• Merel C Broers, Luuk Wieske, Ece Erdag, Cemre Gürlek, Carina Bunschoten, Pieter A van Doorn, Filip Eftimov, Krista Kuitwaard, Juna M de Vries, de WitMarie-Claire YMYDepartment of Pediatric Neurology, Erasmus MC, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands., Mariska Mp Nagtzaam, Suzanne C Franken, Louisa Zhu, Manuela Paunovic, Maurice de Wit, Marco Wj Schreurs, Cinta Lleixà, Lorena Martín-Aguilar, Elba Pascual-Goñi, Luis Querol, Bart C Jacobs, Ruth Huizinga, and Maarten J Titulaer.
• Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
• J. Neurol. Neurosurg. Psychiatr. 2023 Dec 14; 95 (1): 526052-60.

BackgroundThe aim of this study was to determine treatment response and whether it is associated with antibody titre change in patients with autoimmune nodopathy (AN) previously diagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and to compare clinical features and treatment response between AN and CIDP.MethodsSerum IgG antibodies to neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (CASPR1) were detected with cell-based assays in patients diagnosed with CIDP. Clinical improvement was determined using the modified Rankin scale, need for alternative and/or additional treatments and assessment of the treating neurologist.ResultsWe studied 401 patients diagnosed with CIDP and identified 21 patients with AN (10 anti-NF155, 6 anti-CNTN1, 4 anti-CASPR1 and 1 anti-NF155/anti-CASPR1 double positive). In patients with AN ataxia (68% vs 28%, p=0.001), cranial nerve involvement (34% vs 11%, p=0.012) and autonomic symptoms (47% vs 22%, p=0.025) were more frequently reported; patients with AN improved less often after intravenous immunoglobulin treatment (39% vs 80%, p=0.002) and required additional/alternative treatments more frequently (84% vs 34%, p<0.001), compared with patients with CIDP. Antibody titres decreased or became negative in patients improving on treatment. Treatment withdrawal was associated with a titre increase and clinical deterioration in four patients.ConclusionsDistinguishing CIDP from AN is important, as patients with AN need a different treatment approach. Improvement and relapses were associated with changes in antibody titres, supporting the pathogenicity of these antibodies.© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.

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