Gao Pan, Chen Xiaowu, Li Yuhao, Li Fangming, Zhang Hongya
Department of Neurology, Shenzhen University General Hospital, Shenzhen, China.
The Education University of Hong Kong, Tai Po, New Territories, Hong Kong, China.
Medicine (Baltimore). 2025 Jun 20;104(25):e42873. doi: 10.1097/MD.0000000000042873.
With the update of novel autoantibodies and the expansion of the clinical spectrum, our understanding of autoimmune encephalitis (AE) is rapidly evolving. Anti-CASPR2 meningoencephalitis is a relatively rare condition that may be induced by infections.
A young man presented with 3 episodes of meningoencephalitis potentially triggered by possible viral, Salmonella, and severe acute respiratory syndrome coronavirus 2. Neuroimaging revealed a thickening of the cerebral dura mater. Laboratory tests found positive serum CASPR2 antibodies in the third episode.
Across all 3 episodes, similar clinical manifestations and imaging features were observed. Although autoantibodies in the previous 2 phases were negative, the possibility of infection-related anti-CASPR2 meningoencephalitis remains highly suspected.
The treatment regimen comprised antimicrobial agents, corticosteroid therapy, intravenous immunoglobulin, and rituximab administration.
Following treatment, the patient's condition improved with no recurrence to date. Repeat testing showed undetectable anti-CASPR2 immunoglobulin G in both serum and cerebrospinal fluid. Post-treatment contrast-enhanced magnetic resonance imaging demonstrated the resolution of dural thickening.
We further reviewed the mechanism of various infection-related AE and characteristics of CASPR2-related disease. To our knowledge, this is the first report of CASPR2 meningoencephalitis with thickened dura mater, indicating the importance of paying attention to antibody-negative AE and monitor antibodies repeatedly when necessary. In addition to immunotherapy, we recommend comprehensive management throughout the disease process.
随着新型自身抗体的更新以及临床谱的扩展，我们对自身免疫性脑炎（AE）的认识正在迅速演变。抗接触蛋白相关蛋白2（CASPR2）脑膜脑炎是一种相对罕见的疾病，可能由感染诱发。
一名年轻男性出现3次脑膜脑炎发作，可能由病毒、沙门氏菌和严重急性呼吸综合征冠状病毒2诱发。神经影像学检查显示硬脑膜增厚。实验室检查在第三次发作时发现血清CASPR2抗体呈阳性。
在所有3次发作中，观察到相似的临床表现和影像学特征。尽管前两个阶段自身抗体为阴性，但仍高度怀疑感染相关的抗CASPR2脑膜脑炎。
治疗方案包括抗菌药物、皮质类固醇治疗、静脉注射免疫球蛋白和利妥昔单抗给药。
治疗后，患者病情改善，至今未复发。重复检测显示血清和脑脊液中抗CASPR2免疫球蛋白G均检测不到。治疗后增强磁共振成像显示硬脑膜增厚消退。
我们进一步回顾了各种感染相关AE的机制以及CASPR2相关疾病的特征。据我们所知，这是首例硬脑膜增厚的CASPR2脑膜脑炎报告，表明关注抗体阴性AE并在必要时反复监测抗体的重要性。除免疫治疗外，我们建议在疾病全过程进行综合管理。
