Onconeural antibodies
| Antibody | No of patients reported | Identified by more than one laboratory | Paraneoplastic neurological syndrome | Tumours | % of antibody positive patients without cancer* (number of patients studied) | Frequency in cancer patients without PNS (number studied) |
|---|---|---|---|---|---|---|
| *Defined by number of patients with follow up of more than 3 years and no tumour found. | ||||||
| †Unpublished data from Dr J Honnorat. | ||||||
| ‡Data collected from multiple case reports. | ||||||
| §Follow up of patients without cancer in reference 32 not specified. | ||||||
| ¶Data collected in part from unpublished data of Dr Dalmau and Dr Voltz. Patients with brainstem encephalitis/PCD usually associate with tumours different from testicular cancer and their sera also react against Ma1 protein. | ||||||
| **Follow up less than three years but antibody disappeared several months after the onset of the cerebellar syndrome. | ||||||
| Well characterised onconeuronal antibodies | ||||||
| Anti-Hu (ANNA1) | >600 | Yes | Encephalomyelitis; sensory neuronopathy; chronic gastrointestinal pseudo-obstruction; paraneoplastic cerebellar degeneration (PCD); limbic encephalitis | Small cell lung cancer (SCLC) | 2% (200)5 | 16% (196 SCLC) (1% with titres similar to those patients with PNS)22 |
| Anti-Yo (PCA1) | >200 | Yes | Paraneoplastic cerebellar degeneration | Ovary, breast | 2% (125)14,23–25 | 1% (107)26 |
| Anti-CV2 (CRMP5) | >100 | Yes | Encephalomyelitis; chorea; sensory neuronopathy; sensorimotor neuropathy; chronic gastrointestinal pseudo-obstruction; paraneoplastic cerebellar degeneration; limbic encephalitis | SCLC, thymoma27,28 | 4% (47)† | 9% (74 SCLC)29 |
| Anti-Ri (ANNA2) | 61‡ | Yes | Brainstem encephalitis | Breast, SCLC | 3% (61)30–34§ | 4% (181 ovarian cancer)35 |
| Anti-Ma2 (Ta) | 55¶ | Yes | Limbic/diencephalic encephalitis; brainstem encephalitis/PCD¶ | Testicular, lung | 4% (55)36¶ | 0% (350)¶ |
| Anti-amphiphysin | 20‡ | Yes | Stiff person syndrome; various syndromes | Breast SCLC | 5% (20)37,38 | 0% (25 gynaecological cancer)39 1% (146 SCLC)40 |
| Partially characterised onconeuronal antibodies | ||||||
| Anti-Tr (PCA-Tr) | 28 | Yes | Paraneoplastic cerebellar degeneration | Hodgkin’s disease | 11% (28)41** | 0% (30)42 |
| ANNA3 | 11 | No | Various syndromes | SCLC | 9% (11)43 | 0%(58)43 |
| PCA2 | 9 | No | Various syndromes | SCLC | 0% (8)44 | 2% (58)44 |
| Anti-Zic4 | 8 | No | Paraneoplastic cerebellar degeneration | SCLC | 12% (8)29 | 16% (74)29 |
| Anti-mGluR1 | 2 | No | Paraneoplastic cerebellar degeneration | Hodgkin’s disease | 50% (2)45 | ? |