Evidence of a pathogenic role for CD8+ T cells in anti-GABAB receptor limbic encephalitis. Issue 3 (June 2016)

• Record Type: Journal Article
• Authors: Golombeck, Kristin S.; Bönte, Kathrin; Mönig, Constanze; van Loo, Karen M.; Hartwig, Marvin; Schwindt, Wolfram; Widman, Guido; Lindenau, Matthias; Becker, Albert J.; Glatzel, Markus; Elger, Christian E.; Wiendl, Heinz; Meuth, Sven G.; Lohmann, Hubertus; Gross, Catharina C.; Melzer, Nico
• Is Part Of: Neurology. Volume 3:Issue 3(2016)
• Publication Date: 2016-06
• Subjects: Neuroimmunology -- Periodicals; Neurology -- Periodicals; 616.8
• DOI: 10.1212/NXI.0000000000000232
• Languages: English
• ISSNs: 2332-7812

Abstract

Abstract : Objectives: To characterize the cellular autoimmune response in patients with γ-aminobutyric acid (GABA)B receptor antibody–associated limbic encephalitis (GABAB -R LE). Methods: Patients underwent MRI, extensive neuropsychological assessment, and multiparameter flow cytometry of peripheral blood and CSF. Results: We identified a series of 3 cases of nonparaneoplastic GABAB -R LE and one case of paraneoplastic GABAB -R LE associated with small cell lung cancer. All patients exhibited temporal lobe epilepsy, neuropsychological deficits, and MRI findings typical of LE. Absolute numbers of CD19 + B cells, CD138 + CD19 + plasma cells, CD4 + T cells, activated HLADR + CD4 + T cells, as well as CD8 + T cells and HLADR + CD8 + T cells did not differ in peripheral blood but were elevated in CSF of patients with GABAB -R LE compared to controls. Augmented absolute numbers of CD138 + CD19 + plasma cells and activated HLADR + CD8 + T cells in CSF corresponded to higher overall neuropsychological and memory deficits in patients with GABAB -R LE. A histologic specimen of one patient following selective amygdalohippocampectomy revealed perivascular infiltrates of CD138 + plasma cells and CD4 + T cells, whereas cytotoxic CD8 + T cells were detected within the brain parenchyma in close contact to neurons. Conclusion: Our data suggest a pathogenic role for CD8 + T cells in addition to the established role of plasma cell–derived autoantibodies in GABAB -R LE.