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Clinical features of limbic encephalitis with LGI1 antibody

机译:LGI1抗体引起的边缘性脑炎的临床特征

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Objective: The objective of this study was to analyze the clinical manifestation, course, evolution, image manifestation, and treatments of LGI1 limbic encephalitis (LE). Patients and methods: Studies confirmed that LE with the complex antibody of voltage-gated potassium channels is LGI1 LE. Since then, LE cases have been reported. In this study, 10 typical LE cases were searched in PubMed. These cases and one additional case, which we reported herein, were retrospectively analyzed. Results: All the patients suffered from recent memory deterioration. The following cases were observed: eight with faciobrachial dystonic seizures (FBDS), six with different kinds of epileptic seizures (four complex partial seizures, one myoclonus seizure, and one generalized tonic–clonic seizure), four with FBDS and different kinds of epileptic seizures at the same time, five with mental disorders (one visual hallucination, one paranoia, one depression, one anxiety, and one dysphoria), five with hyponatremia, and two with sleep disorder. The brain MRI of nine patients revealed abnormalities in the mediotemporal lobe and the hippocampus. The LGI1 antibodies in the blood and/or cerebrospinal fluid (CSF) were positive. The content of the CSF protein of two patients increased slightly. The tumor marker of all the patients was normal, but capitate myxoma was detected in the combined pancreas duct of one patient. Gamma globulin and hormone treatments were administered to nine patients. Of these patients, six received a combination of antiepileptic drugs. The clinical symptoms of all the patients improved. Conclusion: LGI1 LE is an autoimmune encephalitis whose clinical manifestations are memory deterioration, FBDS, epileptic seizure, mental disorders, and hyponatremia. Brain MRI shows that this autoimmune disease mainly involves the mediotemporal lobe and the hippocampus. This condition can also be manifested with other autoimmune encephalitis cases but can be rarely associated with tumors. After patients with LGI1 LE receive gamma globulin and hormone treatments, their clinical prognosis is good.
机译:目的:本研究的目的是分析LGI1边缘性脑炎(LE)的临床表现,病程,演变,图像表现和治疗。患者和方法:研究证实具有电压门控钾通道复合抗体的LE为LGI1 LE。从那以后,已经报道了LE病例。在这项研究中,在PubMed中搜索了10例典型的LE病例。回顾性分析了这些病例和我们在此报告的另外一种病例。结果:所有患者均近期记忆力减退。观察到以下情况:八例伴发臂臂肌张力性惊厥(FBDS),六例伴有不同类型的癫痫性发作(4例复杂的部分性发作,1例肌阵挛性发作和1例全身性强直-阵挛性发作),4例伴有FBDS和不同类型的癫痫性发作同时,有五种患有精神疾病(一种幻觉,一种偏执狂,一种抑郁,一种焦虑和一种烦躁不安),五种患有低钠血症和两种患有睡眠障碍。 9名患者的脑部MRI显示中颞叶和海马体异常。血液和/或脑脊髓液(CSF)中的LGI1抗体为阳性。两名患者的CSF蛋白含量略有增加。所有患者的肿瘤标志物均正常,但在一名患者的合并胰管中检出了头状粘液瘤。九名患者接受了丙种球蛋白和激素治疗。在这些患者中,有六个接受了抗癫痫药的联合治疗。所有患者的临床症状均得到改善。结论:LGI1 LE是一种自身免疫性脑炎,其临床表现为记忆力减退,FBDS,癫痫发作,精神障碍和低钠血症。脑部MRI显示,这种自身免疫性疾病主要累及中颞叶和海马。这种情况也可以在其他自身免疫性脑炎病例中表现出来,但很少与肿瘤有关。 LGI1 LE患者接受丙种球蛋白和激素治疗后,其临床预后良好。

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