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January 2022; 9 (1) ArticleOpen Access

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

View ORCID ProfileLorena Martín-Aguilar, Cinta Lleixà, Elba Pascual-Goñi, View ORCID ProfileMarta Caballero-Ávila, Laura Martínez-Martínez, Jordi Díaz-Manera, View ORCID ProfileRicard Rojas-García, Elena Cortés-Vicente, Janina Turon-Sans, Noemi de Luna, View ORCID ProfileXavier Suárez-Calvet, Eduard Gallardo, Yusuf Rajabally, Sangeeta Scotton, View ORCID ProfileBart C. Jacobs, View ORCID ProfileAdája Baars, Andrea Cortese, View ORCID ProfileElisa Vegezzi, Romana Höftberger, View ORCID ProfileFritz Zimprich, Cornelia Roesler, Eduardo Nobile-Orazio, View ORCID ProfileGiuseppe Liberatore, Fu Liong Hiew, Alicia Martínez-Piñeiro, Alejandra Carvajal, View ORCID ProfileRaquel Piñar-Morales, Mercedes Usón-Martín, Olalla Albertí, Maria Ángeles López-Pérez, Fabian Márquez, Julio Pardo-Fernández, View ORCID ProfileLaura Muñoz-Delgado, Macarena Cabrera-Serrano, Nicolau Ortiz, Manuel Bartolomé, Özgür Duman, Vera Bril, Darwin Segura-Chávez, Kalliopi Pitarokoili, Claudia Steen, Isabel Illa, View ORCID ProfileLuis Querol
First published November 2, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001098
Lorena Martín-Aguilar
From the Neuromuscular Diseases Unit (L.M.-A., C.L., E.P.-G., M.C.-Á., R.R.-G., E.C.-V., J.T.-S., E.N.-O., I.I., L.Q.), Department of Neurology, Hospital de La Santa Creu I Sant Pau, Universitat Autònoma de Barcelona; Biomedical Research Institute Sant Pau (IIB Sant Pau) (L.M.-A., C.L., E.P.-G., M.C.-Á., J.D.-M., E.C.-V., X.S.-C., E.G.); Immunology Department (L.M.-M.), Hospital de La Santa Creu I Sant Pau, Universitat Autònoma de Barcelona, Spain; Centro para La Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (J.D.-M., R.R.-G., E.C.-V., N.L., X.S.-C., E.G., I.I., L.Q.); University Hospital Birmingham (Y.R., S.S.), UK; Erasmus Medical Center (B.C.J., A.B.), Rotterdam, the Netherlands; IRCCS Mondino Foundation (Andrea Cortese, E.V.), Pavia, Italy; Department of Neurology (R.H., F.Z.), Medical University of Vienna; Paracelsus Medical University (C.R.), Salzburg, Austria; IRCCS Humanitas Research Hospital (G.L.), Milan University, Rozzano, Italy; Kuala Lumpur General Hospital (F.L.H.), Jalan Pahang, Kuala Lumpur, Malaysia; Hospital Universitari Germans Trias I Pujol (A.M.-P.), Badalona; Hospital Universitario Virgen de Las Nieves (Alejandra Carvajal), Granada, Spain; Hospital Universitario Clínico San Cecilio (R.P.-M.), Granada; Hospital Son Llàtzer (M.U.-M.), Palma de Mallorca; Hospital San Jorge (O.A.), Huesca, España; Hospital San Pedro (M.Á.L.-P.), Logroño; Hospital Universitari Josep Trueta (F.M.), Girona; Hospital Clínico Universitario de Santiago (J.P.-F.), Santiago de Compostela; Hospital Universitario Virgen Del Rocío (L.M.-D., M.C.-S.), Sevilla; Hospital Universitari Sant Joan (N.O.), Reus; Complejo Asistencial de Ávila (M.B.), Ávila, Spain; Akdeniz University (Ö.D.), Antalya, Turkey; Toronto General Hospital (V.B.), University Health Network, University of Toronto, Canada; Instituto Nacional de Ciencias Neurológicas. Lima (D.S.-C.), Perú; St. Josef-Hospital (K.P.), Ruhr-University Bochum; and Sant Joseph Hospital (C.S.), Berlin, Germany.
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Cinta Lleixà
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Elba Pascual-Goñi
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Marta Caballero-Ávila
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Laura Martínez-Martínez
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Noemi de Luna
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Mercedes Usón-Martín
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Olalla Albertí
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Maria Ángeles López-Pérez
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Fabian Márquez
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Julio Pardo-Fernández
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Vera Bril
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Luis Querol
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Citation
Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy
Lorena Martín-Aguilar, Cinta Lleixà, Elba Pascual-Goñi, Marta Caballero-Ávila, Laura Martínez-Martínez, Jordi Díaz-Manera, Ricard Rojas-García, Elena Cortés-Vicente, Janina Turon-Sans, Noemi de Luna, Xavier Suárez-Calvet, Eduard Gallardo, Yusuf Rajabally, Sangeeta Scotton, Bart C. Jacobs, Adája Baars, Andrea Cortese, Elisa Vegezzi, Romana Höftberger, Fritz Zimprich, Cornelia Roesler, Eduardo Nobile-Orazio, Giuseppe Liberatore, Fu Liong Hiew, Alicia Martínez-Piñeiro, Alejandra Carvajal, Raquel Piñar-Morales, Mercedes Usón-Martín, Olalla Albertí, Maria Ángeles López-Pérez, Fabian Márquez, Julio Pardo-Fernández, Laura Muñoz-Delgado, Macarena Cabrera-Serrano, Nicolau Ortiz, Manuel Bartolomé, Özgür Duman, Vera Bril, Darwin Segura-Chávez, Kalliopi Pitarokoili, Claudia Steen, Isabel Illa, Luis Querol
Neurol Neuroimmunol Neuroinflamm Jan 2022, 9 (1) e1098; DOI: 10.1212/NXI.0000000000001098

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This article has a correction. Please see:

  • Errata - January 01, 2022
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Abstract

Background and Objectives To study the clinical and laboratory features of antineurofascin-155 (NF155)–positive autoimmune nodopathy (AN).

Methods Patients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up.

Results Forty NF155+ patients with AN were included. Mean age at onset was 42.4 years. Patients presented with a progressive (75%), sensory motor (87.5%), and symmetric distal-predominant weakness in upper (97.2%) and lower extremities (94.5%), with tremor and ataxia (75%). Patients received a median of 3 (2–4) different treatments in 46 months of median follow-up. Response to IV immunoglobulin (86.8%) or steroids (72.2%) was poor in most patients, whereas 77.3% responded to rituximab. HLA-DRB1*15 was detected in 91.3% of patients. IgG4 anti-NF155 antibodies were predominant in all patients; anti-NF155 titers correlated with mRS within the same patient (r = 0.41, p = 0.004). Serum NfL (sNfL) levels were higher in anti-NF155+ AN than in healthy controls (36.47 vs 7.56 pg/mL, p < 0.001) and correlated with anti-NF155 titers (r = 0.43, p = 0.001), with I-RODS at baseline (r = −0.88, p < 0.001) and with maximum I-RODS achieved (r = −0.58, p = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients.

Discussion Anti-NF155 AN presents a distinct clinical profile and good response to rituximab. Autoantibody titers and sNfL are useful to monitor disease status in these patients. The use of untagged-NF155 plasmids minimizes the detection of false anti-NF155+ cases.

Classification of Evidence This study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.

Glossary

CBA=
cell-based assay;
CIDP=
chronic inflammatory demyelinating polyradiculoneuropathy;
CMAP=
compound muscle action potential;
CNTN1=
contactin-1;
EMG=
electromyography;
GBS=
Guillain-Barré syndrome;
HC=
Healthy control;
HLA=
human leukocyte antigen;
ICC=
immunocytochemistry;
I-RODS=
Inflammatory Rasch-built Overall Disability Scale;
IVIg=
IV immunoglobulin;
mRS=
modified Rankin Scale;
NCS=
nerve conduction study;
NF140=
neurofascin-140;
NF155=
neurofascin-155;
NF186=
neurofascin-186;
OD=
optical density;
PLEX=
plasma exchange;
sNfL=
serum neurofilament light chain

Footnotes

  • ↵* These authors contributed equally to this work as co-first authors.

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by authors.

  • Class of Evidence: NPub.org/coe

  • Received March 2, 2021.
  • Accepted in final form September 27, 2021.
  • Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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