title: Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies.
abstract: To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes.
  
  
  
  We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with anti-neurofascin-155 antibodies and 1 patient with anti-contactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings.
  
  
  
  Characteristic light and electron microscopy findings in transverse sections from patients with anti-neurofascin-155 and anti-contactin-1 antibodies indicated a slight reduction in myelinated fibre density, with scattered myelin ovoids, and the absence of macrophage-mediated demyelination or onion bulbs. Teased-fibre preparations revealed that segmental demyelination tended to be found in patients with relatively higher frequencies of axonal degeneration and was tandemly found at consecutive nodes of Ranvier in a single fibre. Assessment of longitudinal sections by electron microscopy revealed that detachment of terminal myelin loops from the axolemma was frequently found at the paranode in patients with anti-neurofascin-155 and anti-contactin-1 antibody-positive CIDP compared with patients with antibody-negative CIDP. Patients with anti-neurofascin-155 antibodies showed a positive correlation between the frequencies of axo-glial detachment at the paranode and axonal degeneration, as assessed by teased-fibre preparations (p<0.05).
  
  
  
  Paranodal dissection without classical macrophage-mediated demyelination is the characteristic feature of patients with CIDP with autoantibodies to paranodal axo-glial junctional molecules.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Haruki
authors.initials: H
authors.lastname: Koike
authors.affiliation: Division of Neurology, Department of Internal Medicine, National Defense Medical College, Saitama, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Kadoya
authors.affiliation: Division of Neurology, Department of Internal Medicine, National Defense Medical College, Saitama, Japan.
authors.forename: Ken-Ichi
authors.initials: KI
authors.lastname: Kaida
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Shohei
authors.initials: S
authors.lastname: Ikeda
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Kawagashira
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Iijima
authors.affiliation: Department of Neurology and Neuroscience, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kato
authors.affiliation: Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Ogata
authors.affiliation: Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Yamasaki
authors.affiliation: Department of Neurology and Neuroscience, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Matsukawa
authors.affiliation: Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Jun-Ichi
authors.initials: JI
authors.lastname: Kira
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Masahisa
authors.initials: M
authors.lastname: Katsuno
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.|Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D001369:Axons / Q000276:immunology / Q000473:pathology*; D001706:Biopsy; D015815:Cell Adhesion Molecules / Q000276:immunology*; D058967:Contactin 1 / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009186:Myelin Sheath / Q000276:immunology / Q000473:pathology*; D009414:Nerve Growth Factors / Q000276:immunology*; D009457:Neuroglia / Q000276:immunology / Q000473:pathology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000276:immunology / Q000473:pathology*; D011901:Ranvier's Nodes / Q000276:immunology / Q000473:pathology*; D012583:Schwann Cells / Q000276:immunology / Q000473:pathology; D013497:Sural Nerve / Q000276:immunology / Q000473:pathology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; C548669:CNTN1 protein, human; D015815:Cell Adhesion Molecules; D058967:Contactin 1; C054305:NFASC protein, human; D009414:Nerve Growth Factors