Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody
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- Fukushima Taiki
- Department of Neurology, Toyota Kosei Hospital, Japan
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- Ikeda Shohei
- Department of Neurology, Toyota Kosei Hospital, Japan
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- Tomita Minoru
- Department of Neurology, Toyota Kosei Hospital, Japan
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- Mori Yu
- Department of Neurology, Gifu Prefectural Tajimi Hospital, Japan
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- Fukami Yuki
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Koike Haruki
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Katsuno Masahisa
- Department of Neurology, Nagoya University Graduate School of Medicine, Japan
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- Ogata Hidenori
- Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Japan
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- Isobe Noriko
- Department of Neurology, Graduate School of Medical Sciences, Kyushu University, Japan
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- Hattori Naoki
- Department of Neurology, Toyota Kosei Hospital, Japan
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Description
<p>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. </p>
Journal
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- Internal Medicine
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Internal Medicine 62 (9), 1341-1344, 2023-05-01
The Japanese Society of Internal Medicine