Table 1.
Neurological diseases associated with Ab reactivities to autoantigen
| Antigen | Protein function | Clinical phenotype | Passive transfer disease model | Common tumor associations |
|---|---|---|---|---|
| AChR (muscle) | Neurotransmitter receptor | Myasthenia gravis | + | Thymoma |
| AChR (ganglionic) | Neurotransmitter receptor | Autonomic dysfunction | + | Breast, prostate, lung, gastrointestinal |
| AMPAR | Neurotransmitter receptor | Limbic encephalitis, seizures, memory loss | + | Breast, lung, thymoma |
| AQP4 | Water channel | NMOSD | + | Breast, lung, thymic, carcinoid, B cell lymphoma |
| CASPR2 | Neural-glial interactions and clustering of potassium channels | Limbic encephalitis (seizures, cognitive impairment), neuromyotonia and Morvan’s syndrome, neuropathic pain | + | Thymoma |
| DPPX | Regulatory Subunit of Kv4.2, voltage-gated potassium channel VGKC | Confusion, hallucinations, prodromal diarrhoea, memory loss, hyperexcitability | B-cell lymphoma | |
| D2R | Dopamin 2 receptor | Parkinsonism, chorea, psychosis, dystonia | ||
| GABAAR | Ligand-gated chloride channel | Seizures, status epilepticus, psychosis | + | Thymoma |
| GABABR | Metabotropic neurotransmitter receptor | Limbic encephalitis, seizures, memory loss | + | Lung, neuroendocrine |
| Glyα1R | Ligand-gated chloride channel | Encephalomyelitis, rigidity, myoclonus, seizures, stiff person syndrome | + | Ovarian, Hodgkin’s lymphoma, thymoma |
| IgLON5 | Neuronal adhesion protein | Parasomnia, sleep apnoea, cognitive impairment, gait abnormalities | Non-Hodgkin’s lymphoma, prostate, breast | |
| LGI-1 | Involved in glutamatergic synapse development | Limbic encephalitis (seizures, cognitive impairment), faciobrachial dystonic seizures, neuromyotonia | + | SCLC, thymoma |
| mGluR1 | Neurotransmitter receptor, G protein-coupled receptor | Cerebellar ataxia | + | Hodgkin’s lymphoma |
| mGluR5 | Neurotransmitter receptor, G protein-coupled receptor | Confusion, psychosis, memory loss, limbic encephalitis | + | Hodgkin’s lymphoma |
| MOG | Member of the immunoglobulin superfamily, expressed on myelin surface | Optic neuritis, myelitis, ADEM | + | Rare |
| NMDAR | Heteromeric ligand-gated calcium ion channel | Encephalitis, psychosis, amnesia, behavioural abnormalities, seizures, dysautonomia | + | Ovarian teratoma, rare carcinoma, medulloblastoma in children |
| Neurexin 3α | Involved in synapse formation and neural adhesion | Clinical overlap with NMDAR encephalitis | ||
| PCA-Tr/DNER | Voltage-dependant ion channel | LEMS, cerebellar degeneration, seizures, encephalopathy | SCLC | |
| VGCC (P/Q or N type) | Voltage-gated calcium channel | Dementia, complex pain syndromes | SCLC, thymoma |
AChR acetylcholine receptor, AMPAR alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionate receptor, AQP4 aqua-porin 4, CASPR2 contactin-associated protein-like 2, LGI-1 leucine-rich glioma inactivated 1, MOG myelin oligodendrocyte glycoprotein, NMDAR NMDA receptor, NMSOD neuromyelitis optica spectrum disorders, SCLC small cell lung carcinoma