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-VGCCa | LEMS, cerebellar degeneration | SCLC |
| Anti-NMDARa | Anti-NMDAR encephalitis | Teratoma in young women (children and men rarely have tumors) |
| Anti-LGI1b | Limbic encephalitis, hyponatremia, faciobrachial tonic or dystonic seizures | Rarely thymoma |
| Anti-Caspr2b | Morvan's syndrome, neuromyotonia, limbic encephalitis | Thymoma, prostate cancer |
| Anti-GABABRc | Limbic encephalitis, seizures | SCLC, neuroendocrine |
| Anti-GABAARa | Encephalitis with prominent seizures and status epilepticus; less often opsoclonus and stiff-person syndrome | Thymoma in ∼30% of pts |
| Anti-AMPARa | Limbic encephalitis with relapses | SCLC, thymoma, breast |
| Glycine receptor | PERM, stiff-person syndrome | Rarely, thymoma, lung, Hodgkin lymphoma |
| Anti-DPPXa | Agitation, myoclonus, tremor, seizures, hyperekplexia, encephalomyelitis with rigidity | No cancer, but frequent diarrhea or cachexia suggesting paraneoplasia |
| Anti-Neurexin 3alpha | Autoimmune encephalitis without distinctive features | No cancer association |
| Anti-Dopamine-2R | Basal ganglia encephalitis | No cancer association |
| Anti-Tr (DNER) | Cerebellar syndrome | Hodgkin lymphoma, or no tumor |
| Anti-mGluR1 | Cerebellar syndrome | Hodgkin lymphoma, or no tumor |
| Anti-mGluR5 | Autoimmune encephalitis without distinctive features | Hodgkin lymphoma, or no tumor |
| IgLON5 | NREM and REM sleep disorder, and brainstem dysfunction | No tumor association |
a A direct pathogenic role of these antibodies has been demonstrated in cultured neurons or animal models.
b Previously 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).
c This antibody is strongly suspected to be pathogenic.
Abbreviations: AChR, acetylcholine receptor; AMPAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; Caspr2, contactin-associated protein-like 2; DNER, delta/notch-like epidermal growth factor-related receptor; DPPX, dipeptidyl-peptidase-like protein-6; GABABR, γ;-aminobutyric acid B receptor; GAD, glutamic acid decarboxylase; mGluR, metabotropic glutamate receptor; LEMS, Lambert-Eaton myasthenic syndrome; LGI1, leucine-rich glioma-inactivated 1; NMDAR, N-methyl-D-aspartate receptor; NREM, non-rapid eye movement; PERM, progressive encephalomyelitis with rigidity and myoclonus; REM, rapid eye movement; SCLC, small-cell lung cancer; VGCC, voltage-gated calcium channel.