TY - JOUR AU - Chefdeville, Aude AU - Treilleux, Isabelle AU - Mayeur, Marie-Eve AU - Couillault, Coline AU - Picard, Géraldine AU - Bost, Chloé AU - Mokhtari, Karima AU - Vasiljevic, Alexandre AU - Meyronet, David AU - Rogemond, Véronique AU - Psimaras, Dimitri AU - Dubois, Bertrand AU - Honnorat, Jérôme AU - Desestret, Virginie PY - 2019 DA - 2019/03/11 TI - Immunopathological characterization of ovarian teratomas associated with anti-N-methyl-D-aspartate receptor encephalitis JO - Acta Neuropathologica Communications SP - 38 VL - 7 IS - 1 AB - Encephalitis with anti-NMDAR antibodies (NMDAR-E) is a severe autoimmune neurological disorder, defined by a clinical presentation of encephalitis and the presence of IgG targeting the GluN1 subunit of NMDA receptors in the CSF. An underlying ovarian teratoma is commonly associated with this autoimmune disease suggesting a role of the tumor in immunopathogenesis. In this study, we characterized the salient histopathological features of 27 ovarian teratomas associated with NMDAR-E (3 immature and 24 mature teratomas) and 40 controls without associated encephalitis. All but one NMDAR-E-associated teratomas contained a nervous tissue component, while less than 40% of control teratomas did (p < 0.001). GluN1 expression by teratomatous nervous tissue seemed to be more often glial in NMDAR-E teratomas than in control teratomas (73% vs. 29%, p < 0.05). Strikingly, 3 out of 24 NMDAR-E-associated mature teratomas contained neuroglial tissue exhibiting histopathological features of central nervous system neuroglial tumor, while such glioma-like features are exceptionally described in the literature on ovarian teratomas. Moreover, NMDAR-E associated teratomas differed from sporadic ovarian teratomas by consistent and prominent infiltration of the nervous tissue component by immune cells, comprised of T- and B-cells and mature dendritic cells organized in tertiary lymphoid structures, with IgG and IgA deposits and plasma cells in close contact to the neuroglial tissue. SN - 2051-5960 UR - https://doi.org/10.1186/s40478-019-0693-7 DO - 10.1186/s40478-019-0693-7 ID - Chefdeville2019 ER -