Martín-Aguilar, Lorena, Lleixà, Cinta, Pascual-Goñi, Elba, Caballero-Ávila, Marta, Martínez-Martínez, Laura, Díaz-Manera, Jordi, Rojas-García, Ricard, Cortés-Vicente, Elena, Turon-Sans, Janina, De Luna, Noemi, Suárez-Calvet, Xavier, Gallardo, Eduard, Rajabally, Yusuf, Scotton, Sangeeta, Jacobs, Bart C., Baars, Adája, Cortese, Andrea, Vegezzi, Elisa, Höftberger, Romana, Zimprich, Fritz, Roesler, Cornelia, Nobile-Orazio, Eduardo, Liberatore, Giuseppe, Hiew, Fu Liong, Martínez-Piñeiro, Alicia, Carvajal, Alejandra, Piñar-Morales, Raquel, Usón-Martín, Mercedes, Albertí, Olalla, López-Pérez, Maria Ángeles, Márquez, Fabian, Pardo-Fernández, Julio, Muñoz-Delgado, Laura, Cabrera-Serrano, MacArena, Ortiz, Nicolau, Bartolomé, Manuel, Duman, Özgür, Bril, Vera, Segura-Chávez, Darwin, Pitarokoili, Kalliopi, Steen, Claudia, Illa, Isabel and Querol, Luis (2022). Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy. Neurology: Neuroimmunology and NeuroInflammation, 9 (1),
Abstract
Background and ObjectivesTo study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN).MethodsPatients 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.ResultsForty 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.DiscussionAnti-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 EvidenceThis study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.
Publication DOI: | https://doi.org/10.1212/NXI.0000000000001098 |
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Divisions: | College of Health & Life Sciences |
Additional Information: | The Article Processing Charge was funded by authors. 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. |
Uncontrolled Keywords: | Neurology,Clinical Neurology |
Publication ISSN: | 2332-7812 |
Last Modified: | 07 Oct 2025 07:21 |
Date Deposited: | 07 Oct 2025 07:21 |
Full Text Link: | |
Related URLs: |
http://www.scop ... tnerID=8YFLogxK
(Scopus URL) https://www.neu ... 000000000001098 (Publisher URL) |
PURE Output Type: | Article |
Published Date: | 2022-01-02 |
Accepted Date: | 2021-09-27 |
Authors: |
Martín-Aguilar, Lorena
Lleixà, Cinta Pascual-Goñi, Elba Caballero-Ávila, Marta Martínez-Martínez, Laura Díaz-Manera, Jordi Rojas-García, Ricard Cortés-Vicente, Elena Turon-Sans, Janina De Luna, Noemi Suárez-Calvet, Xavier Gallardo, Eduard Rajabally, Yusuf ( ![]() Scotton, Sangeeta Jacobs, Bart C. Baars, Adája Cortese, Andrea Vegezzi, Elisa Höftberger, Romana Zimprich, Fritz Roesler, Cornelia Nobile-Orazio, Eduardo Liberatore, Giuseppe Hiew, Fu Liong Martínez-Piñeiro, Alicia Carvajal, Alejandra Piñar-Morales, Raquel Usón-Martín, Mercedes Albertí, Olalla López-Pérez, Maria Ángeles Márquez, Fabian Pardo-Fernández, Julio Muñoz-Delgado, Laura Cabrera-Serrano, MacArena Ortiz, Nicolau Bartolomé, Manuel Duman, Özgür Bril, Vera Segura-Chávez, Darwin Pitarokoili, Kalliopi Steen, Claudia Illa, Isabel Querol, Luis |