Antibodies to Cell Surface or Synaptic Antigens, Syndromes, and Associated Tumors
| Antibody | Neurologic Syndrome | Tumor Type When Associated |
|---|---|---|
| Anti-AChR (muscle)a | Myasthenia gravis | Thymoma |
| Anti-AChR (neuronal)a | Autonomic ganglionopathy | SCLC |
| Anti-VGCCb | LEMS, cerebellar degeneration | SCLC |
| Anti-NMDARa | Anti-NMDAR encephalitis | Teratoma in young women (children and men rarely have tumors) |
| Anti-LGI1c | Limbic encephalitis, hyponatremia, faciobrachial tonic, or dystonic seizures | Rarely thymoma |
| Anti-Caspr2c | Morvan's syndrome, neuromyotonia | Thymoma, prostate cancer |
| Anti-GABABRd | Limbic encephalitis, seizures | SCLC, neuroendocrine |
| Anti-GABAARd | Encephalitis with prominent seizures and status epilepticus; less often opsoclonus and stiff-person syndrome | Rarely thymoma |
| Anti-AMPARd | Limbic encephalitis with relapses | SCLC, thymoma, breast |
| Glycine receptord | Encephalomyelitis with rigidity, stiff-person syndrome | Rarely, thymoma, lung cancer |
| Anti-DPPXd | Agitation, myoclonus, tremor, seizures, hyperekplexia, encephalomyelitis with rigidity | No cancer, but frequent diarrhea or cachexia suggesting paraneoplasia |
aA direct pathogenic role of these antibodies has been demonstrated.
bAnti-VGCC antibodies are pathogenic for LEMS.
cPreviously named voltage-gated potassium channel antibodies (VGKC); currently included under the term VGKC-complex proteins. Of note, the significance of antibodies to VGKC-complex proteins other than LGI1 and Caspr2 is uncertain (the antigens are unknown, and the response to immunotherapy is variable).
dThese antibodies are strongly suspected to be pathogenic.
Abbreviations: AChR, acetylcholine receptor; AMPAR, α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor; Caspr2, contactin-associated protein-like 2; DPPX, dipeptidyl-peptidase-like protein-6; GABABR, 𝛄-aminobutyric acid B receptor; GAD, glutamic acid decarboxylase; LEMS, Lambert-Eaton myasthenic syndrome; LGI1, leucine-rich glioma-inactivated 1; NMDAR, N-methyl-D-aspartate receptor; SCLC, small cell lung cancer; VGCC, voltage-gated calcium channel.