| Antibody (alternative name) | Neurologic phenotypes | Frequency of cancer (%) | Usual tumors | Sex, age-related, and other specificities |
| High-risk antibodies (>70% associated with cancer) | ||||
| Hu (ANNA-1) | Sensory neuronopathy, chronic gastrointestinal pseudoobstruction, encephalomyelitis, limbic encephalitis | 85 | SCLC >> NSCLC, other neuroendocrine tumors, neuroblastoma | Limbic encephalitis is usually nonparaneoplastic in patients <18 years of age. |
| CV2/CRMP5 | Encephalomyelitis, sensory neuronopathy | >80 | SCLC, thymoma | Patients with an associated thymoma are younger and present more frequently with MG and less commonly with neuropathy. |
| SOX1 | LEMS with and without rapidly progressive cerebellar syndrome | >90 | SCLC | Stronger correlation with SCLC than with a particular neurologic presentation. |
| PCA-2 (MAP1B) | Sensorimotor neuropathy, rapidly progressive cerebellar syndrome, encephalomyelitis | 80 | SCLC, NSCLC, breast cancer | |
| Amphiphysin | Polyradiculopathy, sensory neuronopathy, encephalomyelitis, stiff-person syndrome | 80 | SCLC, breast cancer | Associated antibodies commonly coexist. Patients with isolated antiamphiphysin are more likely to be females with breast cancer and stiff-person syndrome. |
| Ri (ANNA-2) | Brainstem/cerebellar syndrome, opsoclonus-myoclonus syndrome | >70 | Breast > lung (SCLC and NSCLC) | Breast cancer in females; lung cancer in males. |
| Yo (PCA-1) | Rapidly progressive cerebellar syndrome | >90 | Ovarian cancer, breast cancer | Almost all female; in males, antigen expression by tumor should be proven. |
| Ma2 and/or Ma | Limbic encephalitis, diencephalitis, brainstem encephalitis | >75 | Testicular cancer, NSCLC | Young males: testicular tumors and isolated Ma2 positivity; older patients: SCLC and both Ma1/2 positivity. |
| Tr (DNER) | Rapidly progressive cerebellar syndrome | 90 | Hodgkin lymphoma | |
| KLHL11 | Brainstem/cerebellar syndrome | 80 | Testicular cancer | Young males. |
| Intermediate-risk antibodies (30 to 70% associated with cancer) | ||||
| AMPAR | Limbic encephalitis | >50 | SCLC, malignant thymoma | Paraneoplastic origin is more likely when other onconeuronal antibodies co-occur. |
| GABABR | Limbic encephalitis | >50 | SCLC | Paraneoplastic cases are more commonly observed in older males, in smokers, and in association with anti-KCTD16 antibodies. Most cases in young patients are not paraneoplastic. |
| mGluR5 | Encephalitis | ~50 | Hodgkin lymphoma | |
| P/Q VGCC | LEMS, rapidly progressive cerebellar syndrome | 50 (LEMS; nearly 90 for rapidly progressive cerebellar syndrome) | SCLC | Co-occurrence with N-type VGCC antibodies might be slightly more common in paraneoplastic LEMS. |
| NMDAR | Anti-NMDAR encephalitis | 38 | Ovarian or extraovarian teratomas | Tumor (mostly ovarian teratomas) predominates in females 25 to 45 years of age (50%). Older patients less frequently have tumors (<25%), usually carcinomas. Paraneoplastic cases in children are very rare (<10%). |
| CASPR2 | Morvan syndrome | 50 | Malignant thymoma | CASPR2 should be considered as an intermediate-risk antibody only in the setting of Morvan syndrome. When associated with other neurologic syndromes, the risk of cancer is very low. |
| Lower-risk antibodies (<30% associated with cancer) | ||||
| mGluR1 | Cerebellar ataxia | 30 | Mostly hematologic | |
| GABAAR | Encephalitis | <30 | Malignant thymoma | Paraneoplastic origin is less frequent in children (10%) than in adults (60%). |
| CASPR2 | Limbic encephalitis, acquired neuromyotonia (Isaac syndrome), Morvan syndrome | <30 | Malignant thymoma | Morvan syndrome is more associated with malignant thymoma (~50%), whereas limbic encephalitis is almost always nonparaneoplastic. |
| GFAP | Meningoencephalitis | ~20 | Ovarian teratomas, adenocarcinomas | May occur as an immunologic accompaniment in anti-NMDAR encephalitis with ovarian teratomas. |
| GAD65 | Limbic encephalitis, stiff-person syndrome, cerebellar ataxia | <15 | SCLC, other neuroendocrine tumors, malignant thymoma | Paraneoplastic origin more likely in older patients, males, and in association with neuronal antibodies or atypical clinical presentations. |
| LGI1 | Limbic encephalitis | <10 | Malignant thymoma, neuroendocrine tumors | Paraneoplastic cases are mainly observed in patients with Morvan syndrome and both serum LGI1 and CASPR2 antibodies. |
| DPPX | Encephalitis with CNS hyperexcitability, PERM | <10 | B cell neoplasms | |
| GlyR | Limbic encephalitis, PERM | <10 | Malignant thymoma, Hodgkin lymphoma | |
| AQP4 | Neuromyelitis optica spectrum disorder | <5 | Adenocarcinomas | Paraneoplastic origin associated with older age, male sex, and severe nausea/vomiting at onset. |
| MOG | MOG antibody-associated disease | 5 cases reported | Mostly ovarian teratomas | |
