Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy

Purpose: Elucidating the potential contribution of specific autoantibodies (Ab's) to the etiology and/or pathology of some human epilepsies. Methods: Six epilepsy patients with Rasmussen's encephalitis (RE) and 71 patients with other epilepsies were test...

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Main Authors: Yonatan Ganor, Hadassa Goldberg-Stern, Dina Amrom, Tally Lerman-Sagie, Vivian I. Teichberg, Dori Pelled, Anthony H. Futerman, Bruria Ben Zeev, Michael Freilinger, Denis Verheulpen, Patrick Van Bogaert, Mia Levite
Format: Article
Language:English
Published: Wiley 2004-01-01
Series:Clinical and Developmental Immunology
Online Access:http://dx.doi.org/10.1080/17402520400001736
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author Yonatan Ganor
Hadassa Goldberg-Stern
Dina Amrom
Tally Lerman-Sagie
Vivian I. Teichberg
Dori Pelled
Anthony H. Futerman
Bruria Ben Zeev
Michael Freilinger
Denis Verheulpen
Patrick Van Bogaert
Mia Levite
author_facet Yonatan Ganor
Hadassa Goldberg-Stern
Dina Amrom
Tally Lerman-Sagie
Vivian I. Teichberg
Dori Pelled
Anthony H. Futerman
Bruria Ben Zeev
Michael Freilinger
Denis Verheulpen
Patrick Van Bogaert
Mia Levite
author_sort Yonatan Ganor
collection DOAJ
description Purpose: Elucidating the potential contribution of specific autoantibodies (Ab's) to the etiology and/or pathology of some human epilepsies. Methods: Six epilepsy patients with Rasmussen's encephalitis (RE) and 71 patients with other epilepsies were tested for Ab's to the –B— peptide (amino acids 372-395) of the glutamate/AMPA subtype 3 receptor (GluR3B peptide), double-stranded DNA (dsDNA), and additional autoimmune disease-associated autoantigens, and for the ability of their serum and cerebrospinal-fluid (CSF) to kill neurons. Results: Elevated anti-GluR3B Ab's were found in serum and CSF of most RE patients, and in serum of 17/71 (24%) patients with other epilepsies. In two RE patients, anti-GluR3B Ab's decreased drastically in CSF following functional-hemispherotomy, in association with seizure cessation and neurological improvement. Serum and CSF of two RE patients, and serum of 12/71 (17%) patients with other epilepsies, contained elevated anti-dsDNA Ab's, the hallmark of systemic-lupus-erythematosus. The sera (but not the CSF) of some RE patients contained also clinically elevated levels of –classical— autoimmune Ab's to glutamic-acid-decarboxylase, cardiolipin, β2-glycoprotein-I and nuclear-antigens SS-A and RNP-70. Sera and CSF of some RE patients caused substantial death of hippocampal neurons. Conclusions: Some epilepsy patients harbor Ab's to GluR3 and dsDNA on both sides of the blood-brain barrier, and additional autoimmune Ab's only in serum. Since all these Ab's may be detrimental to the nervous system and/or peripheral organs, we recommend testing for their presence in epilepsy, and silencing their activity in Ab-positive patients.
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spelling doaj-art-a1a0b92e91d64c2386b1e8c47398e41c2025-08-20T02:20:44ZengWileyClinical and Developmental Immunology1740-25221740-25302004-01-01113-424125210.1080/17402520400001736Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after HemispherotomyYonatan Ganor0Hadassa Goldberg-Stern1Dina Amrom2Tally Lerman-Sagie3Vivian I. Teichberg4Dori Pelled5Anthony H. Futerman6Bruria Ben Zeev7Michael Freilinger8Denis Verheulpen9Patrick Van Bogaert10Mia Levite11Department of Neurobiology, The Weizmann Institute of Science, Rehovot, IsraelEpilepsy Center, Schneider Children's Medical Center of Israel, Petah Tiqwa, IsraelDepartment of Child Neurology, Hôpital Français-ULB, Brussels, BelgiumSackler Faculty of Medicine, Tel Aviv University, aviv, IsraelDepartment of Neurobiology, The Weizmann Institute of Science, Rehovot, IsraelDepartment of Biological Chemistry, The Weizmann Institute of Science, Rehovot, IsraelDepartment of Biological Chemistry, The Weizmann Institute of Science, Rehovot, IsraelSackler Faculty of Medicine, Tel Aviv University, aviv, IsraelDepartment of Pediatrics, University Hospital of Vienna, AustriaDepartment of Pediatric Neurology, ULB-Hopital Erasme, Brussels, BelgiumDepartment of Pediatric Neurology, ULB-Hopital Erasme, Brussels, BelgiumDepartment of Neurobiology, The Weizmann Institute of Science, Rehovot, IsraelPurpose: Elucidating the potential contribution of specific autoantibodies (Ab's) to the etiology and/or pathology of some human epilepsies. Methods: Six epilepsy patients with Rasmussen's encephalitis (RE) and 71 patients with other epilepsies were tested for Ab's to the –B— peptide (amino acids 372-395) of the glutamate/AMPA subtype 3 receptor (GluR3B peptide), double-stranded DNA (dsDNA), and additional autoimmune disease-associated autoantigens, and for the ability of their serum and cerebrospinal-fluid (CSF) to kill neurons. Results: Elevated anti-GluR3B Ab's were found in serum and CSF of most RE patients, and in serum of 17/71 (24%) patients with other epilepsies. In two RE patients, anti-GluR3B Ab's decreased drastically in CSF following functional-hemispherotomy, in association with seizure cessation and neurological improvement. Serum and CSF of two RE patients, and serum of 12/71 (17%) patients with other epilepsies, contained elevated anti-dsDNA Ab's, the hallmark of systemic-lupus-erythematosus. The sera (but not the CSF) of some RE patients contained also clinically elevated levels of –classical— autoimmune Ab's to glutamic-acid-decarboxylase, cardiolipin, β2-glycoprotein-I and nuclear-antigens SS-A and RNP-70. Sera and CSF of some RE patients caused substantial death of hippocampal neurons. Conclusions: Some epilepsy patients harbor Ab's to GluR3 and dsDNA on both sides of the blood-brain barrier, and additional autoimmune Ab's only in serum. Since all these Ab's may be detrimental to the nervous system and/or peripheral organs, we recommend testing for their presence in epilepsy, and silencing their activity in Ab-positive patients.http://dx.doi.org/10.1080/17402520400001736
spellingShingle Yonatan Ganor
Hadassa Goldberg-Stern
Dina Amrom
Tally Lerman-Sagie
Vivian I. Teichberg
Dori Pelled
Anthony H. Futerman
Bruria Ben Zeev
Michael Freilinger
Denis Verheulpen
Patrick Van Bogaert
Mia Levite
Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
Clinical and Developmental Immunology
title Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
title_full Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
title_fullStr Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
title_full_unstemmed Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
title_short Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood-brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy
title_sort autoimmune epilepsy some epilepsy patients harbor autoantibodies to glutamate receptors and dsdna on both sides of the blood brain barrier which may kill neurons and decrease in brain fluids after hemispherotomy
url http://dx.doi.org/10.1080/17402520400001736
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