Contactin-associated protein-2 antibodies in non-paraneoplastic cerebellar ataxia.

Abstract

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.

• Authors: Esther B E Becker, Luigi Zuliani, Rosemary Pettingill, Bethan Lang, Patrick Waters, Anna Dulneva, Frank Sobott, Mark Wardle, Francesc Graus, Luis Bataller, Neil P Robertson, Angela Vincent
• Journal: Journal of neurology, neurosurgery, and psychiatry (J Neurol Neurosurg Psychiatry) Vol. 83 Issue 4 Pg. 437-40 (Apr 2012) ISSN: 1468-330X [Electronic] England
• PMID: 22338029 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• CNTNAP2 protein, human
• Immunoglobulin G
• Membrane Proteins
• Nerve Tissue Proteins

Topics

• Animals
• Autoimmune Diseases (immunology)
• Cerebellar Ataxia (immunology)
• Cerebellum (metabolism)
• Humans
• Immunoglobulin G (blood)
• Membrane Proteins (metabolism)
• Mice
• Nerve Tissue Proteins (metabolism)
• Organ Culture Techniques