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Association of serum neurofilament light chain levels with clinicopathology of chronic inflammatory demyelinating polyneuropathy, including NF155 reactive patients

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To clarify whether serum neurofilament light chains (NfLs) serve as a biomarker of axonal damage in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), especially in patients with anti-neurofascin 155 (NF155) antibodies.


The Simoa system was used to examine serum NfL levels from 58 patients with CIDP, including 13 anti-NF155 antibody-positive patients, and from 14 age- and sex-matched healthy individuals. Serum NfL levels were evaluated before and after treatment in eight patients with anti-NF155 antibodies. Clinical features, electrophysiological findings, and cerebrospinal fluid (CSF) protein levels, were evaluated. The pathological features of sural nerves from 40 patients were also examined.


Serum NfL levels were significantly higher in patients with CIDP than in healthy individuals (median 29.63 vs. 7.71 pg/mL, p < 0.001) and were correlated with both modified Rankin Scale scores (r = 0.584, p < 0.001) and CSF protein levels (r = 0.432, p = 0.001). The NfL levels of anti-NF155 antibody-positive patients were higher than those of antibody-negative patients (p = 0.005). Serum NfL levels were negatively correlated with compound muscle action potential amplitudes of the tibial nerves (r =  − 0.404, p = 0.004) and positively correlated with the degree of active axonal degeneration in the pathological findings (r = 0.485, p = 0.001). In the antibody-positive group, NfL levels and antibody titers decreased after treatment in all examined patients.


Serum NfL correlated with pathological indices of axonal degeneration, and may serve as a biomarker that reflects active axonal damage of CIDP.

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Data availability

The data that support the findings are available from the corresponding author upon reasonable request.


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We thank Bronwen Gardner, PhD, from Edanz Group ( for editing a draft of this manuscript.


Dr. Fukami, Dr. Koike, Dr. Yamada and Dr. Hashizume report no disclosures. Dr. Iijima is supported by grants by JSPS KAKENHI (JP20K07864), the Japan Agency for Medical Research and Development (20lk0201080), Zenyaku Kogyo Co. Ltd., and Japan Blood Products Organization (JBPO). He also received honoraria from CSL Behring Co. Ltd. Dr. Katsuno is supported by a JSPS KAKENHI Grant Number JP20H00527, grants from the Japan Agency for Medical Research and Development (Nos. 19ek0109221, 19ek0109359, 19dk0207027, 19lk0201101, and 19dm0107155), a grant from the Naito Foundation and a grant from the Hori Sciences and Arts Foundation. He received honoraria from Takeda Pharmaceutical Co. Ltd., Alnylam Japan, Daiichi Sankyo Co. Ltd., Otsuka Pharmaceutical Co. Ltd., Novartis Pharma Co. Ltd., Biogen Japan and UCB Japan and grants from Zenyaku Kogyo Co. Ltd., Japan Blood Products Organization, Mitsubishi-Tanabe Pharma, CSL Behring Co. Ltd., Dainippon Sumitomo Pharma Co. Ltd., Otsuka Pharmaceutical Co. Ltd. and Daiichi Sankyo Co. Ltd.

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Drafting/revising the manuscript for content: YF, MI, HK, and MK. Study concept and design: YF, MI, and MK. Acquisition of samples, data, analysis and interpretation of data: YF, MI, HK, SY, AH and MK.

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Correspondence to Masahiro Iijima or Masahisa Katsuno.

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Not commissioned; externally peer reviewed.

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Fukami, Y., Iijima, M., Koike, H. et al. Association of serum neurofilament light chain levels with clinicopathology of chronic inflammatory demyelinating polyneuropathy, including NF155 reactive patients. J Neurol 268, 3835–3844 (2021).

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