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Table 1 Representative autoantibodies to cerebellar antigens in immune-mediated cerebellar ataxias

From: Guidelines for treatment of immune-mediated cerebellar ataxias

Autoantibodies

Conditions (When autoantibodies are associated with neoplasms, frequency of PCD is indicated.)

Localization

Anti-Yo

PCD (53 %), Breast, Uterus, Ovaries

Mainly Purkinje cells and few other neurons in the molecular layer

Anti-Hu

PCD (15 %), SCLC

All neuronal nuclei and cytolpasm

Anti-Tr

PCD (5 %), Hodgkin’s disease

Purkinje cells cytoplasm and dendrites,

Anti-CV2

PCD (4 %), SCLC, thymoma

Oligodendrocytes

Anti-Ri

PCD (2 %), Breast

All neuronal nuclei

Anti-Ma2

PCD (2 %), Testicle, Lung

Nucleoli

Anti-VGCC (P/Q type)

PCD (2 %), SCLC

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-SOX1

PCD (―), SCLC

Bergman glial cell nuclei

Anti-ZIC4

PCD (―), SCLC

Neuronal nuclei

PCA-2

PCD (―), SCLC

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-Homer-3

a

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-CARP VIII

a

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-PKCγ

a

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-Ca/ARHGAP26

a

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-mGluR1

a

Medusa

Anti-Sj/ITPR1

b

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-Nb/AP3B2

b

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-GluRδ2

Para/post infectious

Purkinje cells, cytoplasm, dendrites and dot-staining of the molecular layer

Anti-transglutaminase 2, 6

Gluten ataxia

 

Anti-GAD65

GAD Abs-CA or PCD (―)

GABAergic neurons

  1. Frequency among PCDs was evaluated based on our consensus paper [1]. PCD (―) indicates low frequency.
  2. The localization was based on the review by Jarius and Wildemann [8].
  3. SCLC small cell lung carcinoma, Medusa Immunohistochemistry shows ‘Medusa head’ pattern in some patients.
  4. aAssociation of neoplasms was reported only in 1–2 patients. bConditions that trigger production of Abs are unknown.