Version May 2024
| Antibody (alternative name) | Likely pathogenic mechanism | Neurologic phenotypes | Frequency of cancer (%) | Usual tumors | Sex, age-related, and other specificities |
| High-risk antibodies (>70% associated with cancer) | |||||
| Hu (ANNA-1) | T cell-mediated | Sensory neuronopathy, chronic gastrointestinal pseudo-obstruction, encephalomyelitis, limbic encephalitis | 85 | SCLC >> NSCLC, other neuroendocrine tumors, neuroblastoma | Limbic encephalitis is usually nonparaneoplastic in patients <18 years of age. |
| CV2/CRMP5 | T cell-mediated | 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 | Uncertain | LEMS with and without rapidly progressive cerebellar syndrome | >90 | SCLC | Stronger correlation with SCLC than with a particular neurologic presentation. |
| PCA-2 (MAP1B) | T cell-mediated | Sensorimotor neuropathy, rapidly progressive cerebellar syndrome, encephalomyelitis | 80 | SCLC, NSCLC, breast cancer | |
| Amphiphysin | Uncertain; possibly antibody-mediated | 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) | T cell-mediated | Brainstem/cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome | >70 | Breast > lung (SCLC and NSCLC) | Breast cancer in females; lung cancer in males. |
| Yo (PCA-1) | T cell-mediated | 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 | T cell-mediated | 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) | Uncertain | Rapidly progressive cerebellar syndrome | 90 | Hodgkin lymphoma | |
| KLHL11 | T cell-mediated | Brainstem/cerebellar syndrome | 80 | Testicular cancer | Young males. |
| Intermediate-risk antibodies (30 to 70% associated with cancer) | |||||
| AMPAR | Antibody-mediated | Limbic encephalitis | >50 | SCLC, malignant thymoma | Paraneoplastic origin is more likely when other onconeuronal antibodies co-occur. |
| GABABR | Antibody-mediated | 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 | Antibody-mediated | Encephalitis | ~50 | Hodgkin lymphoma | |
| P/Q VGCC | Antibody-mediated for LEMS; uncertain for cerebellar syndrome | 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 | Antibody-mediated | 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 | Antibody-mediated | Morvan syndrome, limbic encephalitis, acquired neuromyotonia (Isaac syndrome) | 50 (for Morvan syndrome) <30 (for all other syndromes) | 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 | Antibody-mediated | Cerebellar ataxia | 30 | Mostly hematologic | |
| GABAAR | Antibody-mediated | Encephalitis | <30 | Malignant thymoma | Paraneoplastic origin is less frequent in children (10%) than in adults (60%). |
| GFAP | Uncertain | Meningoencephalitis | ~20 | Ovarian teratomas, adenocarcinomas | May occur as an immunologic accompaniment in anti-NMDAR encephalitis with ovarian teratomas. |
| GAD65 | Uncertain | 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 | Antibody-mediated | 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 | Antibody-mediated | Encephalitis with CNS hyperexcitability, PERM | <10 | B cell neoplasms | |
| GlyR | Antibody-mediated | Limbic encephalitis, PERM | <10 | Malignant thymoma, Hodgkin lymphoma | |
| AQP4 | Antibody-mediated | Neuromyelitis optica spectrum disorder | <5 | Adenocarcinomas | Paraneoplastic origin associated with older age, male sex, and severe nausea/vomiting at onset. |
| MOG | Uncertain | MOG antibody-associated disease | 5 cases reported | Mostly ovarian teratomas | |
| AK5 | T cell-mediated | Limbic encephalitis | <1 | No known cancer association | |
| GluK2 | Antibody-mediated | Encephalitis with prominent cerebellar involvement | <1 | 1 of 8 patients had a remote history of Hodgkin lymphoma | |
ANNA: antineuronal nuclear antibody; SCLC: small cell lung cancer; NSCLC: non-small cell lung cancer; CRMP5: collapsin-responsive mediator protein 5; MG: myasthenia gravis; SOX1: SRY-box transcription factor 1; LEMS: Lambert-Eaton myasthenic syndrome; PCA: Purkinje cell antibody; MAP1B: microtubule-associated protein 1B; DNER: delta/notch-like epidermal growth factor-related receptor; KLHL11: Kelch-like protein 11; AMPAR: α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; GABABR: gamma-aminobutyric acid-B receptor; KCTD16: potassium channel tetramerization domain containing; mGluR5: metabotropic glutamate receptor 5; P/Q VGCC: P/Q type voltage-gated calcium channel; NMDAR: N-methyl-D-aspartate receptor; Caspr2: contactin-associated protein-like 2; mGluR1: metabotropic glutamate receptor 1; GABAAR: gamma-aminobutyric acid-A receptor; GFAP: glial fibrillary acidic protein; GAD: glutamic acid decarboxylase; LGI1: leucine-rich glioma inactivated protein 1; DPPX: dipeptidyl-peptidase-like protein; CNS: central nervous system; PERM: progressive encephalomyelitis with rigidity and myoclonus; GlyR: glycine receptor; AQP4: aquaporin 4; MOG: myelin oligodendrocyte glycoprotein; AK5: adenylate kinase 5; GluK2: glutamate kainate receptor subunit 2.
* Antibodies to mGluR2 and SEZ6L2 are not listed as there is an insufficient number of cases to determine risk of cancer association.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
