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

Taiki Fukushima; Shohei Ikeda; Minoru Tomita; Yu Mori; Yuki Fukami; Haruki Koike; Masahisa Katsuno; Hidenori Ogata; Noriko Isobe; Naoki Hattori

doi:10.2169/internalmedicine.9286-21

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

Taiki Fukushima, Shohei Ikeda, Minoru Tomita, Yu Mori, Yuki Fukami, Haruki Koike, Masahisa Katsuno, Hidenori Ogata, Noriko Isobe, Naoki Hattori

著者情報

Taiki Fukushima
Department of Neurology, Toyota Kosei Hospital, Japan
Shohei Ikeda
Department of Neurology, Toyota Kosei Hospital, Japan
Minoru Tomita
Department of Neurology, Toyota Kosei Hospital, Japan
Yu Mori
Department of Neurology, Gifu Prefectural Tajimi Hospital, Japan
Yuki Fukami
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Haruki Koike
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Masahisa Katsuno
Department of Neurology, Nagoya University Graduate School of Medicine, Japan
Hidenori Ogata
Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Japan
Noriko Isobe
Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Japan
Naoki Hattori
Department of Neurology, Toyota Kosei Hospital, Japan

キーワード: Chronic inflammatory demyelinating polyneuropathy, contactin 1, intravenous immunoglobulin therapy, steroid therapy

ジャーナルオープンアクセス早期公開

論文ID: 9286-21

DOI https://doi.org/10.2169/internalmedicine.9286-21

この記事には本公開記事があります。

The final version of this article is now available: Vol. 62 (2023), No. 9 pp. 1341-1344

詳細

抄録

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-contactin1 antibody. Further studies are needed to clarify the significance of IgG4-positive plasma cell infiltration in anti-contactin 1 antibody-positive neuropathies.

責任著者(Corresponding author)

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