Selina M Yogeshwar, Sergio Muñiz-Castrillo, Lidia Sabater, Vicente Peris-Sempere, Vamsee Mallajosyula, Guo Luo, Han Yan, Eric Yu, Jing Zhang, Ling Lin, Flavia Fagundes Bueno, Xuhuai Ji, Géraldine Picard, Véronique Rogemond, Anne Laurie Pinto, Anna Heidbreder, Romana Höftberger, Francesc Graus, Josep Dalmau, Joan Santamaria, Alex Iranzo, Bettina Schreiner, Maria Pia Giannoccaro, Rocco Liguori, Takayoshi Shimohata, Akio Kimura, Yoya Ono, Sophie Binks, Sara Mariotto, Alessandro Dinoto, Michael Bonello, Christian J Hartmann, Nicola Tambasco, Pasquale Nigro, Harald Prüss, Andrew McKeon, Mark M Davis, Sarosh R Irani, Jérôme Honnorat, Carles Gaig, Carsten Finke, Emmanuel Mignot
Anti-IgLON5 disease is a rare and likely underdiagnosed subtype of autoimmune encephalitis. The disease displays a heterogeneous phenotype that includes sleep, movement, and bulbar-associated dysfunction. Presence of IgLON5-antibodies in CSF/serum, together with a strong association with HLA-DRB1*10:01∼DQB1*05:01, support an autoimmune basis. In this study, a multicentric HLA study of 87 anti-IgLON5 patients revealed a stronger association with HLA-DQ than HLA-DR. Specifically, we identified a predisposing rank-wise association with HLA-DQA1*01:05∼DQB1*05:01, HLA-DQA1*01:01∼DQB1*05:01 and HLA-DQA1*01:04∼DQB1*05:03 in 85% of patients...
March 1, 2024: Brain