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LGI1 and CASPR2 neurological autoimmunity in children
Author(s) -
LópezChiriboga A. Sebastian,
Klein Christopher,
Zekeridou Anastasia,
McKeon Andrew,
Dubey Divyanshu,
Flanagan Eoin P.,
Len Vanda A.,
Tillema JanMendelt,
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.
Publication year - 2018
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.25310
Subject(s) - autoimmunity , medicine , encephalopathy , immunology , glioma , neuromyotonia , autoimmune encephalitis , immunotherapy , antibody , autoantibody , immune system , cancer research
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