Immunological reactivity against neuronal and non-neuronal antigens in sporadic adult-onset cerebellar ataxia

Eur Neurol. 2009;62(6):356-61. doi: 10.1159/000242010. Epub 2009 Sep 26.

Abstract

In recent years, the involvement of the immune system in acquired forms of cerebellar ataxia has been frequently demonstrated. In this study, we describe 6 out of 49 patients with subacute or chronic progressive cerebellar ataxia in whom antibodies against neuronal and non-neuronal antigens were identified. Two women had anti-Yo antibodies; two patients had anti-gliadin antibodies in the presence of celiac disease; one patient had a complex autoimmune disorder associated with anti-Ro-52/SS-A and anti-muscle-specific kinase antibodies, and a patient developed subacute cerebellar syndrome associated with the presence of a prostatic adenocarcinoma and atypical antibodies reacting both with cerebellar tissue and with the prostatic tumor. Our study confirms previous findings in paraneoplastic syndromes, and indicates that at least 10% of sporadic cerebellar ataxia may be related to immune-mediated mechanisms.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / complications
  • Adenocarcinoma / immunology
  • Autoantibodies / immunology*
  • Autoimmunity / immunology*
  • Blotting, Western
  • Celiac Disease / complications
  • Celiac Disease / immunology
  • Electromyography
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Gliadin / immunology
  • Glutamate Decarboxylase / immunology
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Nerve Tissue Proteins / immunology
  • Neurons / immunology
  • Prostatic Neoplasms / complications
  • Prostatic Neoplasms / immunology
  • Ribonucleoproteins / immunology
  • Spinocerebellar Degenerations / complications
  • Spinocerebellar Degenerations / immunology*

Substances

  • Autoantibodies
  • CDR2 protein, human
  • Nerve Tissue Proteins
  • Ribonucleoproteins
  • SS-A antigen
  • Gliadin
  • Glutamate Decarboxylase