Anti-neurofascin antibody in patients with combined central and peripheral demyelination
- 20 August 2013
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 81 (8), 714-722
- https://doi.org/10.1212/wnl.0b013e3182a1aa9c
Abstract
Objectives: We aimed to identify the target antigens for combined central and peripheral demyelination (CCPD). Methods: We screened target antigens by immunohistochemistry and immunoblotting using peripheral nerve tissues to identify target antigens recognized by serum antibodies from selected CCPD and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) cases. We then measured the level of antibody to the relevant antigen in 7 patients with CCPD, 16 patients with CIDP, 20 patients with multiple sclerosis, 20 patients with Guillain-Barré syndrome, 21 patients with other neuropathies, and 23 healthy controls (HC) by ELISA and cell-based assays using HEK293 cells. Results: At the initial screening, sera from 2 patients with CCPD showed cross-like binding to sciatic nerve sections at fixed intervals, with nearly perfect colocalization with neurofascin immunostaining at the node and paranode. ELISA with recombinant neurofascin revealed significantly higher mean optical density values in the CCPD group than in other disease groups and HC. Anti-neurofascin antibody positivity rates were 86% in patients with CCPD, 10% in patients with multiple sclerosis, 25% in patients with CIDP, 15% in patients with Guillain-Barré syndrome, and 0% in patients with other neuropathies and HC. The cell-based assay detected serum anti-neurofascin antibody in 5 of 7 patients with CCPD; all others were negative. CSF samples examined from 2 patients with CCPD were both positive. In anti-neurofascin antibody–positive CCPD patients, including those with a limited response to corticosteroids, IV immunoglobulin or plasma exchange alleviated the symptoms. Conclusion: Anti-neurofascin antibody is frequently present in patients with CCPD. Recognition of this antibody may be important, because patients with CCPD who are antibody positive respond well to IV immunoglobulin or plasma exchange.Keywords
This publication has 18 references indexed in Scilit:
- Neurofascin as a target for autoantibodies in peripheral neuropathiesNeurology, 2012
- Diagnostic criteria for multiple sclerosis: 2010 Revisions to the McDonald criteriaAnnals of Neurology, 2011
- Novel forms of neurofascin 155 in the central nervous system: alterations in paranodal disruption models and multiple sclerosisBrain, 2010
- Disruption of neurofascin and gliomedin at nodes of Ranvier precedes demyelination in experimental allergic neuritisBrain, 2008
- Relapsing demyelinating disease affecting both the central and peripheral nervous systemsJournal of Neurology, Neurosurgery & Psychiatry, 2008
- Neurofascin as a novel target for autoantibody-mediated axonal injuryThe Journal of Experimental Medicine, 2007
- Oligoclonal bands in multiple sclerosis cerebrospinal fluid: An update on methodology and clinical usefulnessJournal of Neuroimmunology, 2006
- Neurofascins Are Required to Establish Axonal Domains for Saltatory ConductionNeuron, 2005
- European Federation of Neurological Societies/Peripheral Nerve Society Guideline* on management of chronic inflammatory demyelinating polyradiculoneuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve SocietyJournal of the Peripheral Nervous System, 2005
- Guillain-Barré syndrome in northern China Relationship to Campylobacter jejuni infection and anti-glycolipid antibodiesBrain, 1995