Department of Neurology, Eulji General Hospital, Eulji University College of Medicine, Seoul 139-872, Korea.
Department of Neurology, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul 110-744, Korea.
Exp Neurobiol. 2013 Dec;22(4):337-40. doi: 10.5607/en.2013.22.4.337. Epub 2013 Dec 31.
Anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) is a rare neurological disorder that has a subacute course of progressive encephalopathy and fasciobrachial dystonic seizures. We report a patient with anti-LGI1 LE that presented with atypical manifestations that complicated the diagnosis. A 62-year-old woman presented with a chronic course of memory disturbance and a subsequent relapse with an altered mental status after 10 months. The patient reported frequent chest pain of squeezing and dull nature, typically lasting 10-30 seconds. The chest pain was related to partial seizures, which were confirmed by video-EEG monitoring. Anti-LGI1 antibody was identified in serum and CSF. The patient's symptoms improved by immune modulation treatment. Patients with anti-LGI1 LE can experience atypical partial seizures, and a chronic relapsing course. Clinical suspicions and video-EEG monitoring are helpful for the early diagnosis and effective immune modulation.
抗亮氨酸丰富的胶质瘤失活 1 型(LGI1)边缘性脑炎(LE)是一种罕见的神经疾病,具有亚急性进展性脑病和 fasciobrachial 肌阵挛性癫痫的病程。我们报告了一例抗 LGI1 LE 患者,其表现不典型,使诊断复杂化。一位 62 岁女性以慢性记忆障碍为首发症状,10 个月后出现精神状态改变的再次发作。患者自述频繁出现压榨性和钝痛的胸痛,通常持续 10-30 秒。胸痛与部分性癫痫发作有关,这通过视频脑电图监测得到了证实。在血清和 CSF 中检测到抗 LGI1 抗体。患者的症状通过免疫调节治疗得到改善。抗 LGI1 LE 患者可出现不典型的部分性癫痫发作和慢性复发性病程。临床怀疑和视频脑电图监测有助于早期诊断和有效的免疫调节。