Department of Neurology, The Central Hospital of Shaoyang, Shaoyang, China.
Department of Clinical Pharmacy, The Central Hospital of Shaoyang, Shaoyang, China.
Front Immunol. 2024 Jun 14;15:1424243. doi: 10.3389/fimmu.2024.1424243. eCollection 2024.
Neuromyelitis optica spectrum disorder (NMOSD) is a clinical syndrome characterized by attacks of acute optic neuritis and transverse myelitis. We report a case with paraneoplastic NMOSD that improved after immunosuppressive therapy, surgical resection, and chemotherapy. A 48-year-old woman initially presented with gradual binocular visual loss over the course of one week. The patient was evaluated using magnetic resonance imaging (MRI), computed tomography (CT), visual evoked potential (VEP), pathological biopsy, immunohistochemistry, and autoimmune antibody testing. The brain MRI findings were normal. The VEP revealed prolonged P100 latencies in the right eye and an absence of significant waves in the left eye. Positive serum AQP4-IgG antibodies were found. The patient was diagnosed as NMOSD. Then the patient responded well to treatment with methylprednisolone. An ovarian tumor was found in the patient using abdominal MRI and CT. The tumor was surgically resected, and a pathological biopsy revealed that it was ovarian dysgerminoma. The patient received four rounds of chemotherapy after surgery. One month after the final chemotherapy treatment, a positron emission tomography (PET) scan revealed no tumor. The vision of the patient gradually recovered and serum AQP4 was negative. Furthermore, we summarized the characteristics of patients diagnosed with paraneoplastic NMOSD associated with ovarian neoplasms in previous studies. This is a characteristic case of overlapping NMOSD and ovarian dysgerminoma, demonstrating the importance of tumor therapy in cases of paraneoplastic NMOSD.
视神经脊髓炎谱系疾病（NMOSD）是一种以急性视神经炎和横贯性脊髓炎发作为特征的临床综合征。我们报告了一例副肿瘤性 NMOSD 病例，经免疫抑制治疗、手术切除和化疗后病情改善。一名 48 岁女性最初表现为双眼进行性视力丧失，历时一周。该患者接受了磁共振成像（MRI）、计算机断层扫描（CT）、视觉诱发电位（VEP）、病理活检、免疫组化和自身抗体检测。脑 MRI 检查未见异常。VEP 显示右眼 P100 潜伏期延长，左眼无明显波。血清 AQP4-IgG 抗体阳性。患者被诊断为 NMOSD。随后，患者对甲基强的松龙治疗反应良好。腹部 MRI 和 CT 发现患者卵巢有肿瘤。肿瘤经手术切除，病理活检显示为卵巢无性细胞瘤。患者术后接受了四轮化疗。最后一次化疗治疗一个月后，正电子发射断层扫描（PET）显示无肿瘤。患者的视力逐渐恢复，血清 AQP4 转为阴性。此外，我们总结了既往研究中诊断为伴发卵巢肿瘤的副肿瘤性 NMOSD 患者的特征。这是一例重叠 NMOSD 和卵巢无性细胞瘤的典型病例，表明肿瘤治疗对副肿瘤性 NMOSD 的重要性。
