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A complex and severe encephalitis associated with four co-existing neuronal cell-surface autoantibodies.
J Neuroimmunol
December 2024
Department of Neurology, St. Vincent's University Hospital, Elm Park, Dublin, Ireland. Electronic address:
Many forms of autoimmune encephalitis are mediated by neuronal cell-surface directed autoantibodies. The co-occurrence of four neuronal cell-surface antibodies in a single patient is exceptionally rare. We report a patient who had a severe encephalitis associated with antibodies to NMDA, Glycine, GABA and GABA receptors.
View Article and Find Full Text PDFAutoimmune Encephalitis and Paraneoplastic Neurologic Syndromes: A Nationwide Study on Epidemiology and Antibody Testing Performance.
Neurol Neuroimmunol Neuroinflamm
November 2024
From the Department of Neurology (J.K., J.M.V., R.F.N., J.B., Y.C., R.W.v.S., M.A.A.M.d.B., M.H.v.C.-H., A.E.M.B., M.V., S.C.F., M.M.P.N., M.P., P.A.E.S.S., M.J.T.); Department of Immunology (M.S., S.B., S.V.), Laboratory Medical Immunology, Erasmus University Medical Center, Rotterdam; Department of Neurology (M.A.A.M.D.B.), Elisabeth-TweeSteden Hospital, Tilburg; Department of Neurology (A.v.S.), Haaglanden Medical Center, The Hague; Central Diagnostic Laboratory (J.D.), Maastricht University Medical Center; Central Diagnostic Laboratory (H.G.O.); Department of Neurology (C.J.M.F.), Utrecht University Medical Center; Department of Medical Microbiology and Immunology (B.M.), Saint Antonius Hospital, Nieuwegein; Laboratory of Medical Microbiology and Immunology (A.C.M.P.), Elisabeth-TweeSteden Hospital, Tilburg; Department of Neurology (A.M., C.C.D.); Clinical Laboratory (M.A.C.B.), Máxima Medical Center, Veldhoven; Departments of Neurology and Human Genetics (M.M.V.), Radboud University Medical Center, Nijmegen; and Department of Neurology (E.I.H.), Zuyderland Medical Center, Heerlen, The Netherlands.
Background And Objectives: Autoimmune encephalitis (AIE) and paraneoplastic neurologic syndromes (PNSs) encompass a heterogeneous group of antibody-associated disorders. Both the number of syndromes and commercially available antibody tests have increased considerably over the past decade. High-quality population-based data on epidemiology of these disorders and real-world performance of antibody tests are needed.
View Article and Find Full Text PDFMRI findings in autoimmune encephalitis.
Rev Neurol (Paris)
November 2024
Charité - Universitätsmedizin Berlin, Department of Neurology and Experimental Neurology, Berlin, Germany; Humboldt-Universität zu Berlin, Berlin School of Mind and Brain, Berlin, Germany; Charité - Universitätsmedizin Berlin, Einstein Center for Neurosciences Berlin, Berlin, Germany. Electronic address:
Article Synopsis
- In NMDA receptor encephalitis, most patients have normal MRIs during acute episodes, but subtle lesions can occur; these lesions may signal overlaps with conditions like multiple sclerosis or neuromyelitis optica.
- Other types, such as limbic encephalitis related to LGI1, CASPR2, and GAD antibodies, show specific hyperintensities in the medial temporal lobe on MRI, while GABA receptor encephalitides present different patterns of lesions, underscoring the importance of MRI in diagnosing autoimmune encephalitis and differentiating it from other disorders.
Severe Relapsing Autoimmune Encephalitis with GABA Receptor, Titin, and AchR Antibodies in a Patient with Thymoma: A Case Report.
Case Rep Neurol
May 2024
Department of Neurosciences, Centre Hospitalier de l'Université de Montréal (CHUM), Faculty of Medicine, Université de Montréal, Montreal, QC, Canada.
Article Synopsis
- A 40-year-old man with autoimmune encephalitis and a thymoma experienced severe neurological symptoms and multiple relapses despite initial treatments, which included thymectomy and first-line immunotherapy.
- Initial antibody tests revealed acetylcholine receptor and titin antibodies, but further testing eventually identified anti-GABA receptor antibodies.
- The case highlights the challenge of diagnosing and treating autoimmune encephalitis with coexisting antibodies, underscoring the need for targeted second-line therapies like rituximab in severe cases.
A case of relieving thymoma after conservative management of anti-gamma-aminobutyric acid receptor type A encephalitis.
Thorac Cancer
August 2024
Department of Chest Surgery, Yantaishan Hospital, Yantai, China.
Anti-gamma-aminobutyric acid receptor type A (GABAA) encephalitis is a relatively rare autoimmune encephalitis, and often associated with thymoma. Here, a 44-year-old female was diagnosed as having a thymoma with autoimmune encephalitis. At 4-month follow-up she was without recurrence of symptoms after treatment with methylprednisolone pulse therapy and immunotherapy.
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