Paraneoplastic antibodies associated with rhomboencephalitis
| Antigen location | Antibody | Oncological association | Treatment |
| Intracellular | Anti-Hu (ANNA-1) | Small-cell lung cancer | Cancer targeted therapy±immunosuppression* |
| Anti-Ri (ANNA-2) | Breast, lung | Cancer targeted therapy±immunosuppression | |
| Anti-Ma2 | Testicular germ cell tumour | Cancer targeted therapy±immunosuppression | |
| Less common paraneoplastic antineuronal antibodies: anti-Yo, Anti-Tr, antiamphiphysin, | Breast, gynaecological, small-cell lung cancer and Hodgkin’s lymphoma (anti-Yo); Hodgkin’s lymphoma (anti-Tr); breast and small-cell lung cancer (anti-amphiphysin); | Cancer targeted therapy±immunosuppression | |
| Cell surface | Anti-NMDA | Ovarian teratoma | Immunosuppression±cancer targeted therapy |
| Anti-IgLON5 | None currently | Immunosuppression | |
| Antiglycine receptor | Breast, thymoma, Hodgkin’s lymphoma | Immunosuppression±cancer targeted therapy | |
| Less common: anti-DPPX | Lymphoma | Immunosuppression±cancer targeted therapy |
*Immunosuppression is usually with high-dose corticosteroids and either intravenous immunoglobulin or plasma exchange first line followed by rituximab or cyclophosphamide.
ANNA, antineuronal nuclear antibody; DPPX, dipeptidyl-peptidase-like protein 6; IgLON5, immunoglobulin-like cell adhesion molecule 5; NMDA, N-methyl-D-aspartate.