Case Report: Presenting as optic neuritis—a biopsy-proven IgG4 anti-NF155–positive combined central and peripheral demyelination syndrome

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Combined central and peripheral demyelination (CCPD) features concurrent demyelination in both the central and peripheral nervous systems.
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Optic neuritis can be the initial manifestation of CCPD, particularly in patients positive for anti-neurofascin 155 (anti-NF155) antibodies.
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The case study reports a 21-year-old male with severe optic neuritis, later diagnosed with CCPD through histopathological examination.
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Patients with anti-NF155-positive CCPD often respond poorly to standard treatments like intravenous immunoglobulin.
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Effective management of NF155+ CCPD may require corticosteroids and rituximab due to complications like steroid dependence and relapse.

Frontiers In Immunology

by Yujing Peng, Xiaonan Wang, Weijiao Zhang, Ran Li, Jingting Peng, Jiawei Wang, Hanqiu Jiang
May 22, 2026

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