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Titre du document / Document title

The natural history of Rasmussen's encephalitis

Auteur(s) / Author(s)

BIEN Christian G. (1) ; WIDMAN Guide (1) ; URBACH Horst (2) ; SASSEN Robert (1) ; KUCZATY Stefan (1) ; WIESTLER Otmar D. (3) ; SCHRAMM Johannes (4) ; ELGER Christian E. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Department of Epileptology, University of Bonn, Bonn, ALLEMAGNE
(2) Department of Radiology/Neuroradiology, University of Bonn, Bonn, ALLEMAGNE
(3) Department of Neuropathology, University of Bonn, Bonn, ALLEMAGNE
(4) Department of Neurosurgery, University of Bonn, Bonn, ALLEMAGNE

Résumé / Abstract

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 when future therapeutic approaches to RE are evaluated.

Revue / Journal Title

Brain    ISSN  0006-8950 

Source / Source

2002, vol. 125 (8), pp. 1751-1759 (1 p.1/2)

Langue / Language

Anglais

Editeur / Publisher

Oxford University Press, Oxford, ROYAUME-UNI  (1878) (Revue)

Mots-clés anglais / English Keywords

Medical imagery

;

Motor system disorder

;

Neurological disorder

;

Inflammatory disease

;

Central nervous system disease

;

Nervous system diseases

;

Cerebral disorder

;

Human

;

Evolution

;

Nuclear magnetic resonance imaging

;

Hemiparesis

;

Epilepsy

;

Encephalitis

;

Mots-clés français / French Keywords

Imagerie médicale

;

Trouble moteur

;

Trouble neurologique

;

Maladie inflammatoire

;

Système nerveux central pathologie

;

Système nerveux pathologie

;

Encéphale pathologie

;

Encéphalite Rasmussen

;

Homme

;

Evolution

;

Imagerie RMN

;

Hémiparésie

;

Epilepsie

;

Encéphalite

;

Mots-clés espagnols / Spanish Keywords

Imaginería médica

;

Trastorno motor

;

Trastorno neurológico

;

Enfermedad inflamatoria

;

Sistema nervosio central patología

;

Sistema nervioso patología

;

Encéfalo patología

;

Hombre

;

Evolución

;

Imaginería RMN

;

Hemiparesia

;

Epilepsia

;

Encefalitis

;

Localisation / Location

INIST-CNRS, Cote INIST : 998, 35400010443530.0070

Nº notice refdoc (ud4) : 13797818



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