Xu Wendeng, Zhang Wei, Zhang Xiaofeng
Department of Neurology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua Medicine, Tsinghua University, Beijing, 102218, China.
BMC Neurol. 2025 Jul 17;25(1):296. doi: 10.1186/s12883-025-04311-4.
Leucine-rich glioma inactivated 1 (LGI1) antibodies are associated with a limbic encephalitis syndrome characterized by faciobrachial dystonic seizures (FBDSs) that is responsive to immunotherapy. The precise pathophysiology, neural origins, and underlying mechanisms of FBDS remain incompletely understood.
We present a 54-year-old Chinese female patient with LGI1-related isolated FBDS triggered by swallowing.
FBDS in LGI1-antibody encephalitis exhibit overlapping features with both paroxysmal kinesigenic dyskinesia (PKD) and epileptic motor seizures, both in their clinical manifestations and underlying neural mechanisms. This unique intersection suggests that FBDS represents a pathophysiological borderland between epileptic motor seizures and paroxysmal movement disorders, bridging the gap between these two diagnostic categories.
FBDS triggered by swallowing expands the clinical spectrum of FBDS and provides new insights into its potential reflex mechanisms, offering a valuable insight for future research into its pathophysiology.
富含亮氨酸的胶质瘤失活1（LGI1）抗体与一种以面臂肌张力障碍性癫痫发作（FBDS）为特征的边缘性脑炎综合征相关，该综合征对免疫治疗有反应。FBDS的确切病理生理学、神经起源和潜在机制仍不完全清楚。
我们报告一名54岁中国女性患者，其LGI1相关的孤立性FBDS由吞咽引发。
LGI1抗体脑炎中的FBDS在临床表现和潜在神经机制方面均表现出与发作性运动诱发性运动障碍（PKD）和癫痫运动发作的重叠特征。这种独特的交叉表明，FBDS代表了癫痫运动发作和发作性运动障碍之间的病理生理交界地带，弥合了这两个诊断类别之间的差距。
吞咽引发的FBDS扩展了FBDS的临床谱，并为其潜在的反射机制提供了新的见解，为未来对其病理生理学的研究提供了有价值的线索。
