Comparative Study of Paraneoplastic and Nonparaneoplastic Autoimmune Encephalitis With GABA <sub>B</sub> R Antibodies
Florian Lamblin, Jeroen Kerstens, Sergio Muñiz‐Castrillo, Alberto Vogrig, David Gonçalves, Véronique Rogemond, Géraldine Picard, Marine Villard, Anne‐Laurie Pinto, Marleen H. van Coevorden‐Hameete, M. Bruijn, Juna M. de Vries, Marco W.J. Schreurs, Louise Tyvaert, Lucie Hopes, Jérôme Aupy, Cécile Marchal, Dimitri Psimaras, Laurent Kremer, Veronique Bourg, Jean‐Christophe Antoine, Adrien Wang, Philippe Kahane, Sophie Demeret, Guido Ahle, Vicente Peris Sempere, Noémie Timestit, Mikaïl Nourredine, Aurélien Maureille, Marie Bénaiteau, Bastien Joubert, Emmanuel Mignot, Maarten J. Titulaer, Jérôme Honnorat
Abstract
BACKGROUND AND OBJECTIVES: R-AE) have poor functional outcomes and high mortality, the prognosis of nonparaneoplastic cases has not been well studied. METHODS: R-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples. RESULTS: = 0.008), respectively. Neurologic outcome no longer differed after adjustment for confounding factors but seemed to be negatively associated with increased age and ICU admission. A better survival was associated with nonparaneoplastic cases, a younger age, and the use of immunosuppressive drugs. DISCUSSION: R-AE, which might be due to a more intensive treatment strategy. A better understanding of immunologic mechanisms underlying both forms is needed.