. 2022 Nov;29(11):3347-3357.

doi: 10.1111/ene.15496. Epub 2022 Jul 25.

Association of plasma neurofilament light chain with disease activity in chronic inflammatory demyelinating polyradiculoneuropathy

Mahima Kapoor^{1

2}, Aisling Carr^{1

3}, Martha Foiani^{4

5}, Amanda Heslegrave^{4

5}, Henrik Zetterberg^{4

5

6

7

8}, Andrea Malaspina^{1

9

10

11}, Laura Compton³, Elspeth Hutton², Alexander Rossor^{1

3}, Mary M Reilly^{1

3}, Michael P Lunn^{1

3

12}

Affiliations

¹ Department of Neuromuscular Diseases, University College London Queen Square Institute of Neurology, London, UK.
² Department of Neurosciences, Central Clinical School, Monash University, Alfred Centre, Melbourne, Victoria, Australia.
³ Centre for Neuromuscular diseases, National Hospital for Neurology and Neurosurgery, London, UK.
⁴ Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology, London, UK.
⁵ UK Dementia Research Institute at University College London, London, UK.
⁶ Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden.
⁷ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska Academy at University of Gothenburg, Mölndal, Sweden.
⁸ Hong Kong Center for Neurodegenerative Diseases, Hong Kong, China.
⁹ University College London Queen Square Motor Neuron Disease Centre, Queen Square Institute of Neurology, London, UK.
¹⁰ Centre for Neuroscience and Trauma, Blizard Institute, Barts and London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
¹¹ ALS Biomarkers Study, University College London, London, UK.
¹² Neuroimmunology and CSF Laboratory, University College London Queen Square Institute of Neurology, London, UK.

PMID: 35837802
PMCID: PMC9796374
DOI: 10.1111/ene.15496

Association of plasma neurofilament light chain with disease activity in chronic inflammatory demyelinating polyradiculoneuropathy

Mahima Kapoor et al. Eur J Neurol. 2022 Nov.

. 2022 Nov;29(11):3347-3357.

doi: 10.1111/ene.15496. Epub 2022 Jul 25.

Authors

Affiliations

¹ Department of Neuromuscular Diseases, University College London Queen Square Institute of Neurology, London, UK.
² Department of Neurosciences, Central Clinical School, Monash University, Alfred Centre, Melbourne, Victoria, Australia.
³ Centre for Neuromuscular diseases, National Hospital for Neurology and Neurosurgery, London, UK.
⁴ Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology, London, UK.
⁵ UK Dementia Research Institute at University College London, London, UK.
⁶ Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden.
⁷ Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska Academy at University of Gothenburg, Mölndal, Sweden.
⁸ Hong Kong Center for Neurodegenerative Diseases, Hong Kong, China.
⁹ University College London Queen Square Motor Neuron Disease Centre, Queen Square Institute of Neurology, London, UK.
¹⁰ Centre for Neuroscience and Trauma, Blizard Institute, Barts and London School of Medicine and Dentistry, Queen Mary University of London, London, UK.
¹¹ ALS Biomarkers Study, University College London, London, UK.
¹² Neuroimmunology and CSF Laboratory, University College London Queen Square Institute of Neurology, London, UK.

PMID: 35837802
PMCID: PMC9796374
DOI: 10.1111/ene.15496

Abstract

Background and purpose: This study was undertaken to explore associations between plasma neurofilament light chain (pNfL) concentration (pg/ml) and disease activity in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and examine the usefulness of pNfL concentrations in determining disease remission.

Methods: We examined pNfL concentrations in treatment-naïve CIDP patients (n = 10) before and after intravenous immunoglobulin (IVIg) induction treatment, in pNfL concentrations in patients on maintenance IVIg treatment who had stable (n = 15) versus unstable disease (n = 9), and in clinically stable IVIg-treated patients (n = 10) in whom we suspended IVIg to determine disease activity and ongoing need for maintenance IVIg. pNfL concentrations in an age-matched healthy control group were measured for comparison.

Results: Among treatment-naïve patients, pNfL concentration was higher in patients before IVIg treatment than healthy controls and subsequently reduced to be comparable to control group values after IVIg induction. Among CIDP patients on IVIg treatment, pNfL concentration was significantly higher in unstable patients than stable patients. A pNFL concentration > 16.6 pg/ml distinguished unstable treated CIDP from stable treated CIDP (sensitivity = 86.7%, specificity = 66.7%, area under receiver operating characteristic curve = 0.73). Among the treatment withdrawal group, there was a statistically significant correlation between pNfL concentration at time of IVIg withdrawal and the likelihood of relapse (r = 0.72, p < 0.05), suggesting an association of higher pNfL concentration with active disease.

Conclusions: pNfL concentrations may be a sensitive, clinically useful biomarker in assessing subclinical disease activity.

Keywords: CIDP; IVIg; disease activity; neurofilament light chain.

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Conflict of interest statement

M.K. reports Grifols sponsorship for meeting attendance. A.C. reports Grifols sponsorship for meeting attendance and honoraria from CSL and Lupin for an advisory role. M.P.L. was a Primary Investigator in studies for CSL Behring, UCB Pharma, Novartis, Octapharma. He has also received ad hoc consulting fees from CSL Behring and UCB and an honorarium from Terumo BCT. H.Z. reports, outside the submitted work, institutional research support and support to attend scientific meetings from Bayer Healthcare, with honoraria for lectures from Bayer Healthcare and consultancy fees from UCB Biopharma paid to University College London Hospitals Charity. H.Z. has served on scientific advisory boards and/or as a consultant for Abbvie, Alector, Annexon, Artery Therapeutics, AZTherapies, CogRx, Denali, Eisai, Nervgen, Novo Nordisk, Pinteon Therapeutics, Red Abbey Labs, Passage Bio, Roche, Samumed, Siemens Healthineers, Triplet Therapeutics, and Wave, has given lectures in symposia sponsored by Cellectricon, Fujirebio, Alzecure, Biogen, and Roche, and is a cofounder of Brain Biomarker Solutions in Gothenburg, which is a part of the GU Ventures Incubator Program (outside submitted work). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

**FIGURE 1**
Plasma neurofilament light (NfL) in controls, before and after intravenous immunoglobulin (IVIg) induction in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. ns, not significant. *Statistically significant

**FIGURE 2**
Plasma neurofilament light (NfL) in chronic inflammatory demyelinating polyradiculoneuropathy patients before and after intravenous immunoglobulin (IVIg) induction

**FIGURE 3**
Plasma neurofilament light (NfL) concentration in relation to Inflammatory Rasch‐built Overall Disability Scale (I‐RODS) before and after intravenous immunoglobulin (IVIg) induction. CIDP, chronic inflammatory demyelinating polyradiculoneuropathy

**FIGURE 4**
Plasma neurofilament light (NfL) in stable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients compared with unstable CIDP patients (both groups on maintenance intravenous immunoglobulin). *Statistically significant

**FIGURE 5**
Plasma neurofilament light (NfL) concentration in relation to age of controls and stable and unstable chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. ns, not significant

**FIGURE 6**
Plasma neurofilament light (NfL) at time of intravenous immunoglobulin cessation in chronic inflammatory demyelinating polyradiculoneuropathy patients who did and did not relapse

See this image and copyright information in PMC

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Association of plasma neurofilament light chain with disease activity in chronic inflammatory demyelinating polyradiculoneuropathy

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Association of plasma neurofilament light chain with disease activity in chronic inflammatory demyelinating polyradiculoneuropathy

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