Destruction of neurons by cytotoxic T cells: A new pathogenic mechanism in rasmussen's encephalitis

Rasmussen's encephalitis is a progressive epileptic disorder characterized by unihemispheric lymphocytic infiltrates, microglial nodules, and neuronal loss leading to the destruction of the affected hemisphere. In this study, immunohistochemical evaluation of specimens from 11 patients revealed...

Full description

Saved in:
Bibliographic Details
Published in:Annals of neurology 2002-03, Vol.51 (3), p.311-318
Main Authors: Bien, Christian G., Bauer, Jan, Deckwerth, Thomas L., Wiendl, Heinz, Deckert, Martina, Wiestler, Otmar D., Schramm, Johannes, Elger, Christian E., Lassmann, Hans
Format: Article
Language:English
Subjects:
Adolescent
Adult
Apoptosis - physiology
Biological and medical sciences
Brain - pathology
Brain - physiopathology
CD3 Complex - metabolism
CD8-Positive T-Lymphocytes - pathology
Child
Child, Preschool
Encephalitis - pathology
Encephalitis - physiopathology
Female
Granzymes
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Histocompatibility Antigens Class I - metabolism
Humans
Immunohistochemistry
Infant
Male
Medical sciences
Microscopy, Confocal
Nervous system (semeiology, syndromes)
Neurology
Neurons - physiology
Serine Endopeptidases - metabolism
T-Lymphocytes, Cytotoxic - pathology
T-Lymphocytes, Cytotoxic - physiology
Tissue Distribution
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Rasmussen's encephalitis is a progressive epileptic disorder characterized by unihemispheric lymphocytic infiltrates, microglial nodules, and neuronal loss leading to the destruction of the affected hemisphere. In this study, immunohistochemical evaluation of specimens from 11 patients revealed lymphocytic infiltrates that consisted mainly of CD3+CD8+ T cells. Of these cells, 7.0% lay in direct apposition to MHC class I+ neurons. Confocal laser microscopy revealed that these lymphocytes contained granzyme B in a polar orientation toward these perikarya. Single neurons underwent apoptosis. These findings indicate that a T‐cell–mediated cytotoxic reaction induces neuronal death in Rasmussen's encephalitis. This study directly shows, for what we believe is the first time, that a cytotoxic T‐cell mechanism contributes to loss of neurons in human brain disease.
ISSN:0364-5134
1531-8249
DOI:10.1002/ana.10100