Table 2

 Onconeural antibodies

AntibodyNo of patients reportedIdentified by more than one laboratoryParaneoplastic neurological syndromeTumours% 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†Unpublished data from Dr J Honnorat.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¶Data collected in part from unpublished data of Dr Dalmau and Dr Voltz.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)>600YesEncephalomyelitis; sensory neuronopathy; chronic gastrointestinal pseudo-obstruction; paraneoplastic cerebellar degeneration (PCD); limbic encephalitisSmall cell lung cancer (SCLC)2% (200)516% (196 SCLC) (1% with titres similar to those patients with PNS)22
Anti-Yo (PCA1)>200YesParaneoplastic cerebellar degenerationOvary, breast2% (125)14,23–251% (107)26
Anti-CV2 (CRMP5)>100YesEncephalomyelitis; chorea; sensory neuronopathy; sensorimotor neuropathy; chronic gastrointestinal pseudo-obstruction; paraneoplastic cerebellar degeneration; limbic encephalitisSCLC, thymoma27,284% (47)†9% (74 SCLC)29
Anti-Ri (ANNA2)61‡YesBrainstem encephalitisBreast, SCLC3% (61)30–34§4% (181 ovarian cancer)35
Anti-Ma2 (Ta)55¶YesLimbic/diencephalic encephalitis; brainstem encephalitis/PCD¶Testicular, lung4% (55)360% (350)¶
Anti-amphiphysin20‡YesStiff person syndrome; various syndromesBreast SCLC5% (20)37,380% (25 gynaecological cancer)39 1% (146 SCLC)40
Partially characterised onconeuronal antibodies
Anti-Tr (PCA-Tr)28YesParaneoplastic cerebellar degenerationHodgkin’s disease11% (28)41**0% (30)42
ANNA311NoVarious syndromesSCLC9% (11)430%(58)43
PCA29NoVarious syndromesSCLC0% (8)442% (58)44
Anti-Zic48NoParaneoplastic cerebellar degenerationSCLC12% (8)2916% (74)29
Anti-mGluR12NoParaneoplastic cerebellar degenerationHodgkin’s disease50% (2)45?