Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1
ObjectivesAutoantibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) and leucine-rich glioma-inactivated 1 (Lgi1) are associated with autoimmune encephalitis. We described an acetylcholine receptor (AChR)-positive patient with myasthenia gravis who developed limbic e...
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Frontiers Media S.A.
2023-10-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2023.1237140/full |
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| author | Magne Solberg Nes Mette Haugen Hans Kristian Haugland Nils Erik Gilhus Nils Erik Gilhus Christian Alexander Vedeler Christian Alexander Vedeler |
| author_facet | Magne Solberg Nes Mette Haugen Hans Kristian Haugland Nils Erik Gilhus Nils Erik Gilhus Christian Alexander Vedeler Christian Alexander Vedeler |
| author_sort | Magne Solberg Nes |
| collection | DOAJ |
| description | ObjectivesAutoantibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) and leucine-rich glioma-inactivated 1 (Lgi1) are associated with autoimmune encephalitis. We described an acetylcholine receptor (AChR)-positive patient with myasthenia gravis who developed limbic encephalitis with antibodies to AMPAR and Lgi1.MethodsA single-case report with detailed, prospective clinical and biomarker data including serial laboratory testing and histopathology.ResultsA 49-year-old woman was diagnosed with anti-AChR antibody-positive generalized myasthenia gravis in 1983. After 9 months of the removal of thymoma in 1984, she developed influenza-like symptoms and then symptoms of limbic encephalitis. Retrospective analysis of serum showed high concentrations of anti-AMPAR and lower concentrations of anti-Lgi1 antibodies. Cerebral CT was normal, EEG showed bifrontal dysrhythmia, and CSF showed mild pleocytosis. Immuno-histochemical examination of the thymoma confirmed staining for Glur2, a subunit of AMPAR. The patient recovered with mild sequelae, but low levels of anti-AMPAR and anti-Lgi1 antibodies were detectable for over 25 years subsequently.DiscussionThis case confirms earlier reports of AMPAR-associated autoimmune encephalitis co-occurring with thymoma and myasthenia gravis and is unique in its observational length. It shows, moreover, that antibodies to AMPAR and Lgi1 can persist despite clinical recovery. |
| first_indexed | 2024-03-11T18:36:28Z |
| format | Article |
| id | doaj.art-02fef01bffa841d2a542ef3b196738ee |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-03-11T18:36:28Z |
| publishDate | 2023-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-02fef01bffa841d2a542ef3b196738ee2023-10-12T16:53:06ZengFrontiers Media S.A.Frontiers in Neurology1664-22952023-10-011410.3389/fneur.2023.12371401237140Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1Magne Solberg Nes0Mette Haugen1Hans Kristian Haugland2Nils Erik Gilhus3Nils Erik Gilhus4Christian Alexander Vedeler5Christian Alexander Vedeler6Department of Neurology, Haukeland University Hospital, Bergen, NorwayDepartment of Neurology, Haukeland University Hospital, Bergen, NorwayDepartment of Pathology, Haukeland University Hospital, Bergen, NorwayDepartment of Neurology, Haukeland University Hospital, Bergen, NorwayDepartment of Clinical Medicine, University of Bergen, Bergen, NorwayDepartment of Neurology, Haukeland University Hospital, Bergen, NorwayDepartment of Clinical Medicine, University of Bergen, Bergen, NorwayObjectivesAutoantibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) and leucine-rich glioma-inactivated 1 (Lgi1) are associated with autoimmune encephalitis. We described an acetylcholine receptor (AChR)-positive patient with myasthenia gravis who developed limbic encephalitis with antibodies to AMPAR and Lgi1.MethodsA single-case report with detailed, prospective clinical and biomarker data including serial laboratory testing and histopathology.ResultsA 49-year-old woman was diagnosed with anti-AChR antibody-positive generalized myasthenia gravis in 1983. After 9 months of the removal of thymoma in 1984, she developed influenza-like symptoms and then symptoms of limbic encephalitis. Retrospective analysis of serum showed high concentrations of anti-AMPAR and lower concentrations of anti-Lgi1 antibodies. Cerebral CT was normal, EEG showed bifrontal dysrhythmia, and CSF showed mild pleocytosis. Immuno-histochemical examination of the thymoma confirmed staining for Glur2, a subunit of AMPAR. The patient recovered with mild sequelae, but low levels of anti-AMPAR and anti-Lgi1 antibodies were detectable for over 25 years subsequently.DiscussionThis case confirms earlier reports of AMPAR-associated autoimmune encephalitis co-occurring with thymoma and myasthenia gravis and is unique in its observational length. It shows, moreover, that antibodies to AMPAR and Lgi1 can persist despite clinical recovery.https://www.frontiersin.org/articles/10.3389/fneur.2023.1237140/fullmyasthenia gravisautoimmune encephalitisneuroimmunologyimmunologythymoma |
| spellingShingle | Magne Solberg Nes Mette Haugen Hans Kristian Haugland Nils Erik Gilhus Nils Erik Gilhus Christian Alexander Vedeler Christian Alexander Vedeler Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 Frontiers in Neurology myasthenia gravis autoimmune encephalitis neuroimmunology immunology thymoma |
| title | Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 |
| title_full | Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 |
| title_fullStr | Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 |
| title_full_unstemmed | Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 |
| title_short | Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1 |
| title_sort | case report seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to ampar and lgi1 |
| topic | myasthenia gravis autoimmune encephalitis neuroimmunology immunology thymoma |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2023.1237140/full |
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