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Case Reports
. 2023 Feb 15:10:2329048X221149618.
doi: 10.1177/2329048X221149618. eCollection 2023 Jan-Dec.

Autoantibodies to a Nodal Isoform of Neurofascin in Pediatric Chronic Inflammatory Demyelinating Polyneuropathy

Eline Chauvet  1 , Geraldine Blanchard Rohner  2 , Véroniqu Manel  3 , Emilien Delmont  4 , Joseph Boucraut  5 , Stephanie Garcia-Tarodo  1
Affiliations
Case Reports

Autoantibodies to a Nodal Isoform of Neurofascin in Pediatric Chronic Inflammatory Demyelinating Polyneuropathy

Eline Chauvet et al. Child Neurol Open. .

Abstract

Pediatric chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder of the peripheral nervous system with a number of diagnostic pitfalls. A subset of treatment-resistant CIDP adult patients have been found with antibodies against paranodal proteins. We report the first pediatric case in a 14 year-old adolescent with a severe CIDP phenotype in whom positive anti-neurofascin 155 antibodies were found in his serum. Resistant to conventional therapies, he showed dramatic improvement when treated with Rituximab with mild to moderate functional motor disability at 24 month follow-up. In pediatric CIDP patients that remain refractory to conventional treatments, the presence of antibodies to paranodal proteins warrants investigation as it can have potential therapeutic guidance.

Keywords: paranodal antibodies; pediatric chronic inflammatory demyelinating polyradiculoneuropathy; rituximab.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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