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- Title
LGI1 and CASPR2 neurological autoimmunity in children.
- 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.; López-Chiriboga, A Sebastian; Tillema, Jan-Mendelt
- 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.
- Subjects
NEUROLOGICAL disorders; AUTOIMMUNITY; IMMUNOTHERAPY; HEPATIC encephalopathy; IMMUNOPRECIPITATION; PROTEIN metabolism; PROTEINS; AUTOANTIBODIES; RESEARCH; NERVE tissue proteins; RESEARCH methodology; AUTOIMMUNE diseases; POTASSIUM; EVALUATION research; MEDICAL cooperation; COMPARATIVE studies; IMMUNITY; RESEARCH funding; MEMBRANE proteins
- Publication
Annals of Neurology, 2018, Vol 84, Issue 3, p473
- ISSN
0364-5134
- Publication type
journal article
- DOI
10.1002/ana.25310