LGI1 and CASPR2 neurological autoimmunity in children

A. Sebastian López-Chiriboga, Christopher Klein, Anastasia Zekeridou, Andrew McKeon, Divyanshu Dubey, Eoin P. Flanagan, Vanda A. Lennon, Jan Mendelt Tillema, Elaine C. Wirrell, Marc C. Patterson, Avi Gadoth, J. Gregory Aaen, J. Nicholas Brenton, Jonathan D. Bui, Amanda Moen, Catherine Otten, Amanda Piquet, Sean J. Pittock

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)473-480
Number of pages8
JournalAnnals of neurology
Issue number3
StatePublished - Sep 2018

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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