title: Anti-neurofascin antibody in patients with combined central and peripheral demyelination.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Kawamura
authors.forename: Ryo
authors.initials: R
authors.lastname: Yamasaki
authors.forename: Tomomi
authors.initials: T
authors.lastname: Yonekawa
authors.forename: Takuya
authors.initials: T
authors.lastname: Matsushita
authors.forename: Susumu
authors.initials: S
authors.lastname: Kusunoki
authors.forename: Shigemi
authors.initials: S
authors.lastname: Nagayama
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Fukuda
authors.forename: Hidenori
authors.initials: H
authors.lastname: Ogata
authors.forename: Dai
authors.initials: D
authors.lastname: Matsuse
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Murai
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Kira
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D020278:Demyelinating Autoimmune Diseases, CNS / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors / Q000276:immunology*; D011129:Polyradiculoneuropathy / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015815:Cell Adhesion Molecules; C054305:NFASC protein, human; D009414:Nerve Growth Factors