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. 2025 Dec;30(4):e70077.
doi: 10.1111/jns.70077.

Clinical and Radiological Heterogeneity in Anti-Neurofascin-155 Autoimmune Nodopathy: A Case Series Analysis

Michie Imamura  1 , Hironori Mizutani  1 , Keiichi Nakahara  1 , Hidenori Ogata  2 , Tomoaki Taguchi  1 , Masao Imura  1 , Toshiya Nomura  1 , Yohei Misumi  1 , Noriko Isobe  2 , Mitsuharu Ueda  1
Affiliations

Clinical and Radiological Heterogeneity in Anti-Neurofascin-155 Autoimmune Nodopathy: A Case Series Analysis

Michie Imamura et al. J Peripher Nerv Syst. 2025 Dec.

Abstract

Background and aims: Autoimmune nodopathy with anti-neurofascin-155 (NF155) antibodies is increasingly recognized as a distinct disease entity. Although these patients have been described, not all aspects have been fully elucidated. We investigated clinical phenotypes, biomarker profiles, and treatment outcomes in patients with anti-NF155 antibody-positive autoimmune nodopathy.

Methods: We retrospectively analyzed seven Japanese patients (aged 13-27 years) with anti-NF155 antibody-positive autoimmune nodopathy treated at Kumamoto University Hospital between 2017 and 2020. Clinical, electrophysiological, radiological, and biomarker data were evaluated. One refractory case was followed up for over 80 months, tracking serum neurofilament light chain (sNfL) and anti-NF155 antibody levels in the serum and cerebrospinal fluid (CSF).

Results: All patients exhibited severe sensory ataxia and motor dysfunction. Trigeminal nerve hypertrophy was observed in five individuals, without corresponding symptoms, and pes cavus in one patient. All patients demonstrated a pronounced demyelinating neuropathy phenotype. The mean CSF protein level was 343 mg/dL. Intravenous immunoglobulin (IVIg) had minimal efficacy, while corticosteroids produced good responses in four patients and partial responses in three patients. Rituximab markedly improved a refractory case. Anti-NF155 antibody and sNfL levels correlated with clinical status. Magnetic resonance imaging frequently revealed persistent nerve root hypertrophy despite functional improvement.

Interpretation: Anti-NF155 nodopathy is a distinct, biomarker-trackable entity; corticosteroids and rituximab outperform IVIg, challenging conventional practice and highlighting the need for antibody testing, especially in young patients with severe demyelinating neuropathy.

Keywords: anti‐neurofascin‐155 antibodies; autoimmune nodopathy; neurofilament light chain; rituximab; treatment response.

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References

    1. L. Appeltshauser and K. Doppler, “Pan‐Neurofascin Autoimmune Nodopathy – A Life‐Threatening, but Reversible Neuropathy,” Current Opinion in Neurology 36 (2023): 394–401, https://doi.org/10.1097/WCO.0000000000001195.
    1. S. Tait, F. Gunn‐Moore, J. M. Collinson, et al., “An Oligodendrocyte Cell Adhesion Molecule at the Site of Assembly of the Paranodal Axo‐Glial Junction,” Journal of Cell Biology 150 (2000): 657–666, https://doi.org/10.1083/jcb.150.3.657.
    1. V. Amor, K. Feinberg, Y. Eshed‐Eisenbach, et al., “Long‐Term Maintenance of Na+ Channels at Nodes of Ranvier Depends on Glial Contact Mediated by Gliomedin and NrCAM,” Journal of Neuroscience 34 (2014): 5089–5098, https://doi.org/10.1523/JNEUROSCI.4752‐13.2014.
    1. P. Y. K. Van den Bergh, P. A. van Doorn, R. D. M. Hadden, et al., “European Academy of Neurology/Peripheral Nerve Society Guideline on Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Report of a Joint Task Force—Second Revision,” Journal of the Peripheral Nervous System 26 (2021): 242–268, https://doi.org/10.1111/jns.12455.
    1. L. Martín‐Aguilar, C. Lleixà, and E. Pascual‐Goñi, “Autoimmune Nodopathies, an Emerging Diagnostic Category,” Current Opinion in Neurology 35, no. 5 (2022): 579–585, https://doi.org/10.1097/WCO.0000000000001107.

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