Division of Rheumatology, Department of Internal Medicine, Loma Linda University School of Medicine, Loma Linda, California, USA.
Department of Internal Medicine, University of California, Riverside San Bernardino, California, USA.
Curr Rheumatol Rev. 2024;20(2):208-212. doi: 10.2174/0115733971261420230919110103.
Transverse myelitis is considered one of the cardinal features of neuromyelitis optica spectrum disorder (NMOSD), an immune-mediated inflammatory condition of the CNS characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. We describe a case in which a diagnosis of NMOSD was established, associated with West Nile Virus (WNV) infection.
A healthy 18-year-old female presented with intractable hiccups and rapidly progressing paraparesis. MRI demonstrated T2 edema extending from the medulla to the conus, consistent with longitudinally extensive transverse myelitis. Serum and CSF Aquaporin-4 IgG (AQP4) were both positive with high titers. In conjunction with antiviral therapy, immunomodulatory treatment was initiated using pulse methylprednisolone, plasmapheresis and Rituximab. A month and a half after admission, the patient was fully ambulatory with no residual symptoms. On her rheumatology follow-up visit, West Nile Virus-specific IgM in CSF was found to be positive from the patient's initial presentation.
We propose that West Nile Virus may have been the autoimmune trigger to the patient's development of NMOSD, highlighting the importance of evaluating viral triggers in autoimmune diseases.
横贯性脊髓炎被认为是视神经脊髓炎谱系疾病（NMOSD）的主要特征之一，这是一种中枢神经系统的免疫介导的炎症性疾病，其特征是严重的、免疫介导的脱髓鞘和轴索损伤，主要针对视神经和脊髓。我们描述了一例 NMOSD 诊断与西尼罗河病毒（WNV）感染相关的病例。
一位健康的 18 岁女性因顽固性呃逆和迅速进展的截瘫就诊。MRI 显示从延髓延伸到圆锥的 T2 水肿，符合广泛的横贯性脊髓炎。血清和 CSF 水通道蛋白 4 抗体（AQP4）均为阳性，且滴度较高。在进行抗病毒治疗的同时，采用脉冲甲基强的松龙、血浆置换和利妥昔单抗进行免疫调节治疗。入院一个半月后，患者完全可以行走，没有残留症状。在她的风湿病随访就诊时，发现患者最初发病时 CSF 中的西尼罗河病毒特异性 IgM 呈阳性。
我们提出西尼罗河病毒可能是导致该患者 NMOSD 发展的自身免疫触发因素，这突出了在自身免疫性疾病中评估病毒触发因素的重要性。
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