LGI1 and CASPR2 neurological autoimmunity in children. Issue 3 (24th September 2018)

• Record Type: Journal Article
• Authors: López‐Chiriboga, A. Sebastian; Klein, Christopher; Zekeridou, Anastasia; McKeon, Andrew; Dubey, Divyanshu; Flanagan, Eoin P.; Lennon, Vanda A.; Tillema, Jan‐Mendelt; Wirrell, Elaine C.; Patterson, Marc C.; Gadoth, Avi; Aaen, J. Gregory; Brenton, J. Nicholas; Bui, Jonathan D.; Moen, Amanda; Otten, Catherine; Piquet, Amanda; Pittock, Sean J.
• Is Part Of: Annals of neurology. Volume 84:Issue 3(2018)
• Page Start: 473
• Page End: 480
• Publication Date: 2018-09-24
• Subjects: Neurology -- Periodicals; Pediatric neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; 616.8
• DOI: 10.1002/ana.25310
• Languages: English
• ISSNs: 0364-5134

Abstract

Abstract : The clinical phenotype of leucine‐rich glioma‐inactivated protein 1 (LGI1) and contactin‐associated proteinlike 2 (CASPR2) autoimmunity is well defined in adults. Data for children are limited (<10 cases). Among 13, 319 pediatric patients serologically tested for autoimmune neurological disorders (2010–2017), 264 were seropositive for voltage‐gated potassium channel‐complex–IgG (radioimmunoprecipitation). Only 13 (4.9%) were positive by transfected cell‐binding assay for LGI1‐IgG (n = 7), CASPR2‐IgG (n = 3), or both (n = 3). This is significantly less than in adults. Encephalopathy, seizures, and peripheral nerve hyperexcitability were common, as was coexisting autoimmunity. No faciobrachial dystonic seizures or cancers were identified. Functional neurologic disorders were frequently the initial diagnosis, and immunotherapy appeared beneficial. Ann Neurol 2018;84:473–480