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Antibodies to the Caspr1/contactin-1 complex in chronic inflammatory demyelinating polyradiculoneuropathy by Elba Pascual-Goñi; Janev Fehmi; Cinta Lleixà; Lorena Martín-Aguilar; Jérôme Devaux; Romana Höftberger; Emilien Delmont; Kathrin Doppler; Claudia Sommer; Aleksandar Radunovic; Alejandra Carvajal; Shane Smyth; Laura Williams; Radim Mazanec; Veronika Potočková; Nigel Hinds; Julien Cassereau; Karine Viala; Mathilde Lefilliatre; Guillaume Nicolas; Peter Foley; Frank Leypoldt; Stephen Keddie; Michael P Lunn; Fritz Zimprich; Vharoon Sharma Nunkoo; Wolfgang N Löscher; Laura Martínez-Martínez; Jordi Díaz-Manera; Ricard Rojas-Garcia; Isabel Illa; Simon Rinaldi; Luis Querol is a Neuroscience article available to read on EtoBox.

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## Abstract Previous studies have described the clinical, serological and pathological features of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and antibodies directed against the paranodal proteins neurofascin-155, contactin-1 (CNTN1), contactin-associated protein-1 (Caspr1), or nodal forms of neurofascin. Such antibodies are useful for diagnosis and potentially treatment selection. However, antibodies targeting Caspr1 only or the Caspr1/CNTN1 complex have been reported in few patients with CIDP. Moreover, it is unclear if these patients belong to the same pathophysiological subgroup. Using cell-based assays in routine clinical testing, we identified sera from patients with CIDP showing strong membrane reactivity when both CNTN1 and Caspr1 were co-transfected (but not when CNTN1 was transfected alone). Fifteen patients (10 male; aged between 40 and 75) with antibodies targeting Caspr1/CNTN1 co-transfected cells were enrolled for characterization. The prevalence of anti-Caspr1/CNTN1 antibodies was 1.9% (1/52) in the Sant Pau CIDP cohort, and 4.3% (1/23) in a German cohort of acute-onset CIDP. All patients fulfilled European Federation of Neurologic

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Author

Elba Pascual-Goñi; Janev Fehmi; Cinta Lleixà; Lorena Martín-Aguilar; Jérôme Devaux; Romana Höftberger; Emilien Delmont; Kathrin Doppler; Claudia Sommer; Aleksandar Radunovic; Alejandra Carvajal; Shane Smyth; Laura Williams; Radim Mazanec; Veronika Potočková; Nigel Hinds; Julien Cassereau; Karine Viala; Mathilde Lefilliatre; Guillaume Nicolas; Peter Foley; Frank Leypoldt; Stephen Keddie; Michael P Lunn; Fritz Zimprich; Vharoon Sharma Nunkoo; Wolfgang N Löscher; Laura Martínez-Martínez; Jordi Díaz-Manera; Ricard Rojas-Garcia; Isabel Illa; Simon Rinaldi; Luis Querol

Publisher

Oxford University Press (OUP)

Published

2021

Language

EN

Field

Neuroscience (Life Sciences)