Case report: Seropositive myasthenia gravis complicated by limbic encephalitis positive for antibodies to AMPAR and Lgi1

Frontiers in Neurology

Neurology

Magne Solberg Nes¹  Mette Haugen¹  Nils Erik Gilhus²  Christian Alexander Vedeler²  Hans Kristian Haugland³ 

[1] Department of Neurology, Haukeland University Hospital, Bergen, Norway;Department of Neurology, Haukeland University Hospital, Bergen, Norway;Department of Clinical Medicine, University of Bergen, Bergen, Norway;Department of Pathology, Haukeland University Hospital, Bergen, Norway;

关键词: myasthenia gravis;    autoimmune encephalitis;    neuroimmunology;    immunology;    thymoma;   

DOI  :  10.3389/fneur.2023.1237140

 received in 2023-06-08, accepted in 2023-09-15,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

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Copyright © 2023 Nes, Haugen, Haugland, Gilhus and Vedeler.