Clinical Characterization and Long-Term Outcome in Children and Adults With Anti-AMPA Receptor Encephalitis
Chiara Milano, Ezgi Saylam, Claudia Papi, Laura Marmolejo, Alexandra Sankovic, Raphael Reinecke, Jeroen Kerstens, Chiara Pizzanelli, Mateus Mistieri Simabukuro, Marie Bénaiteau, Bastien Joubert, Matteo Gastaldi, Lívia Almeida Dutra, Frank Leypoldt, Mareike Jansen, Izumi Kawachi, Hisanao Akiyama, Nikolas Boy, Silvia Bozzetti, Peter Christensen, Pietro Businaro, Alessandro Dinoto, Tal Friedman‐Korn, Urara Fujiwara, Tatsuya Fukumoto, Kenshiro Fuse, Sam Hooshmand, Lauren Hurst, Raffaele Iorio, Isabelle Korn‐Lubetzki, André Filipe Lucchi Rodrigues, Sara Mariotto, J A Michael, Yuko Morimoto, Rinze F. Neuteboom, Amanda L. Piquet, Andrea O. Rossetti, Sabine Rumpler-Kreiner, Jonathan D. Santoro, Florian Schwendinger, Brenda Shen, Steffen Syrbe, J Tröger, Ingrid Wagner, Christian G. Bien, Eugenia Martínez‐Hernández, Thaís Armangué, Romana Höftberger, Takahiro Iizuka, Maarten J. Titulaer, Jérôme Honnorat, Francesc Graus, Josep Dalmau, Setty M. Magaña, Marianna Spatola
Abstract
BACKGROUND AND OBJECTIVES: Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptor (anti-AMPAR) encephalitis manifests as limbic encephalitis in adults and is often associated with cancer. Although some reports suggest that it may occur in children, the clinical features in this population, as well as the prognostic factors and long-term outcomes in children and adults, are unknown. METHODS: We performed a retrospective, international collaborative study of patients with anti-AMPAR encephalitis. Clinical information was reviewed, together with data from published pediatric patients. Clinical features of children and adults were compared with nonparametric tests. Survival rates (Kaplan-Meier curves) were compared using log-rank tests. Prognostic factors of poor outcome (modified Rankin Scale score >2) were identified using logistic regression models. RESULTS: = 0.008). DISCUSSION: Children and adults with anti-AMPAR encephalitis show distinct clinical-radiologic features. At long-term follow-up, 68% of patients, all adults, have neurologic sequelae, with failure to respond to first-line immunotherapy being associated with worse outcomes.