Skip to main content

Table 1 Selected antibodies to cerebellar antigens reported in patients with cerebellar ataxia

From: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 1: Anti-mGluR1, anti-Homer-3, anti-Sj/ITPR1 and anti-CARP VIII

Target structures Comments Ref
Purkinje cells   
MGluR1/calcium pathway-related   
  Anti-mGluR1 Tumour-associated in some cases [3336]
  Anti-Homer-3 Lung cancer-associated in one unpublished cases [85, 86]
  Anti-Sj/ITPR1 NSCLC-associated in one unpublished case [100]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 [149151]
  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 [154157]
  Anti-Yo/CDR2 (PCA-1)a Typical paraneoplastic syndrome [15, 158162]
  Anti-Nb/AP3B2/beta-NAP Tumour-association unknown [163, 164]
  PCA-2 (target antigen not known) Tumour-associated in almost all published cases [154]
  Anti-Tr/DNER HD-associated in almost all cases [165169]
Molecular and granular layer, PCs spared   
 Anti-amphiphysin Tumour-associated in most cases [170]
 Anti-GABABR Tumour-associated in many cases [5052]
 Anti-DPPX Reported in association with B cell neoplasm in a few patients [171174]
 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, 178181]
 Anti-CV2/CRMP5 Typical paraneoplastic syndrome [182184]
 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 [189191]
 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]
 Anti-Ma2/Ta (PNMA2) Typical paraneoplastic syndrome [200, 201]
 Anti-Ma1 (PNMA1) Typical paraneoplastic syndrome [200, 201]
 Anti-γγ-enolase, -pericentrin, -ninein, -PCM1, -Mob1 Para-/post varicella zoster virus [202]
 Anti-centriolar antibodies Para-/post M. pneumoniae [203]
 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’ [208211]
  1. DM diabetes mellitus, HD Hodgkin’s disease
  2. aFurther target antigens reported in the literature: CDR34, CDR3, CDR2L
  3. bWhether AGNA and SOX1 are identical is controversial; recent evidence suggests that they may represent different reactivities