LGI1 and CASPR2 neurological autoimmunity in children |
| |
| Authors: | A. Sebastian López‐Chiriboga MD Christopher Klein MD Anastasia Zekeridou MD Andrew McKeon MD Divyanshu Dubey MD Eoin P. Flanagan MD Vanda A. Lennon MD PhD Jan‐Mendelt Tillema MD Elaine C. Wirrell MD Marc C. Patterson MD Avi Gadoth MD J. Gregory Aaen MD J. Nicholas Brenton MD Jonathan D. Bui MD PhD Amanda Moen MD Catherine Otten MD Amanda Piquet MD Sean J. Pittock MD |
| |
| Affiliation: | 1. Departments of Neurology;2. Laboratory Medicine and Pathology;3. Immunology, Mayo Clinic, Rochester, MN;4. Department of Pediatrics and Neurology, Loma Linda University Children's Hospital, Loma Linda, CA;5. Department of Neurology and Pediatrics, University of Virginia, Charlottesville, VA;6. Department of Neurosciences, University of California, San Diego and Division of Child Neurology, Rady Children's Hospital, San Diego, CA;7. Department of Pediatric Neurology, Gillette Children's Specialty Healthcare, St Paul, MN;8. Department of Pediatric Neurology, Seattle Children's Hospital, Seattle, WA;9. Department of Neurology, University of Colorado, Aurora, CO |
| |
| 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 |
| |
| Keywords: | |
|
|
