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Case Reports
. 2023 May 1;62(9):1341-1344.
doi: 10.2169/internalmedicine.9286-21. Epub 2022 Jul 29.

Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody

Taiki Fukushima  1 , Shohei Ikeda  1 , Minoru Tomita  1 , Yu Mori  2 , Yuki Fukami  3 , Haruki Koike  3 , Masahisa Katsuno  3 , Hidenori Ogata  4 , Noriko Isobe  4 , Naoki Hattori  1
Affiliations
Case Reports

Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody

Taiki Fukushima et al. Intern Med. .

Abstract

We herein report a 77-year-old man with a 4-month history of progressive gait and sensory disturbances of the extremities. A nerve conduction study indicated demyelinating polyneuropathy. Serum IgG4 levels and anti-contactin 1 IgG4 antibodies were markedly increased. The sural nerve biopsy specimen showed IgG4-positive plasma cell infiltration in the epineurium. Treatment with steroids resulted in an amelioration of functional status, improvement of nerve conduction parameters, decreased serum IgG4 levels, and negative conversion of anti-contactin 1 antibody. Further studies are needed to clarify the significance of IgG4-positive plasma cell infiltration in anti-contactin 1 antibody-positive neuropathies.

Keywords: Chronic inflammatory demyelinating polyneuropathy; contactin 1; intravenous immunoglobulin therapy; steroid therapy.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure.
Figure.
Pathological findings of the sural nerve biopsy. (A) Transverse sections stained with toluidine blue showed a mild decrease in myelinated fiber density. (B) Hematoxylin and Eosin staining showed infiltration of plasma cells and lymphocytes in the epineurium. (C) IgG4 immunostaining showed infiltration of IgG4-positive plasma cells in the epineurium. Scale bar indicates 50 μm.
See this image and copyright information in PMC

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