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Case Reports
. 2022 Nov 20;12(11):1587.
doi: 10.3390/brainsci12111587.

Anti-NF155/NF186 IgG4 Antibody Positive Autoimmune Nodopathy

Lijun Wang  1   2 , Jing Pan  1 , Huanyu Meng  1 , Zhao Yang  3 , Lili Zeng  1 , Jun Liu  1   4
Affiliations
Case Reports

Anti-NF155/NF186 IgG4 Antibody Positive Autoimmune Nodopathy

Lijun Wang et al. Brain Sci. .

Abstract

Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) seropositive for autoantibodies against nodal and paranodal proteins display distinct clinical presentations. In the latest study, CIDP with autoantibodies against paranodal proteins was defined as autoimmune nodopathy (AN). We herein present a case of 39-year-old male with anti- neurofascin (NF) 155 and NF186 IgG4 antibody with gait disturbance and tremor, who was followed up for 4 months and demonstrated clinical improvements after apparently effective rituximab therapy. In addition, a literature review was conducted to investigate the clinical characteristics of anti-NF155/NF186-positive AN.

Keywords: IgG4; autoimmune nodopathy; neurofascin 155; neurofascin 186; rituximab.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Reactivity to NF 155 and NF186 by CBA Anti-NF 155 IgG4 antibodies detected in the patient’s serum (A) (titer 1:1000) and CSF (B) (titer 1:100) using CBA method. Anti-NF186 IgG4 antibodies detected in the patient’s serum (C) (titer 1:100) and CSF (D) (titer 1:100) using CBA method. CBA: cell-based assay; CSF: cerebrospinal fluid; Ig: immunoglobulin; NF: neurofascin.
Figure 2
Figure 2
Pathological images of sural nerve biopsy from the patient (A) Hematoxylin-eosin (HE) staining of sural nerve biopsy. (B) Modified Gomori trichrome (MGT) staining showed no obvious abnormality in the density of myelinated nerve fibers, and no significant demyelination. (C) Neurofilament (NF) staining showed that the axonal density was well. (A–C) Original magnification ×10.
Figure 3
Figure 3
The repeat serum and CSF antibody tests for NF 155 and NF186 by CBA after 4 months follow-up visit. Anti-NF 155 IgG4 antibodies detected in the patient’s serum (A) (titer 1:1000) and CSF (B) (titer 1:10) using CBA method. Anti-NF186 IgG4 antibodies detected in the patient’s serum (C) (titer 1:1000) and CSF (D) (titer 1:10) using CBA method. CBA: cell-based assay; CSF: cerebrospinal fluid; Ig: immunoglobulin; NF: neurofascin.
See this image and copyright information in PMC

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