Estiasari Riwanti, Firdausia Salsabila, Wulandari Dewi, Maharani Kartika, Sitorus Freddy, Pangeran David, Imran Darma
Department of Neurology.
Department of Clinical Pathology, Faculty of Medicine, Universitas Indonesia, dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia.
Neurol Int. 2019 Mar 11;11(1):7958. doi: 10.4081/ni.2019.7958.
Neuromyelitis Optic (NMO) is an inflammatory disorder involving central nervous system which often co-exists with other autoimmune diseases such as Sjögren's syndrome (SS). NMO manifestation could precede or follow SS, but the role of anti-SSA in the pathogenesis of NMO remains unclear. We present a case of NMO with anti-AQP4 anti-SSA antibody positive. A-44-year-old female presented with right side weakness. The symptoms began with numbness that improved spontaneously. She also complained pain and dry sensations on her eyes. Schirmer test on her left eye, antinuclear antibody (ANA) and anti-SSA antibody were positive. Cervical MRI revealed intramedullary lesion on T2-weighted-image at C2-C5 level. She was diagnosed as NMO with positive anti-AQP4 and probable SS. She received 1g methylprednisolone for 5 days proceeded with mycophenolic acid. One-year observation showed clinical improvement. Systemic autoantibodies must substansially be evaluated in NMO. Comprehensive diagnosis and providing appropriate immuno-suppressant might prevent further disability and relapse.
视神经脊髓炎(NMO)是一种累及中枢神经系统的炎性疾病,常与干燥综合征(SS)等其他自身免疫性疾病共存。NMO的表现可能先于或后于SS出现,但抗SSA在NMO发病机制中的作用仍不清楚。我们报告一例抗水通道蛋白4(AQP4)和抗SSA抗体阳性的NMO病例。一名44岁女性出现右侧肢体无力。症状始于麻木,后自行缓解。她还诉说眼睛疼痛和干涩感。左眼施密特试验、抗核抗体(ANA)和抗SSA抗体均呈阳性。颈椎磁共振成像(MRI)显示C2 - C5水平T2加权像上的脊髓内病变。她被诊断为抗AQP4阳性的NMO且可能患有SS。她接受了5天的1g甲泼尼龙治疗,随后使用霉酚酸。一年的观察显示临床症状有所改善。在NMO中必须充分评估全身自身抗体。综合诊断并提供适当的免疫抑制剂可能预防进一步的残疾和复发。