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Case Reports
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, and Naoki Hattori.
- Department of Neurology, Toyota Kosei Hospital, Japan.
- Intern. Med. 2023 May 1; 62 (9): 134113441341-1344.
AbstractWe 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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