TY - JOUR
T1 - Persistence of parenchymal and perivascular T-cells in treatment-refractory anti- N -methyl- d -aspartate receptor encephalitis
AU - Filatenkov, Alexander
AU - Richardson, Timothy E.
AU - Daoud, Elena
AU - Johnson-Welch, Sarah F.
AU - Ramirez, Denise M.
AU - Torrealba, Jose
AU - Greenberg, Benjamin
AU - Monson, Nancy L.
AU - Rajaram, Veena
N1 - Publisher Copyright:
© 2017 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2017
Y1 - 2017
N2 - Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disease mediated by IgG1 or IgG3 antibodies to the GluN1 subunit of the NMDAR, resulting in downregulation of NMDARs. Early diagnosis, prompt reduction of anti-NMDAR antibodies, and removal of associated ovarian tumors when identified are important drivers of prognosis. Immunohistochemical studies were carried out to evaluate B cell, plasma cell, and T-cell infiltrates in the brain of a 3-year-old patient with anti-NMDAR encephalitis who failed to show improvement after plasma exchange and Rituximab treatment. Complement activation was evaluated by C4d staining. Plasma cells and B-cells were rarely detected in the brain. In contrast, persistent intraparenchymal infiltrates and perivascular CD3+ T cells and evidence of complement activation were detected. Activated microglia and microglial nodules were also detected in the frontal lobes and the basal ganglia. The role of T cells and complement activation should be investigated in patients who do not respond to plasma exchange and Rituximab treatment.
AB - Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disease mediated by IgG1 or IgG3 antibodies to the GluN1 subunit of the NMDAR, resulting in downregulation of NMDARs. Early diagnosis, prompt reduction of anti-NMDAR antibodies, and removal of associated ovarian tumors when identified are important drivers of prognosis. Immunohistochemical studies were carried out to evaluate B cell, plasma cell, and T-cell infiltrates in the brain of a 3-year-old patient with anti-NMDAR encephalitis who failed to show improvement after plasma exchange and Rituximab treatment. Complement activation was evaluated by C4d staining. Plasma cells and B-cells were rarely detected in the brain. In contrast, persistent intraparenchymal infiltrates and perivascular CD3+ T cells and evidence of complement activation were detected. Activated microglia and microglial nodules were also detected in the frontal lobes and the basal ganglia. The role of T cells and complement activation should be investigated in patients who do not respond to plasma exchange and Rituximab treatment.
KW - N -methyl- d -aspartate receptor
KW - N -methyl- d -aspartate receptor encephalitis
KW - T cells
KW - autoimmune encephalitis
KW - complement activation
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U2 - 10.1097/WNR.0000000000000851
DO - 10.1097/WNR.0000000000000851
M3 - Article
C2 - 28777260
AN - SCOPUS:85030855159
SN - 0959-4965
VL - 28
SP - 890
EP - 895
JO - NeuroReport
JF - NeuroReport
IS - 14
ER -