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What is the proposed mechanism of neurologic paraneoplastic syndromes
Paraneoplastic syndromes are thought to occur when an immunologic response is focused against common antigens present on the tumor but also expressed in the nervous system.
Patients suspected of having a paraneoplastic syndrome should be evaluated for paraneoplastic antibodies in their serum and CSF
Antibody Testing for Paraneoplastic Syndrome
| Antibody | Tumor | Paraneoplastic Symptoms |
|---|---|---|
| Anti-Hu (ANNA-1) | Small-cell lung cancer, neuroblastoma, prostate cancer | Paraneoplastic encephalomyelitis, paraneoplastic sensory neuronopathy, paraneoplastic cerebellar degeneration, autonomic dysfunction |
| Anti-Yo (PCA-1) | Ovarian, breast, and lung cancers | Paraneoplastic cerebellar degeneration |
| Anti-Ri | Breast, gynecologic, lung, and bladder cancers | Ataxia with or without opsoclonus–myoclonus |
| Anti-Tr | Hodgkin’s lymphoma | Paraneoplastic cerebellar degeneration |
| Anti-VGCC | Small-cell lung cancer | Lambert–Eaton myasthenic syndrome |
| Antiretinal (antirecoverin protein) | Small-cell lung cancer, melanoma, gynecologic cancers | Cancer-associated retinopathy, melanoma-associated retinopathy |
| Antiamphiphysin | Breast and small-cell lung cancers | Stiff person syndrome, paraneoplastic encephalomyelitis |
| Anti-CRMP5 (anti-CV2) | Small-cell lung cancer, thymoma | Paraneoplastic encephalomyelitis, cerebellar degeneration, chorea, sensory neuropathy |
| Anti-PCA-2 | Small-cell lung cancer | Paraneoplastic encephalomyelitis, cerebellar degeneration; Lambert–Eaton myasthenic syndrome |
| Anti-Ma1 | Lung and other cancers | Brainstem encephalitis, cerebellar degeneration |
| Anti-Ma2 (-Ta) | Testicular cancer | Limbic brain stem encephalitis |
| ANNA-3 | Lung cancer | Sensory neuronopathy, encephalomyelitis |
| Anti-mGluR1 | Hodgkin’s lymphoma | Paraneoplastic cerebellar degeneration |
| Anti-VGKC | Thymoma, small-cell lung cancer | Neuromyotonia |
| Anti-MAG | Waldenström macroglobulinemia | Peripheral neuropathy |
Sources
From Darnell RB, Posner JB: Paraneoplastic syndromes involving the nervous system. N Engl J Med 349:1543-1554, 2003.
