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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

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Lung cancer-associated in one unpublished cases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NSCLC-associated in one unpublished case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]

Others

  

  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]

Oligodendrocytes

  

 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]

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’

[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