范思远, 任海涛, 林楠, 卢强, 金丽日, 黄颜, 侯波, 有慧, 冯逢, 崔瑞雪, 朱以诚, 关鸿志, 崔丽英. 抗LGI1脑炎:一项国人单中心队列研究[J]. 罕见病研究, 2022, 1(2): 122-129. DOI: 10.12376/j.issn.2097-0501.2022.02.005
引用本文: 范思远, 任海涛, 林楠, 卢强, 金丽日, 黄颜, 侯波, 有慧, 冯逢, 崔瑞雪, 朱以诚, 关鸿志, 崔丽英. 抗LGI1脑炎:一项国人单中心队列研究[J]. 罕见病研究, 2022, 1(2): 122-129. DOI: 10.12376/j.issn.2097-0501.2022.02.005
FAN Siyuan, REN Haitao, LIN Nan, LU Qiang, JIN Liri, HUANG Yan, HOU Bo, YOU Hui, FENG Feng, CUI Ruixue, ZHU Yicheng, GUAN Hongzhi, CUI Liying. Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis: A Single-Center Cohort Study in China[J]. Journal of Rare Diseases, 2022, 1(2): 122-129. DOI: 10.12376/j.issn.2097-0501.2022.02.005
Citation: FAN Siyuan, REN Haitao, LIN Nan, LU Qiang, JIN Liri, HUANG Yan, HOU Bo, YOU Hui, FENG Feng, CUI Ruixue, ZHU Yicheng, GUAN Hongzhi, CUI Liying. Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis: A Single-Center Cohort Study in China[J]. Journal of Rare Diseases, 2022, 1(2): 122-129. DOI: 10.12376/j.issn.2097-0501.2022.02.005

抗LGI1脑炎:一项国人单中心队列研究

Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis: A Single-Center Cohort Study in China

  • 摘要:
      目的  抗LGI1脑炎是最常见的自身免疫性边缘性脑炎,本研究拟评估中国抗LGI1脑炎患者的临床特点、免疫治疗方案及长期预后。
      方法  2013年7月至2018年1月于北京协和医院就诊且存在近事记忆下降、精神症状、癫痫发作、意识障碍等症状,临床怀疑脑炎的患者,进行血清和/或脑脊液抗LGI1抗体检测,纳入抗LGI1抗体阳性的患者。登记入组患者的一般情况、临床表现、辅助检查、免疫治疗、随访与预后等信息,并进行分析。
      结果  共纳入120例患者。66.7%为男性,中位发病年龄为61岁(四分位距:49~66岁)。最常见的首发症状是癫痫发作(65.0%)。95.0%的患者随病情进展出现癫痫发作(其中54.2%出现特征性的面臂肌张力障碍发作)、近事记忆下降(92.5%)和精神行为异常(69.1%)等症状。头磁共振与18F-FDG PET/CT检查提示病变主要位于单侧或者双侧颞叶内侧,和/或基底节区。95.0%的患者接受静脉注射免疫球蛋白和/或糖皮质激素治疗,47.5%接受吗替麦考酚酯长程免疫治疗,无患者接受血浆置换或二线免疫治疗。中位随访时间为34.2个月(四分位距:22.0~45.6个月)。91.2%的患者预后良好(改良Rankin评分≤2分)。47.8%的患者遗留轻微近事记忆下降。9例患者死亡。发病后首年复发率为24.8%。队列中24例(20%)患者为青年(发病年龄≤45岁),与中老年患者相比,青年患者中男性更少见(37.5% vs. 74.0%,P<0.01),精神行为异常(50.0% vs. 74.0%,P=0.02)、低钠血症(33.3% vs. 68.8%,P<0.01)和头18F-FDG PET/CT异常改变(20.8% vs. 47.9%,P=0.02)也更少见,无复发生存概率高于中老年患者。
      结论  抗LGI1脑炎患者以中老年为主,多以典型的边缘性脑炎或者FBDS为主要表现,青年患者可能症状不典型。神经影像学提示边缘系统或者基底节受累。该病患者对免疫治疗反应良好,多数患者预后良好。

     

    Abstract:
      Objective  To evaluate the clinical and paraclinical features of Chinese patients with anti- LGI1 encephalitis.
      Methods  Patients with memory deficits, psychiatric symptoms, seizures or altered level of consciousness, suspicious of encephalitis, at presentation to Peking Union Medical College Hospital were recruited between July 2013 and January 2018, and tested for anti-LGI1 antibodies in their serum and/or cerebrospinal fluid(CSF) samples. Patients with anti-LGI1 antibodies were enrolled. The demographic characteristics, clinical manifestations, laboratory examination results, neuroimaging features, immunotherapy, follow-up practices and outcomes for included patients were registered and analyzed.
      Results  The study enrolled 120 patients, of whom 66.7% were male. The median age was 61 years (interquartile range IQR: 49-66 years). Seizures(65.0%) were the most common initial symptoms. Most patients developed seizures (95.0%), including faciobrachial dystonic seizures (54.2%), memory deficits (92.5%), and psychiatric symptoms (69.1%). Brain MRI and 18F-FDG PET / CT showed that the lesions were mainly located in unilateral or bilateral medial temporal lobes, and (or) basal ganglia. Of the patients, 95.0% received intravenous immunoglobulin (IVIg) or corticosteroids, 47.5% received mycophenolate mofetil as long-term immunotherapy, and no one received second-line immunotherapy. The median follow-up was 34.2 months(IQR: 22.0-45.6 months). 91.2% had a good outcome (modified Rankin Scale score≤2 points). Residual mild memory deficits were present in 47.8% of the patients. Nine deaths were documented. Relapses occurred in 24.8% of the patients in the first year. In total, 24 (20%)cases were young patients(onset age ≤45 years).There were fewer males among the younger patients(37.5% vs. 74.0%, P < 0.01). Besides, there were fewer younger patients with psychiatric symptoms(50.0% vs. 74.0%, P=0.02), hyponatremia(33.3% vs. 68.8%, P < 0.01), and abnormalities on brain 18F-FDG PET/CT(20.8% vs. 47.9%, P=0.02). The relapse-free survival rate was significantly higher in the young patients.
      Conclusions  Elderly males were predominant in patients with anti-LGI1 encephalitis. Most patients developed symptoms of limbic encephalitis and/or FDBS during the disease course. Several patients were young adults and lacked typical symptoms. Neuroimaging features were consistent with the involvement of limbic system or basal ganglia. Patients with anti-LGI1 encephalitis respond well to immunotherapy, irrespective of the age.

     

/

返回文章
返回