LAUSR

BACKGROUND AND PURPOSE Faciobrachial dystonic seizures (FBDS) and hyponatraemia are the distinct clinical features of autoimmune encephalitis (AE) caused by antibodies against leucine-rich glioma-inactivated 1 (LGI1). The pathophysiological pattern and neural mechanisms underlying these symptoms remain largely unexplored. METHODS We included 30 patients with anti-LGI1 AE and 30 controls from a retrospective observational cohort. Whole-brain metabolic pattern analysis was performed to assess the pathological network of anti-LGI1 AE, as well as the symptomatic networks of FBDS. Logistic regression was applied to explore independent predictors of FBDS. Finally, we applied multiple regression model to investigate the hyponatraemia-associated brain network and its effect on serum sodium levels. RESULTS The pathological network of anti-LGI1 AE involved a hypermetabolism in cerebellum, subcortical structures, and Rolandic area, as well as a hypometabolism in the medial prefrontal cortex. The symptomatic network of FBDS shown a hypometabolism in cerebellum and Rolandic area (PFDR < 0.05). Hypometabolism in the cerebellum was an independent predictor of FBDS (P < 0.001). Hyponatraemia-associated network highlighted a negative effect on caudate nucleus, frontal and temporal white matter. Serum sodium level had the negative trend with metabolism of hypothalamus (Pearson's R = -0.180, P = 0.342) but the mediation was not detected (path c' = -7.238, 95% CI = -30.947 to 16.472). CONCLUSIONS Our results provide insights into the whole-brain metabolic patterns of patients with anti-LGI1 AE, including the symptomatic network FBDS and the hyponatraemia-associated brain network, which is conducive to understanding the neural mechanisms and evaluating disease progress of anti-LGI1 AE.