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: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2004-01-01
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| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1080/17402520400001736 |
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| Summary: | 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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| ISSN: | 1740-2522 1740-2530 |