LGI1 and CASPR2 neurological autoimmunity in children

AS López‐Chiriboga, C Klein, A Zekeridou… - Annals of …, 2018 - Wiley Online Library
Annals of neurology, 2018Wiley Online Library
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) …
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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