Jayarangaiah Apoorva, Sehgal Rahul, Epperla Narendranath
BMC Neurol. 2014 Oct 9;14:200. doi: 10.1186/s12883-014-0200-5.
Neuromyelitis optica (NMO) is a rare relapsing auto-immune disease of the central nervous system which is sometimes found in association with other autoimmune disorders including Sjogren's syndrome. We present the case of a middle aged female with Sjogren's syndrome (SS) and Neuromyelitis optica spectrum disorders (NMOSD) who had a rapidly declining neurological illness that responded to immunosuppressive therapy.
A 51-year-old female with Sjogren's syndrome and recent history of varicella zoster infection presented with right upper and lower extremity weakness of one week duration. She was noted to have contrast enhancement at C2-C4 cord levels on cervico-thoracic MRI. Comprehensive work up was negative except for presence of a mild lymphocytic pleocytosis and oligoclonal bands in the CSF. She was diagnosed with transverse myelitis secondary to varicella zoster infection and was treated with high dose steroids in addition to acyclovir with improvement in her symptoms. Two months later she developed left upper and lower extremity weakness, bilateral dysesthesias and urinary incontinence. Repeat MRI of the cervico-thoracic spine revealed worsening enhancement at lower cervical cord levels (C5-7) with extension to T1. CSF analysis was unchanged; however immunological work up was abnormal for elevated NMO-IgG/AQP4 antibody. She was diagnosed with NMOSD and was treated with immunosuppressive therapy. Initially with IV methylprednisone and Cyclophosphamide therapy followed by Mycophenolate mofetil (MMF) maintenance therapy with good response. Repeat MRI 6 months later showed near complete resolution of previous abnormal cord signal changes.
One needs to recognize the relationship between autoimmune diseases especially SS and NMOSD. The presence of NMO antibody has been associated with a relapsing disease course and a careful follow-up, besides use of remission maintenance agents such as MMF and Azathioprine are important to consider.
视神经脊髓炎（NMO）是一种罕见的中枢神经系统复发性自身免疫性疾病，有时与包括干燥综合征在内的其他自身免疫性疾病相关。我们报告一例患有干燥综合征（SS）和视神经脊髓炎谱系障碍（NMOSD）的中年女性病例，其神经系统疾病迅速恶化，但对免疫抑制治疗有反应。
一名51岁患有干燥综合征且近期有带状疱疹感染史的女性，出现持续一周的右上肢和下肢无力。在颈胸段MRI上，发现其C2 - C4脊髓节段有强化表现。全面检查除脑脊液中存在轻度淋巴细胞增多和寡克隆带外均为阴性。她被诊断为带状疱疹感染继发横贯性脊髓炎，并接受了高剂量类固醇及阿昔洛韦治疗，症状有所改善。两个月后，她出现左上肢和下肢无力、双侧感觉异常及尿失禁。颈胸段脊柱的重复MRI显示下颈段脊髓节段（C5 - 7）强化加重并延伸至T1。脑脊液分析无变化；然而免疫检查发现NMO - IgG/AQP4抗体升高异常。她被诊断为NMOSD，并接受免疫抑制治疗。最初采用静脉注射甲基强的松龙和环磷酰胺治疗，随后用霉酚酸酯（MMF）维持治疗，反应良好。6个月后的重复MRI显示先前异常的脊髓信号变化几乎完全消失。
需要认识到自身免疫性疾病尤其是SS和NMOSD之间的关系。NMO抗体的存在与疾病复发过程相关，除使用MMF和硫唑嘌呤等缓解维持药物外，仔细的随访也很重要。
