Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis
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Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis. / 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.
in: NEUROL-NEUROIMMUNOL, Jahrgang 3, Nr. 3, 06.2016, S. e232.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Evidence of a pathogenic role for CD8(+) T cells in anti-GABAB receptor limbic encephalitis
AU - Golombeck, Kristin S
AU - Bönte, Kathrin
AU - Mönig, Constanze
AU - van Loo, Karen M
AU - Hartwig, Marvin
AU - Schwindt, Wolfram
AU - Widman, Guido
AU - Lindenau, Matthias
AU - Becker, Albert J
AU - Glatzel, Markus
AU - Elger, Christian E
AU - Wiendl, Heinz
AU - Meuth, Sven G
AU - Lohmann, Hubertus
AU - Gross, Catharina C
AU - Melzer, Nico
PY - 2016/6
Y1 - 2016/6
N2 - 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.
AB - 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.
KW - Journal Article
U2 - 10.1212/NXI.0000000000000232
DO - 10.1212/NXI.0000000000000232
M3 - SCORING: Journal article
C2 - 27213174
VL - 3
SP - e232
JO - NEUROL-NEUROIMMUNOL
JF - NEUROL-NEUROIMMUNOL
SN - 2332-7812
IS - 3
ER -