Antibodies to AMPA receptors in Rasmussen's encephalitis.
Rasmussen's encephalitis is a rare progressive childhood disorder characterized by frequent severe seizures, hemiparesis, encephalitis and mental deterioration, and associated with severe unilateral neuroinflammation. Autoantibodies, particularly to the GluA3 subtype of the alpha-amino-3-hydrox...
| Main Authors: | , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
Elsevier
2016
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| _version_ | 1797080001279950848 |
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| author | Nibber, A Clover, L Pettingill, P Waters, P Elger, C Bien, C Vincent, A Lang, B |
| author_facet | Nibber, A Clover, L Pettingill, P Waters, P Elger, C Bien, C Vincent, A Lang, B |
| author_sort | Nibber, A |
| collection | OXFORD |
| description | Rasmussen's encephalitis is a rare progressive childhood disorder characterized by frequent severe seizures, hemiparesis, encephalitis and mental deterioration, and associated with severe unilateral neuroinflammation. Autoantibodies, particularly to the GluA3 subtype of the alpha-amino-3-hydroxy-5-methyl-isoxazole-4-propinonic acid receptor (AMPAR) were reported in the 1990s but not always confirmed. To explore further, sera from 52 patients with Rasmussen's encephalitis were tested by cell-based assays for antibodies to AMPAR GluA1/2/3, N-methyl-d-aspartate (NMDA NR1/2b), γ-aminobutyric acid type A and B (GABAAR α1/γ2/β2 and GABABR b1/b2) receptors, for potassium channel complex proteins, and for binding to live cortical and hippocampal neuronal cultures. Two patients' sera (3.8%) bound to HEK cells co-transfected with the GluA2 and GluA3 subunits. One additional patient had a low level of VGKC-complex antibodies. These three, and seven additional, sera bound to hippocampal cultures. No other antibodies were detected. Thus, despite the rarity of GluA2/3 antibodies, 10 patients (19.2%) had evidence of antibodies to neuronal antigens. Whether these antibodies play a primary role in RE, or appear secondary to the neuro-inflammatory damage in this highly destructive disease, requires further study. |
| first_indexed | 2024-03-07T00:53:52Z |
| format | Journal article |
| id | oxford-uuid:8756c10c-d252-404c-93a7-c4acb1601061 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T00:53:52Z |
| publishDate | 2016 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | oxford-uuid:8756c10c-d252-404c-93a7-c4acb16010612022-03-26T22:10:08ZAntibodies to AMPA receptors in Rasmussen's encephalitis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:8756c10c-d252-404c-93a7-c4acb1601061EnglishSymplectic Elements at OxfordElsevier2016Nibber, AClover, LPettingill, PWaters, PElger, CBien, CVincent, ALang, BRasmussen's encephalitis is a rare progressive childhood disorder characterized by frequent severe seizures, hemiparesis, encephalitis and mental deterioration, and associated with severe unilateral neuroinflammation. Autoantibodies, particularly to the GluA3 subtype of the alpha-amino-3-hydroxy-5-methyl-isoxazole-4-propinonic acid receptor (AMPAR) were reported in the 1990s but not always confirmed. To explore further, sera from 52 patients with Rasmussen's encephalitis were tested by cell-based assays for antibodies to AMPAR GluA1/2/3, N-methyl-d-aspartate (NMDA NR1/2b), γ-aminobutyric acid type A and B (GABAAR α1/γ2/β2 and GABABR b1/b2) receptors, for potassium channel complex proteins, and for binding to live cortical and hippocampal neuronal cultures. Two patients' sera (3.8%) bound to HEK cells co-transfected with the GluA2 and GluA3 subunits. One additional patient had a low level of VGKC-complex antibodies. These three, and seven additional, sera bound to hippocampal cultures. No other antibodies were detected. Thus, despite the rarity of GluA2/3 antibodies, 10 patients (19.2%) had evidence of antibodies to neuronal antigens. Whether these antibodies play a primary role in RE, or appear secondary to the neuro-inflammatory damage in this highly destructive disease, requires further study. |
| spellingShingle | Nibber, A Clover, L Pettingill, P Waters, P Elger, C Bien, C Vincent, A Lang, B Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title | Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title_full | Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title_fullStr | Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title_full_unstemmed | Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title_short | Antibodies to AMPA receptors in Rasmussen's encephalitis. |
| title_sort | antibodies to ampa receptors in rasmussen s encephalitis |
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