Contactin-associated protein-2 antibodies in non-paraneoplastic cerebellar ataxia. Issue 4 (15th February 2012)

• Record Type: Journal Article
• Authors: Becker, Esther B E; Zuliani, Luigi; Pettingill, Rosemary; Lang, Bethan; Waters, Patrick; Dulneva, Anna; Sobott, Frank; Wardle, Mark; Graus, Francesc; Bataller, Luis; Robertson, Neil P; Vincent, Angela
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 83:Issue 4(2012)
• Page Start: 437
• Page End: 440
• Publication Date: 2012-02-15
• Subjects: CASPR2 -- autoimmune -- cerebellar ataxia -- VGKC-complex -- antibody -- neuroimmunology -- neurooncology -- NMDA -- paraneoplastic syndrome -- stiff man syndrome -- biochemistry -- molecular biology -- multiple sclerosis -- neuroepidemiology -- neurogenetics -- myasthenia -- channels -- lambert eaton syndrome -- stiff man syndr -- limbic system; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2011-301506
• Languages: English
• ISSNs: 0022-3050

Abstract

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.