Siddiqui Amna, Ross Dylan, Jani Ronak H, Prabhu Vikram C, Lo Shelly, Wainwright Derek A, Rouse Stasia, Refaat Tamer, Zhu Yirong, Thakkar Jigisha P
Department of Neurology of Loyola University Stritch School of Medicine, Maywood, Illinois, USA.
Department of Neurological Surgery of Loyola University Stritch School of Medicine, Maywood, Illinois, USA.
Clin Exp Neuroimmunol. 2024 Feb;15(1):55-60. doi: 10.1111/cen3.12767. Epub 2023 Sep 18.
We report a rare case of paraneoplastic neurological syndrome with dual seropositivity of anti-aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in a 40 year-old woman with metastatic triple-negative breast cancer. She received multiple lines of anti-neoplastic treatment, including immunotherapy with pembrolizumab, as well as cytotoxic chemotherapy. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis followed by left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid analysis during both episodes showed normal glucose and protein, and elevated white blood cell count. Cytology was negative for malignancy. Cerebrospinal fluid was positive for neuromyelitis optica immunoglobulin G antibody anti-aquaporin-4, and autoimmune myelopathy panel was positive for myelin oligodendrocyte glycoprotein antibody. The patient had significant clinical and radiographic improvement after completion of five cycles of plasmapheresis followed by intravenous immunoglobulin. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every 6 months and daily low-dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case shows that these antibodies can occur concurrently and cause clinical features, such as both neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody disease, in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or cerebrospinal fluid profile negative for leptomeningeal carcinomatosis.
我们报告了一例罕见的副肿瘤性神经综合征病例，该病例为一名40岁患有转移性三阴性乳腺癌的女性，其抗水通道蛋白4抗体和髓鞘少突胶质细胞糖蛋白抗体呈双重血清阳性。她接受了多线抗肿瘤治疗，包括帕博利珠单抗免疫治疗以及细胞毒性化疗。在乳腺癌诊断后2年以及停用帕博利珠单抗免疫治疗1年后，发生了副肿瘤性脑膜脑脊髓炎。她最初出现纵向延伸的横贯性脊髓炎，随后出现左眼视神经炎和脑膜脑炎，脑部和脊髓软脑膜有新的强化病灶。两次发作期间的脑脊液分析显示葡萄糖和蛋白质正常，白细胞计数升高。细胞学检查未发现恶性肿瘤。脑脊液中视神经脊髓炎免疫球蛋白G抗体抗水通道蛋白4呈阳性，自身免疫性脊髓病检测显示髓鞘少突胶质细胞糖蛋白抗体呈阳性。在完成五个周期的血浆置换并随后静脉注射免疫球蛋白后，患者的临床和影像学表现有显著改善。每6个月使用利妥昔单抗维持治疗并每日服用低剂量泼尼松后，她未再出现副肿瘤综合征复发。在乳腺癌诊断4.5年后，她死于进行性全身转移性疾病。该病例表明，这些抗体可同时出现，并在单一类型癌症患者中引发临床特征，如视神经脊髓炎谱系障碍和髓鞘少突胶质细胞糖蛋白抗体疾病。我们强调，对于患有影像学表现为脑膜脑脊髓炎或脑膜炎且伴有脑膜癌病非典型症状和/或脑脊液检查结果阴性的癌症患者，检测副肿瘤病因的重要性。
