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

doi:10.1002/ana.25310

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

54 Scopus citations

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.

Original language	English (US)
Pages (from-to)	473-480
Number of pages	8
Journal	Annals of Neurology
Volume	84
Issue number	3
DOIs	https://doi.org/10.1002/ana.25310
State	Published - Sep 2018

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© 2018 American Neurological Association

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López-Chiriboga, A. S., Klein, C., Zekeridou, A., McKeon, A., Dubey, D., Flanagan, E. P., Lennon, V. A., Tillema, J. M., Wirrell, E. C., Patterson, M. C., Gadoth, A., Aaen, J. G., Brenton, J. N., Bui, J. D., Moen, A., Otten, C., Piquet, A., & Pittock, S. J. (2018). LGI1 and CASPR2 neurological autoimmunity in children. Annals of Neurology, 84(3), 473-480. https://doi.org/10.1002/ana.25310

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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.",

author = "L{\'o}pez-Chiriboga, {A. Sebastian} and Christopher Klein and Anastasia Zekeridou and Andrew McKeon and Divyanshu Dubey and Flanagan, {Eoin P.} and Lennon, {Vanda A.} and Tillema, {Jan Mendelt} and Wirrell, {Elaine C.} and Patterson, {Marc C.} and Avi Gadoth and Aaen, {J. Gregory} and Brenton, {J. Nicholas} and Bui, {Jonathan D.} and Amanda Moen and Catherine Otten and Amanda Piquet and Pittock, {Sean J.}",

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AU - Patterson, Marc C.

AU - Gadoth, Avi

AU - Aaen, J. Gregory

AU - Brenton, J. Nicholas

AU - Bui, Jonathan D.

AU - Moen, Amanda

AU - Otten, Catherine

AU - Piquet, Amanda

AU - Pittock, Sean J.

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N2 - 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.

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LGI1 and CASPR2 neurological autoimmunity in children

Abstract

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