Wang Congcong, Kong Fanyu, Yu Xianwen, Li Yan, Yang Meihe, Si Zhihua, Liu Rutao, Hu Ke, Yang Bing
Internal Medicine of Traditional Chinese Medicine, Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China.
Department of Neurology, Shandong Provincial Qianfoshan Hospital, The First Affiliated Hospital of Shandong First Medical University, Jinan, Shandong, China.
Front Hum Neurosci. 2025 Jul 15;19:1586005. doi: 10.3389/fnhum.2025.1586005. eCollection 2025.
Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis is a rare autoimmune encephalitis, with only a few series describing its typical clinical manifestations and prognosis. Here, we present three newly identified patients with anti-AMPAR encephalitis and were followed up for prognostic evaluation. The mean age of the patients was 47 years (range, 32-57). All three patients experienced memory issues, with two showing signs of typical limbic encephalitis (LE). Cranial magnetic resonance imaging (MRI) in two patients demonstrated lesions in the bilateral temporal lobes, insula, and cingulate gyrus as well as significant cortical atrophy after 1 month of follow-up. No acute lesions were observed on cranial MRI in the third patient at the onset of symptoms. One patient had positive antibodies for both AMPAR1 and AMPAR2 in cerebrospinal fluid (CSF), while the other two patients only had positive antibodies for AMPAR2. Severe clinical symptoms and high CSF antibody levels were found in two patients. Immunotherapy demonstrated partial efficacy in all three patients. Two patients exhibited favorable responses to first-line immunotherapy. In contrast, the third patient experienced a suboptimal response to the initial treatment, with no remission and subsequent disease relapse. Following second-line immunotherapy, her condition stabilized; however, she continued to suffer from significant cognitive impairment. One patient was diagnosed with a viral infection, but no tumors were found in any patients. Besides its typical manifestation as LE, anti-AMPAR2 encephalitis may also present as simple amnesia. It is advised to monitor CSF antibodies and their level changes. While first-line immunotherapy is partially effective, some patients may need additional second-line therapy. Viral infections could be a predisposing factor; thus, routine CSF virus testing is recommended. Additionally, screening for tumors and follow-up assessments are also important.
抗α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体脑炎是一种罕见的自身免疫性脑炎，仅有少数系列报道描述其典型临床表现和预后。在此，我们报告3例新确诊的抗AMPAR脑炎患者，并对其进行随访以评估预后。患者的平均年龄为47岁（范围32 - 57岁）。所有3例患者均有记忆问题，其中2例表现出典型边缘叶脑炎（LE）的体征。2例患者的头颅磁共振成像（MRI）显示双侧颞叶、岛叶和扣带回有病变，随访1个月后出现明显的皮质萎缩。第3例患者在症状发作时头颅MRI未观察到急性病变。1例患者脑脊液（CSF）中AMPAR1和AMPAR2抗体均为阳性，而另外2例患者仅AMPAR2抗体阳性。2例患者出现严重的临床症状和脑脊液抗体水平升高。免疫治疗在所有3例患者中均显示出部分疗效。2例患者对一线免疫治疗反应良好。相比之下，第3例患者对初始治疗反应欠佳，未缓解且随后疾病复发。二线免疫治疗后，她的病情稳定；然而，她仍持续存在明显的认知障碍。1例患者被诊断为病毒感染，但所有患者均未发现肿瘤。除了表现为典型的LE外，抗AMPAR2脑炎也可能表现为单纯失忆。建议监测脑脊液抗体及其水平变化。虽然一线免疫治疗部分有效，但一些患者可能需要额外的二线治疗。病毒感染可能是一个诱发因素；因此，建议常规进行脑脊液病毒检测。此外，肿瘤筛查和随访评估也很重要。
