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

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...

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Autores principales: Fukushima, Taiki, Ikeda, Shohei, Tomita, Minoru, Mori, Yu, Fukami, Yuki, Koike, Haruki, Katsuno, Masahisa, Ogata, Hidenori, Isobe, Noriko, Hattori, Naoki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10208785/
https://www.ncbi.nlm.nih.gov/pubmed/35908968
http://dx.doi.org/10.2169/internalmedicine.9286-21
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author Fukushima, Taiki
Ikeda, Shohei
Tomita, Minoru
Mori, Yu
Fukami, Yuki
Koike, Haruki
Katsuno, Masahisa
Ogata, Hidenori
Isobe, Noriko
Hattori, Naoki
author_facet Fukushima, Taiki
Ikeda, Shohei
Tomita, Minoru
Mori, Yu
Fukami, Yuki
Koike, Haruki
Katsuno, Masahisa
Ogata, Hidenori
Isobe, Noriko
Hattori, Naoki
author_sort Fukushima, Taiki
collection PubMed
description 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.
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spelling pubmed-102087852023-05-25 Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody Fukushima, Taiki Ikeda, Shohei Tomita, Minoru Mori, Yu Fukami, Yuki Koike, Haruki Katsuno, Masahisa Ogata, Hidenori Isobe, Noriko Hattori, Naoki Intern Med Case Report 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. The Japanese Society of Internal Medicine 2022-07-29 2023-05-01 /pmc/articles/PMC10208785/ /pubmed/35908968 http://dx.doi.org/10.2169/internalmedicine.9286-21 Text en Copyright © 2023 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Fukushima, Taiki
Ikeda, Shohei
Tomita, Minoru
Mori, Yu
Fukami, Yuki
Koike, Haruki
Katsuno, Masahisa
Ogata, Hidenori
Isobe, Noriko
Hattori, Naoki
Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title_full Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title_fullStr Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title_full_unstemmed Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title_short Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
title_sort demyelinating neuropathy with markedly elevated serum igg4 levels and anti-contactin 1 igg4 antibody
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10208785/
https://www.ncbi.nlm.nih.gov/pubmed/35908968
http://dx.doi.org/10.2169/internalmedicine.9286-21
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