Table 1.
Selected antibodies to cerebellar antigens reported in patients with cerebellar ataxia
| Target structures | Comments | Ref |
|---|---|---|
| Purkinje cells | ||
| MGluR1/calcium pathway-related | ||
| Anti-mGluR1 | Tumour-associated in some cases | [33–36] |
| Anti-Homer-3 | Lung cancer-associated in one unpublished cases | [85, 86] |
| Anti-Sj/ITPR1 | NSCLC-associated in one unpublished case | [100] |
| Anti-CARP VIII | Reported in association with melanoma and ovarian cancer | [134, 135] |
| Anti-PKCγ | Reported in association with SCLC and liver cancer | [147, 148] |
| Anti-GluRδ2 | Mostly para/postinfectious | [149–151] |
| Anti-Ca/ARHGAP26 | Tumour-associated in a few cases | [74, 152, 153] |
| Anti-P/Q-type VGCC | Tumour-associated in many cases | [13, 14] |
| Anti-N type VGCC | Often associated with anti-P/Q-type VGCC | [154–157] |
| Anti-Yo/CDR2 (PCA-1)a | Typical paraneoplastic syndrome | [15, 158–162] |
| Anti-Nb/AP3B2/beta-NAP | Tumour-association unknown | [163, 164] |
| Others | ||
| PCA-2 (target antigen not known) | Tumour-associated in almost all published cases | [154] |
| Anti-Tr/DNER | HD-associated in almost all cases | [165–169] |
| Molecular and granular layer, PCs spared | ||
| Anti-amphiphysin | Tumour-associated in most cases | [170] |
| Anti-GABABR | Tumour-associated in many cases | [50–52] |
| Anti-DPPX | Reported in association with B cell neoplasm in a few patients | [171–174] |
| Anti-Caspr2 | Facultatively paraneoplastic | [175, 176] |
| Pinceau formation/Basket cells | ||
| Anti-LGI1 | Mainly not tumour-associated | [177] |
| Granular layer | ||
| Anti-GAD | DM-associated (mostly DM type I) and, in neurological patients, often tumour-associated | [119, 178–181] |
| Oligodendrocytes | ||
| Anti-CV2/CRMP5 | Typical paraneoplastic syndrome | [182–184] |
| Anti-MOG | Usually non-paraneoplastic | [185, 186] |
| Astrocytic endfeet | ||
| Anti-AQP4 | Very rarely causing cerebellar ataxia, usually non-paraneoplastic | [187, 188] |
| Neuronal nuclei | ||
| ANNA-1 (Anti-Hu/HuD) | Neuronal nuclei in the CNS and PNS paraneoplastic | [189–191] |
| ANNA-2 (Anti-Ri) | Neuronal nuclei in the CNS paraneoplastic | [192, 193] |
| ANNA-3 (unknown antigen) | Typical paraneoplastic syndrome | [194] |
| Anti-Zic4 | Typical paraneoplastic syndrome | [195, 196] |
| Anti-Zic2 | Mostly SCLC-associated | [197] |
| Anti-Zic1 | Mostly SCLC-associated | [197] |
| Bergman glial cell nuclei | ||
| AGNA/Anti-SOX1b | Typically tumour-associated | [198, 199] |
| Nucleoli | ||
| Anti-Ma2/Ta (PNMA2) | Typical paraneoplastic syndrome | [200, 201] |
| Anti-Ma1 (PNMA1) | Typical paraneoplastic syndrome | [200, 201] |
| Centrosome | ||
| Anti-γγ-enolase, -pericentrin, -ninein, -PCM1, -Mob1 | Para-/post varicella zoster virus | [202] |
| Centriols | ||
| Anti-centriolar antibodies | Para-/post M. pneumoniae | [203] |
| Others | ||
| Anti-transglutaminase6 | Associated with celiac disease | [204, 205] |
| Anti-triophosphate isomerase | Post-EBV | [206] |
| Anti-20 S proteasome | Associated with anti-Yo | [207] |
| Anti-GQ1b | ‘Ataxic Guillaine Barré syndrome’ | [208–211] |
DM diabetes mellitus, HD Hodgkin’s disease
aFurther target antigens reported in the literature: CDR34, CDR3, CDR2L
bWhether AGNA and SOX1 are identical is controversial; recent evidence suggests that they may represent different reactivities