency of focal onset seizures and mostly no hemiparesis. The occurrence of this stage was mainly observed in the adolescent and adult patients. All patients went through an acute phase with a median duration of 8 months. During this stage, there were frequent simple partial motor seizures, development of hemiparesis and volume loss of the affected hemisphere. After this, the patients passed into a residual stage with a marked decrease in seizure frequency. Twelve months after the onset of the acute stage, the average HR was 0.72. These data allow an estimation of the prognosis of newly affected patients, and demonstrate that most of the brain damage in RE occurs during the first 8-12 months. These findings should be taken into consideration whe
Editeur
Oxford University Press
Identifiant
ISSN : 0006-8950
Source
Brain A. 2002, vol. 125, 8, pp. 1751-1759 [bibl. : 1 p.1/2]
Langue
Anglais
Pour les membres de la communauté du CNRS, ce document est autorisé à la reproduction à titre gratuit.
Pour les membres des communautés hors CNRS, la reproduction de ce document à titre onéreux sera fournie sous réserve d’autorisation du Centre Français d’exploitation du droit de Copie.

Pour bénéficier de nos services (strictement destinés aux membres de la communauté CNRS (Centre National de la Recherche Scientifique), de l'ESR français (Enseignement Supérieur et Recherche), et du secteur public français & étranger) :