104 Overlapping autoimmunity: a case of concomitant aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibody positivity in neuromyelitis optica spectrum disorder

M. Wronski, Justine Wang
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Abstract

Objectives To describe a rare case of double antibody positive Neuromyelitis Optica Spectrum Disorder (NMOSD) with both Aquaporin-4 and MOG antibodies, occurring following a Pertussis infection in a patient with a history of auto-immunity. Methods Retrospective review of clinical records. Results A 41-year-old Chinese woman with a history of Systemic Lupus Erythematosus presented with a sub-acute onset of progressive gait ataxia and urinary retention occurring seven days after a confirmed Bordetella pertussis infection. Magnetic resonance imaging revealed extensive subcortical and thalamic T2/FLAIR hyperintensities with subtle enhancement, and a longitudinally extensive non-enhancing spinal cord lesion (T1-T7), without optic nerve involvement. Cerebrospinal fluid protein was raised (0.55 g/L) with 7 mononuclear cells and matched oligoclonal bands. Viral PCRs were negative including JC virus and Pertussis. Established live cell-based immunoassays revealed positivity for both Aquaporin-4 antibodies (in CSF and serum) and MOG antibodies in high titres. Our patient meets the 2015 Consensus Diagnostic Criteria for NMOSD.1 Treatment with high dose corticosteroids and rituximab lead to clinical and radiological improvement, but she had a clinical relapse 10 months later with new LETM (T3-T7), necessitating increased immunosuppression with more rigorous rituximab dosing of 1000mg every 6 months. Conclusions Double positivity for both Aquaporin-4 and MOG antibodies in NMOSD is rare. We describe a case of double-positive NMOSD occurring following an infective illness. This case demonstrates that NMOSD may occasionally masquerade as post-infectious Acute Disseminated Encephalomyelitis and highlights the importance of checking antibodies in these patients, given the treatment strategies and risk of relapse differs considerably.
重叠自身免疫:视神经脊髓炎谱系障碍伴水通道蛋白-4和髓鞘少突胶质细胞糖蛋白抗体阳性1例
目的报道一例罕见的双抗体阳性视谱神经脊髓炎(NMOSD),同时伴有水通道蛋白-4和MOG抗体,发生在有自身免疫史的百日咳感染后。方法回顾性分析临床资料。结果一名41岁的中国女性,有系统性红斑狼疮病史,在确诊百日咳杆菌感染7天后出现亚急性进行性步态共济失调和尿潴留。磁共振成像显示广泛的皮质下和丘脑T2/FLAIR高信号伴轻微强化,以及纵向广泛的非强化脊髓病变(T1-T7),未累及视神经。用7个单核细胞和匹配的寡克隆条带培养脑脊液蛋白(0.55 g/L)。病毒pcr阴性,包括JC病毒和百日咳。已建立的基于活细胞的免疫分析显示水通道蛋白-4抗体(CSF和血清)和MOG抗体均呈高滴度阳性。我们的患者符合2015年nmos1共识诊断标准。1高剂量皮质类固醇和利妥昔单抗治疗导致临床和影像学改善,但10个月后临床复发,出现新的LETM (T3-T7),需要增加免疫抑制,利妥昔单抗剂量更严格,每6个月1000mg。结论水通道蛋白-4和MOG抗体双阳性在NMOSD中是罕见的。我们描述了一个病例双阳性NMOSD发生在感染性疾病。该病例表明,NMOSD可能偶尔伪装成感染后急性播散性脑脊髓炎,并强调了在这些患者中检查抗体的重要性,因为治疗策略和复发风险有很大差异。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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