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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



Purkinje cells


MGluR1/calcium pathway-related



Tumour-associated in some cases



Lung cancer-associated in one unpublished cases

[85, 86]


NSCLC-associated in one unpublished case



Reported in association with melanoma and ovarian cancer

[134, 135]


Reported in association with SCLC and liver cancer

[147, 148]


Mostly para/postinfectious



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


  Anti-Yo/CDR2 (PCA-1)a

Typical paraneoplastic syndrome

[15, 158162]


Tumour-association unknown

[163, 164]



  PCA-2 (target antigen not known)

Tumour-associated in almost all published cases



HD-associated in almost all cases


Molecular and granular layer, PCs spared



Tumour-associated in most cases



Tumour-associated in many cases



Reported in association with B cell neoplasm in a few patients



Facultatively paraneoplastic

[175, 176]

Pinceau formation/Basket cells



Mainly not tumour-associated


Granular layer



DM-associated (mostly DM type I) and, in neurological patients, often tumour-associated

[119, 178181]




Typical paraneoplastic syndrome



Usually non-paraneoplastic

[185, 186]

Astrocytic endfeet



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


 ANNA-2 (Anti-Ri)

Neuronal nuclei in the CNS paraneoplastic

[192, 193]

 ANNA-3 (unknown antigen)

Typical paraneoplastic syndrome



Typical paraneoplastic syndrome

[195, 196]


Mostly SCLC-associated



Mostly SCLC-associated


Bergman glial cell nuclei



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




 Anti-centriolar antibodies

Para-/post M. pneumoniae





Associated with celiac disease

[204, 205]

 Anti-triophosphate isomerase



 Anti-20 S proteasome

Associated with anti-Yo



‘Ataxic Guillaine Barré syndrome’


  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