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

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

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説明

<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>

収録刊行物

  • Internal Medicine

    Internal Medicine 62 (9), 1341-1344, 2023-05-01

    一般社団法人 日本内科学会

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