Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
China National Clinical Research Center for Neurological Diseases, Beijing, China.
Front Immunol. 2021 May 13;12:652864. doi: 10.3389/fimmu.2021.652864. eCollection 2021.
To examine the clinical characteristics of autoimmune encephalitis associated with the contactin-associated protein-2 (CASPR2) antibody.
Medical records of all patients diagnosed with CASPR2 antibody-associated encephalitis were retrospectively analysed. Data regarding demographic features, neurological symptoms and signs, laboratory tests, imaging results, treatments, and prognosis were collected.
A total of 25 patients aged from 3 to 79 years old were enrolled in this study, with a median age of 43. Eight of 25 (32%) were female, and 17 of 25 (68%) were male. The median age of symptom onset was 42 years old with the course of disease from onset to hospital admission ranging from 2 days to 6 months (median was 17 days). Six patients (6/25) had fever as an onset symptom. During the course of disease, cognitive disturbance was the most common symptom, which was observed in 17 patients (17/25) in total. Eight patients (8/25) met the criteria for limbic encephalitis. Epileptic seizure occurred in six of these eight patients. Four patients (4/25) were diagnosed as Morvan syndrome. All patients were positive for anti-CASPR2 antibody in the serum (1:10-1:300). In six patients, antibodies were detected both in the blood and CSF (1:32-1:100). White blood cell (WBC) counts in the CSF were elevated in eight patients (8/25). The concentration of proteins in CSF increased in 10 patients (ranging from 480 to 1,337.6 mg/dl), decreased in seven patients (ranging from 23.2 to 130.5 mg/dl) and remained at a normal range in the other eight patients (ranging from 150 to 450 mg/dl). Abnormal electroencephalogram (EEG) activities included slow background activity and epileptic patterns. Abnormal signals in the bilateral hippocampus were detected by magnetic resonance imaging (MRI) in three patients presenting cognitive disturbance. In one patient who had limbic encephalitis, increased metabolism of bilateral basal ganglia and the mesial temporal lobe was revealed by PET-CT. Eleven of 15 patients receiving immunotherapy experienced varying degrees of improvement. Relapse occurred in four of 25 patients (4/25) after 2 months.
CASPR-antibody-mediated autoimmune encephalitis is characterized by diverse clinical manifestations. The most prominent conclusion revealed by this retrospective analysis is the involvement of both central and peripheral nerve systems, as well as a lower relapse rate, a good response to immunotherapy, and favorable short-term prognosis after treatment was also demonstrated. Besides, additional work is necessary to evaluate the long-term prognosis.
研究接触蛋白相关蛋白 2（CASPR2）抗体相关自身免疫性脑炎的临床特征。
回顾性分析所有诊断为 CASPR2 抗体相关脑炎的患者的病历。收集人口统计学特征、神经症状和体征、实验室检查、影像学结果、治疗和预后等数据。
本研究共纳入 25 例年龄 3 至 79 岁的患者，中位年龄为 43 岁。25 例患者中 8 例（32%）为女性，17 例（68%）为男性。症状发作的中位年龄为 42 岁，从发病到入院的病程为 2 天至 6 个月（中位时间为 17 天）。6 例（6/25）以发热为首发症状。在病程中，认知障碍是最常见的症状，25 例患者中有 17 例（17/25）存在认知障碍。8 例（8/25）符合边缘性脑炎的标准。其中 6 例（6/8）癫痫发作。4 例（4/25）被诊断为莫尔万综合征。所有患者血清中均检测到抗 CASPR2 抗体（1:10-1:300）。在 6 例患者中，血液和脑脊液中均检测到抗体（1:32-1:100）。8 例患者（8/25）脑脊液白细胞（WBC）计数升高。10 例患者脑脊液蛋白浓度升高（480-1337.6mg/dl），7 例患者脑脊液蛋白浓度降低（23.2-130.5mg/dl），8 例患者脑脊液蛋白浓度在正常范围内（150-450mg/dl）。异常脑电图（EEG）活动包括背景活动减慢和癫痫样模式。3 例表现为认知障碍的患者，磁共振成像（MRI）显示双侧海马区信号异常。1 例边缘性脑炎患者，PET-CT 显示双侧基底节和内侧颞叶代谢增加。15 例接受免疫治疗的患者中有 11 例病情不同程度改善。25 例患者中有 4 例（4/25）在 2 个月后复发。
CASPR 抗体介导的自身免疫性脑炎表现为多种临床表现。本回顾性分析的最显著结论是中枢和周围神经系统均受累，复发率较低，免疫治疗反应良好，治疗后短期预后良好。此外，还需要进一步的工作来评估长期预后。
