摘要
目的 探讨抗富亮氨酸胶质瘤失活蛋白1(LGI1)抗体脑炎的临床特征。方法 回顾性分析收住的4例确诊为LGI1脑炎患者的临床资料。结果 4例LGI1脑炎患者中男性3例,女性1例,中位发病年龄59岁,主要临床症状为认知障碍4例,癫痫发作2例,面臂肌张力障碍发作(FBDS)1例,睡眠障碍1例,精神异常2例,低钠血症2例。辅助检查颅脑核磁共振异常1例,脑电图异常2例。脑脊液及血清LGI1抗体双阳性4例,其中1例2次复发时仅血清LGI1抗体阳性。接受皮质类固醇治疗2例,接受免疫球蛋白治疗2例,伴有癫痫发作的2例同时接受抗癫痫药物治疗。3例患者临床症状好转,延误诊治的1例对治疗反应差并多次复发,遗留认知障碍和癫痫发作。结论 LGI1脑炎的临床表现以锥体外系症状为主,颅脑核磁共振在海马和颞叶内侧可有异常信号,早期有效的免疫治疗能及时阻止病情进展,对于复发的患者,应长期维持免疫治疗或及时采用二线治疗。
Objective[HJ2mm]To investigate the clinical features of Leucine-rich Glioma Inactivated 1(LGI1)antibody encephalitis.Methods The clinical data of 4 patients diagnosed with LGI1 encephalitis were retrospectively analyzed.Results The main clinical symptoms were cognitive impairment,seizures,faciobrachial dystonic seizures(FBDS),sleep disorder,mental abnormalities and hyponatremia.Auxiliary examination of cranial magnetic resonance abnormalities and EEG abnormalities.Cerebrospinal fluid and serum LGI1 antibodies were both positive in four cases,and only serum LGI1 antibodies were positive in one case with two recurrences.Two patients received corticosteroids,and two received immunoglobulin therapy,and two patients with seizures received antiepileptic drugs at the same time.The clinical symptoms of three patients improved,and one case who delayed diagnosis and treatment had poor response to treatment and repeated relapses,leaving cognitive impairment and seizures.Conclusion The clinical manifestations of LGI1 encephalitis are mainly extrapyramidal symptoms,cranial MRI may have abnormal signals in the hippocampus and medial temporal lobe,early effective immunotherapy can prevent the progression of the disease in time,for patients with relapse,long-term maintenance immunotherapy or timely second-line therapy should be used.
作者
张妍
范学文
郭芸芸
ZHANG Yan;FAN Xuewen;GUO Yunyun(School of Clinical Medicine,Ningxia Medical University,Yinchuan 750004,China;Department of Neurology,General Hospital of Ningxia Medical University,Yinchuan 750004,China)
出处
《宁夏医学杂志》
CAS
2024年第1期9-11,共3页
Ningxia Medical Journal
