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BACKGROUND: Relatively few studies have searched for potentially pathogenic antibodies in non-paraneoplastic patients with cerebellar ataxia. METHODS AND RESULTS: We first screened sera from 52 idiopathic ataxia patients for binding of serum IgG antibodies to cerebellar neurons. One strong-binding serum was selected for immunoprecipitation and mass spectrometry, which resulted in the identification of contactin-associated protein 2 (CASPR2) as a major antigen. CASPR2 antibodies were then found by a cell-based assay in 9/88 (10%) ataxia patients, compared to 3/144 (2%) multiple sclerosis or dementia controls (p=0.011). CASPR2 is strongly expressed in the cerebellum, only partly in association with voltage-gated potassium channels. CONCLUSIONS: Prospective studies are now needed to see whether identification of CASPR2 antibodies has relevance for the diagnosis and treatment of idiopathic cerebellar ataxia.

Original publication




Journal article


J Neurol Neurosurg Psychiatry

Publication Date





437 - 440


Animals, Autoimmune Diseases, Cerebellar Ataxia, Cerebellum, Humans, Immunoglobulin G, Membrane Proteins, Mice, Nerve Tissue Proteins, Organ Culture Techniques