PDF(Pubmed)
Abstract：
Neuromyelitis optica, an autoimmune inflammatory disorder affecting the central nervous system, can occur in a paraneoplastic context, although rare. We report an intriguing case of a 71-year-old woman with a history of triple-negative infiltrating ductal breast carcinoma, manifesting with paraneoplastic neuromyelitis optica that led to significant respiratory failure and required a cervical laminectomy. The patient presented with pain in the left breast, weakness in the lower extremities, and neck pain. The neurological evaluation showed 2/5 muscle strength in all extremities, diffuse hyperreflexia, and loss of multimodal sensation below the shoulder. She developed acute respiratory failure that required mechanical ventilation. Magnetic resonance imaging highlighted a diffuse abnormal increase in T2 signal intensity throughout the posterior and central portion of the cervical and thoracic spinal cord consistent with longitudinally extensive transverse myelitis, and significant cervical cord compression at C3-C4. Magnetic resonance imaging of the brain showed non-enhancing T2/fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities and cerebellar hemispheres. The serum cell-based assay study demonstrated a high anti-aquaporin-4 immunoglobulin G titer (>1:160) confirming the diagnosis of neuromyelitis optica. She was taken for bilateral laminectomy from C3 to C6. Despite intravenous methylprednisolone and plasmapheresis treatment, no significant recovery was achieved, necessitating tracheostomy and percutaneous endoscopic gastrostomy. Subsequent rituximab treatment led to a mild improvement, with no new lesions on repeat magnetic resonance imaging. This case raises suspicion of the potential for neuromyelitis optica to occur as a paraneoplastic phenomenon, strengthening the need for vigilance in patients with malignancies.
摘要：
视神经脊髓炎,一种影响中枢神经系统的自身免疫性炎症性疾病，可能发生在副肿瘤环境中，虽然罕见。我们报道了一个有三阴性浸润性导管癌病史的71岁女性的有趣病例，表现为副肿瘤性视神经脊髓炎，导致严重的呼吸衰竭，需要进行子宫颈椎板切除术。病人出现左乳房疼痛，下肢无力，颈部疼痛。神经系统评估显示所有四肢有2/5的肌肉力量，弥漫性反射亢进,肩下多模态感觉的丧失。她出现了急性呼吸衰竭，需要机械通气。磁共振成像显示，在整个颈椎和胸椎脊髓后部和中央部分，T2信号强度出现弥漫性异常增加，与纵向广泛性横贯性脊髓炎一致。在C3-C4处有明显的颈索压迫。大脑的磁共振成像显示无增强的T2/液体衰减的倒置恢复（FLAIR）白质高强度和小脑半球。基于血清细胞的测定研究证明了高抗水通道蛋白-4免疫球蛋白G滴度（>1：160），证实了视神经脊髓炎的诊断。她从C3到C6接受双侧椎板切除术。尽管静脉注射甲基强的松龙和血浆置换治疗，没有明显的恢复，需要气管造口术和经皮内镜胃造瘘术。随后的利妥昔单抗治疗导致轻度改善，重复磁共振成像没有新的病变。这种情况引起了人们对视神经脊髓炎作为副肿瘤现象发生的可能性的怀疑。加强恶性肿瘤患者警惕的必要性。
