Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neurosurgery, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
Eur J Neurol. 2022 Aug;29(8):2376-2385. doi: 10.1111/ene.15384. Epub 2022 May 29.
Faciobrachial dystonic seizures (FBDS) and hyponatremia are the distinct clinical features of autoimmune encephalitis (AE) caused by antibodies against leucine-rich glioma-inactivated 1 (LGI1). The present study aims to explore the pathophysiological patterns and neural mechanisms underlying these symptoms.
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 symptom networks associated with FBDS. Logistic regression was applied to explore independent predictors of FBDS. Finally, we used a multiple regression model to investigate the hyponatremia-associated brain network and its effect on serum sodium levels.
The pathological network of anti-LGI1 AE involved hypermetabolism in the cerebellum, subcortical structures and Rolandic area, as well as hypometabolism in the medial prefrontal cortex. The symptom network of FBDS included hypometabolism in the cerebellum and Rolandic area (p <0.05). Hypometabolism in the cerebellum was an independent predictor of FBDS (p < 0.001). Hyponatremia-associated network highlighted a negative effect on the caudate nucleus, frontal and temporal white matter. The metabolism of the hypothalamus was negatively associated with (Pearson's R = -0.180, p = 0.342), while not the independent predictor for serum sodium level (path c' = -7.238, 95% confidence interval = -30.947 to 16.472).
Our results provide insights into the whole-brain metabolic patterns of patients with anti-LGI1 AE, including the symptom network associated with FBDS and the hyponatremia-associated brain network. The findings help us to understand the neural mechanisms underlying anti-LGI1 AE and to evaluate the progress of this disease.
面臂肌张力障碍发作（FBDS）和低钠血症是抗富亮氨酸胶质瘤失活 1 抗体（LGI1）自身免疫性脑炎（AE）的独特临床特征。本研究旨在探讨这些症状的病理生理模式和神经机制。
我们纳入了 30 例抗 LGI1AE 患者和 30 名来自回顾性观察队列的对照者。进行全脑代谢模式分析，以评估抗 LGI1AE 的病理网络，以及与 FBDS 相关的症状网络。应用逻辑回归探讨 FBDS 的独立预测因子。最后，我们使用多元回归模型研究低钠血症相关的脑网络及其对血清钠水平的影响。
抗 LGI1AE 的病理网络包括小脑、皮质下结构和 Rolandic 区的代谢亢进，以及内侧前额叶皮质的代谢减退。FBDS 的症状网络包括小脑和 Rolandic 区的代谢减退（p<0.05）。小脑代谢减退是 FBDS 的独立预测因子（p<0.001）。低钠血症相关网络强调对尾状核、额颞叶白质的负面影响。下丘脑的代谢与（Pearson's R = -0.180，p = 0.342）呈负相关，而不是血清钠水平的独立预测因子（路径 c' = -7.238，95%置信区间 = -30.947 至 16.472）。
我们的研究结果提供了关于抗 LGI1AE 患者全脑代谢模式的见解，包括与 FBDS 相关的症状网络和与低钠血症相关的脑网络。这些发现有助于我们了解抗 LGI1AE 的神经机制，并评估该疾病的进展。
